Movement Disorders
Deep brain stimulation in movement disorders
Nov. 27, 2023
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Rating scales of movement disorders …
- Updated 11.27.2023
- Released 12.16.1998
- Expires For CME 11.27.2026
Rating scales of movement disorders
Introduction
Overview
Measuring disease severity is critical from a clinical and research perspective. Utilizing standardized rating scales allows for a universally understood means of quantifying disease states regarding both disease progression and treatment response. Detecting changes in an underlying disease process commonly relies on changes in rating scales as a surrogate marker. In this article, the authors provide an introduction to several of the rating scales that have been used to quantify specific involuntary movements (ie, tremor) as a symptom or within the context of a defined movement disorder (ie, Parkinson disease). Scales for each of the following involuntary movements or diseases are discussed: Parkinson disease, tics, chorea, dystonia, myoclonus, ataxia, tremor, medication-induced dyskinesia, and gait and balance in any movement disorder. The scales are summarized and presented to highlight clinical relevance, statistical rigor, and limitations using current published evaluations. There are numerous scales available; however, the ones discussed are the ones that have been most used and thoroughly tested. This article also reflects the most updated revisions and critiques of these scales.
Key points
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• The ability to measure movement disorder characteristics over time allows for universally accepted, standardized outcome measures. | |
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• The ability of scales to produce consistent results and, therefore, to be useful instruments in monitoring patients and conducting research, depends on the investigator’s experience administering and interpreting each scale as well as the psychometric properties of each scale. | |
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• Rating scales in movement disorders focus on two primary concepts: impairment and disability. | |
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• Items rated by the investigator and based on the neurologic examination assess impairment and relate to objective deficits. | |
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• Interviews that involve the patient’s, the caregiver’s, and the investigator’s assessments of activities of daily living or quality of life rate disability and, thus, assess the impact of the disease on daily function. |
About rating scales for movement disorders
To continue to advance the field of neurodegenerative conditions, it is critical to utilize standardized measures assessing disease state and treatment response. Rating scales serve as a surrogate marker for changes in underlying disease process. Utilizing such measures allows for a more objective means of quantifying outcomes across research efforts.
A rating scale should meet three primary criteria of psychometric accuracy standards (85). First, the scale should be objective, with the results being independent of the examiner. Second, different observers or the same observer should repeatedly reach the same endpoint when using the scale, also referred to as reliability. Third, the scale should be valid; that is, it accurately measures the variable it is purported to object. Secondary criteria should also be met and include standardization, comparability, economy, and usefulness. In addition to the quality criteria, there are the relative criteria of degrees of sensitivity and specificity (109).
Movement disorders comprise a group of disorders characterized by abnormal, involuntary movements that do not result from weakness or sensory deficits. Despite their diversity, movement disorders may be divided into two types: (1) hyperkinetic, characterized by an excess of movement, and (2) hypokinetic, characterized by a paucity of movement (162; 89). The abnormal movement stems from dysfunction of what may be defined anatomically as the basal ganglia and cerebellum or functionally as the extrapyramidal motor system (162; 89).
This article introduces several of the rating scales that have been used to quantify specific involuntary movements (ie, tremor) or specific disease entities characterized by involuntary movements (ie, Parkinson disease). The purpose is to provide a reasonable overview of the more commonly used scales rather than an exhaustive list of the numerous rating scales that have ever been developed. Scales for each of the following involuntary movements or diseases will be discussed: Parkinson disease, tics, chorea, dystonia, myoclonus, ataxia, tremor, medication-induced movement disorders, and gait and balance. For each scale, the following basic issues will be addressed:
(1) What are the practical applications of this scale? |
No single scale is perfect. Scales that aim to obtain a general overview may be criticized as being nonspecific, whereas those that aim to address a particular problem may be criticized as being too limited. Those that are brief may be viewed as incomplete, whereas those that are longer may be viewed as cumbersome and impractical.
Parkinson disease. A review of the clinimetrics of all Parkinson disease scales available between 1960 and 2002 was reviewed (124). The authors identified 11 rating scales used for Parkinson disease, including three impairment scales (Webster, Columbia University Rating Scale [CURS], and Parkinson Disease Impairment Scale), four disability scales (Schwab and England, Northwestern University Disability Scale [NUDS], Intermediate Scale for Assessment of Parkinson Disease, and Extensive Disability Scale), and four scales evaluating both impairment and disability (New York University, University of California Los Angeles, Unified Parkinson's Disease Rating Scale [UPDRS], and Short Parkinson Evaluation Scale). The authors concluded that the CURS, NUDS, and UPDRS have moderate to good reliability and validity, but most of the rating scales had either not been subjected to an extensive clinimetric evaluation or had demonstrated clinimetric shortcomings. In addition to the scales presented in detail in this article, readers may consider using the following scales (a partial list of available scales) based on their need.
Scales for general measures of Parkinson disease. SCales for Outcomes in PArkinson's disease (SCOPA) has multiple subscales including for psychosocial, motor, cognitive, and autonomic features of Parkinson disease (97; 98; 158; 159; 101).
Scales for disease-specific issues in Parkinson disease.
• The Parkinson Disease Sleep Scale (17) |
Multiple scales have been used to assess dementia in Parkinson disease. Some are general measures of cognition adapted for Parkinson disease, including the Folstein Mini-Mental Status Examination (38), Montreal Cognitive Assessment (MoCA) (117), pentagon drawing alone (23), Cambridge Cognitive Assessment (revised) (62), and Mattis scale for dementia (27; 87). Other scales have been specifically developed to be used in Parkinson disease, including SCOPA-COG, the Parkinson neuropsychometric dementia assessment (PANDA) instrument (72), and the Parkinson disease cognitive rating scale (118).
Mild cognitive impairment in Parkinson disease is an indicator of Parkinson disease dementia (61). The Movement Disorder Society formed a task force that developed guidelines for mild cognitive impairment in Parkinson disease (PD-MCI) (86; 152; 88). The pattern of progression seen is from normal cognition to mild cognitive impairment and, eventually, Parkinson disease dementia (45). The PD-MCI guidelines can identify patients at risk for Parkinson disease dementia (26). It is an optimal and efficient neuropsychological assessment of PD-MCI (53). Several studies have analyzed the application, validation, and utility of the criteria. Geurtsen and colleagues validated the criteria by analyzing cross-sectional and neuropsychological databases of patients with Parkinson disease and a number of controls by predicting the conversion to dementia (46). Szeto and associates used a range of cutoff scores to study the utility of the criteria (149). Currently, there is no optimal cutoff score for defining PD-MCI. Yet, this tool can be used as an important assessment of cognition in Parkinson disease. The MoCA is a general cognitive measure considered sensitive in Parkinson disease (171). It may be used without permission for clinical and educational noncommercial purposes and is available in multiple languages and with instructions at www.mocatest.org.
There have also been evaluations in Parkinson disease of psychiatric scales used to assess depression. The Beck Inventory-II is commonly used, but due to psychomotor influence on this scale, there can be a high rate of false positives. Alternatively, the Geriatric Depression Scale (GDS) (110) and the Hamilton Depression scale have been independently evaluated in Parkinson disease and measured against each other and are comparable (164; 136).
Psychosis is one of the most troublesome non-motor symptoms of Parkinson disease, which has no specific, validated scale, but there is a clear need for one (37). The Scale for Evaluation of Neuropsychiatric Disorders in Parkinson’s disease (SEND-PD) assesses the severity of neuropsychiatric symptoms (103). The scale has been validated and shown to correlate with the MDS-UPDRS (128). As the pathophysiology of Parkinson disease is elucidated and better biomarkers are developed, scales may be validated against more objective measures. As a supplement to the revised UPDRS (presented below), the Movement Disorders Society Task force summarized and rated multiple nonmotor aspects of Parkinson disease.
The scales presented and discussed are the following:
For patients with Parkinson disease: | |
• Movement Disorder Society - Unified Parkinson Disease Rating Scale | |
For patients with tic disorders: | |
• Yale Global Tic Severity Scale | |
For patients with chorea or Huntington disease: | |
• Unified Huntington Disease Rating Scale | |
For patients with dystonia: | |
• Burke-Fahn-Marsden Evaluation Scale for Dystonia | |
For patients with myoclonus: | |
• Myoclonus Evaluation | |
• The Unified Myoclonus Rating Scale | |
For patients with ataxia: | |
• International Cooperative Ataxia Rating Scale | |
For patients with essential tremor: | |
• Washington Heights-Inwood Tremor Rating Scale | |
For patients with tardive dyskinesia: | |
• Abnormal Involuntary Movement Scale | |
For most movement disorders: | |
• Berg Balance Scale | |
Scales used to assess patients with Parkinson disease
Parkinson disease is a disorder of the basal ganglia, specifically the nigrostriatal system characterized clinically by rest tremor, bradykinesia, rigidity, postural instability, and response to levodopa. Pathologically, it is characterized by the presence of Lewy bodies and neuronal loss in the substantia nigra (66; 93). Although primarily sporadic, familial forms of the disease exist (52) and genetic mutations associated with Parkinson disease are emerging (18). Three complementary rating scales will be discussed. The first is the Unified Parkinson Disease Rating Scale (33; 51). This scale underwent a significant revision and clinimetric testing by the Movement Disorder Society Task Force for Rating Scales in Parkinson’s disease (51). The modified Hoehn and Yahr Staging Scale (63) and the Schwab and England Activities of Daily Living Scale (161; 137) remain the main scales to stage Parkinson disease and give an overall sense of functioning.
Movement Disorder Society - Unified Parkinson Disease Rating Scale (MDS-UPDRS)
The revised MDS-UPDRS (available here: www.movementdisorders.org) is divided into the following sections:
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1. Non-motor aspects of experiences of daily living (nM-EDL) | |
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a. Complex behaviors | |
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2. Motor aspects of experiences of daily living (M-EDL) | |
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3. Motor examination | |
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4. Motor Complications | |
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a. Dyskinesias | |
Parts 1b and 2 were designed to be self-administered but can be confirmed by the rater administering the scale. Similar to the initial version, parts 1 and 2 do not have separate on and off ratings.
Although we are unable to reproduce the new UPDRS in this article, you can follow this link to the Movement Disorder Society website for the UPDRS and other copyrighted rating scales: www.movementdisorders.org.
(1) What are the practical applications of this scale?
The Unified Parkinson Disease Rating Scale is perhaps the most commonly used scale for the assessment of the functional capacity and motor manifestations of patients with Parkinson disease. The initial version of the Unified Parkinson Disease Rating Scale (UPDRS) consisted of four sections for the assessment of: (1) mentation, behavior, and mood; (2) activities of daily living; (3) motor examination; and (4) complications of therapy (33). The revised version has followed a similar format.
The updated MDS-UPDRS has more specific directions and descriptions of each measure. The motor section will look familiar to those who have used the older version of the UPDRS, with a few additions including toe-tapping, freezing of gait, postural and kinetic tremor of the hands, and constancy of rest tremor. In total, there are nine new items not captured on the original UPDRS that are on the revised version, including anxious mood, dopamine dysregulation syndrome, urinary problems, constipation, fatigue, doing hobbies, getting in and out of bed, toe-tapping, and freezing. The “complications of therapy” section also assesses functional impact as well as time and severity of the complications. A convenient scoring sheet is included at the end of the published scale.
