Neurological Disorders: Dystonia

Introduction

Dystonia is an example for an extra pyramidal type of neurological disorder; it also falls under the category of movement disorders.

Extra pyramidal is the term given to a group of structures within the brain that are involved with controlling the tone and functioning of the muscles and includes structures like basal ganglia.

Extra pyramidal dysfunction can produce various movement disorders including posturing, tremors, chorea etc.

The dystonic-movement-disorder is characterized by prolonged abnormal contraction of the muscles resulting in twisting or posturing of the involved part of the body.

It may be focal or generalized.

What causes it?

It may be hereditary or acquired. There are myriad causes for dystonic-conditions under both these categories and include traumas, infections, medication side effects (acute dystonic-reactions), hereditary/ degenerative disorders, idiopathic etc.

Common Types

The following are the common types;

• Wry neck or torticollis
• Hemi-facial spasm
• Blepharospasm
• Generalized type
• One of the manifestations of Wilson's, Parkinsonian and Huntington's Disease etc

Diagnosis

The diagnosis is mostly clinical however its cause needs to be identified and accomplished by carefully chosen investigations like CT/MRI of the brain, various blood tests (e.g. ceruloplasmin levels for Wilson disease), Genetic tests (DMD),

Treatment

Treatment would be mostly symptomatic focusing on the movement disorder, however if any treatable condition is identified it is managed accordingly.

For the symptomatic management drugs like anticholinergics (e.g. benztropine), benzodazepines, antihistaminic, dopaminergic, antidopaminergic etc can be used.

Botulinum toxin injection can be tried for focal dystonic conditions.

More specific treatments include chelating agents for Wilson disease, stopping the offending agents(for drug induced) etc.

A neurologist is frequently involved in the care of patients with these movement disorder.



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