Clinical Features

A combination of cerebellar dysfunction, parkinsonian features, and autonomic failure characterize the clinical picture. In some patients, cerebellar, and in others, par-kinsonian symptoms predominate. The Shy-Drager syndrome may occur with either one.

Ataxia of gait is usually the presenting cerebellar symptom, followed by ataxia of limbs, dysarthria, and ocular signs such as nystagmus, ocular dysmetria, fixation instability, and jerky pursuit movements. The parkinsonian features include rigidity, bradykinesia-akinesia, postural instability, hypokinetic dysarthria, and tremor. The autonomic failure consists of orthostatic hypotension, bladder dysfunction (urinary frequency, urgency, retention), bowel dysfunction (constipation), and sexual (male erectile) dysfunction. Spasticity, hyper-reflexia, sleep disorder, respiratory stridor, and hypoventilation add to the syndrome. The course averages from 6 to 10 years. MRI demonstrates cerebellar atrophy and enlargement of the fourth ventricle, indicating pontine atrophy (Fig. 5.35).

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