It should be emphasized that the MDS-UPDRS was designed to be more comprehensive than the original UPDRS. There is a greater emphasis on distinguishing relatively mild impairments and disabilities, drawing distinctions between slight and mild, whereas former distinctions between severe and marked are now collapsed into the severe rating. This decision was made due to the focus of clinical trials on early disease, and for the general outpatient population, functional differences between severe and marked impairments may not be clinically relevant. As a result of this decision, for several items in the MDS-UPDRS, moderate impairment and disability is now rated as 3 instead of 2. In a study, Stebbins and colleagues developed algorithms to identify tremor dominant versus postural instability or gait difficulty in patients with Parkinson disease based on MDS-UPDRS to facilitate transition from the UPDRS to MDS-UPDRS (145).
A study that was part of the Movement Disorder Society looked at longitudinal changes that may be predicted using the MDS-UPDRS. In this study, Lang and colleagues found that parts I and II of the scale, the non-motor (nm-EDL) and motor experiences of daily living (M-EDL), can be utilized to measure symptom changes early in the disease course (77). This will be particularly important for providers’ assessment of the disease process. Further studies have shown that the MDS-UPDRS, specifically the evaluation of motor and nonmotor experiences of daily living, can relate to impaired health-related quality of life (HRQoL). This suggests an even broader range of applications of this scale (106).
Using the MDS-UPDRS screening tool to assess sleep-related problems in patients with Parkinson disease is an example of using the scale for nonmotor symptoms. A study showed that the MDS-UPDRS is equally effective as the commonly used screening tools, such as the Parkinson’s Disease Sleep Scale, second version, and the Epworth Sleepiness Scale (65).
The MDS-UPDRS has validated Italian and Spanish versions, which are structurally equivalent to the original English version (03; 105). There are also Chinese, French, German, Estonian, and Slovak versions are currently available, and language teams are involved in establishing numerous other versions. Remote administration of MDS-UPDRS has been assessed as telemedicine has become standard during COVID-19 pandemic. It was determined that the total-sum score of the MDS-UPDRS motor subscale could be accurately computed even without the two measures that require in-person assessment (ie, rigidity and postural instability) (96).
(2) Has the reliability of this scale been assessed?
For the original UPDRS, several studies have confirmed moderate to excellent interrater reliability of the historical section (104; 126; 94). The motor section had similar reliability (126; 49). The scale also has high reliability in patients with advanced disease (113).
While adapting the MDS-UPDRS into a Spanish version, the first independent testing of the updated MDS-UPDRS was performed. Authors confirmed reliability and other clinimetric parameters from the original study (105). Additionally, aspects of clinimetric properties were explored and have not been previously reported.
(3) Has the validity of this scale been assessed?
The original version of the UPDRS has well-demonstrated convergent validity with other rating scales for Parkinson disease, such as the Hoehn and Yahr and Schwab and England scales (104; 156; 144; 40).
Internal consistency was computed for each of the MDS-UPDRS parts [Part I (13 items), alpha = 0.79; Part II (13 items), alpha = 0.90; Part III (33 items), alpha = 0.93; Part IV (6 items), alpha = 0.79]. The distributions of the total scores in the MDS-UPDRS and original UPDRS were similar. The MDS-UPDRS showed strong concurrent validity based on high correlations between the two scales, both total and individual parts. As a measure of internal validity, correlations among the MDS-UPDRS parts were examined. These analyses confirmed that each part assesses a different aspect of Parkinson disease, with most parts, except Parts I and II, having relatively low correlations. In summary, the validity of the revised MDS-UPDRS is intrinsically excellent and correlates with the well-established and validated original version of the UPDRS. The validity of the MDS-UPDRS was confirmed in a study that also showed it can be used for individual measurements over time (105). In addition to the internal validity, the MDS-UPDRS part 1 nM-EDL also demonstrated convergent validity with the NMSS (102).
(4) What are the limitations of this scale?
The temporal stability, sensitivity to change, and interrater reliability have yet to be established. This scale was also specifically changed to address the needs of earlier and milder Parkinson disease, meaning that more advanced disease states may be harder to track and measure using the MDS-UPDRS.
In addition to revising the UPDRS, the Movement Disorder Society task force also summarized and made recommendations on the use of additional scales for multiple non-motor aspects of Parkinson disease including:
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• Depression |
Modified Hoehn and Yahr Staging Scale
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Stage 0 = No signs of disease | |
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(63; Unified Parkinson’s Disease Rating Scale) | |
(1) What are the practical applications of this scale?
This is a brief, easy-to-administer scale that may be used to appropriately stage patients with Parkinson disease, giving a general sense about the severity of disease, bilaterality of involvement, and impairment of postural reflexes. Stages 1.5 and 2.5 were added as part of the Unified Parkinson’s Disease Rating Scale, and the scale was intended for clinical, not research use. It can, however, be reliably used for inclusion criteria in clinical research.
(2) Has the reliability of this scale been assessed?
The interrater reliability has been assessed, and the weighted kappa was 0.71, indicating substantial agreement (47).
(3) Has the validity of this scale been assessed?
Ginanneschi and colleagues demonstrated convergent validity with other Parkinson disease rating scales, including the Columbia University Rating Scale (28), the Northwestern University Disability Scale (14), and the Extensive Disability Scale (47). However, no clinimetric information is available regarding the modified portion of the scale (stages 1.5 and 2.5).
(4) What are the limitations of this scale?
The scale provides little specific information about the presence or absence of tremor, rigidity, or bradykinesia. It is more appropriate as a staging instrument and global measure of disease.
Schwab and England Activities of Daily Living Scale
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100% = Completely independent, able to do all chores without slowness, difficulty, or impairment. Essentially normal, unaware of any difficulty. | |
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90% = Completely independent, able to do all chores with some degree of slowness, difficulty, and impairment, might take twice as long. Beginning to be aware of difficulty. | |
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80% = Completely independent in most chores, takes twice as long. Conscious of difficulty and slowness. | |
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70% = Not completely independent, more difficulty with some chores; takes three to four times as long in some; must spend a large part of the day with chores. | |
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60% = Some dependency; can do most chores, but exceedingly slowly and with much effort and errors; some impossible. | |
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50% = More dependent; help with half, slower, etc.; difficulty with everything. | |
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40% = Very dependent; can assist with all chores, but few alone. | |
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30% = With effort, now and then does a few chores alone or begins alone; much help needed. | |
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20% = Nothing alone; can be a slight help with some chores; severe invalid. | |
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10% = Totally dependent, helpless; complete invalid. | |
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0% = Vegetative functions such as swallowing, bladder, and bowel functions are not functioning; bedridden. | |
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(137) | |
(1) What are the practical applications of this scale?
This scale can be rapidly administered and may be used to stage the degree of functional dependence of patients with chronic diseases, particularly those characterized by bradykinesia. One review recommended this scale, along with MDS-UPDRS (previously discussed), to be the most suitable for evaluating activities of daily living in people with Parkinson disease (08)
(2) Has the reliability of this scale been assessed?
The test-retest (ICC 0.7) and inter-rater reliabilites (ICC 0.6) are both adequate (08).
(3) Has the validity of this scale been assessed?
The convergent validity between this scale and other rating scales (eg, the Unified Parkinson Disease Rating Scale) is high (104).
(4) What are the limitations of this scale?
It is often difficult for patients to determine whether they perform chores twice as slowly or four times as slowly as normal; this distinction may be difficult. In addition, for the most part, patients are not questioned regarding their ability to perform a specific set of chores, and functional abilities may vary from chore to chore.
Unified Dyskinesia Rating Scale (UDysRS)
(Available here: www.movementdisorders.org.)
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Part 1A: On-dyskinesia (exclusive of off-dystonia) | ||
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Time spent with on-dyskinesia | ||
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0 = Normal: no dyskinesia | ||
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Score | |
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1. Total hours on: ____ | ||
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Speech | |
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0 = Normal: not at all, no problems | ||
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Chewing and swallowing | |
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0 = Normal: not at all, no problems | ||
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Eating tasks | |
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0 = Normal: not at all, no problems | ||
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Dressing | |
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0 = Normal: not at all, no problems | ||
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Hygiene | |
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0 = Normal: not at all, no problems | ||
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Handwriting | |
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0 = Normal: not at all, no problems | ||
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Doing hobbies and other activities | |
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0 = Normal: not at all, no problems | ||
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Walking and balance | |
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0 = Normal: not at all, no problems | ||
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Public and social settings | |
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0 = Normal: not at all, no problem | ||
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Exciting or emotional settings | |
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0 = Normal: not at all, no problem | ||
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Time spent with off-dystonia | ||
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0 = Never | |
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Effects of spasms or cramps, called off-dystonia, separate from pain on activities | ||
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0 = Normal: not at all | |
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Effects of pain from off-dystonia on daily activities | ||
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0 = Normal: not at all, no pain from off-dystonia | |
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Dystonia pain | ||
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0 = Normal: not painful | |
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Intensity Scale | ||
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Rating by body part that includes both choreic dyskinesia and dystonia during communication, drinking from a cup, dressing, ambulation | ||
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0 = No dyskinesia | ||
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Communication | ||
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0 = No dyskinesia observed | |
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Drinking from a cup | ||
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0 = No dyskinesia observed | ||
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Dressing | ||
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0 = No dyskinesia observed | ||
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Ambulation (walking) | ||
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0 = No dyskinesia observed | ||
(1) What are the practical applications of this scale?
The Unified Dyskinesia Rating Scale (UDysRS) is a rating scale developed specifically for the assessment of dyskinesia in Parkinson disease. The UDysRS contains both self-evaluation questions (by the patient alone or with their caregivers) and items that are assessed directly by the physician to objectively rate the abnormal movements associated with Parkinson disease (48; 19). The UDysRS has important clinical and research applications. By using the UDysRS, physicians can determine the clinical relevance perceived by patients and their caregivers, particularly the onset and remission of dyskinesia (111).
(2) Has the reliability of this scale been assessed?
The intrarater and interrater reliability has been assessed and was found to be satisfactory (48). Further studies have confirmed the intrarater and interrater reliability to be very good. It also has high internal consistency for both subjective and objective rating sections (19).
(3) Has the validity of this scale been assessed?
The validity of this scale has been established. The scale’s temporal stability, which is that it is consistent for a given patient over time, was established. This is important as it can be used to assess patients in ON or OFF states. Also, it can be reliably used in video assessments using on-site raters or centralized off-site rater (50).
(4) What are the limitations of this scale?
The scale has not been tested to measure its sensitivity to changes and has not been studied by other groups, independent of the many investigators who participated in its development. It is nevertheless the most widely used scale currently used to assess dyskinesia.
Scales used to assess patients with tic disorders
Tics are sudden, rapid, repetitive, nonrhythmic motor movements or sounds that vary from simple tics (eg, eye blinking or head jerking) to complex acts (patterned, multi-stepped, coordinated movements) (68; 70; 123). Tics are typically preceded by an urge and are suppressible for variable periods. Tourette syndrome is characterized by both chronic motor and vocal tics (138). The International Parkinsonism and Movement Disorder Society published a systematic review of rating scales for tics in 2017 (108). They identified 16 scales for tics or tic-related sensory phenomena. Of those 16, the authors recommended five for use in primary tic disorders. An additional six rating scales were labeled as “suggested,” and five more as “listed.” The five recommended scales for assessment of tics are the Yale Global Tic Severity Scale (YGTSS) (78), the Shapiro Tourette Syndrome Severity Scale (STSS) (138), the Tourette Syndrome Clinical Global Impression (TS-CGI) (79), the Tourette’s Disorder Scale (TODS) (140), and the Premonitory Urges for Tics Scale (PUTS) (167). These rating scales are also endorsed by the European Clinical Guidelines for Tourette Syndrome and Other Tic Disorders 2.0 (148).
