Movement Disorders
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Feb. 26, 2023
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Rating scales of movement disorders …
- Updated 06.04.2018
- Released 12.16.1998
- Expires For CME 06.04.2021
Rating scales of movement disorders
Introduction
This article includes discussion rating scales of movement disorders, rating scales, movement disorders, and Parkinson disease (anxiety). The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
Overview
Measuring severity of disease and changes in disease state over time is critical for evaluating treatments and providing prognosis. When evaluating new treatments in clinical trials or comparing symptoms across time, methods of assessment need to be employed to ensure that we are improving patients and providing accurate information while minimizing harm. Detecting changes in disease commonly relies on changes in rating scales as a surrogate marker for changes in underlying disease processes. Rating scales also provide a standardized method of characterizing disease such that they can be reliably used by physicians worldwide for clinical and research purposes. In this article, the author provides an introduction to several of the rating scales that have been used to quantify specific involuntary movements (eg, tremor, chorea) or specific disease entities characterized by involuntary movements (eg, Huntington disease, Parkinson disease). Scales for each of the following involuntary movements or diseases are discussed: Parkinson disease, tics, chorea, dystonia, myoclonus, ataxia, tremor, drug-induced dyskinesia, and gait and balance in any movement disorder. The scales are summarized and presented in the text or linked to another site. The uses, reliability, validity, and limitations of each scale are discussed using current published evaluations. There are numerous scales available; however, the ones discussed are the ones that have been most commonly 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 comparability of outcomes, helps with the interpretation of results, and minimizes errors of measurement. | |
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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 their psychometric properties. | |
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• Rating scales in movement disorders focus on 2 primary concepts of dysfunction: 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
When treatments are given to patients, we should be able to measure the change in the disease. When evaluating new treatments in clinical trials or comparing symptoms across time, methods of assessment need to be employed to ensure that we are improving patients while minimizing harm. Detecting changes in disease commonly relies on changes in rating scales as a surrogate marker for changes in underlying disease processes.
An appropriate rating scale is 1 that has met 3 primary psychometric accuracy standards criteria (79). First, the scale should be objective, with the results being independent from 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. Of course, in addition to the quality criteria, there are the relative criteria of degrees of sensitivity and specificity (102).
Movement disorders are the abnormal, involuntary movements that are not the result of weakness or sensory deficits. The movement stems from dysfunction of what may be defined anatomically as the basal ganglia and cerebellum or functionally as the extrapyramidal motor system (154; 83). Despite their diversity, movement disorders may be divided conveniently into 2 types: (1) hyperkinesias, characterized by an excess of movement; and (2) hypokinesias, characterized by a paucity of movement (154; 83).
The current article will provide an introduction to several of the numerous rating scales that have been used to quantify specific involuntary movements (eg, tremor, chorea) or specific disease entities characterized by involuntary movements (eg, Huntington disease, Parkinson disease). The purpose is to provide a reasonable overview of the more commonly used scales rather than a complete and comprehensive list of all 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, and drug-induced movement disorders. For each scale, the following basic issues will be addressed:
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(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 unfocussed, 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.
Too many scales are associated with movement disorders to thoroughly present and discuss them all in this article. The purpose of this section is to provide an introduction to the more commonly used scales. A review of the clinimetrics of all Parkinson disease scales available between 1960 and 2002 was reviewed (115). The authors identified 11 rating scales used for Parkinson disease, including 3 impairment scales (Webster, Columbia University Rating Scale [CURS], and Parkinson Disease Impairment Scale), 4 disability scales (Schwab and England, Northwestern University Disability Scale [NUDS], Intermediate Scale for Assessment of Parkinson Disease, and Extensive Disability Scale), and 4 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 the majority 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 (90; 91; 148; 149; 94).
Scales for disease-specific issues in Parkinson disease.
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• The Parkinson Disease Sleep Scale (13) |
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 (35), Montreal Cognitive Assessment (MoCA) (108), pentagon drawing alone (19), Cambridge Cognitive Assessment (revised) (61), and Mattis scale for dementia (23; 81). Other scales have been specifically developed to be used in Parkinson disease, including SCOPA-COG, the Parkinson neuropsychometric dementia assessment (PANDA) instrument (69), and the Parkinson disease cognitive rating scale (109).
Mild cognitive impairment in Parkinson disease is an indicator of Parkinson disease dementia (60). The Movement Disorder Society formed a task force that developed guidelines for mild cognitive impairment in Parkinson disease (PD-MCI) (80; 141; 82). The pattern of progression seen is from normal cognition to mild cognitive impairment, and eventually Parkinson disease dementia (42). The PD-MCI guidelines can identify patients at risk for Parkinson disease dementia (22). It is considered to be an optimal and efficient neuropsychological assessment of PD-MCI (52). 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 (43). Szeto and associates used a range of cutoff scores to study the utility of the criteria (138). 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 (161). It may be used without permission for clinical and educational non-commercial 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) (103) and the Hamilton Depression scale have been independently evaluated in Parkinson disease and measured against each other and are comparable (156; 126).
