Alcoholic cerebellar degeneration

Alcoholic cerebellar degeneration is a rare syndrome that occurs in long-term alcoholics (over 140 g of alcohol/day for more than 10 years), often malnourished. The direct toxicity of alcohol and vitamin deficiencies are implicated.

Clinical Presentation

The onset usually occurs over a few months to several years. However, cases with onset over a few days to weeks have also been described.

The dominant abnormality is truncal ataxia (vermis involvement) affecting gait and coordination of the lower limbs. Rare and late manifestations may include dysarthria, tremors, coordination problems of the upper limbs, intermittent diplopia, and nystagmus. Any other abnormalities should prompt consideration of an alternative diagnosis.

The differential diagnosis can be challenging, particularly distinguishing it from a mild form of Wernicke's limited to ataxia, multisystem atrophy, and chronic sedative intoxication.

Additional Examinations

Additional examinations are only justified to rule out differential diagnoses.

MRI may show predominant cerebellar atrophy in the vermis. However, more than a quarter of chronic alcoholics present similar radiological abnormalities without associated clinical symptoms.

Management

• Alcohol abstinence is crucial. If achieved, small series demonstrate stabilization or even long-term improvement.
• Vitamin supplementation as a principle (B vitamins and folates) should be maintained if abstinence is not achieved.

Bibliography

Bradley WG et al., Neurology in clinical practice, 5th ed., Butterworth-Heinemann, e-dition, 2007

Charness ME, Overview of the chronic neurologic complications of alcohol, UpToDate, 2022

EMC, Traité de neurologie, 2022