Yale Global Tic Severity Scale (YGTSS)
(Available here: www.sciencedirect.com.)
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Tic Inventory | |||
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1. Description of motor tics (check motor tics present during the past week) | |||
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a. Simple motor tics: rapid, darting, "meaningless" | |||
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2. Description of phonic tic symptoms (check phonic tics present over the past week) | |||
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a. Simple phonic symptoms: fast, "meaningless" sounds | |||
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Number | |||
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Motor score, phonic score (rated separately) | |||
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0 = None | |||
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1 = Single tic | |||
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2 = Multiple discrete tics (2 to 5) | |||
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3 = Multiple discrete tics (greater than 5) | |||
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4 = Multiple discrete tics plus at least one orchestrated pattern of multiple, simultaneous, or sequential tics where it is difficult to distinguish discrete tics | |||
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5 = Multiple discrete tics plus several (greater than 2) orchestrated patterns of multiple, simultaneous, or sequential tics where it is difficult to distinguish discrete tics | |||
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Frequency | |||
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0 = None: no evidence of specific tic behaviors | |||
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1 = Rarely: specific tic behaviors have been present during the previous week; these behaviors occur infrequently, often not on a daily basis; if bouts of tics occur, they are brief and uncommon | |||
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2 = Occasionally: specific tic behaviors are usually present on a daily basis, but there are long tic-free intervals during the day; bouts of tics may occur on occasion and are not sustained for more than a few minutes at a time | |||
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3 = Frequently: specific tic behaviors are present on a daily basis; tic-free intervals as long as 3 hours are not uncommon; bouts of tics occur regularly, but may be limited to a single setting | |||
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4 = Almost always: specific tic behaviors are present virtually every waking hour of every day, and periods of sustained tic behaviors occur regularly; bouts of tics are common and are not limited to a single setting | |||
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5 = Always: specific tic behaviors are present virtually all the time; Tic-free intervals are difficult to identify and do not last more than 5 to 10 minutes at most | |||
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Intensity | |||
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Motor score, phonic score (rated separately) | |||
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0 = Absent | |||
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1 = Minimal intensity: tics not visible or audible (based solely on patient's private experience), or tics are less forceful than comparable voluntary actions and are typically not noticed because of their intensity | |||
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2 = Mild intensity: tics are not more forceful than comparable voluntary actions or utterances and are typically not noticed because of their intensity | |||
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3 = Moderate intensity: tics are more forceful than comparable voluntary actions but are not outside the range of normal expression for comparable voluntary actions or utterances; they may call attention to the individual because of their forceful character | |||
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4 = Marked intensity: tics are more forceful than comparable voluntary actions or utterances and typically have an "exaggerated" character; such tics frequently call attention to the individual because of their forceful and exaggerated character | |||
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5 = Severe intensity: tics are extremely forceful and exaggerated in expression; these tics call attention to the individual and may result in risk of physical injury (accidental, provoked, or self-inflicted) because of their forceful expression | |||
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Complexity | |||
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Motor score, phonic score (rated separately) | |||
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0 = None: if present, all tics are clearly "simple" (sudden, brief, purposeless) in character | |||
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1 = Borderline: some tics are not clearly "simple" in character | |||
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2 = Mild: some tics are clearly "complex" (purposive in appearance) and mimic brief "automatic" behaviors, such as grooming, syllables, or brief meaningful utterances such as "ah huh," "hi," that could be readily camouflaged | |||
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3 = Moderate: some tics are more "complex" (more purposive and sustained in appearance) and may occur in orchestrated bouts that would be difficult to camouflage but could be rationalized or "explained" as normal behavior or speech (picking, tapping, saying "you bet" or "honey,” brief echolalia) | |||
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4 = Marked: some tics are very "complex" in character and tend to occur in sustained orchestrated bouts that would be difficult to camouflage and could not be easily rationalized as normal behavior or speech because of their duration or their unusual, inappropriate, bizarre, or obscene character (a lengthy facial contortion, touching genitals, echolalia, speech atypicalities, longer bouts of saying "what do you mean" repeatedly, or saying "fu" or "sh") | |||
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5 = Severe: some tics involve lengthy bouts of orchestrated behavior or speech that would be impossible to camouflage or successfully rationalize as normal because of their duration or extremely unusual, inappropriate, bizarre, or obscene character (lengthy displays or utterances often involving copropraxia, self-abusive behavior, or coprolalia) | |||
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Interference | |||
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Motor score, phonic score (rated separately) | |||
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0 = None | |||
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1 = Minimal: when tics are present, they do not interrupt the flow of behavior or speech | |||
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2 = Mild: when tics are present, they occasionally interrupt the flow of behavior or speech | |||
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3 = Moderate: when tics are present, they frequently interrupt the flow of behavior or speech | |||
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4 = Marked: when tics are present, they frequently interrupt the flow of behavior or speech, and they occasionally disrupt intended action or communication | |||
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5 = Severe: when tics are present, they frequently disrupt intended action or communication | |||
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Impairment | |||
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Motor score, phonic score (rated separately) | |||
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0 = None | |||
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1 = Minimal: tics associated with subtle difficulties in self-esteem, family life, social acceptance, or school or job functioning (infrequent upset or concern about tics vis-a-vis the future; periodic, slight increase in family tensions because of tics; friends or acquaintances may occasionally notice or comment about tics in an upsetting way) | |||
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2 = Mild: tics associated with minor difficulties in self-esteem, family life, social acceptance, or school or job functioning | |||
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3 = Moderate: tics associated with some clear problems in self-esteem, family life, social acceptance, or school or job functioning (episodes of dysphoria, periodic distress, and upheaval in the family; frequent teasing by peers or episodic social avoidance; periodic interference in school or job performance because of tics) | |||
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4 = Marked: tics associated with major difficulties in self-esteem, family life, social acceptance, or school or job functioning | |||
|
5 = Severe: tics associated with extreme difficulties in self-esteem, family life, social acceptance, or school or job functioning (severe depression with suicidal ideation, disruption of the family [separation or divorce, residential placement], disruption of social ties; severely restricted life because of social stigma and social avoidance, removal from school, or loss of job) | |||
(1) What are the practical applications of this scale?
The Yale Global Tic Severity Scale (YGTSS) includes a separate rating of severity for motor and phonic tics along five dimensions: number, frequency, intensity, complexity and interference. It also includes a checklist for specific types of motor and vocal tics (range = 0 to 25 for each motor and phonic tics). An independent rating of impairment, which focuses on the impact of the tic disorder over the previous week (range = 0 to 50), is added to the total tic score to obtain a final score (range = 0 to 100) (78). It is the most extensively deployed and recommended tic severity rating scale globally and takes approximately 15 to 20 minutes to complete (108).
(2) Has the reliability of this scale been assessed?
The scale has been found to have good interrater reliability in three studies (78; 160; 146). The scale was also shown to have good to very good internal consistency and stability over time (146).
(3) Has the validity of this scale been assessed?
The YGTSS has shown a strong convergent and divergent validity with robust correlations with the Tourette syndrome global scale (TSGS) scores (rs ranging from 0.86 to 0.90), strong correlations with clinician impairment ratings (rs ranging from 0.65 to 0.82), and moderate to strong relations with the Tourette syndrome severity scale (TSSS) (rs ranging from 0.54 to 0.76). Support for discriminant validity was demonstrated by weak to moderate relations with clinician impairment ratings of other forms of psychopathology (78; 160).
Storch and colleagues investigated the reliability and validity of the YGTSS in a study of 28 children and adolescents with Tourette syndrome and found it sufficient (146).
(4) What are the limitations of this scale?
Disadvantages of this scale include a lack of differentiation of historical information from observations made by the examiner and a lack of inclusion of specific tic characteristics, such as tic distribution, type, and suppression. The impairment score reflects overall coping and functioning that may be worsened by coexisting depression, obsessive-compulsive disorder, or attention deficit hyperactivity disorder. Finally, the YGTSS can be lengthy to complete and requires a trained clinician.
Shapiro Tourette Syndrome Severity Scale (STSS)
(Available here: psycnet.apa.org.)
(1) What are the practical applications of this scale?
The STSS is a simple and quick five-question assessment for tic severity.
|
(1) Are the tics noticeable to others? | |
|
(2) Do the tics elicit comments? | |
|
(3) Is the patient considered odd or bizarre? | |
|
(4) Do the tics interfere with functioning? | |
|
(5) Is the patient incapacitated, homebound, or hospitalized? | |
|
(138) | |
(2) Has the reliability of this scale been assessed?
The STSS was shown to have very good interrater reliability (rs = 0.85) (160).
(3) Has the validity of this scale been assessed?
It has also been found to have good concurrent validity compared to YGTSS and TS-CGI (rs = 0.85 and rs = 0.87) (160).
(4) What are the limitations of this scale?
This rating scale is less comprehensive than the YGTSS. It does not assess frequency, complexity, or distribution. The timeframe for which tic severity is measured is uncertain (108).
Tourette Syndrome Clinical Global Impression (TS-CGI)
(Available here: link.springer.com.)
(1) What are the practical applications of this scale?
The TS-CGI is a brief seven-point scale that ranks severity from none to severe. It is quick and easy to administer, typically taking fewer than 2 minutes (108).
(2) Has the reliability of this scale been assessed?
The TS-CGI has very good interrater reliability (rs = 0.89) (160).
(3) Has the validity of this scale been assessed?
The TS-CGI was shown to be valid with good concurrent validity compared to YGSS and STSS (rs = 0.79 and rs = 0.89) (160).
(4) What are the limitations of this scale?
The main limitation of the TS-CGI is that this scale does not assess individual tic dimensions separately (108).
Tourette’s Disorder Scale (TODS)
(Available here: journals.sagepub.com.)
(1) What are the practical applications of this scale?
The TODS rating scale is the only recommended scale that also measures comorbid behavioral symptoms. It has two parts: (1) a parent-administered questionnaire (TODS-PR) and (2) a clinician-administered assessment (TODS-CR) (140).
(2) Has the reliability of this scale been assessed?
The interrater reliability for TODS-CR was found to be good (range r=0.70 to 0.94), with reliability between TODS-PR and TODS-CR (r=0.88) (140).
(3) Has the validity of this scale been assessed?
The TODS-CR correlated with the motor and phonic tic scores of the YGTSS and other rating scales measuring psychometric properties (140).
(4) What are the limitations of this scale?
The TODS rating scale does not assess individual dimensions of tics separately and has a longer time of administration, approximately 20 minutes (108).
Premonitory Urges for Tics Scale (PUTS)
(Available here: journals.lww.com.)
(1) What are the practical applications of this scale?
The PUTS is the only recommended scale that assesses premonitory urges in tics. It can be administered quickly, in approximately 5 to 10 minutes (108). It consists of 10 questions that pertain to premonitory urges related to tic disorders (167).
(2) Has the reliability of this scale been assessed?
The PUTS was shown to have internal consistency in patients older than 10 years of age (alpha = 0.89) but not in patients younger than 10 years of age (alpha = 0.57) (167). The 2-week test-retest results were reliable (r42 = 0.86) (167).
(3) Has the validity of this scale been assessed?
