Recurrent Wernicke's encephalopathy in a 16year-old girl with atypical clinical and radiological features

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.

Thyrotoxicosis With Development of Wernicke Encephalopathy in an Adolescent

“…Classical MRI features have been reported in children, but it is more common to see atypical features in younger nonalcoholic patients (17). Atypical radiological features have been also been reported in young patients (14). Our patient showed classic WE imaging findings seen in older patients with alcoholism.…”

“…Rehydrating her with dextrose solution induced her neurologic deficit, which progressed to full-blown picture WE. This clinical picture has been reported in similar scenarios in older patients and during pregnancy (14). However, presentation in children and adolescents is not always classical but can be atypical, resulting in delayed diagnosis (4).…”

“…She continued on antithyroid medication and was educated on the predisposing factors of the disease. Although uncommon, recurrence has been reported in adolescents (14). Hence, proper education and follow-up are necessary.…”

Wernicke encephalopathy hearing loss and palinacousis

“…Deafness associated with WE is rare. There have only been four cases in the literature, which report bilateral sensorineural hearing loss associated in WE, and only one case with documentation of an audiogram . Zhang et al .…”

Pediatric thiamine deficiency disorders in high‐income countries between 2000 and 2020: a clinical reappraisal

“…Mammillary bodies and periaqueductal area abnormalities seem to be more frequent in children and adolescents than in young infants. 23,60,61,68,82 Similarly, nutrition, environment, and genetic diseases (such as Leigh syndrome) potentially overlapping with the TD spectrum might also contribute to these differences. 188,189,197 MRI protocols, such as contrast enhancement, might increase the sensitivity for mammillary bodies detection and has not been used systematically in some studies.…”