Tuesday, June 19, 2018

Wernicke encephalopathy in a 15-year-old girl

While attending the 2018 American Academy of Neurology (AAN) meeting in Los Angeles, California, Medscape contributor Andrew N. Wilner, MD, interviewed Ariel Lyons-Warren, MD, PhD, about her case report of a teenager with pediatric Wernicke encephalopathy.  Dr Lyons-Warren is a pediatric neurology resident at Baylor College of Medicine and Texas Children's Hospital in Houston.

Andrew N. Wilner, MD: I just saw a young pregnant woman on our inpatient service who had hyperemesis gravidarum and developed Wernicke encephalopathy, so your AAN Resident Poster on that topic interested me. I see you have MRI scans with the typical findings. Could you describe those findings?

Ariel Lyons-Warren, MD, PhD: The typical MRI findings in Wernicke encephalopathy in children and adults include a T2 FLAIR hyperintensity—brightness on the T2-weighted MRI in the bilateral medial thalami and the periaqueductal region. The mammillary bodies also can be involved.
Wilner: I'm an adult neurologist so I was intrigued: Why would a child have Wernicke encephalopathy?

Lyons-Warren: Wernicke encephalopathy is actually believed to be just as prevalent in children as in adults and, as in adults, it is underdiagnosed. Our patient, a 15-year-old girl, developed Wernicke encephalopathy because she had undergone a Roux-en-Y gastric bypass, which is a common risk factor for Wernicke encephalopathy. She was not taking her vitamins and became vitamin deficient.

Wilner: What clued you in to the diagnosis?

Lyons-Warren: I was moonlighting in the pediatric ICU when this patient came to us. Her only symptom was an isolated ophthalmoplegia and we didn't know why. Her history of Roux-en-Y gastric bypass made me think about a vitamin deficiency. But the classic triad of Wernicke encephalopathy is altered mental status, ophthalmoplegia, and ataxia, and she did not have the other two symptoms. So it wasn't first on our diagnostic list, but we ordered the MRI and saw the typical findings.
Wilner: Were you able to get a thiamine level (vitamin B1) to document the deficiency?

Lyons-Warren: The team checked the thiamine level the next day. The results came back a few days later at 26 nmol/L, which is very low.

Wilner: I imagine you gave this unfortunate 15-year-old some thiamine. Did it help?

Lyons-Warren: Yes. She is completely back to baseline. My coauthor, Dr Danielle Takacs, sees her in clinic, and other than continuing to be noncompliant, she's completely back to herself.


Danielle Takacs, Ariel Lyons-Warren.  Isolated ophthalmoplegia as presenting sign of pediatric Wernicke encephalopathy.  Abstract.  American Academy of Neurology Annual Meeting,  April 2018.

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.

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