Wernicke-Korsakoff syndrome (WKS) is caused by thiamine deficiency due to malnutrition or malabsorption and can be recognized classically by a clinical triad: (1) encephalopathy (disorientation, indifference, and inattentiveness); (2) ocular motor abnormalities (nystagmus, lateral reclus palsy, and conjugate gaze palsies); and (3) gait ataxia. Nevertheless, only one third of patients diagnosed with WKS presented with the classical triad. WKS is an uncommon disease which occurs primarily in chronic alcoholics, but is also seen in gastrointestinal diseases, anorexia nervosa, peritoneal dialysis or hemodialysis, and human immunodeficiency virus infection. The WKS may, therefore, be underdiagnosed, especially in nonalcoholic patients: unfortunately, most diagnoses are not made clinically but rather at autopsy. Treatment is based on immediate restoration of thiamine. Clinical diagnosis can be elusive but MRI may be able to detect cerebral lesions, whose type and distribution are characteristic of the WKS We present a female adult, nonalcoholic subject with a previous history of rettocolic resection for rectal carcinoma. After 1 year the patient was submitted to a second surgical procedure for clinical symptoms of a subocclusive intestinal condition. In the postoperative period, during total parenteral nutrition, the patient developed clinical signs and symptoms suggestive of WKS, associated with flaccid paraparesis on lower limbs. CT scan was normal. MRI, in the acute phase, demonstrated hyperintensity located in the mammillary bodies and mammillo-thalamic tracts, mainly evident on FLAIR sequences and enhancing on T1-weighted sequences after intravenous gadolinium administration. The patient was treated with vitamin supplementation but she improved only with parenteral thiamine therapy. Spine MRI and echocardiography were normal. A gradually recovery of general, gastrointestinal, and neurological conditions appeared. Now, after 4 months, the patient is attending a neuroreahabilitation program. She shows ataxia and mild cognitive dysfunctions. Neuropathic beriberi involvement is also present. Recovery from cerebellar ataxia following thiamine treatment may be very slow and rarely complete, suggesting the existence of both reversible "biochemical" as well as irreversible neuropathologic damage. Purpose of the case presentation is to focalize the variability of clinical presentations of this underdiagnosed and rarely considered condition associated with high morbidity and mortality and cognitive and neurological sequelae. The literature concerning WKS is reviewed, especially for nonalcoholic conditions.
|Issue number||4 SUPPL.|
|Publication status||Published - 2000|
ASJC Scopus subject areas
- Clinical Neurology