Psychosis is 1 of the most troublesome non-motor symptoms of Parkinson disease, which has no specific, validated scale, but there is a clear need for one (34). The Scale for Evaluation of Neuropsychiatric Disorders in Parkinson’s disease (SEND-PD) assesses the severity of neuropsychiatric symptoms (96). The scale has been validated and shown to have a correlation with the MDS-UPDRS (119). 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 non-motor aspects of Parkinson disease.
The scales presented and discussed are the following:
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For patients with Parkinson disease: | |
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• Movement Disorder Society - Unified Parkinson Disease Rating Scale | |
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For patients with tic disorders: | |
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• Yale Global Tic Severity Scale | |
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For patients with chorea or Huntington disease: | |
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• Unified Huntington Disease Rating Scale | |
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For patients with dystonia: | |
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• Burke-Fahn-Marsden Evaluation Scale for Dystonia | |
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For patients with myoclonus: | |
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• Myoclonus Evaluation | |
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• The Unified Myoclonus Rating Scale | |
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For patients with ataxia: | |
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• International Cooperative Ataxia Rating Scale | |
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For patients with essential tremor: | |
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• Washington Heights-Inwood Tremor Rating Scale | |
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For patients with tardive dyskinesia: | |
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• Abnormal Involuntary Movement Scale | |
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For most movement disorders: | |
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• Gait and 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 (65; 87). Although primarily sporadic, familial forms of the disease exist (51) and genetic mutations associated with sporadic Parkinson disease are emerging (14). Three complementary rating scales will be discussed. The first is the Unified Parkinson Disease Rating Scale (30; 50). This scale underwent a significant revision and clinimetric testing by the Movement Disorder Society Task Force for Rating Scales in Parkinson’s disease (50). The modified Hoehn and Yahr Staging Scale (62) and the Schwab and England Activities of Daily Living Scale (153; 127) 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/publications/rating_scales/.
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 |
(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 4 sections for the assessment of: (1) mentation, behavior, and mood; (2) activities of daily living; (3) motor examination; and (4) complications of therapy (30). 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 9 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/gait difficulty in Parkinson patients based on MDS-UPDRS to facilitate transition from the UPDRS to MDS-UPDRS (135).
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 change in symptoms early in the disease course (73). This will be particularly important for providers’ assessment of the disease process. Further studies have shown that the MDS-UPDRS, specifically the assessment of motor and non-motor EDL, can relate to impaired health-related quality of life (HRQoL). This suggests an even broader range of application of this scale (99).
The MDS-UPDRS screening tool for sleep-related problems in Parkinson disease patients is an example of using the scale for nonmotor symptoms. A study showed MDS-UPDRS is as equally effective when compared to the commonly used screening tools, such as the Parkinson disease sleep scale, second version, and the Epworth sleepiness scale (64).
The MDS-UPDRS has validated Italian and Spanish versions, which are structurally equivalent to the original English version (02; 98). There are also Chinese, French, German, Estonian, and Slovak versions currently available and language teams involved in establishing numerous other versions.
(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 (97; 117; 88). There was similar reliability of the motor section (117; 47). The scale also has high reliability in patients with advanced disease (105).
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 (98). Additionally, there were aspects of clinimetric properties that 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 (97; 146; 134; 37).
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 2 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. Validity of the MDS-UPDRS was confirmed in a study that also showed that it can be used for individual measurements over time (98). In addition to the internal validity, the MDS-UPDRS part 1 nM-EDL also demonstrated convergent validity with the NMSS (95).
(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.
Modified Hoehn and Yahr Staging Scale
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Stage 0 = No signs of disease |
(62; 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 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 (44).
(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 (25), the Northwestern University Disability Scale (10), and the Extensive Disability Scale (44). 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 3 to 4 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. |
(127)
(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.
(2) Has the reliability of this scale been assessed?
This has not been formally assessed.
(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 (97).
(4) What are the limitations of this scale?
It is often difficult for patients to determine whether they perform chores twice as slowly or 4 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)
Part 1A: On-dyskinesia (exclusive of off-dystonia)
Time spent with on-dyskinesia
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0 = Normal: no dyskinesia |
Score
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1. Total hours on: ____ |
Part 1B: Patient dyskinesia questionnaire
Speech
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0 = Normal: not at all, no problems |
Chewing and swallowing
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0 = Normal: not at all, no problems |
Eating tasks
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0 = Normal: not at all, no problems |
Dressing
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0 = Normal: not at all, no problems |
Hygiene
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0 = Normal: not at all, no problems |
Handwriting
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0 = Normal: not at all, no problems |
Doing hobbies and other activities
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0 = Normal: not at all, no problems |
Walking and balance
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0 = Normal: not at all, no problems |
Public and social settings
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0 = Normal: not at all, no problem |
Exciting or emotional settings
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0 = Normal: not at all, no problem |
Part 2A: Off-dystonia ratings
Time spent with off-dystonia
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0 = Never |
Part 2B: Patient questionnaire
Effects of spasms or cramps, called off-dystonia, separate from pain on activities
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0 = Normal: not at all |
Effects of pain from off-dystonia on daily activities
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0 = Normal: not at all, no pain from off-dystonia |
Dystonia pain
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0 = Normal: not painful |
Part 3: Objective evaluation of dyskinesia disability
Intensity Scale
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 |
Disability scale
Communication
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0 = No dyskinesia observed |
Drinking from a cup
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0 = No dyskinesia observed |
Dressing
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0 = No dyskinesia observed |
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 (45; 15). A clinically important change in phase 2 trials for dyskinesia was determined in Parkinson disease with a standard intravenous levodopa infusion model. By using the UDysRS rating scale physicians are able to determine the clinical relevance perceived by patients and their caregivers, particularly the onset and remission of dyskinesia (104).
