A 51-year-old independently living white male with chronic paranoid schizophrenia was admitted to the hospital following 2 witnessed generalized tonic-clinic seizures. Paramedics measured his blood glucose at 60 mg/dL. He had a second seizure in the emergency department. He had no history of neurological abnormalities, gait disturbances, or tardive dyskinesia, and at baseline was well functioning, and performing his activities of daily living. Three months before his admission, he stopped his chlorpromazine resulting in increased isolation and paranoia, decreased appetite, and a 40-pound weight loss. He had a remote alcohol abuse history but no history of alcohol dependence, delirium tremens, or seizures. As per his parents and case worker who visited him regularly, he had remained alcohol free the past several years and reported no evidence of him drinking alcohol during the weeks before admission. He had no tobacco or illicit drug use. Review of system was negative for paresthesia, paresis, chest pain, or shortness of breath.
His examination was notable for a temperature of 36.6°F, blood pressure 95/74, heart rate 118, respiration 20, with a room air oxygen saturation of 97%. He was markedly disoriented, writhing with agitation, emaciated, and disheveled. He had mild dysarthria, a flat affect, randomly responded “not much, no, or yes” to questions, and increased nonpurposeful head and arm movements. Pupils were equal, round, and reactive to light, with marked limitation of bilateral vertical and horizontal gaze with no nystagmus. His neck and extremity tone was increased, and deep tendon reflexes could not be elicited. The plantar responses were flexor. He was unable to stand, ambulate, or cooperate with cerebellar, sensory, or motor examination due to confusion but was able to move all 4 extremities. He had a 3 cm forehead laceration with ecchymosis, multiple perifollicular petechiae on his posterior legs (). He had no changes in gums or tooth mobility. He had no appreciable ocular or retinal abnormalities.
Follicular petechiae on the patient's lower extremities.
Laboratory studies revealed hyponatremia, hypochloremia, an anion gap acidosis, moderate renal failure, and mild rhabdomyolysis (). His CBC values were within normal range except for a mildly increased white blood cells (WBC) of 13.5 with 85% neutrophils. His MCV, methylmalonic acid, homocysteine, urine dipstick and microanalysis, urine drug screen, blood cultures, prothrombin time (by INR), partial thromboplastin time, troponin, electrocardiogram, arterial blood gas, chest radiograph were normal, and cerebral spinal fluid (CSF) fluid were unrevealing. On admission he had a mildly elevated aspartate aminotransaminase (AST) and total bilirubin but normal alanine aminotransaminase, alkaline phosphatase, total protein and albumin. His anion gap, AST, and bilirubin normalized on hospital day 2, but his albumin fell to 1.9 g/dL following hydration. A blood alcohol level was not obtained. A noncontrast head computed tomography (CT) showed mild cortical atrophy but no evidence of intracranial bleed or trauma.
He was admitted with seizures, mild rhabdomyolysis, metabolic acidosis (thought secondary to his seizures), and laboratory and clinical features of malnutrition, scurvy, andWE. He was treated with intravenous 0.9% saline with 5% dextrose, thiamine, multivitamins, and phosphenytoin. Several hours after receiving 100 mg IV thiamine, his mental status, dyskinesia, rigidity, and ophthalmoplegia improved; however, marked vertical and horizontal nystagmus developed. He experienced a third witnessed generalized tonic-clonic seizure. An awake, interictal electroencephalogram (EEG) showed mild generalized slowing. A cranial magnetic resonace imaging (MRI) with gadolinium demonstrated moderate cortical atrophy and scattered bihemispheric subcortical and deep white matter nonenhancing foci. These changes were interpreted as consistent with chronic small vessel ischemic changes. Further review of the MRI revealed increased FLAIR T2 signal involving the medial aspect of the anterior thalamus extending into the hypothalamus and the massa intermedia (), all consistent with his clinical diagnosis of WE. His vitamin analysis revealed low vitamin A, slightly low vitamin E, and C ().
FIGURE 2 (A and B) Increased signal on axial T2 images involving the medial aspect of the thalamus and the massa intermedia. The arrows on “A” indicate the hyperintensity in the medial thalamus and anterior hypothalamus. The arrow on “B” (more ...)
Olanzapine was initiated for his schizophrenia. The thiamine dose was maintained at 100 mg/d throughout hospitalization and continued upon discharge. A repeat EEG on hospital day 4 showed improvement. On day 14, he was discharged at his baseline functioning mental status with mild ataxia, and persistent vertical nystagmus.