The PUTS scores correlated with YGTSS total scores in patients older than 10 years old, as well as the number of tics, complexity, and interference subscores of the YGTSS (r = 0.52, r = 0.52, r = 0.62 and r = 0.54 respectively) (167).
(4) What are the limitations of this scale?
The PUTS scale is only valid in individuals that are older than 10 years old (167; 108). This is consistent with previous research noting that patients younger than 10 years old are not consistent in reporting premonitory urge sensory phenomenon (167).
Scales used to assess patients with chorea or Huntington disease
Chorea may be characterized as semi-purposeful but involuntary, flowing movements that flow from one part of the body to another in a continuous and random pattern. Chorea may be a feature of several neurologic disorders, including Huntington disease (89), an autosomal dominant disorder characterized by chorea, dystonia, motor impersistence, depression, psychosis, cognitive decline, and change in personality (59). Chorea is also the main feature of Sydenham chorea, a delayed complication following A beta-hemolytic streptococcal pharyngitis (02). Several scales have been developed for the assessment of Huntington chorea; however, only the Unified Huntington Disease Rating Scale (UHDRS-TMS) is recommended for use by the International Parkinson and Movement Disorder Society (112). Only one rating scale has been developed and validated specifically for Sydenham chorea, the UFMG Sydenham’s Chorea Rating Scale (USCRS) (150).
Unified Huntington Disease Rating Scale
Please refer to the Huntington Study Group publication to view the scale (151). The UHDRS is composed of six sections (motor, cognitive, behavioral, functional assessment, independence, and total functional capacity).
(1) What are the practical applications of this scale?
The scale provides a detailed and comprehensive approach for assessing and rating multiple domains for clinical or research purposes, including a motor assessment (eg, abnormal eye movements, involuntary movements), a behavioral assessment (eg, mood, self-esteem, anxiety, hallucinations), a functional assessment checklist (ability to engage in gainful employment, manage finances, drive, etc.), and an independence scale. The Total Motor Score is quick and easy to use in clinical settings, taking approximately 5 minutes to administer (112).
(2) Has the reliability of this scale been assessed?
The interrater reliability for the motor scores was excellent (intraclass correlation coefficient for the total motor score = 0.94, for chorea score = 0.82, and for dystonia score = 0.62) (151). A reevaluation of the scale using a videotaped protocol demonstrated a weighted kappa of 0.62 among 75 HD expert clinicians but only 0.28 of nonclinicians (64).
(3) Has the validity of this scale been assessed?
The degree of motor abnormality highly correlates with changes in basal ganglia metabolism in positron emission tomography studies (170). The motor, cognitive, and functional components and the validated Total Functional Capacity scale were highly intercorrelated (151).
(4) What are the limitations of this scale?
Because the scale is so broad in scope, several more specific items, particularly in motor assessment (eg, dystonia), are covered only briefly, and others (eg, tics, activities of daily living) not at all. A follow-up study in 1997, however, did find that the motor assessment questions could be reduced from 31 to 15 questions without a reduction in validity (141). A study investigated the ability of individual items of the UHDRS to discriminate individual differences in motor or behavioral manifestations in Huntington disease gene expansion carriers without a motor-defined clinical diagnosis (155). In this population, depressed mood, anxiety, and irritable behavior demonstrated good discriminative properties, whereas motor scores failed to do so.
UFMG Sydenham’s Chorea Rating Scale (USCRS)
The scale is available here: movementdisorders.onlinelibrary.wiley.com. The scale is comprised of 27 items each scored 0 to 4 (150).
(1) What are the practical applications of this scale?
This scale provides a detailed quantitative assessment of activities of daily living, behavioral abnormalities, and motor function in patients with Sydenham chorea.
(2) Has the reliability of this scale been assessed?
Interrater reliability has been established in one study between three clinician raters, intraclass correlation coefficient (median for all 27 items, 0.98). It was also found to have internal consistency with Cronbach’s of 0.97 (150).
(3) Has the validity of this scale been assessed?
The validity of the USCRS has not been assessed.
(4) What are the limitations of this scale?
Some items assessing behavioral symptoms of Sydenham chorea had poor correlation with other variables (150).
Scales used to assess patients with dystonia
Dystonic movements are characterized by sustained muscle contractions, frequently resulting in twisting movements sustained for variable periods of time. Dystonia may involve a number of different body regions, including the eyes (blepharospasm), the face (Meige syndrome), the jaw (jaw opening or jaw closing dystonia), the vocal cords (spasmodic dysphonia), the neck (cervical dystonia or torticollis), the limbs, or the trunk (34). Occupational, often task-specific dystonias, such as writer's cramp, are also common. The International Parkinson and Movement Disorder Society Task Force Dystonia Rating Scales recommends five clinical rating scales for use in dystonic syndromes (01). These are the Blepharospasm Disability Index (BSDI), the Cervical Dystonia Impact Scale (CDIP-58, Toronto Western Spasmodic Torticollis Rating Scale, TWSTRS), the Voice Handicap Index (VHI) for laryngeal dystonia, and the Burke-Fahn-Marsden Dystonia Rating Scale (FMDRS) for generalized dystonia (12; 22; 67; 13; 69).
The following two rating scales will be discussed: the Burke-Fahn-Marsden Evaluation Scale for Dystonia (100; 12) and the Toronto Western Spasmodic Torticollis Rating Scale (22). However, since the publication of the Burke-Fahn-Marsden scale, two other dystonia rating scales have been developed and validated, including the Unified Dystonia Rating Scale (UDRS) and the Global Dystonia Rating Scale (GDS) (20). Clinimetric testing showed that the Comprehensive Cervical Dystonia Rating Scale is internally consistent with a logical factor structure (21). The scales can be used for patients with generalized, segmental, or focal dystonias, and all three scales have acceptable levels of reliability. The interrater agreement was good to excellent for all three scales (29). The Jankovic Rating Scale has been used to assess the effects of botulinum toxin on the severity and frequency of involuntary eyelid contractions in treating blepharospasm (129; 69). In addition, health-related quality-of-life instruments have found that blepharospasm can markedly impair the functioning of the affected individuals, who are often subject to social stigmatization and prejudice (58; 127). The craniocervical dystonia questionnaire (CDQ-24) has been developed to evaluate the effects of botulinum toxin treatment on blepharospasm and cervical dystonia (116). The reliability and validity of the Barry-Albright Dystonia Scale (BADS), the Burke-Fahn-Marsden Movement Scale (BFMMS), and the Unified Dystonia Rating Scale (UDRS) in patients with bilateral dystonic cerebral palsy was assessed (114). Moderate to good interrater reliability was found for total scores of the three scales (ICC: BADS = 0.87; BFMMS = 0.86; UDRS = 0.79). However, many sub-items showed low reliability, particularly for the UDRS. High internal consistency was found, although content validity showed insufficient accordance with the new cerebral palsy definition and classification.
A new classification scale has been developed for cervical dystonia, differentiating head and neck subtypes (125). However, the current scales do not adequately address these subtypes. Additionally, there is a need for a scale to evaluate the effect of therapeutic interventions, such as botulinum toxin injections, on symptom improvement. It would be useful to have such a standardized scale guide intervention in terms of dose, targeted muscles, and number of sites (71). There is a need for clinical trials for the development of novel therapeutics for dystonia because the existing treatments are empirical. As these clinical trials come underway, there is a necessity for validated rating scales for dystonias such as spasmodic, task-specific, limb, oromandibular, and trunk dystonias (44). More recently, an oromandibular dystonia-specific rating scale was developed, the Oromandibular Dystonia Rating Scale (OMDRS), which modified elements of the TWSTRS and CIDP-58 scales to oromandibular dystonia (169). This scale has been shown to be both reliable and valid (169).
Burke-Fahn-Marsden Evaluation Scale for Dystonia
(Available here: www.cambridge.org.)
Dystonia movement scale:
Region | Provoking factor | Severity factor | Weight | Product |
Eyes | 0 - 4 | x 0 - 4 | 0.5 | 0 - 8 |
| ||||
I. Provoking Factor | ||
A. General | ||
0 = No dystonia at rest or with action | ||
B. Speech and swallowing | ||
0 = No dystonia | ||
II. Severity factors | ||
A. Eyes | ||
0 = No dystonia present | ||
B. Mouth | ||
0 = No dystonia present | ||
C. Speech and swallowing | ||
0 = Normal | ||
D. Neck | ||
0 = No dystonia present | ||
E. Arm | ||
0 = No dystonia present | ||
F. Trunk | ||
0 = No dystonia present | ||
G. Leg | ||
0 = No dystonia present | ||
Disability scale:
Function | Score |
Speech Sum: (Maximum = 30) | 0 - 4 |
A. Speech | ||
0 = Normal | ||
B. Handwriting (tremor or dystonia) | ||
0 = Normal | ||
C. Feeding | ||
0 = Normal | ||
D. Eating and swallowing | ||
0 = Normal | ||
E. Hygiene | ||
0 = Normal | ||
F. Dressing | ||
0 = Normal | ||
G. Walking | ||
0 = Normal | ||
(12) | ||
The following vidso demonstrates an example of sensory “tricks”:
Dystonic movements are characterized by sustained muscle contractions, frequently resulting in twisting movements sustained for variable periods of time. Dystonia may involve a number of different body regions, including the eyes (blepharospasm), the face (Meige syndrome), the jaw (jaw opening or jaw closing dystonia), the vocal cords (spasmodic dysphonia), the neck (cervical dystonia or torticollis), the limbs, or the trunk (34). Occupational, often task-specific dystonias, such as writer's cramp, are also common. The International Parkinson and Movement Disorder Society Task Force Dystonia Rating Scales recommends five clinical rating scales for use in dystonic syndromes (01). These are the Blepharospasm Disability Index (BSDI), the Cervical Dystonia Impact Scale (CDIP-58, Toronto Western Spasmodic Torticollis Rating Scale, TWSTRS), the Voice Handicap Index (VHI) for laryngeal dystonia, and the Burke-Fahn-Marsden Dystonia Rating Scale (FMDRS) for generalized dystonia (12; 22; 67; 13; 69).
The following two rating scales will be discussed: the Burke-Fahn-Marsden Evaluation Scale for Dystonia (100; 12) and the Toronto Western Spasmodic Torticollis Rating Scale (22). However, since the publication of the Burke-Fahn-Marsden scale, two other dystonia rating scales have been developed and validated, including the Unified Dystonia Rating Scale (UDRS) and the Global Dystonia Rating Scale (GDS) (20). Clinimetric testing showed that the Comprehensive Cervical Dystonia Rating Scale is internally consistent with a logical factor structure (21). The scales can be used for patients with generalized, segmental, or focal dystonias, and all three scales have acceptable levels of reliability. The interrater agreement was good to excellent for all three scales (29). The Jankovic Rating Scale has been used to assess the effects of botulinum toxin on the severity and frequency of involuntary eyelid contractions in treating blepharospasm (129; 69). In addition, health-related quality-of-life instruments have found that blepharospasm can markedly impair the functioning of the affected individuals, who are often subject to social stigmatization and prejudice (58; 127). The craniocervical dystonia questionnaire (CDQ-24) has been developed to evaluate the effects of botulinum toxin treatment on blepharospasm and cervical dystonia (116). The reliability and validity of the Barry-Albright Dystonia Scale (BADS), the Burke-Fahn-Marsden Movement Scale (BFMMS), and the Unified Dystonia Rating Scale (UDRS) in patients with bilateral dystonic cerebral palsy was assessed (114). Moderate to good interrater reliability was found for total scores of the three scales (ICC: BADS = 0.87; BFMMS = 0.86; UDRS = 0.79). However, many sub-items showed low reliability, particularly for the UDRS. High internal consistency was found, although content validity showed insufficient accordance with the new cerebral palsy definition and classification.