(2) Has the reliability of this scale been assessed?
The intrarater and interrater reliability has been assessed and was found to be satisfactory (45). 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 (15).
(3) Has the validity of this scale been assessed?
The validity of this scale has not been examined. However, the temporal stability of the scale, 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 (48).
(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 large number of investigators who participated in its development. It is nevertheless the most widely used scale currently used to assess dyskinesia, and its validation outside the group that developed it is underway.
Scales used to assess patients with tic disorders
Tics are rapid, nonrhythmic movements or sounds that vary from simple tics (eg, eye blinking, head jerking) to complex acts (patterned, multi-stepped, coordinated movements) (66; 154). Tics are typically preceded by an urge and are suppressible for variable periods. Tourette syndrome is characterized by both chronic motor and vocal tics (129). The following 3 ratings scales will be discussed: (1) the Yale Global Tic Severity Scale (YGTSS) (74), (2) the Tic Rating Scale (49), and (3) the Tourette Syndrome Global Scale (57).
Yale Global Tic Severity Scale (YGTSS)
(Available here: YGTSS
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Tic Inventory | ||
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1. Description of motor tics (check motor tics present during 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 | ||
Number
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 1 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 |
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 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 |
Intensity
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 |
Complexity
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 and/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 and/or extremely unusual, inappropriate, bizarre, or obscene character (lengthy displays or utterances often involving copropraxia, self-abusive behavior, or coprolalia) |
Interference
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 |
Impairment
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 | |
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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/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 5 dimensions: number, frequency, intensity, complexity and interference. It also includes a checklist for specific types of motor and vocal tics. An independent rating of impairment, which focuses on the impact of the tic disorder over the previous week, is added to the total tic score to obtain a final score (74).
(2) Has the reliability of this scale been assessed?
The scale has been assessed and was found to have good reliability (136).
(3) Has the validity of this scale been assessed?
The YGTSS has shown a strong convergent validity vis-a-vis robust correlations with corresponding 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 (74; 150).
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 to be sufficient (136).
(4) What are the limitations of this scale?
Disadvantages of this scale include lack of differentiation of historical information from observations made by the examiner and 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.
Tic Rating Scale
Motor tic distribution. Tics should be rated as either present or absent in each of the following 11 body regions:
|
1. Eyes |
Motor tic severity:
|
0 = Absent |
Motor tic frequency:
The number of discrete motor tics is counted during a short videotape segment.
Vocal tic severity:
|
0 = Absent |
Vocal tic frequency:
The number of discrete vocal tics is counted during a short videotape segment.
(49)
(1) What are the practical applications of this scale?
This scale is easy to administer and bases its ratings exclusively on objectively collected data. Tic phenomenology is conveniently divided into 5 domains: (1) motor tic distribution, (2) motor tic severity, (3) motor tic frequency, (4) vocal tic severity, and (5) vocal tic frequency, although some would argue that vocal tics are simply motor tics of the respiratory system. A systematic review article noted that the scale can determine clinical exacerbations of tics (101).
(2) Has the reliability of this scale been assessed?
The interrater reliability for each of the 5 domains was high (49).
(3) Has the validity of this scale been assessed?
The correlation between the tic rating scores and the scale of Shapiro and colleagues was moderate (129). The scale was compared to the Yale Global Tic Severity Scale with high correlation (46).
(4) What are the limitations of this scale?
Tics are difficult to rate because of their variability in character and severity. It may be difficult to assess tic frequency when tics occur in flurries or seem to flow together.
