Lamdhade S, Almulla A, Alroughani R
Division of Neurology, Department of Medicine, Amiri Hospital, Arabian Gulf Street, Kuwait City, Kuwait.
Division of Neurology, Department of Medicine, Amiri Hospital, Arabian Gulf Street, Kuwait City, Kuwait ; Neurology Clinic, Dasman Diabetes Institute, Dasman, Kuwait.
Case Rep Neurol Med. 2014;2014:582482. doi: 10.1155/2014/582482. Epub 2014 Feb 10.
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.
背景。韦尼克脑病（WE）是一种具有严重神经后果的临床诊断疾病。其在非酒精性患者中的发生率被低估，且在青少年中并不常见。我们旨在引起对一例罕见病例的关注，该病例具有额外的临床和影像学特征。病例。一名16岁女孩因肠梗阻出现三周的呕吐病史。住院后不久出现复视。神经学评估显示双侧外直肌受限伴水平眼球震颤，以及双侧肢体辨距不良。脑部MRI正常。给予硫胺素后她迅速好转。四个月后，她出现头痛、双侧严重耳聋和耳鸣。临床上，她有严重的感音神经性听力损失、双侧外直肌麻痹和步态共济失调。头颅CT显示双侧尾状核低密度影。脑部MRI显示乳头体和蚓部钆增强。静脉注射硫胺素后她有显著改善。头痛完全缓解，而眼球运动、听力和耳鸣在72小时内部分改善。结论。青少年复发性韦尼克脑病并不常见。头痛、耳鸣和耳聋是罕见的临床特征。尽管MRI研究显示韦尼克脑病的典型特征，但CT扫描中双侧尾状核低密度影并不常见。对于疑似病例，应及时给予硫胺素治疗以预防永久性神经后遗症。
