Wernicke’s encephalopathy because of pancreatitis in a young boy

Abstract

Editor, W ernicke’s encephalopathy (WE) is a potentially life-threatening and acute neurological disorder resulting from a lack of thiamine (vitamin B1). Early diagnosis is important to prevent morbidity and mortality. Symptoms are ocular dysfunction, ataxia and changes in consciousness. WE is often associated with alcoholism or malnutrition of the elderly, but can occur in other settings such as hyperemesis gravidarum (Leitner et al. 2006), gastric bypass surgery (Longmuir et al. 2007; Bohnsack & Patel 2010) and other causes of malnutrition (Kesler et al. 2005). WE is suspected to be underdiagnosed in children (Vasconcelos et al. 1999). We wish to present a case where the ophthalmological examination led to the early detection of WE, thereby reducing the patient’s chances of severe complications. An 11-year-old boy was referred to the ophthalmological department with a 1-week history of bilateral eye pain, intermittent diplopia and bilateral temporal headache. The mother had also noted a slight ptosis of the upper left eyelid. The patient had been diagnosed with recurring pancreatitis 5 months prior to presentation at the ophthalmological department and had been experiencing abdominal pain, nausea and vomiting for a long time. He had been discharged from the hospital 2 weeks before the ophthalmological consultation after an episode of pancreatitis, but had been troubled by daily nausea and vomiting since then. The eye examination revealed a mixed horizontal ⁄ vertical end-point nystagmus. The horizontal nystagmus was observed at right gaze and left gaze and was characterized as a coarse, moderate frequency, jerk nystagmus with fast component in the direction of gaze. The vertical nystagmus was only observed at up gaze and had the same characteristics as the horizontal nystagmus, with the exception of a smaller amplitude. In addition, there was a slight ptosis of the upper left eyelid. No malalignment of the eyes was noted. Pupillary reactions were normal, and there was no anisocoria. Funduscopic examination showed symmetrical optic nerve heads with normal appearance, and there were no signs of retinal pathology. Best corrected visual acuity was 0.8 (Snellen chart) in both eyes. The ophthalmologist advised further examination with a cerebral MRI. The images showed bilateral vasogenic brain oedema in the medial thalamus in addition to a pathologically increased and symmetrical signal in both corpora mamillaria (Fig. 1). The radiologist concluded that the changes were consistent with WE. The patient was immediately admitted to the hospital and treated with intravenous thiamine and vitamin B complex tablets. Blood tests taken before commencing treatment confirmed a low serum thiamine level of 72.5 nm (normal range 100–190 nm). One day after the initiation of treatment, the vertical nystagmus had disappeared. At 2 weeks after treatment, the ptosis was no longer apparent, but the patient still had a horizontal nystagmus. Visual acuity was unchanged. A follow-up MRI about 11 weeks after the initial examination was described as normal with complete resolution of the previously observed changes. We wish to publish this case report as a reminder of a potentially lifethreatening diagnosis where the ophthalmologist can be the first to aid in early detection and prevention of the serious consequences that WE entails.