Dystonias focal segmental idiopathic

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SPASMODIC TORTICOLLIS (Wry neck)

Unilateral deviation of the head.

Aetiology is unknown. Vestibular abnormalities occur on testing, but it is uncertain whether these cause torticollis or result from the abnormal head posture.

/ Dystonic contraction of the left sternomastoid produces head turning to j the right.

/ Pressure of the index finger on the right side of the chin may turn the / head back to the neutral position (gestc antagoniste).

Turning of the head is specially noticeable when the patient is walking. Eventually hypertrophy of the sternomastoid occurs. Pathology: unknown. Diagnosis is based on clinical findings. Treatment: anticholinergics and phenothiazincs produce some benefit in 50% of patients. Injection of Botulinum toxin into the sternomastoid muscle gives variable symptomatic relief though requires regular repetition.

(Operative techniques no longer performed).

Prognosis: Remission occurs in 20% of patients. Dystonia may spread into other muscle groups. In the long term, psychological disturbance often occurs.

WRITER'S CRAMP

Variable age of onset.

Muscles of the hand and forearm tighten on attempting to write and pain may occur in the forearm muscles. Previously regarded as an 'occupational neurosis' but now classified as a partial dystonia. May be a precursor of Parkinson's disease.

Treatment: Benzodiazepines and anticholinergics arc of limited value. OROMANDIBULAR DYSTONIA

Constant involuntary prolonged tight eye closure (blepharospasm) is associated with dystonia of mouth, tongue or jaw muscles (jaw clenching and tongue protrusion). Response to treatment is poor though phenothiazines should be tried. Section of the nerves to orbicularis oculi muscles will relieve blepharospasm. Botulinum toxin injection is also effective.

DRUG INDUCED DYSTONIA

Acute adoption of abnormal dystonic posture - usually head and neck or oculogyric crisis (upward deviation of eyes) - caused by phenothiazines, butyrophenones, e.g. haloperidol, metoclopramide. Anticholinergics, e.g. benztropine for 24-48 hours helps symptoms settle.

LEVODOPA RESPONSIVE DYSTONIA

This disorder presents in childhood and generally involves the legs only. Falls are frequent and the response to levodopa is maintained over many years.

PROGRESSIVE SUPRANUCLEAR PALSY

A condition characterised by gaze palsies, extrapyramidal features, axial dystonia (truncal dystonia) and progressive pseudobulbar palsy. Onset in the 5th to 6th decade. Aetiology: unknown.

Pathology: Neuronal loss is evident in periaqueductal grey matter, brain stem, nuclei, subthalamic nuclei and the superior colliculi.

Neurofibrillary tangles as seen in Alzheimer's disease arc also found.

Signs: Downward eye movement is initially impaired followed by all other voluntary eye movement. Lid retraction is common. Pseudobulbar signs develop (sec page 534).

The head then hyperextends (dystonia) and rigidity ensues in the limbs.

Treatment: Levodopa and anticholinergics give disappointing results.

The course is relentless with progression and death in 2-5 years.

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