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  • Updated 09.01.2021
  • Released 10.10.2004
  • Expires For CME 09.01.2024

Peripheral dystonia

Introduction

Overview

Peripheral dystonia is defined as sustained muscle contractions, frequently causing twisting and repetitive movements, or abnormal postures triggered by trauma to the peripheral or cranial nerves. Although a cause-and-effect relationship between central nervous system injury and subsequent dystonia is well established, the existence of such a relationship following peripheral injury is still a subject of controversy. There is increasing evidence, largely from clinical reports based on a strong temporal-anatomical relationship, supporting the association between peripheral nerve trauma and dystonia. In this article, the authors discuss the mechanisms and the clinical characteristics of peripherally induced movement disorders and address the controversies existent in the field. This review focuses on the following fundamental questions: (1) What are possible underlying mechanisms of peripherally induced dystonia? (2) What factors predict or predispose an individual to the development of dystonia following peripheral injury? (3) What are the clinical characteristics of dystonic movements in peripheral dystonia compared to primary dystonia? (4) What are the prognoses and long-term outcomes in patients with peripheral dystonia? In addition, the latest evidence in the mechanisms of peripherally induced dystonia and treatment options are reviewed.

Key points

• Peripheral dystonia is a secondary dystonia produced by trauma to peripheral and cranial nerves.


• Peripheral dystonia is the result of maladaptive changes in the cerebral cortex in response to altered peripheral input.


• In contrast to primary dystonia, peripherally induced dystonia is characterized by a fixed posture, contractures, and absence of sensory tricks.


• Peripheral dystonia is often associated with psychogenic movement disorders.


• Peripheral dystonia is often associated with pain and autonomic dysfunction including complex regional pain syndrome (CRPS).

Historical note and terminology

Dystonia refers to a syndrome of sustained or intermittent muscle contractions, frequently causing twisting and repetitive movements or abnormal postures (20). In primary dystonia the cause is genetic or unknown, whereas in secondary dystonia an identifiable trigger is present, such as a metabolic, heredodegenerative, or toxic trigger. Peripheral dystonia is considered a form of secondary dystonia induced by trauma to the peripheral nerves, nerve roots, or cranial nerves. Secondary dystonia is often accompanied by neurologic deficits, begins and occurs suddenly at rest, and develops mainly as the result of environmental factors that cause insult to the brain (08). Secondary dystonias can be caused by focal brain lesions, neurodegenerative disorders, metabolic disorders, and several drugs and chemicals that affect the basal ganglia, thalamus, or brainstem (Table 1). Although a cause-and-effect relationship between central nervous system injury and subsequent dystonia is well established, the existence of such a relationship following peripheral injury is still a subject of controversy (34; 74).

Table 1. List of Known Causes of Secondary Dystonias

• Perinatal cerebral injury
• Encephalitis, infectious and postinfectious
• Head trauma
• Thalamotomy
• Cervical cord injuries or lesions
• Peripheral injury
• Brainstem lesion, including pontine myelinolysis
• Primary antiphospholipid syndrome
• Focal cerebral vascular injury, arteriovenous malformations
• Hypoxia
• Brain tumor
• Multiple sclerosis
• Drug-induced (dopamine D2 receptor blocking agents)
• Toxicants (eg, Mn, CO, carbon disulfide, cyanide, methanol, disulfiram, 3-nitropropionic acid)
• Metabolic, such as hypoparathyroidism
• Neurodegenerative such as neurodegeneration with brain iron accumulation, Niemann-Pick type C, Wilson disease, neuroachantocythosis, etc.
• Psychogenic


Adapted from (20)

The concept of peripheral trauma as a causative factor of dystonia is not new. In 1888, Gowers reported a case of cervical dystonia after a neck injury and mentioned a case of a naval officer who developed writer’s cramp after spraining his thumb (27). Wilson also believed that occupational cramps were sometimes precipitated by injury such as a sprain (75). There is growing support for the hypothesis that peripheral trauma may cause movement disorders, such as tremor, dystonia, and parkinsonism. However, no prospective data have been reported, and the evidence comes largely from clinical reports based on a strong temporal-anatomic relationship between trauma and dystonia. Studies have linked peripheral dystonia with complex regional pain syndrome (CRPS) and provided new evidence to support the cause-effect relationship between trauma and peripheral dystonia (71; 35; 46; 40; 51; 54). This review will focus on the following fundamental questions: (1) What are possible underlying mechanisms of peripherally induced dystonia? (2) What factors predict or predispose an individual to the development of dystonia following peripheral injury? (3) What are the clinical characteristics of dystonic movements in peripheral dystonia compared to primary dystonia? (4) What are the prognoses and long-term outcomes in patients with peripheral dystonia?

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