Tourette Syndrome Global Scale
|
1. Simple Motor Tics (non-purposeful, tics, jerks or movements) | ||
|
A. Frequency | ||
|
0 = None | ||
|
B. Disruption | ||
|
1 = Camouflaged | ||
|
2. Complex motor tics (purposeful, thoughtful actions, systematic actions, rituals, touching self, others, or objects) | ||
|
A. Frequency | ||
|
0 = None | ||
|
B. Disruption | ||
|
1 = Camouflaged | ||
|
3. Simple phonic tics (non-purposeful noises, throat clearing, coughing) | ||
|
A. Frequency | ||
|
0 = None | ||
|
B. Disruption | ||
|
1 = Camouflaged | ||
|
4. Complex phonic tics (purposeful, insults, coprolalia, words, distinguishable speech) | ||
|
A. Frequency | ||
|
0 = None | ||
|
B. Disruption | ||
|
1 = Camouflaged | ||
|
5. Behavior (conduct) | ||
|
0 = No problem | ||
|
6. Motor restlessness | ||
|
0 = Normal movement | ||
|
7. School and learning problems | ||
|
0 = No problem | ||
|
8. Work and occupation problems | ||
|
0 = No problem | ||
Global score = [(item 1 + item 2)/2 + (item 3 + item 4)/2 ] divided by (0.67)(item 5 + item 6 + item 7 or item 8).
(57)
(1) What are the practical applications of this scale?
The scale looks not only at the frequency of motor and phonic tics separately but also divides each of these into simple versus complex tics. The scale also assesses the impact of tics on behavior (conduct), school and learning problems, and work and occupational problems.
(2) Has the reliability of this scale been assessed?
The agreement between those who rated was good, with weighted kappa coefficients for each of the 8 items being between 0.65 and 0.85. However, a literature of rating scales for tics found that the interrater reliability is lower than the Yale Global Tic Severity Scale (101).
(3) Has the validity of this scale been assessed?
Construct validity was demonstrated when the authors demonstrated a reasonable level of agreement between the Tourette Syndrome Global Scale scores and the Children's Global Assessment Scale scores (128).
(4) What are the limitations of this scale?
The scale is based on historical information rather than objective ratings; patients are asked to assess their symptoms over the preceding 1 week. There is no video component to evaluate tic frequency and distribution associated with this scale.
Scales used to assess patients with chorea or Huntington disease
Chorea may be characterized as semi-purposeful, but involuntary, flowing movements that flit from 1 part of the body to another in a continuous and random pattern. Chorea may be a feature of several neurologic disorders, including Huntington disease (83), an autosomal dominant disorder characterized by chorea, dystonia, motor impersistence, depression, psychosis, cognitive decline, and change in personality (58). Two rating scales will be discussed: the Unified Huntington Disease Rating Scale (139) and Quantified Staging of Functional Capacities in Huntington Disease (130).
Unified Huntington Disease Rating Scale
Please refer to the Huntington Study Group publication to view the scale (139). The components include motor, cognitive, behavioral, functional, and independence assessments. A 13-point total functional capacity scale is included and has been used to monitor the progression of early disease.
(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.
(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) (139). 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 non-clinicians (63).
(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 (160). The motor, cognitive, and functional components and the validated Total Functional Capacity scale were highly intercorrelated (139).
(4) What are the limitations of this scale?
Because the scale is so broad in its scope, several more specific items, particularly in motor assessment (eg, dystonia) are covered only briefly, and others (eg, tics, ADLs) 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 (131).
Quantified Staging of Functional Capacities in Huntington Disease
|
Engagement in occupation | |
|
3 = Usual level: full time salaried employment, actual or potential (eg, job offer or qualified), with normal work expectations and satisfactory performance. | |
|
2 = Lower level: full- or part-time salaried employment, actual or potential, with a lower-than-usual work expectation (relative to patient's training and education) but with satisfactory performance). | |
|
1 = Marginal level: part-time voluntary or salaried employment, actual or potential, with lower expectation and less-than-satisfactory work performance. | |
|
0 = Unable: totally unable to engage in voluntary or salaried employment. | |
|
Capacity to handle financial affairs | |
|
3 = Full: normal capacity to handle personal and family finances (income tax, balancing checkbook, paying bills, budgeting, shopping). | |
|
2 = Requires slight assistance: mildly impaired ability to handle financial affairs, such that accustomed routine responsibilities require some organization and assistance from family member or financial advisor. | |
|
1 = Requires major assistance: moderately impaired ability to handle financial affairs, such that patient comprehends the nature and purpose of routine financial procedures and is competent to handle funds but requires major assistance in the performance of these tasks. | |
|
0 = Unable: patient is unable to comprehend the financial process and is totally unable to perform task-related routine financial procedures. | |
|
Capacity to manage domestic responsibilities | |
|
2 = Full: no impairment in performance of routine domestic tasks (cleaning, laundering, dishwashing, table setting, recipes, lawn care, answering mail, civic responsibilities). | |
|
1 = Impaired: moderate impairment in performance of routine domestic tasks, such that patient requires some assistance in carrying out these tasks. | |
|
0 = Unable: marked impairment in function and marginal performance; requires major assistance. | |
|
Capacity to perform activities of daily living | |
|
3 = Full: complete independence in eating, dressing, and bathing. | |
|
2 = Mildly impaired: somewhat labored performance in eating (avoids certain foods that cause chewing and swallowing problems), in dressing (difficulty in fine tasks only, eg, buttoning or tying shoes), in bathing (difficulty in fine performance only, eg, brushing teeth); requires only slight assistance. | |
|
1 = Moderately impaired: substantial difficulty in eating (swallowing only liquid or soft foods and requires considerable assistance), in dressing (performs only gross dressing activities and requires assistance with everything else), in bathing (performs only gross bathing tasks; otherwise, requires assistance). | |
|
0 = Severely impaired: requires total care in activities of daily living. | |
|
Care can be provided at: | |
|
2 = Home: patient living at home and family readily able to meet care needs. | |
|
1 = Home or extended care facility: patient may be living at home, but care needs would be better provided at an extended care facility. | |
|
0 = Total care facility only: patient requires full-time, skilled nursing care. | |
(130)
(1) What are the practical applications of this scale?