A new classification scale has been developed for cervical dystonia, differentiating head and neck subtypes (125). However, the current scales do not adequately address these subtypes. Additionally, there is a need for a scale to evaluate the effect of therapeutic interventions, such as botulinum toxin injections, on symptom improvement. It would be useful to have such a standardized scale guide intervention in terms of dose, targeted muscles, and number of sites (71). There is a need for clinical trials for the development of novel therapeutics for dystonia because the existing treatments are empirical. As these clinical trials come underway, there is a necessity for validated rating scales for dystonias such as spasmodic, task-specific, limb, oromandibular, and trunk dystonias (44). More recently, an oromandibular dystonia-specific rating scale was developed, the Oromandibular Dystonia Rating Scale (OMDRS), which modified elements of the TWSTRS and CIDP-58 scales to oromandibular dystonia (169). This scale has been shown to be both reliable and valid (169).
Burke-Fahn-Marsden Evaluation Scale for Dystonia
(Available here: www.cambridge.org.)
Dystonia movement scale:
|
Region |
Provoking factor |
Severity factor |
Weight |
Product |
|
Eyes |
0 - 4 |
x 0 - 4 |
0.5 |
0 - 8 |
|
| ||||
|
I. Provoking Factor | ||
|
A. General | ||
|
0 = No dystonia at rest or with action | ||
|
B. Speech and swallowing | ||
|
0 = No dystonia | ||
|
II. Severity factors | ||
|
A. Eyes | ||
|
0 = No dystonia present | ||
|
B. Mouth | ||
|
0 = No dystonia present | ||
|
C. Speech and swallowing | ||
|
0 = Normal | ||
|
D. Neck | ||
|
0 = No dystonia present | ||
|
E. Arm | ||
|
0 = No dystonia present | ||
|
F. Trunk | ||
|
0 = No dystonia present | ||
|
G. Leg | ||
|
0 = No dystonia present | ||
Disability scale:
|
Function |
Score |
|
Speech |
0 - 4 |
|
| |
|
A. Speech | ||
|
0 = Normal | ||
|
B. Handwriting (tremor or dystonia) | ||
|
0 = Normal | ||
|
C. Feeding | ||
|
0 = Normal | ||
|
D. Eating and swallowing | ||
|
0 = Normal | ||
|
E. Hygiene | ||
|
0 = Normal | ||
|
F. Dressing | ||
|
0 = Normal | ||
|
G. Walking | ||
|
0 = Normal | ||
|
(12) | ||
The following vidso demonstrates an example of sensory “tricks”:
Dystonia with camptocormia helped by a postural maneuver in primary generalized dystonia
This patient has primary generalized dystonia manifested mostly by dystonic camptocormia. Her dystonia is markedly relieved by stepping with one foot onto a chair ("geste antagonistique" or "geste antagoniste")--analogous to a "se...
(1) What are the practical applications of this scale?
The scale allows the investigator to rate dystonic movements, taking many different aspects of dystonia into account, including the role of provoking factors, the severity of dystonia in multiple body regions, and the functional disability resulting from the dystonia. Due to the various factors incorporated into this scale, it is more complex compared to others, but it can be easily administered for studies, such as when evaluating the effect of GPi deep-brain stimulation for dystonia (157). Also, this scale is highly correlated with the Unified Dystonia Rating Scale and Global Dystonia Rating Scale (01).
(2) Has the reliability of this scale been assessed?
Both interrater and intrarater (test-retest) reliability were high (12). The intra- and interrater reliability were again tested as part of the SPIDY trial and confirmed good to very good intraclass correlation coefficient (75).
(3) Has the validity of this scale been assessed?
The correlation between this scale and global impressions of dystonia severity was high (12).
(4) What are the limitations of this scale?
Although the scale stratifies dystonia according to body region (eg, arm, leg, etc.), it does not reflect the fact that within each of these regions, different types of dystonia may exist (eg, foot inversion, foot eversion, toe-curling, great toe extension). Similarly, it does not assess individual body areas in detail (01).
Toronto Western Spasmodic Torticollis Rating Scale
(Available here: www.neurosurgeryresident.net.)
I. Torticollis Severity Scale
A. Maximal excursion. Rate the maximum amplitude of excursion by asking the patient not to oppose the abnormal movement; the examiner may use distracting or aggravating maneuvers. When the degree of deviation is between scores, chose the higher of the two. | |||
1. Rotation (turn: right or left) | |||
0 = None | |||
2. Laterocollis (tilt: right or left) (exclude shoulder elevation) | |||
0 = None | |||
3. Anterocollis or retrocollis (a or b) | |||
a. Anterocollis | |||
0 = None | |||
b. Retrocollis | |||
0 = None | |||
4. Lateral shift (right or left) | |||
0 = Absent | |||
5. Sagittal shift (forward or backward) | |||
0 = Absent | |||
B. Duration factor. Provide an overall score estimated throughout the course of the standardized examination after estimating maximal excursion (exclusive of asking the patient to allow the head to deviate maximally). Weighted times 2. | |||
0 = None | |||
C. Effect of sensory tricks | |||
0 = Complete relief by one or more "trick" | |||
D. Shoulder elevation and anterior displacement | |||
0 = Absent | |||
E. Range of motion (without the aid of sensory tricks). If limitations occur in more than one plane of motion, use the individual score that is highest. | |||
0 = Able to move to the extreme opposite position. | |||
F. Time. Time (up to 60 seconds) that the patient is able to maintain the head within 10 degrees of the neutral position without the use of sensory "tricks" (the mean of two attempts). | |||
0 = greater than 60 seconds | |||
II. Disability Scale
A. Work | |
0 = No difficulty | |
B. Activities of daily living (eg, feeding, dressing, or hygiene, including washing, shaving, makeup, etc.) | |
0 = No difficulty with any activity | |
C. Driving | |
0 = No difficulty (or has never driven a car) | |
D. Reading | |
1 = Unlimited ability to read in normal seated position but bothered by torticollis | |
E. Television | |
0 = No difficulty | |
F. Activities outside the home (eg, shopping, walking about, movies, dining, and other recreational activities) | |
0 = No difficulty | |
III. Pain Scale (maximum = 20)
A. Severity of pain | |
Rate the severity of neck pain due to ST during the last week on a scale of 0 to 10, in which a score of 0 represents no pain, and 10 represents the most excruciating pain imaginable. | |
Score calculated as: (worst + best + (2*usual))/4 Best ____ | |
B. Duration of pain | |
0 = None | |
C. Disability due to pain | |
0 = No limitation or interference from pain | |
| |
(1) What are the practical applications of this scale?
The scale allows the examiner to evaluate torticollis in a focused and detailed manner, taking into account multiple different aspects of clinical phenomenology including maximal excursion, different types of movement (eg, rotation, tilt, shift), duration of involuntary movement, and effect of sensory tricks. In a revision of the scale, non-motor features were incorporated. Disability and pain are important components of this scale (71). Also, it includes a videotape protocol providing standardization of evaluation. This is the most widely used scale for cervical dystonia and has been used in assessments of treatment trials for botulinum toxin, pharmacotherapy, and surgery (01).
(2) Has the reliability of this scale been assessed?
Interrater agreement, assessed by interclass correlation coefficients, was substantial (20; 01).
(3) Has the validity of this scale been assessed?
Validity was demonstrated by assessing the correlation between rating scores and self-reported improvement in disability and pain scores.
(4) What are the limitations of this scale?
It may be difficult for some examiners to estimate duration using percentages (eg, 25% of time vs. 50% of time). This scale may be time consuming and complex to administer in clinical practice (71).
(1) What are the practical applications of this scale?
The scale allows the investigator to rate dystonic movements, taking many different aspects of dystonia into account, including the role of provoking factors, the severity of dystonia in multiple body regions, and the functional disability resulting from the dystonia. Due to the various factors incorporated into this scale, it is more complex compared to others, but it can be easily administered for studies, such as when evaluating the effect of GPi deep-brain stimulation for dystonia (157). Also, this scale is highly correlated with the Unified Dystonia Rating Scale and Global Dystonia Rating Scale (01).
(2) Has the reliability of this scale been assessed?
Both interrater and intrarater (test-retest) reliability were high (12). The intra- and interrater reliability were again tested as part of the SPIDY trial and confirmed good to very good intraclass correlation coefficient (75).
(3) Has the validity of this scale been assessed?
The correlation between this scale and global impressions of dystonia severity was high (12).
(4) What are the limitations of this scale?
Although the scale stratifies dystonia according to body region (eg, arm, leg, etc.), it does not reflect the fact that within each of these regions, different types of dystonia may exist (eg, foot inversion, foot eversion, toe-curling, great toe extension). Similarly, it does not assess individual body areas in detail (01).
Toronto Western Spasmodic Torticollis Rating Scale
(Available here: www.neurosurgeryresident.net.)
I. Torticollis Severity Scale
A. Maximal excursion. Rate the maximum amplitude of excursion by asking the patient not to oppose the abnormal movement; the examiner may use distracting or aggravating maneuvers. When the degree of deviation is between scores, chose the higher of the two. | |||
1. Rotation (turn: right or left) | |||
0 = None | |||
2. Laterocollis (tilt: right or left) (exclude shoulder elevation) | |||
0 = None | |||
3. Anterocollis or retrocollis (a or b) | |||
a. Anterocollis | |||
0 = None | |||
b. Retrocollis | |||
0 = None | |||
4. Lateral shift (right or left) | |||
0 = Absent | |||
5. Sagittal shift (forward or backward) | |||
0 = Absent | |||
B. Duration factor. Provide an overall score estimated throughout the course of the standardized examination after estimating maximal excursion (exclusive of asking the patient to allow the head to deviate maximally). Weighted times 2. | |||
0 = None | |||
C. Effect of sensory tricks | |||
0 = Complete relief by one or more "trick" | |||
D. Shoulder elevation and anterior displacement | |||
0 = Absent | |||
E. Range of motion (without the aid of sensory tricks). If limitations occur in more than one plane of motion, use the individual score that is highest. | |||
0 = Able to move to the extreme opposite position. | |||
F. Time. Time (up to 60 seconds) that the patient is able to maintain the head within 10 degrees of the neutral position without the use of sensory "tricks" (the mean of two attempts). | |||
0 = greater than 60 seconds | |||
II. Disability Scale
A. Work | |
0 = No difficulty | |
B. Activities of daily living (eg, feeding, dressing, or hygiene, including washing, shaving, makeup, etc.) | |
0 = No difficulty with any activity | |
C. Driving | |
0 = No difficulty (or has never driven a car) | |
D. Reading | |
1 = Unlimited ability to read in normal seated position but bothered by torticollis | |
E. Television | |
0 = No difficulty | |
F. Activities outside the home (eg, shopping, walking about, movies, dining, and other recreational activities) | |
0 = No difficulty | |
III. Pain Scale (maximum = 20)
A. Severity of pain | |
Rate the severity of neck pain due to ST during the last week on a scale of 0 to 10, in which a score of 0 represents no pain, and 10 represents the most excruciating pain imaginable. | |
Score calculated as: (worst + best + (2*usual))/4 Best ____ | |
B. Duration of pain | |
0 = None | |
C. Disability due to pain | |
0 = No limitation or interference from pain | |
(22) | |
(1) What are the practical applications of this scale?
