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Título

Status epilepticus in a patient with anorexia nervosa-related Wernicke-Korsakoff syndrome: case report

RESUMO

Case: A 42-year-old female underwent exploratory laparotomy due to acute intestinal obstruction, and nineteen days later lost consciousness. She had been severely depressed and anorexic for over four months by that time. There was no report of previous seizures, other neurological disorders, smoking or regular alcohol use. Physical examination revealed pupillary hippus, vertical and horizontal nistagmus, tachyarrhythmia, irregular breathing pattern and bilateral focal neurological signs – particularly on the left – findings suggestive of focal status epilepticus, which was successfully treated with IV midazolam. The hypothesis of status epilepticus complicating Wernicke-korsakoff syndrome (WKS) was raised. MRI displayed several T2/Flair hyperintense areas and “faint” hyperintense DWI areas in the mammilary bodies, thalamus, parieto-occipital zones and cerebellum, as well as gyriform oedema within the telencephalic sites. In addition, a medium right-sided parieto-occipital area of DWI hyperintensity (with corresponding ADC map hypointensity) was found. The radiological findings were suggestive of peri-ictal epileptic state and WKS + secondary parieto-occipital stroke. EEG, performed three days after seizure control was obtained with phenytoin and levetiracetam, was compatible with toxic-metabolic encephalopathy and focal slowing on the right posterior cerebral quadrant due to stroke. Discussion: WKS is a disorder caused by thiamine defficiency, usually related to binge alcohol drinking, or, alternatively, to anorexia nervosa, hepatic disfunction, hyperthyroidism or hyperemesis gravidarum. The recommended daily thiamine intake is supplied by a normal healthy diet. However, malnourished people undergoing medium-large surgeries (which demand a good storage of B1), are especially prone to this condition. Nevertheless, associated focal cortical lesions are rather rare, and among these cases 70% are non-alcohol-related and 30% present seizures at some point of clinical follow-up. Final coments: we have reported an atypical evolution of anorexia nervosa-related WKS, which led to permanent multifocal encephalic damage following status epilepticus. Although its atypical course here, WKS should be considered - as a standard of care – in every single patient at the emergency room with reduced conscious level. WKS must always be a “don’t miss” diagnosis, and, as soon as possible, treated with thiamine. Therefore, the burden of permanent brain damage is usually minimal.

Palavras Chave

Epilepsy. Status epilepticus. Wernicke-Korsakoff syndrome

Área

Epilepsia