This scale provides a detailed assessment of various domains of functional disability associated with Huntington disease, allowing the clinician to assess the patient's stage of illness and requirements for additional functional assistance.
(2) Has the reliability of this scale been assessed?
Interrater reliability has been established (139).
(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 (160).
(4) What are the limitations of this scale?
It may be difficult to rate some patients who, for example, may never have handled financial affairs or participated in household chores, even in the pre-disease state. 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 (145). In this population, depressed mood, anxiety, and irritable behavior demonstrated good discriminative properties, whereas motor scores failed to do so.
Scales used to assess patients with dystonia
Dystonic movements are characterized by sustained muscle contractions, frequently resulting in twisting movements that are 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 (31). Occupational dystonias such as writer's cramp are also common. The following 2 rating scales will be discussed: the Burke-Fahn-Marsden Evaluation Scale for Dystonia (93; 09) and the Toronto Western Spasmodic Torticollis Rating Scale. However, since the publication of the Burke-Fahn-Marsden scale, 2 other dystonia rating scales have been developed and validated including the Unified Dystonia Rating Scale (UDRS) and the Global Dystonia Rating Scale (GDS) (16). Clinimetric testing showed that the Comprehensive Cervical Dystonia Rating Scale is internally consistent with a logical factor structure (17). The scales can be used for patients with generalized, segmental or focal dystonias and all 3 scales have acceptable levels of reliability. The interrater agreement was good to excellent for all 3 scales (26). The Jankovic Rating Scale has been used to assess the effects of botulinum toxin on the severity and frequency of involuntary eyelid contractions in the treatment of blepharospasm (120; 67). In addition, health-related quality-of-life instruments have found that blepharospasm can markedly impair functioning of the affected individuals, who are often subject to social stigmatization and prejudice (56; 118). The craniocervical dystonia questionnaire (CDQ-24) has been developed to evaluate the effects of botulinum toxin treatment on blepharospasm and cervical dystonia (107). 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 (106). Moderate to good interrater reliability was found for total scores of the 3 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.
There has been development of a new classification scale for cervical dystonia differentiating head and neck subtypes (116). However, the current scales do not adequately address these subtypes. Additionally, there is a need for a scale to evaluate the effect of therapeutic intervention, 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 (68). There is a need for clinical trials for the development of novel therapeutics for dystonia as 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 (41).
Burke-Fahn-Marsden Evaluation Scale for Dystonia
(Available here: www.mdvu.org)
1. Dystonia movement scale:
|
Region |
Provoking factor |
Severity factor |
Weight |
Product |
|
Eyes |
0 - 4 |
x 0 - 4 |
0.5 |
0 - 8 |
Sum: (maximum = 120)
|
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 | ||
2. Disability scale:
|
Function |
Score |
|
Speech |
0 - 4 |
|
A. Speech | ||
|
0 = Normal | ||
|
B. Handwriting (tremor or dystonia) | ||
|
0 = Normal | ||
|
C. Feeding | ||
|
0 = Normal | ||
|
D. Eating/swallowing | ||
|
0 = Normal | ||
|
E. Hygiene | ||
|
0 = Normal | ||
|
F. Dressing | ||
|
0 = Normal | ||
|
G. Walking | ||
|
0 = Normal | ||
(09)
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 (147). 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 (09). The intra- and interrater reliability were again tested as part of the SPIDY trial and confirmed good to very good intraclass correlation coefficient (71).
(3) Has the validity of this scale been assessed?
The correlation between this scale and global impressions of dystonia severity was high (09).
(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.mdvu.org)
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/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 1 or more "trick" | |||
|
D. Shoulder elevation/anterior displacement | |||
|
0 = Absent | |||
|
E. Range of motion (without the aid of sensory tricks). If limitations occur in more than 1 plane of motion, use the individual score that is highest. | |||
|
0 = Able to move to the extreme opposite position. | |||
|
F. 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 2 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 | |
(18)
(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 (68). 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 (16; 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 (68).
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 (92; 32; 83). The Myoclonus Evaluation Scale (93; 11) and the Unified Myoclonus Rating Scale (39) will be discussed.
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 1 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 | ||
(93; 11)
(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)
(Available here: www.neurology.org/content/suppl/2005/12/20/01.wnl.0000188670.38576.bd.DC2/E2.doc)
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. | ||
Section 2. Myoclonus at rest
|
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.
