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907-1. Alcoholic Cerebellar Degeneration. Clinical Syndrome. The clinical syndrome of alcoholic cerebellar degeneration is remarkably stereotyped. The usual presentation, as in this patient, is a progressive unsteadiness in walking evolving over months and years. Clinical Syndrome.

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907-1

Alcoholic Cerebellar Degeneration

clinical syndrome
Clinical Syndrome

The clinical syndrome of alcoholic cerebellar degeneration is remarkably stereotyped. The usual presentation, as in this patient, is a progressive unsteadiness in walking evolving over months and years.

clinical syndrome3
Clinical Syndrome

The cerebellar syndrome predominantly affects stance, eye movements, and gait, sometimes with trunkal ataxia and titubation. Dysarthria and upper limb ataxia are rare.

pathophysiology
Pathophysiology

Ataxia may develop during periods of abstinence.

Identical cerebellar degeneration has been observed in non-alcoholic patients with severe malnutrition.

eye movements
Eye Movements

Square Wave Jerks

Horizontal Saccadic Hypermetria

Horizontal Gaze Evoked Nystagmus

Saccadic Pursuit

slide6

Deficits Caused by Lesions of Dorsal Vermis, Fastigial Nucleus, and Uncinate Fasciculus

Box 12-4. Leigh RJ, Zee DS. The Neurology of Eye Movements 4th Edition. Oxford University Press, New York 2006 with permission.

pathological changes
Pathological Changes

Selective atrophy of the anterior and superior parts of the cerebellar vermis

Involvement of the cerebellar hemispheres less extensive

Loss of neurons in the cerebellum involves all types but Purkinje’s cells are the most seriously affected

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MRI

Figure 1: Sagittal T1WI shows striking atrophy of the superior vermis.

mri findings
MRI Findings

Figure 2: Axial T2WI through the upper midbrain and vermis shows the cerebellar folia are thinned and the CSF spaces increased.

Courtesy Anne Osborn, M.D