Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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June 9, 2016
Nonalcoholic Wernicke Encephalopathy
- Background:
- Wernicke encephalopathy is secondary to thiamine deficiency and represents a medical emergency. Most patients have a background of alcohol abuse, but there are many other causes for this disease, including GI tumors, hyperemesis, chronic uremia, and total parenteral nutrition.
- In this case, Wernicke encephalopathy developed after prolonged parenteral nutrition.
- Clinical Presentation:
- Presents with a clinical triad of altered consciousness, opthalmoplegia, and ataxia, but only 16–38% of patients show the classic triad.
- Key Diagnostic Features:
- Symmetric high signal intensity in the medial thalami, mamillary bodies, and periaqueductal grey matter
- Differential Diagnoses:
- Ischemia due to artery of Percheron occlusion: Clinically is characterized by a triad of altered mental status, vertical gaze palsy, and memory impairment. The medial thalami can be symmetrically involved but does not affect the mammillary bodies.
- Acute disseminated encephalomyelitis: Typically presents in children following viral infection or vaccination. MRI demonstrates multifocal bilateral but asymmetric white matter lesions and involvement of the basal ganglia and posterior fossa.
- Creutzfeldt-Jakob disease: A rapidly progressive dementia associated with myoclonic jerks and akinetic mutism. The classic finding on MRI is bilateral symmetrical restricted diffusion of the striatum, plus pulvinar nuclei of the thalami (hockey stick sign) and cortical gray matter.
- Treatment:
- Administration of intravenous thiamine
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