Neuroscience Clerkship

 

 

WERNICKE’S ENCEPHALOPATHY

 
Alcohol accounts for an estimated 100,000 deaths/year in the U.S. Wernicke’s encephalopathy is a condition most commonly seen in chronic alcoholics in the U.S. This condition reflects the effect of severe and sustained thiamine depletion in the presence of continued calorie intake. Wernicke's is a medical emergency. If not recognized and treated promptly, it can lead to permanent neurologic disability or death.
 

Thiamine is required for oxidative carboxylation of pyruvate and a-ketoglutarate which are required for energy metabolism of most cells, especially nervous tissue cells. The dehydrogenase reactions require thiamine in order to produce ATP.

Nervous Tissue Physiological Changes:

Axonal demyelination

Neuronal loss

Glial proliferation

Petechial pericapillary hemorrhages

Above: Pathologic specimens in Wernicke's encephalopathy. Note the hemorrhagic lesions in the medial thalamus adjacent ot the third ventricle (left) and in the mamillary bodies (right).

The oculomotor, vestibular, medullary autonomic nuclei, and the brainstem reticular formation are the most affected. At higher levels, the mamillary bodies, the mediodorsal thalamic nuclei, and scattered cortical regions, including hippocampus are also affected.
Clinical Presentation


Classic Triad:

Mental State Changes

Confused to stupor to coma

Ataxia

Ophthalmoplegia

Partial or complete external opthalmoplegia

Nystagmus

Pupils may only sometimes be affected

Any motor cranial nerve can be partially affected

Other Clinic Findings That May Be Present:

Tachycardia

Orthostatic hypotension

Hypothermia

Seizures (including status)

Above: MRI images; (Left) Axial flair, (Middle) Axial T1 weighted, (Right) Axial T1 weighted with gadolinium in a patient with Wernicke's. Note the enhancement of the mamillary bodies on the post-gadolinium scan and the prominent signal changes in the hypothalamus and mamillary bodies on the flair image.
Treatment


Administer thiamine, 100 mg parenterally, upon suspicion of diagnosis, followed by replenishment of blood volume and electrolytes. The response to thiamine administration is diagnostic. The opthalmoplegia should begin to improve within a matter of hours to a day.

Glucose replenishment should not precede thiamine because it may actually precipitate acute worsening of this condition

Patients should also be observed for any withdrawal seizures or delirium tremens