Section 3. Stimulus sensitivity
|
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 – 17. | |
Section 4. Myoclonus with action
|
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.
Section 5. Performance on functional tests
|
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 2 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 0-28. | |
Section 6. Score global disability
|
0 = Normal. |
Section 7: Is negative myoclonus present or absent?
|
0 = Absent (less than 50% likely). |
Section 8: If negative myoclonus is present, how severe is it?
|
0 = Not present. |
Total scores for sections 1, 3, 5, 6, 7, and 8 are calculated by addition.
(39)
(1) What are the practical applications for this scale?
It is easy to use and can be completed in less than 15 minutes (114).
(2) Has the reliability of this scale been assessed?
Interrater reliability was calculated for each section of the UMRS and confirmed as excellent (114). 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 (39).
(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 (07).
Scales used to assess patients with ataxia
There are perhaps hundreds of causes of ataxia, from inherited disorders to medications to neurodegenerative disorders. When coordination becomes the primary clinical concern, the International Cooperative Ataxia Rating Scale (ICARS) (144) is 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 (123; 124). However, a brief version of the ICARS (BARS) has been developed for clinical practice and validated against the SARA and full ICARS (122). The SARA has been validated against the ICARS in nonspinocerebellar ataxic patients and is reliable (158). In studying patients with spinocerebellar ataxia, the SARA was shown to have high interrater reliability and internal consistency. SARA score increased 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) (124). In 1 study, the SARA also correlated with measures of activities of daily living (08). Due to its convenience and emerging validity and reliability, the SARA may become the newer standard for measuring ataxia (159). The Modified International Cooperative Ataxia Rating Scale (MICARS) was developed by adding 7 tests to the ICARS. It has been validated and shown to have high interrater reliability (122). The Friedrich’s Ataxia Rating Score (FARS) shows significant correlation with the ICARS when evaluating Friedrich’s ataxia (FDA) patients. The FARS, ICARS, and SARA have strong direct correlation in multiple studies of FRDA patients (29; 08). The SARA and the ICARS are the most studied and validated scales, and it is recommended that they are utilized in long-term multicenter studies (121). Currently, ICARS, SARA, and BARS found a high reliability of early onset ataxia, but low discriminative validity (06).
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 Slightly reduced | |
|
C. Standing capacities, eyes open (the patient is asked first to try to stay on 1 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 1 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 1 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 3 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; 3 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 2 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 2 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 2 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 (143). 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 (137). An evaluation of the scale demonstrated very good interrater reliability and high test-retest reliability and Cronbach’s alpha (123; 124). 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 (123; 124). The validity of this scale was also investigated comparing acute versus chronic and focal versus degenerative disease with excellent correlation to clinical state (125).
(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.
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. The most commonly involved body region is the arms, although the head or voice may also be involved (86). The following 2 rating scales will be discussed: (1) the Washington Heights-Inwood Tremor Rating Scale (89; 85) and (2) the Fahn-Tolosa-Marin Tremor Rating Scale (FTM-TRS) (33).
Washington Heights-Inwood Tremor Rating Scale:
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 1 another in a midline position.
|
1. Dominant arm postural tremor: | |
|
0 = No visible tremor | |
|
2. Nondominant arm postural tremor: | |
|
0 = No visible tremor | |
Maneuver B:
Pouring water between 2 cups (10 repetitions using cups filled to the three-quarters mark).
|
3. Dominant arm pouring kinetic tremor: | |
|
0 = No visible tremor | |
|
4. Nondominant arm pouring kinetic tremor: | |
|
0 = No visible tremor | |
Maneuver C:
Drinking water from a cup (cup filled to the three-quarter mark and raised 10 times from lap level to the mouth).
|
5. Dominant arm drinking kinetic tremor: | |
|
0 = No visible tremor | |
|
6. Nondominant arm drinking kinetic tremor: | |
|
0 = No visible tremor. | |
Maneuver D:
Using a spoon to drink water (10 repetitions, hand raised from lap level to mouth).
|
7. Dominant arm spoon kinetic tremor: | |
|
0 = No visible tremor | |
|
8. Nondominant arm spoon kinetic tremor: | |
|
0 = No visible tremor | |
Maneuver E:
Finger-to-nose movements (10 repetitions).
|
9. Dominant arm finger-to-nose kinetic tremor: | |
|
0 = No visible tremor | |
|
10. Nondominant arm finger-to-nose kinetic tremor: | |
|
0 = No visible tremor | |
Maneuver F:
Drawing Archimedes spirals (2 slow, neat, free-hand spirals drawn on standard 8.5 x 11 inch sheets of paper, starting at the center of the page).
|
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.
(89; 85)
(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 severity tremor based on amplitude, presence of oscillations, and constancy of tremor.
(2) Has the reliability of this scale been assessed?
Interrater and intrarater reliability were high (84).
(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 (85).
