Recurrent Wernicke's Encephalopathy in a 16-Year-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.

Figure 1

Axial images of Computed Tomography (CT) of brain showing bilateral hypodensities of the caudate nuclei.

Figure 2

Magnetic Resonance Images performed during the second episode. (a) T2 coronal images showing hyperintense signals in caudate nuclei bilaterally. (b)-(c) Axial FLAIR images showing hyperintense signals in vermis and periaqueductal areas. (d) Axial DWI showing restricted diffusion in caudate nuclei. (e) Sagittal T1 with contrast images showing enhancement of the vermis.

Figure 3

An audiogram revealed bilateral sensorineural deafness (performed during the second episode).

Figure 4

(a) Sagittal T1 with contrast image showed persistence of gadolinium in a follow-up study conducted 4 months later. (b)–(d) Sequential coronal T1 images showing the baseline mamillary body when MRI was performed during the first episode (b), enhancement of mamillary body during the second episode (c), and atrophy of mamillary body in a follow-up MRI performed 4 months later (d).