Bilateral Sixth Nerve Nuclear Lesions In Wernicke's Encephalopathy (WE) - Recovery with Thiamine

Ophthalmoplegia is an integral part of the classic WE triad. Horizontal gaze palsy (h-GP) in previous WE series was present in 102 of 232 cases. We examined a patient with vertical and bilateral h-GP and video-monitored the daily evolution of the ophthalmoplegia while on high-dose intravenous thiamine.

Poster 60 BILATERAL SIXTH NERVE NUCLEAR LESIONS IN WERNICKE'S ENCEPHALOPATHY (WE). RECOVERY WITH THIAMINE Joshua Chisholm1, Maxwell Nyce1, Julia Szmada1, Gregory Blume1, Jorge Kattah1 1 Illinois Neurological Institute. University of Illinois College of Medicine, Peoria, Illinois, USA Introduction: Ophthalmoplegia is an integral part of the classic WE triad. Horizontal gaze palsy (h-GP) in previous WE series was present in 102 of 232 cases. We examined a patient with vertical and bilateral h-GP and video-monitored the daily evolution of the ophthalmoplegia while on high-dose intravenous thiamine. Description of Case(s): A 64- year old woman status post vertical band sleeve gastroplasty ~ 25 years earlier presented with generalized weakness. Two months prior to admission, she developed persistent nausea and vomiting, and underwent gastric dilatation, without improvement. The day of admission, she was awake, though verbally unresponsive to her husband. Initial examination showed encephalopathy, unstable station and complete horizontal and vertical gaze palsy, sparing lids and pupils. She had paralysis of saccades and pursuit, but was not alert enough to test the slow vestibulo-ocular reflex (VOR). In attempted right gaze, she had a few degrees of adduction of the left eye, suggesting greater involvement of motoneurons over interneurons in the left sixth nerve nucleus. Baseline thiamine level was 28 mg/Dl. Intravenous thiamine induced rapid, albeit asymmetric gaze recovery. The next day, she had left gaze paretic nystagmus and only partial right gaze. Vertical gaze became normal. Two days later, she had bilateral h-gaze holding failure but ophthalmoplegia resolved; the h-head impulse test became bilaterally positive with reduced gain, the vertical VOR was normal. Conclusions, including unique features of the case(s): Absence of pupillary or eyelid involvement is characteristic for WE associated ophthalmoplegia. In this case, motoneurons in the left sixth nerve nucleus (cholinergic) had greater involvement than the (glutamatergic) interneurons. Vertical gaze improved rapidly. Recovery was initially asymmetric, but once h-GP recovered, she had gaze holding failure (Medial vestibular (MVN) and prepositus hypoglossi nucleus), without abducens paresis. At this point, she showed bilaterally impaired h-VOR (MVN compromise) . Vertical gaze palsy resolved, the vertical neural integrator function and VOR were normal. References: 1. Horn A K E, et al Identification of functional cell groups in the abductees nucleus of monkey and humans by perineural nets and Choline Acetyltransferase Immunolabelling. Frontiers of Neurology 2018;12:75 2. Victor M, Adams R, Collins G. The Wernicke's Korsakoff's Syndrome F.A Davis Co , Philadelphia , 1978.pp 25-26 3. Kattah J C. The Spectrum of Ocular Motor and Vestibular abnormalities in Thiamine Deficiency. Curr Neurol Neuroscience Rep. 2017 May; 17(5):40 Keywords: Ocular motility, Ocular manifestations of vestibular disorders, Nystagmus, Miscellaneous Financial Disclosures: The authors had no disclosures. Grant Support: None. Contact Information: Jorge C Kattah: [e-mail] 2020 Annual Meeting Syllabus | 115