The scale allows the examiner to evaluate torticollis in a focused and detailed manner, taking into account multiple different aspects of clinical phenomenology including maximal excursion, different types of movement (eg, rotation, tilt, shift), duration of involuntary movement, and effect of sensory tricks. In a revision of the scale, non-motor features were incorporated. Disability and pain are important components of this scale (71). Also, it includes a videotape protocol providing standardization of evaluation. This is the most widely used scale for cervical dystonia and has been used in assessments of treatment trials for botulinum toxin, pharmacotherapy, and surgery (01).
(2) Has the reliability of this scale been assessed?
Interrater agreement, assessed by interclass correlation coefficients, was substantial (20; 01).
(3) Has the validity of this scale been assessed?
Validity was demonstrated by assessing the correlation between rating scores and self-reported improvement in disability and pain scores.
(4) What are the limitations of this scale?
It may be difficult for some examiners to estimate duration using percentages (eg, 25% of time vs. 50% of time). This scale may be time consuming and complex to administer in clinical practice (71).
Scales used to assess patients with myoclonus
Myoclonus is a sudden, brief, jerking movement. The movements have classically been associated with metabolic derangements, as seen in liver or renal failure. However, there are many causes of myoclonus, including medications. Myoclonic movements may be focal, multifocal, or generalized. Myoclonus may originate from cortical, subcortical, or spinal cord pathology (99; 35; 89). The Myoclonus Evaluation Scale (100; 15) and the Unified Myoclonus Rating Scale (42) will be discussed. The Unified Myoclonus Rating Scale is currently the most widely used rating scale to assess myoclonus symptoms.
Myoclonus Evaluation Scale
|
A. Score sustained posture (an individual score in given for each limb tested): | ||
|
1. Of outstretched arm: | ||
|
0 = Normal | ||
|
2. Of flexed arm in front of face: | ||
|
0 = Normal | ||
|
3. Of leg elevated from bed while lying down: | ||
|
0 = Normal | ||
|
4. Of body while standing on one leg: | ||
|
0 = Normal | ||
|
5. Of face while pursing lips: | ||
|
0 = Normal | ||
|
B. Score dynamic function (an individual score in given for each limb tested): | ||
|
1. Finger-nose test: | ||
|
0 = Normal | ||
|
2. Rapid hand tapping: | ||
|
0 = Normal | ||
|
3. Rapid-pronation-supination hand movements: | ||
|
0 = Normal | ||
|
4. Heel-shin test: | ||
|
0 = Normal | ||
|
5. Gait: | ||
|
0 = Normal | ||
|
6. Speech: | ||
|
0 = Normal | ||
|
7. Handwriting | ||
|
0 = Normal | ||
|
8. Drawing of Archimedes spiral: | ||
|
0 = Normal | ||
|
(100; 15) | ||
(1) What are the practical applications of this scale?
The scale provides data on multiple body regions as well as postures and dynamic tests (eg, finger-to-nose test, gait, etc.)
(2) Has the reliability of this scale been assessed?
This has not been formally assessed.
(3) Has the validity of this scale been assessed?
This has not been formally assessed.
(4) What are the limitations of this scale?
Terms that describe severity ("mild," "moderate," and "severe") may be interpreted differently by various users.
The Unified Myoclonus Rating Scale (UMRS)
|
Section 1. Patient questionnaire | |
|
A. Speech | |
|
0 = My speech is normal. | |
|
B. Reading (silently) | |
|
0 = My ability to read is normal. | |
|
C. Handwriting | |
|
0 = My handwriting is normal. | |
|
D. Eating | |
|
0 = I eat normally. | |
|
E. Drinking | |
|
0 = I drink normally. | |
|
F. Swallowing | |
|
0 = I swallow without difficulty. | |
|
G. Hygiene | |
|
0 = I bathe (or shower), brush my teeth and comb my hair normally. | |
|
H. Dressing | |
|
0 = I can get dressed without a problem. | |
|
I. Arising | |
|
0 = I arise from a chair without difficulty. | |
|
J. Standing | |
|
0 = I can stand by myself without difficulty. | |
|
K. Walking | |
|
0 = I have no disability. | |
|
| |
|
A. Upper face, lower face, neck, trunk, right arm, left arm, right leg, left leg | |
|
Frequency at rest | |
|
The total score (maximum = 128) is calculated by multiplying frequency x amplitude for each body region. | |
|
| |
|
Score 1 if a stimulus produces a jerk in any body part; score 0 if no jerk is elicited. Each stimulus is performed only once. | |
|
• Threat: thrust hands towards patient’s face unexpectedly. | |
|
A total stimulus-sensitivity is 0 to 17. | |
|
| |
|
Close eyelids, neck, trunk, right arm, left arm, right leg, left leg, arising, standing, and walking (ask the patient to walk down the corridor for 15 seconds, turn, then walk back and sit down. Patients who are unsteady or at risk for falling will walk with the examiner at their side, holding one arm if necessary). | |
|
0 = No jerks. | |
|
The total score (maximum = 160) is calculated by multiplying frequency x amplitude for each body part. | |
|
| |
|
A. Writing. Ask the patient to write “London, England”, in script with their hand reading on the desk. Patients who do not write in script with their hand resting on the desk. Patients who do not write in script may print. Circle the hand used to write. | |
|
0. Normal | |
|
B. Right hand spiral. Ask the patient to complete the spiral connecting the dots with the right hand in one continuous motion. The hand remains off the desk during the task. | |
|
0. Normal. | |
|
C. Left hand spiral. Ask the patient to complete the spiral connecting the dots with the left hand in one continuous motion. The hand remains off the desk during the task. | |
|
0. Normal. | |
|
D-E. Pouring water. Ask the patient to pour an 8-ounce glass of water into an empty 8-ounce glass with his dominant hand, without touching the two glasses. Use clear plastic glasses. | |
|
0 = Normal, no spill. | |
|
F-G. Soup spoon. Ask the patient to use a soupspoon to bring water from a cup to his mouth with his dominant hand. | |
|
0 = Normal, no spill. | |
|
A total score for functional tests is 0 to 28. | |
|
| |
|
0 = Normal. | |
|
| |
|
0 = Absent (less than 50% likely). | |
|
| |
|
0 = Not present. | |
|
Total scores for sections 1, 3, 5, 6, 7, and 8 are calculated by addition. (42) | |
(1) What are the practical applications for this scale?
It is easy to use and can be completed in less than 15 minutes (122).
(2) Has the reliability of this scale been assessed?
Interrater reliability was calculated for each section of the UMRS and confirmed as excellent (122). Intrarater reliability has not been formally assessed.
(3) Has the validity of this scale been assessed?
Statistical validation of the UMRS was performed by members of the Myoclonus Study Group (42).
(4) What are the limitations of this scale?
Many of the sections (1, 3, 4, 6, 7, and 8) are potentially biased by the underlying illness or symptoms (10).
Scales used to assess patients with ataxia
There are numerous, varied causes of ataxia, from inherited disorders to autoimmune conditions to neurodegenerative disorders. When coordination becomes the primary clinical concern, the International Cooperative Ataxia Rating Scale (ICARS) (154) had been the most commonly used quantification method, but many studies are beginning to adopt the easier-to-use Scale for the Assessment and Rating of Ataxia (SARA). SARA was developed to take less time and addresses some of the concerns related to research use (133; 134). The original ICARS was modified by adding seven ataxia tests (modified ICARS or MICARS). A five-test subset of MICARS was developed, namely the Brief Ataxia Rating Scale (BARS) (132). The BARS was developed for use in clinical practice, given ease of administration. It has been validated against the SARA and full ICARS (132). BARS has high interrater reliability (132). The SARA and the ICARS are the most studied and validated scales, and it is recommended that they be utilized in long-term multicenter studies (131). All three scales, namely SARA, ICARS, and BARS, have been shown to have a high reliability of early-onset ataxia but a low discriminative validity (09).
International Cooperative Ataxia Rating Scale:
|
I. Posture and gait disturbances | |||
|
A. Walking capacities (observed during a 10-meter test including a half turn near a wall at about 1.5 meter) | |||
|
0 = Normal | |||
|
B. Gait speed (observed in patients with preceding scores of 1 to 3; a preceding score of 4 or more automatically gives a score of 4 on this test.) | |||
|
0 = Normal | |||
|
C. Standing capacities, eyes open (the patient is asked first to try to stay on one foot; if that is impossible, he is asked to stand with the feet in tandem position; if impossible, he is asked to stand with his feet together; for the natural position, the patient is asked to find a comfortable standing position.) | |||
|
0 = Normal: able to stand on one foot more than 10 seconds | |||
|
D. Spread of feet in natural position without support and eyes open (the patient is asked to find a comfortable position, and then, the distance between medial malleoli is measured.) | |||
|
0 = Normal (< 10 cm) | |||
|
E. Body sway with feet together and eyes open | |||
|
0 = Normal | |||
|
F. Body sway with feet together and eyes closed | |||
|
0 = Normal | |||
|
G. Quality of sitting position (thighs together, on a hard surface, arms folded). | |||
|
0 = Normal | |||
|
Posture and gait score (static score): result/34 | |||
|
II. Kinetic functions | |||
|
H. Knee-tibia test (decomposition of movement and intention tremor). The test is performed in the supine position, but the head is tilted so that visual control is possible. The patient is asked to raise one leg and place the heel on the knee, then to slide the heel down the anterior tibial surface of the resting leg toward the ankle. On reaching the ankle joint, the leg is again raised in the air to a height of approximately 40 cm, and the action is repeated. At least three movements of each limb must be performed for proper assessment. | |||
|
0 = Normal | |||
|
I. Action tremor in the heel-to-knee test. This is the same test as preceding one; the action tremor of the heel on the knee is specifically observed when the patient holds the heel on the knee for a few seconds before sliding down the anterior tibial surface; visual control is required. | |||
|
0 = No trouble | |||
|
J. Finger-to-nose test (decomposition and dysmetria) The subject sits on a chair; the hand is resting on the knee before the beginning of the movement; visual control is required. Three movements of each limb must be performed for proper assessment. | |||
|
0 = No trouble | |||
|
K. Finger-to-nose test (intention tremor of the finger) The studied tremor is that appearing during the ballistic phase of the movement; the patient is sitting comfortably with his hand resting on his thigh; visual control is required; three movements of each limb must be performed for proper assessment. | |||
|
0 = No trouble | |||
|
L. Finger-finger test (action tremor or instability). The sitting patient is asked to maintain medially his two index fingers pointing at each other for about 10 seconds, at a distance of about 1 cm, at the level of the thorax, under visual control. | |||
|
0 = Normal | |||
|
M. Pronation-supination alternating movements. The subject, sitting comfortably on a chair, is asked to raise his forearm vertically and to make alternative movements of the hand. Each hand is moved and assessed separately. | |||
|
0 = Normal | |||
|
N. Drawing of the Archimedes spiral on a predrawn pattern. The subject is settled comfortably in front of a table; the sheet of paper is fixed to avoid artifacts. The subject is asked to perform the task without timing requirements. The same conditions of examination must be used at each examination: same table, same pen. The dominant hand is examined. | |||
|
0 = Normal | |||
|
Kinetic score (limb coordination): result/52 | |||
|
III. Speech disorders | |||
|
O. Dysarthria (fluency of speech). The patient is asked to repeat a standard phrase several times. The phrase is always the same; for instance, a patient may be asked to repeat “a mischievous spectacle in Czechoslovakia.” | |||
|
0 = Normal | |||
|
P. Dysarthria (clarity of speech) | |||
|
0 = Normal | |||
|
Dysarthria score: result/8 | |||
|
IV. Oculomotor disorders | |||
|
Q. Gaze-evoked nystagmus. The subject is asked to look laterally at the finger of the examiner; the movements assessed are mainly horizontal, but they may be oblique, rotatory, or vertical. | |||
|
0 = Normal | |||
|
R. Abnormalities of the ocular pursuit. The subject is asked to follow the slow lateral movement performed by the finger of the examiner. | |||
|
0 = Normal | |||
|
S. Dysmetria of the saccade. The two index fingers of the examiner are placed in each temporal visual field of the patient, whose eyes are in the primary position; the patient is then asked to look laterally at the finger, on the right and on the left; the average overshoot or undershoot of the two sides is then estimated. | |||
|
0 = Absent | |||
|
Oculomotor movement score: result/6 TOTAL ATAXIA SCORE: results/I00 | |||
(1) What are the practical applications of this scale?
