Abstract
Background. Wernicke's Encephalopathy (WE) is a clinical diagnosis with serious neurological consequences. Its occurrence is underestimated in nonalcoholics and is uncommon in adolescents. We aim to draw the attention to a rare case, which had additional clinical and radiological features. Case. A 16-year-old girl presented with three-week history of vomiting secondary to intestinal obstruction. She developed diplopia soon after hospitalization. Neurological evaluation revealed restriction of bilateral lateral recti with horizontal nystagmus, and bilateral limb dysmetria. Brain MRI was normal. She had prompt improvement to thiamine. Four months later, she presented with headache, bilateral severe deafness, and tinnitus. Clinically, she had severe sensorineural hearing loss, bilateral lateral recti paresis, and gait ataxia. CT head showed bilateral caudate nucleus hypodensities. MRI brain revealed gadolinium enhancement of mamillary bodies and vermis. She had significant improvement after IV thiamine. Headache completely resolved while the ocular movements, hearing, and tinnitus improved partially in 72 hours. Conclusions. Recurrent WE in adolescence is uncommon. Headache, tinnitus, and deafness are rare clinical features. Although MRI study shows typical features of WE, the presence of bilateral caudate nuclei hypodensities on CT scan is uncommon. Prompt treatment with thiamine is warranted in suspected cases to prevent permanent neurological sequelae.
Highlights
The clinical description of Wernicke’s encephalopathy (WE) was first given by Karl Wernicke in 1881, who described 3 cases; one of them was interestingly nonalcoholic
In the last two decades, nonalcoholic Wernicke’s Encephalopathy (WE) had been described with various etiological conditions secondary to wrong feeding formula in infants, acute pancreatitis, anorexia nervosa, Crohn’s disease, thyrotoxicosis, long term use of TPN following bariatric or major surgeries, bone marrow transplant, AIDS, and chronic kidney disease with hemo- and peritoneal dialysis [1, 2]
We describe a case of recurrent nonalcoholic WE with atypical clinical features of severe bilateral hearing impairment and atypical radiological MRI findings of mammillary body atrophy
Summary
Wernicke’s Encephalopathy (WE) is a clinical diagnosis with serious neurological consequences. A 16-year-old girl presented with three-week history of vomiting secondary to intestinal obstruction. Four months later, she presented with headache, bilateral severe deafness, and tinnitus. She presented with headache, bilateral severe deafness, and tinnitus She had severe sensorineural hearing loss, bilateral lateral recti paresis, and gait ataxia. MRI brain revealed gadolinium enhancement of mamillary bodies and vermis. She had significant improvement after IV thiamine. Tinnitus, and deafness are rare clinical features. MRI study shows typical features of WE, the presence of bilateral caudate nuclei hypodensities on CT scan is uncommon. Prompt treatment with thiamine is warranted in suspected cases to prevent permanent neurological sequelae
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