(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 utilization of this scale and also standardization of the props used (Elble et al 2013).
Fahn-Tolosa-Marin Tremor Rating Scale (FTM-TRS)
(Available here: http://neurocirugiacontemporanea.com/lib/exe/fetch.php?media=fahn_tolosa_marin.pdf)
Part A. Tremor location/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
Part B. Specific motor tasks/function rating (scores 11-15)
|
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
Part C. Functional disabilities resulting from tremor
|
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 3 parts (A, B, and C)
Global assessment of tremor-related disability (5-point scale)
0 = No functional disability
1 = mild disability (1%-24% impaired)
2 = moderate disability (25%-49% impaired)
3 = markedly impaired (50%-74%)
4 = severe disability (75%-100%)
(28)
(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 (114). This scale includes disability ratings and clinician-based and patient-based ratings (Elble et al 2013).
(2) Has the reliability of this scale been assessed?
The retest reliability for each scale was considered good, but part C was considered least reliable (28). Interrater reliability was fair in the upper limb compared to poor in the lower limb (133). A study determined the intrarater reliability was high except for trunk tremor (114).
(3) Has the validity of this scale been assessed?
The scale has good validity and correlates strongly with transducer measures of tremor (Elble et al 2013).
(4) What are the limitations of this scale?
The scale is difficult to use, time consuming, and there is a poor concordance among raters who are nonneurologists.
Scales used to assess patients with tardive dyskinesia
Tardive dyskinesia is a well-known complication of exposure to antipsychotic medications (151). Any dopamine-blocking agent used for an extended period of time may cause these oral-buccal-facial dyskinesias. The movements are more likely to involve the face but may involve any portion of the body, and these patients are frequently referred to neurologists for evaluation and treatment options. 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 dyskinesias or chorea (Guy 1976). The AIMS is divided into the examination and scoring procedures. The AIMS test 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 scale of severity from 0 to 4. The scale is rated from 0 (none), 1 (minimal), 2 (mild), 3 (moderate), and 4 (severe). Two of the 12 items refer to dental care. Thus, the total maximum score is 48.
Read more: www.minddisorders.com
Abnormal Involuntary Movement Scale
(Available here: AIMS)
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, 1 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 3 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, 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, 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?
The scale is the most commonly used evaluation for dyskinesias and chorea and provides a communication tool between psychiatrists and neurologists.
(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 (72).
(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 (72).
(4) What are the limitations of this scale?
Tardive dyskinesias fluctuate with time, and this scale provides only a glimpse of the spectrum in 1 person. This scale was devised to measure specifically the dyskinesias associated with neuroleptic use, but clearly there are other tardive syndromes including dystonia, akathisia, myoclonus, tremor, tics, chorea and a withdrawal syndrome. The scale also fails to examine other aspects of neuroleptic use such as withdrawal syndrome, neuroleptic malignant syndrome, and parkinsonism (140). The Barnes Akathisia Rating Scale has been used to assess akathisia, which is 1 of several movement disorders characterized by both motor and sensory components (04; 110).
Scales that can be used for any movement disorder
Gait and Balance Scale (GABS) (140). This scale was developed to measure gait and balance in a practical fashion without extensive physiologic equipment. Gait is a complex function and can be affected in many disorders, including Parkinson disease, Huntington disease, stroke, peripheral neuropathy, and many others. A common referral to movement disorder specialists is gait disorders; therefore, we thought this scale would be appropriately discussed in this chapter.
Historical Information
|
1. Level of care | ||
|
0 = entirely independent | ||
|
2. Walking environment | ||
|
0 = able to walk anywhere, able to negotiate any terrain | ||
|
3. Ambulation | ||
|
0 = normal | ||
|
4. Falls (UPDRS, item 13) | ||
|
0 = no falls | ||
|
5. Limitation of activity due to fear of falling | ||
|
0 = no limitation | ||
|
6. Freezing (motor blocks) (UPDRS, item 14) | ||
|
0 = no freezing | ||
|
7. Freezing (motor blocks) - modifying factors | ||
|
0 = no freezing | ||
Subscore of items 1 through 7:
Physical examination
Performance items
|
8. Rising from a chair (UPDRS, item 27): patient attempts to arise from a straight-back wood or metal chair with arms folded across chest) | |||
|
0 = normal | |||
|
9. Posture (UPDRS, item 28) | |||
|
0 = normal | |||
|
10. Postural stability (UPDRS, item 30): response to sudden posterior displacement produced by pull on shoulders while patient erect and prepared with eyes open and feet slightly apart | |||