This scale is easy to administer and assesses the key areas typically affected in ataxic disorders. There are some overlapping and interdependent items, however. It also weighs the various components based on functional impact, with limb ataxia most heavily, then stance and gait. The scale can be used for a diffuse process, such as in neurodegenerative diseases, or more focal cerebellar lesions.
(2) Has the reliability of this scale been assessed?
In patients with multiple system atrophy and cerebellar signs, this scale had an excellent internal consistency rate (Cronback = 0.93); however, some test items had overlap with parkinsonism (153). In an analysis of the scale in patients with various spinocerebellar ataxias and Friedreich ataxia, Kendall’s omega was 0.990 indicating a highly significant interrater correlation (147). An evaluation of the scale demonstrated very good interrater reliability and high test-retest reliability and Cronbach’s alpha (133; 134). In addition, there was excellent reliability in patients with degenerative diseases, focal ischemia, and surgically induced ataxia (Schoch e al 2007).
(3) Has the validity of this scale been assessed?
ICARS has been validated against the Barthel index (inversely related) and disease duration with significant correlation (133; 134). The validity of this scale was also investigated comparing acute versus chronic and focal versus degenerative disease with excellent correlation to clinical state (135). ICARS shows a significant correlation with the Friedrich’s Ataxia Rating Score (FARS) when evaluating patients with Friedrich ataxia (32; 11).
(4) What are the limitations of this scale?
This scale may be less accurate in patients with parkinsonism and may vary between ataxic conditions. Due to the variability of diseases, this scale may need to be individually evaluated for patients with spinocerebellar ataxias, Friedrich ataxia, and other forms of ataxia. ICARS may be preferred in focal lesions, but whether ICARS or the Scale for the Assessment and Rating of Ataxia (SARA) is preferred for degenerative diseases needs further study.
Scale for Assessment and Rating of Ataxia (SARA)
The SARA is an eight-item performance-based scale, yielding total severity of ataxia (0 [no ataxia] to 40 [severe ataxia]). Items assessed include gait, stance, sitting, speech disturbance, finger chase, nose-finger test, fast alternating hand movements, and heel-shin slide. The scale does not assess oculomotor function. The aggregate score is then translated to performance of daily living activities (< 5.5 mild dependence, < 10 minimal dependence, < 14.25 moderate dependence, > 23 maximal dependence).
|
1. Gait. Proband is asked to walk at a safe distance parallel to a wall, including a half-turn, and to walk in tandem without support. | ||
|
0 = Normal | ||
|
2. Stance. Stand in natural position, with feet together in parallel and in tandem. Three trials are allowed. Best trial is rated. | ||
|
0 = Normal, able to stand in tandem for >10 seconds | ||
|
3. Sitting. Sit without support of feet, eyes open, and arms outstretched to front. | ||
|
0 = Normal, no difficulties sitting >10 seconds | ||
|
4. Speech disturbance. | ||
|
0 = Normal | ||
|
5. Finger chase. Follow movement of examiner’s index finger, as fast and precisely as possible. | ||
|
0 = No dysmetria | ||
|
6. Nose-finger test. Point repeatedly with his index finger from his nose to examiner’s finger. | ||
|
0 = No tremor | ||
|
7. Fast alternating hand movements. Perform 10 cycles of repetitive alternation of pro- and supinations of hand on his or her thigh as fast and as precise as possible. | ||
|
0 = Normal, no irregularities (performs < 10 seconds) | ||
|
8. Heel-shin slide. Supine position. Lift one leg, point with heel to the opposite knee, and slide down along the shin to the ankle. Repeat three times. | ||
|
0 = Normal | ||
(1) What are the practical applications of this scale?
SARA is a short and easy-to-use clinical score to assess the core symptoms of ataxia (54). Due to its ease of administration and strong psychometric properties, SARA has become the cornerstone for measuring disease severity in different ataxia conditions (168; 130).
(2) Has the reliability of this scale been assessed?
The SARA has excellent test/retest reliability in spinocerebellar ataxia (SCA) (ICC = 0.90) (133) and Friedreich ataxia (FA) (ICC = 0.99) (11). The interrater reliability has been established in spinocerebellar ataxia (133) as well as sporadic ataxia disorders (166). The intrarater reliability is also excellent (ICC – 0.90) in sporadic ataxia (166). The SARA has been shown to have excellent internal consistency in spinocerebellar ataxia (Cronbach’s alpha =0.94) (133), in Friedreich ataxia (Cronbach’s alpha = 0.89) (11), and in sporadic ataxia disorders (Cronbach’s alpha = 0.97) (166).
(3) Has the validity of this scale been assessed?
SARA has demonstrated excellent validity with ICARS (r = 0.953) and Friedreich’s Ataxia Rating Scale (r = 0.938) (131). SARA score has been shown to increase with disease stage (p< 0.001) and was closely correlated with the Barthel index (r=0.80) and part IV (functional assessment) of the Unified Huntington’s Disease Rating Scale (UHDRS-IV, r=0.89) (134). The SARA also correlated with measures of activities of daily living (11).
(4) What are the limitations of this scale?
Although the SARA instructions are very precise, there is room for discussion on how to correctly perform and rate single items. This increases interrater variability, especially in multicenter studies. This was aggravated by a lack of standardized teaching material or certification for application of the SARA until 2023, when a video-based training and certification program was developed (54).
Scales used to assess patients with essential tremor
Essential tremor is a 4 to 12 Hz kinetic and postural tremor that may be sporadic or familial. Tremor is most commonly present in the arms, although the head or voice may also be involved (92). The following two rating scales will be discussed: (1) the Washington Heights-Inwood Tremor Rating Scale (89; 91) and (2) the Fahn-Tolosa-Marin Tremor Rating Scale (FTM-TRS) (36).
Washington Heights-Inwood Genetic Study of Essential Tremor (WHIGET) Tremo Rating Scale
The original WHIGET tremor rating scale was developed for distinguishing abnormal upper extremity tremor from normal tremor in population studies of essential tremor. The scale was later revised so that kinetic tremor was rated from 0 to 4 (instead of 0 to 3, whereas posture tremor retained 0 to 3). This modification was felt to broaden the applicability of the scale to clinical trials (30).
|
Maneuver A: First sustained arm extension with the arms held in front of the body and the wrists pronated, and then sustained arm extension with the arms flexed at the elbow and the fingers opposing one another in a midline position. | ||
|
1. Dominant arm postural tremor: | ||
|
0 = No visible tremor | ||
|
2. Nondominant arm postural tremor: | ||
|
0 = No visible tremor | ||
|
| ||
|
3. Dominant arm pouring kinetic tremor: | ||
|
0 = No visible tremor | ||
|
4. Nondominant arm pouring kinetic tremor: | ||
|
0 = No visible tremor | ||
|
| ||
|
5. Dominant arm drinking kinetic tremor: | ||
|
0 = No visible tremor | ||
|
6. Nondominant arm drinking kinetic tremor: | ||
|
0 = No visible tremor. | ||
|
| ||
|
7. Dominant arm spoon kinetic tremor: | ||
|
0 = No visible tremor | ||
|
8. Nondominant arm spoon kinetic tremor: | ||
|
0 = No visible tremor | ||
|
| ||
|
9. Dominant arm finger-to-nose kinetic tremor: | ||
|
0 = No visible tremor | ||
|
10. Nondominant arm finger-to-nose kinetic tremor: | ||
|
0 = No visible tremor | ||
|
| ||
|
11. Dominant arm drawing kinetic tremor: | ||
|
0 = No visible tremor | ||
|
12. Nondominant arm drawing kinetic tremor: | ||
|
0 = No visible tremor | ||
|
A total tremor score (maximum = 36) is calculated for each subject by addition of the scores from each of the 12 bedside tests. | ||
(1) What are the practical applications of this scale?
This scale allows the rater to evaluate postural and kinetic tremor separately, to evaluate kinetic tremor during multiple explicitly defined maneuvers, to rate the dominant and nondominant arms separately, and to rate the tremor severity based on amplitude, presence of oscillations, and constancy of tremor.
(2) Has the reliability of this scale been assessed?
Interrater and intrarater reliability of version 2 using videotape was high (weighted kappa > 0.97) (90).
(3) Has the validity of this scale been assessed?
The correlation between the total tremor score and self-reported assessments of tremor severity was high (90).
(4) What are the limitations of this scale?
Because the ratings range from 0 to 4 rather than from 0 to 10, subtle differences in tremor severity may not be picked up. Also, the scale is limited to upper extremity tremor. Reliability may improve with more specific instructions on the utilization of this scale and standardization of the props used (30).
Fahn-Tolosa-Marin Tremor Rating Scale (FTM-TRS)
The FTM-TRS scale contains sections for assessing rest, postural, and kinetic or intention tremor amplitude in specific anatomic locations (part A); tremor in writing, drawing, and pouring (part B); activities of daily living (part C); and global assessments by the patient and examiner, with each item rated on a scale from 0 to 4 (30).
(Available here: http://neurocirugiacontemporanea.com.)
|
Part A. Tremor location and severity rating | ||
|
|
Tremor distribution. Tremors should be rated at rest, with posture, action and intention in each of the 10 body regions. | |
|
1. Face | ||
|
Tremor severity rating | ||
|
0 = None. | ||
|
Total score for part A = 88 | ||
|
| ||
|
11. Handwriting. Have patient wrist the standard sentence: “This is a sample of my best handwriting”, sign his or her name and write the date. | ||
|
0 = Normal. | ||
|
12-14. Figures A, B, and C. Ask the patient to join both points of the various drawings without crossing the lines. Test each hand, beginning with the lesser, without leaning the hand or the arm on the table. | ||
|
0 = Normal. | ||
|
15. Ask the patient to pour water from a firm plastic cup (8 cm tall) filled with water to 1 cm from top to another cup. Test each hand separately. | ||
|
0 = Normal. | ||
|
Total score for part B = 36 | ||
|
| ||
|
16. Speech. Asking the person to speak, which includes dysphonia if present. | ||
|
0 = Normal. | ||
|
17. Feeding (other than liquids) | ||
|
0 = Normal. | ||
|
18. Bringing liquids to mouth | ||
|
0 = Normal. | ||
|
19. Hygiene | ||
|
0 = Normal. | ||
|
20. Dressing | ||
|
0 = Normal. | ||
|
21. Writing | ||
|
0 = Normal. | ||
|
22. Working | ||
|
0 = Tremor does not interfere with the job. | ||
|
23. Social activities | ||
|
0 = No changes. | ||
|
Total score for part C = 32 A total tremor score (maximum = 156) is calculated by addition of the scores from each of the three parts (A, B, and C) | ||
|
| ||
|
0 = No functional disability | ||
|
(31) | ||
(1) What are the practical applications of this scale?
