Isolated ophthalmoplegia as presenting sign of pediatric Wernicke encephalopathy

Neurology(2018)

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摘要
Objective: To describe a case of isolated ophthalmoplegia as the presenting sign of Wernicke encephalopathy in a teenager with recent history of Roux-en-Y gastric bypass. Background: Wernicke encephalopathy is present in up to 3% of the population with increased prevalence in patients with history of alcoholism, malabsorption, or poor dietary intake of thiamine. It is classically characterized by altered mental status, oculomotor dysfunction and gait ataxia. However, the full triad is present in less than one third of patients leading to delays in diagnosis. Pediatric Wernicke encephalopathy is rare and of the ten previously reported cases occurring after weight loss surgery, none had isolated ophthalmoplegia. Design/Methods: A 15 year old female with morbid obesity was admitted with dizziness and hypotension 2 months after her Roux-en-Y gastric bypass. Symptoms improved with normal saline fluid bolus without thiamine. Within 24 hours she developed hypertension and subjectively blurred vision. On physical examination patient was alert and oriented with cranial nerve exam significant for prominent vertical nystagmus with vertical gaze and ophthalmoplegia (bilateral medial and lateral rectus palsy). Strength and coordination were intact. CT head was negative for hemorrhage, subacute ischemia or mass lesion. Results: MRI brain demonstrated hyperintensities in the periaqueductal parenchyma along the dorsal margin of the medulla consistent with thiamine deficiency. A diagnosis of Wernicke encephalopathy was subsequently confirmed by low thiamine level. The patient later developed mild alterations in mental status and transient gait ataxia. Her symptoms improved with thiamine supplementation. Conclusions: Clinicians should have a high index of suspicion for Wernicke encephalopathy in pediatric patients after gastric bypass procedures. We recommend monitoring for clinical signs of nutritional deficiencies, educating patients on clinical signs of thiamine deficiency, and early multivitamin supplementation. Prevention and early identification to ensure treatment of this potentially fatal yet easily treated condition are essential. Disclosure: Dr. Takacs has nothing to disclose. Dr Lyons-Warren has nothing to disclose.
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