Neuroscience Clerkship

 

 

ALCOHOLIC CEREBELLAR DEGENERATION

The prevalence of alcoholic cerebellar degeneration is not known exactly (around 1% of alcoholics), but it is the most common cause acquired degeneration of the cerebellum, and its clinical syndrome is easily identifiable. It present more commonly in males. Most often, these patients have a long-standing history of alcohol abuse. In addition to, and compounding the syndrome, is commonly the presence of polyneuropathy as well.
 

Clinical Presentation

• Progressive unsteadiness when walking over weeks to months

• Truncal ataxia with wide-based gait and tandem difficulty

• Mild limb ataxia possible, legs > arms due to anterior vermis involvement

• Nystagmus / ocular dysmetria (less common but present sometimes; more often in Wernike’s encephalopathy)

• Rare findings of dysarthria, tremor, and hypotonia
 

Above: Mid-sagittal MRI showing prominent vermal atrophy, especially of the anterior lobe (yellow arrow)

Above: Standard axial head CT at the level of the midbrain. Note the prominent atrophy of the cerebellum.
 

Pathology Findings

Atrophy of the cerebellum ,especiallly the anterior and superior parts of the cerebellar vermis (see figures above) which is responsible for axial and lower extremity coordination

• Histological examination shows cell loss of all neuron types in the cerebellum, especially Purkinje cells

• Secondary loss of neurons is common in the deep cerebellar nuclei and inferior olivary nucleus

• Patients often have concomitant changes due to Wernicke’s encephalopathy as well

 

Treatment

• Improvement in cerebellar symptoms occurs slowly with abstinence from alcohol and with nutritional supplementation, but it is not always complete