A study analyzed 12 rating scales that were classified based on the Movement Disorder Society Task Force rating scales for Parkinson disease. The Fahn-Tolosa-Marin Tremor Rating Scale (FTM-TRS) and the Washington Heights-Inwood Genetic Study Essential Tremor (WHIGET) scales met the criteria for recommendation (122). This scale includes disability ratings and clinician-based and patient-based ratings (30).
(2) Has the reliability of this scale been assessed?
The interrater reliability is high for part A (p = 0.93), part B (p = 0.94), part C (p = 0.99), total score (p = 0.97), and global assessment by the examiner (0.99). Intrarater reliability was fair in the upper limb compared to poor in the lower limb (143).
(3) Has the validity of this scale been assessed?
The scale has good validity and correlates strongly with transducer measures of tremor (30).
(4) What are the limitations of this scale?
The scale is difficult to use and time-consuming, and there is a poor concordance among raters who are untrained. Upper extremity tremor has a potential ceiling effect (30).
Scales used to assess patients with tardive dyskinesia
Tardive dyskinesia is a chronic disorder characterized by involuntary, stereotyped, choreic, athetoid, or dystonic movements in one or more body areas, including the orofacial region, extremities, and torso. Tardive dyskinesia results from exposure to dopamine receptor-blocking agents, such as antipsychotics and antiemetics (73). The Abnormal Involuntary Movement Scale (AIMS) was developed in 1976 by W. Guy at the National Institute of Mental Health for specific use in patients with tardive dyskinesia but can be applied to any patient with dyskinesia or chorea (Guy 1976). The AIMS is divided into the examination and scoring procedures and has a total of 12 items rating involuntary movements of various areas of the patient's body. These items are rated on a 5-point severity scale from 0 to 4 (0 = none, 1 = minimal, 2 = mild, 3 = moderate, and 4 = severe). The first seven items are used to measure the severity of abnormal movements. Items 8 through 12 are related to clinical global judgment of severity, patient awareness, incapacitation due to abnormal movements, and dental status. A later version of AIMS contains 14 items, including two additional items for edentulousness and disappearance of abnormal movements during sleep. The total score for severity of abnormal movement (0 to 28) in this variation of the original AIMS is the sum of items 1 through 7 (73).
Abnormal Involuntary Movement Scale
(Available here: https://psychiatry.uw.edu.)
Examination procedure:
1. Either before or after completing the examination procedure, observe the patient unobtrusively at rest (eg, in the waiting room). | |
2. The chair to be used in this examination should be hard and firm, without arms. | |
3. Ask the patient whether there is anything in his or her mouth (such as gum or candy) and, if so, to remove it. | |
4. Ask about the current condition of the patient's teeth. Ask if he or she wears dentures. Ask whether teeth or dentures bother the patient now. | |
5. Ask whether the patient notices any movements in his or her mouth, face, hands, or feet. If yes, ask the patient to describe them and to indicate to what extent they currently bother the patient or interfere with activities. | |
6. Have the patient sit in the chair with hands on knees, legs slightly apart, and feet flat on floor. (Look at the entire body for movements while the patient is in this position.) | |
7. Ask the patient to sit with hands hanging unsupported—if male, between his legs, if female and wearing a dress, hanging over her knees. (Observe hands and other body areas). | |
8. Ask the patient to open his or her mouth. (Observe the tongue at rest within the mouth.) Do this twice. | |
9. Ask the patient to protrude his or her tongue. (Observe abnormalities of tongue movement.) Do this twice. | |
10. Ask the patient to tap his or her thumb with each finger as rapidly as possible for 10 to 15 seconds, first with right hand, then with left hand. (Observe facial and leg movements.) | |
11. Flex and extend the patient's left and right arms, one at a time. | |
12. Ask the patient to stand up. (Observe the patient in profile. Observe all body areas again, hips included.) | |
13. Ask the patient to extend both arms out in front, palms down. (Observe trunk, legs, and mouth.) | |
14. Have the patient walk a few paces, turn, and walk back to the chair. (Observe hands and gait.) Do this twice. |
Scoring procedure:
Complete the examination procedure before making ratings. For the movement ratings (the first three categories below), rate the highest severity observed.
0 = none |
According to the original Abnormal Involuntary Movement Scale instructions, 1 point is subtracted if movements are seen only on activation, but not all investigators follow that convention.
Facial and oral movements
1. Muscles of facial expression, eg, movements of forehead, eyebrows, periorbital area, cheeks. Include frowning, blinking, and grimacing of upper face. | |
2. Lips and perioral area, eg, puckering, pouting, smacking. | |
3. Jaw, eg, biting, clenching, chewing, mouth opening, lateral movement. | |
4. Tongue. Rate only increase in movement both in and out of mouth, not inability to sustain movement. |
Extremity movements
5. Upper (arms, wrists, hands, fingers). Include movements that are choreic (rapid, objectively purposeless, irregular, spontaneous) or athetoid (slow, irregular, complex, serpentine). Do not include tremor (repetitive, regular, rhythmic movements). | |
6. Lower (legs, knees, ankles, toes), eg, lateral knee movement, foot tapping, heel dropping, foot squirming, and inversion and eversion of foot. |
Trunk movements
7. Neck, shoulders, hips, eg, rocking, twisting, squirming, pelvic gyrations. Include diaphragmatic movements. |
Global judgments
Severity of abnormal movements based on the highest single score on the above items: | ||
Incapacitation due to abnormal movements: | ||
0 = none, normal | ||
Patient's awareness of abnormal movements: | ||
0 = no awareness | ||
Dental status
Current problems with teeth or dentures? | ||
0 = no | ||
Does patient usually wear dentures? | ||
0 = no | ||
(1) What are the practical applications of this scale?
AIMS is strictly a tool for measuring the anatomic distribution and severity of abnormal movements. It does not provide criteria for diagnosing tardive dyskinesia. The AIMS is used both in research and clinical realms (73).
(2) Has the reliability of this scale been assessed?
Lane and colleagues report the interrater reliability was 0.87 with a test-retest reliability of 0.86 (76).
(3) Has the validity of this scale been assessed?
This scale had a high degree of shared variance with the St. Hans scale, a scale used extensively outside the United States. The scale was also assessed by Lane and colleagues (76).
(4) What are the limitations of this scale?
The AIMS scores provide reliable information about the severity of abnormal movement, both in research and clinical practice, but may not be sufficient for indicating clinical severity and functional impact of tardive dyskinesia. Although there is a generally accepted range for the AIMS total score (0 to 28), the range itself is not linear because each constituent item is scored separately. Therefore, the AIMS total score may be a useful index to measure treatment effect, but its applicability in clinical practice is limited. As such, in clinical trials, a mean change in total AIMS score is reported to evaluate treatment effect. In addition, instructions and refinements for the global and distress measures (items 8 through 10) and dental pathology (items 11 and 12) have not been standardized. Global judgment items have not correlated with responses on the anatomic measure of the severity of tardive dyskinesia (items 1 through 7) and may not be reliable given the lack of awareness and insight reported in tardive dyskinesia (73).
Scales that can be used for any movement disorder
Berg Balance Scale (BBS)
This scale is a 14-item objective measure that assesses static balance and fall risk in adults. Item level scores range from 0 to 4, determined by the ability to perform the activity. The maximum score is 56 (06; 05; 07).
|
Sitting balance | ||
|
Task 1. Sitting unsupported | ||
|
0 = Unable to sit without support 10 seconds | ||
|
Standing balance | ||
|
Task 2. Standing unsupported | ||
|
0 = Unable to stand without support 10 seconds | ||
|
Task 3. Standing with eyes closed | ||
|
0 = Needs help to keep from falling | ||
|
Task 4. Standing with feet together | ||
|
0 = Needs help to attain position and is unable to hold for 15 seconds | ||
|
Task 5. Standing on one foot | ||
|
0 = Unable to try or needs assistance to prevent fall | ||
|
Task 6. Turning to look behind | ||
|
0 = Needs assistance to keep from losing balance or falling | ||
|
Task 7. Grab an object from the floor | ||
|
0 = Unable to try or needs assistance to keep from losing balance or falling | ||
|
Task 8. Reaching forward with outstretched arms while standing | ||
|
0 = Loses balance while trying or requires external support | ||
|
Task 9. Placing one foot in front of the other | ||
|
0 = Loses balance while stepping or standing | ||
|
Dynamic balance | ||
|
Task 10. Going from sitting to standing | ||
|
0 = Needs moderate or maximal assist to stand | ||
|
Task 11. Going from standing to sitting | ||
|
0 = Needs assistance to sit | ||
|
Task 12. Transfer from a seat with an armrest to a seat without an armrest | ||
|
0 = Needs two people to assist or supervise to be safe | ||
|
Task 13. Turn 360 degrees | ||
|
0 = Needs assistance with turning | ||
|
Task 14. Place alternating foot on a step or stool while standing unsupported | ||
|
0 = Needs assistance to keep from falling or unable to try | ||
|
Interpretation | ||
|
0 to 20, wheelchair-bound | ||
(1) What are the practical applications of this scale?
Strong evidence supports using BBS to assess changes in static and dynamic sitting and standing balance (115). In older adults, a score of 56 indicates functional balance, whereas a score of less than 45 indicates an increased risk of falls (05; 07). The BBS initially was used to assess individuals with stroke but has since demonstrated high reliability and validity in various neurologic conditions such as Parkinson disease, multiple sclerosis, and traumatic brain injury. The BBS can assess, an increased risk for falls as well as the need for an assistive device.
(2) Has the reliability of this scale been assessed?
The scale has been determined to have excellent intrarater reliability (ICC = 0.98) and interrater reliability (ICC = 0.98 for community-dwelling older adults) (05; 07).
(3) Has the validity of this scale been assessed?
BBS has demonstrated an excellent correlation with the Dyamic Gait Index (r = 0.67) in older adults (139).
(4) What are the limitations of this scale?
It may have limited utility in middle-stage Parkinson disease (Hoehn and Yahr stage 2 to 3) due to ceiling effects (80). Patients at more advanced stages (Hoehn and Yahr stage 4 to 5) cannot complete the test because an assistive device cannot be utilized during testing.
Media
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Contributors
All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.
Authors
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Sheila Eichenseer MD MS
Dr. Eichenseer of the Medical College of Wisconsin has no relevant financial relationships to disclose.
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Ryan Brennan DO
Dr. Brennan of the Medical College of Wisconsin received a consulting fee from Medtronic and speaker and consulting fees from Abbvie.
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Robert Fekete MD
Dr. Fekete of New York Medical College received consultation fees from Acadia Pharmaceutical, Acorda, Adamas/Supernus Pharmaceuticals, Amneal/Impax, Kyowa Kirin, Lundbeck Inc., Neurocrine Inc., and Teva Pharmaceutical, Inc.
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Former Authors
- Chantale Branson MD
- Joseph Jankovic MD
- Elan D Louis MD (original author), Katie A Kompoliti MD, Samuel Frank MD, Anindita Deb MD, and Chantale Branson MD
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