PC2146: Korsakoff Syndrome in a Late Adolescent with Wernicke Encephalopathy

Introduction: Wernicke encephalopathy (WE) is a neurological emergency defined by acute encephalopathy, oculomotor dysfunction, and ataxia. While often considered an alcohol-related disease in adults, in pediatrics, disorders leading to deficient intake (anorexia nervosa, parenteral nutrition), malabsorption (gastrointestinal surgery, colitis, food allergy), and altered thiamine metabolism (leukemia, SLC19A3 mutation) are the main non-alcohol related causes of WE. Suspecting thiamine deficiency and promptly treating with high-dose intravenous thiamine improves prognosis.

Case Description: An 18-year-old male with disorganized type schizophrenia non-adherent to antipsychotic medication for eight months was involuntarily admitted for psychiatric assessment after he refused to leave his room for four weeks, was defecating and voiding in containers, and vomiting daily in the context of cannabis use. Food remnants were found in his bedroom and he had no history of alcohol use. He presented with dehydration, hypokalemia, and features of retarded catatonia, and was given fluids (including dextrose), lorazepam and then olanzapine. Six days later, there was acute worsening of mental status (GCS 7), exacerbated catatonia, horizontal nystagmus, and hypothermia. Brain MRI showed findings suggestive of WE (Figure 1). Multiple investigations eliminated alternative causes of altered consciousness, and of nutrient deficiency. Thiamine 500 mg intravenous three times daily was started along with oral lorazepam, reverting the altered consciousness. It was later confirmed that thiamine plasma levels were undetectable ( < 7 ng/mL) with concurrent vitamin B6, D, and C deficiencies. Following multivitamin supplementation, there was limited improvement of catatonia, ultimately requiring a prolonged course of thrice weekly ECT. As catatonia improved, schizophrenia symptomatology emerged, and improved significantly following paliperidone therapy. As schizophrenia symptoms improved, complete anterograde and partial retrograde amnesia, apathy, and anosognosia emerged indicating – to our knowledge – the youngest individual with Korsakoff syndrome reported to date.

Discussion: Pediatric cases of WE are underdiagnosed and suspected to have a similar incidence to adults. Our patient’s untreated schizophrenia likely contributed to deficient vitamin intake, possibly exacerbated by cannabinoid hyperemesis syndrome, with the presentation of catatonia masking the WE. Since only 16% of patients present with the classical WE triad, recognizing the high risk for thiamine deficiency is important, and in this case could have prevented the administration of dextrose prior to thiamine, possibly preventing acute WE. Korsakoff syndrome has been described in non-alcohol related adult psychiatric patients, but never in the pediatric population, and is associated with poor prognosis.

Conclusion: Pediatric WE is underrecognized; its early consideration in patients with risk factors can improve prognosis.