|
0 = normal | |||
|
11. Balance during stance; feet close together with eyes open | |||
|
0 = no impairment | |||
|
12. Romberg test (with eyes closed) | |||
|
0 = no difficulty, > 20s | |||
|
13. One limb stance | |||
|
0 = no difficulty, > 20s | |||
|
14. Tandem stance | |||
|
0 = no difficulty, > 20s | |||
|
15. Gait (UPDRS, item 29) – walking 5m | |||
|
0 = normal | |||
|
16. Turning 180 degrees after walking | |||
|
0 = normal pivoting | |||
|
17. Turning 360 degrees (turn completely around in a full circle, pause, and then turn a full circle in the other direction)** | |||
|
0 = able to turn 360 degrees in both directions, 4s per turn | |||
|
18. Walking on heels* | |||
|
0 = normal | |||
|
19. Walking on toes* | |||
|
0 = normal | |||
|
20. Walking in tandem* | |||
|
0 = normal | |||
|
21. Arm swing (vertical wrist displacement)* | |||
|
0 = normal | |||
|
22. Provocative test for freezing, motor blocks (rise from a chair and walk 5 m, between 2 chairs spaced 24 inches apart, turn 180 degrees, walk back and sit down)* | |||
|
A. Start hesitation* | |||
|
0 = no | |||
|
B. Sudden transient blocks interrupting gait* | |||
|
0 = no | |||
|
C. Motor blocks on turning* | |||
|
0 = no | |||
|
D. Motor blocks on reaching a target (chair)* | |||
|
0 = no | |||
|
E. Motor blocks when walking through narrow spaces (24 inches)* | |||
|
0 = no | |||
|
23. Functional reach (in inches) (24) | |||
|
0 = normal (> 10 in) | |||
|
1= Impaired (< 10 in) | |||
|
24. Modified Performance Oriented Gait Assessment Scale (Total score 0-12)#. Examine patient while walking a 10m distance including a turn, from the side for items a-d and from the back for items e-g. | |||
|
A. Initiation of gait | |||
|
0= No hesitancy | |||
|
B. Step length and height | |||
|
(i) Right swing foot | |||
|
0= Passes left stance foot | |||
|
(ii) Right step | |||
|
0= Right foot completely clears floor with step | |||
|
(iii) Left swing foot | |||
|
0= Passes right stance foot | |||
|
(iv) Left step | |||
|
0= Left foot completely clears floor=0 | |||
|
C. Step symmetry | |||
|
0= Right and left step appear equal | |||
|
D. Step continuity and rhythmicity | |||
|
0= Steps appear continuous | |||
|
E. Path (estimated in relation to floor tiles, 12-in. diameter; observe excursion of 1 foot over about 5 m of the course) | |||
|
0= Straight without walking aid | |||
|
F. Trunk | |||
|
0= No sway, no flexion, no use of arms, and no use of walking aid | |||
|
G. Walking distance | |||
|
0= Heels almost touching while walking | |||
|
25. Foam Posturography (stand barefooted with eyes closed on a 5 inch, medium density foam pad for 15 sec, with examiner next to the subject to prevent falls)+ | |||
|
0 = yes | |||
Sub score of Items 8 through 25:
Total score for all items 1 through 25:
Timed Tasks
|
26. Timed walking at usual speed (5m) | ||
|
Time in seconds: | ||
|
27. Timed walking as fast as possible (5m) | ||
|
Time in seconds: | ||
|
28. Stand-walk-sit time (total 10 m, in sec)***. Rise from a chair and walk 5m , turn 180 degrees, walk back and sit down. | ||
|
Time in seconds: | ||
(140)
Notes for Gait and Balance Scale:
* All items rated on a 0 - 4 scale: 0 = normal, 1 = mildly abnormal or rare, 2 = moderately abnormal or occasional, 3 = markedly abnormal or frequent, 4 = unable to perform or continuous, unless specified as UPDRS (30)
** Modified from balance scale (05)
***Rise from armless chair, walk 5 m, turn, walk back and sit
# Score each item on the following scale: 12= worst, 0= normal (142)
+ Detects vestibulospinal dysfunction, correlates well with moving-platform posturography (152)
(1) What are the practical applications of this scale?
GABS is specifically useful in assessing the gait and balance problems, and the various items can identify abnormalities in either gait or balance. It can be used in studies specifically evaluating progression gait and balance abnormalities. GABS can also be a useful assessment tool to evaluate the response to medication. It is a comprehensive, easy-to-use clinical scale, which reliably measures gait, balance, posture, freezing of gait and gait cycle.
(2) Has the reliability of this scale been assessed?
The data for validity were compared to data gathered from 2 commercial gait and balance systems. Intrarater reliability was fair to good with kappa statistic k > 0.41 for several items.
(3) Has the validity of this scale been assessed?
All items on the gait and balance testing had significant correlations with the corresponding computerized testing.
(4) What are the limitations of this scale?
This scale has only been tested in Parkinson disease patients. Future studies are planned to assess this scale in other diseases.
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.
Author
-
Chantale Branson MD
Dr. Branson of Boston Medical Center has no relevant financial relationships to disclose.
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Editor
-
Joseph Jankovic MD
Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received consulting/advisory board honorariums from Neurocrine Biosciences, Revance, Teva, and Aeon.
See Profile
Former Authors
- Elan D Louis MD (original author), Katie A Kompoliti MD, Samuel Frank MD, and Anindita Deb MD
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