Wernicke’s encephalopathy precipitated by neuromyelitis optica spectrum disorder and Graves’ disease: A tale of clinical and radiological dilemmas

Background Neuromyelitis optica spectrum disorder (NMOSD), an autoimmune astrocytopathy, may share common clinico‐radiological features with Wernicke's encephalopathy (WE). A variant of NMOSD, known as area postrema syndrome (APS), that presents with intractable hiccups and associated vomiting,...

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Published in:Clinical & experimental neuroimmunology 2022-05, Vol.13 (2), p.67-71
Main Authors: Ghosh, Ritwik, Dubey, Souvik, Ray, Adrija, Roy, Dipayan, De, Kaustav, Mandal, Arpan, Naga, Dinobandhu, Swaika, Bikash Chandra, Pandit, Alak, Benito‐León, Julián
Format: Article
Language:English
Subjects:
Area postrema
area postrema syndrome
astrocytopathy
Encephalopathy
Graves disease
Hyperthyroidism
Immunomodulation
Metabolic disorders
Neuroimaging
Neuromyelitis
neuromyelitis optica
Thiamine
Thyroid
Thyrotoxicosis
Vomiting
Wernicke's encephalopathy
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Summary:Background Neuromyelitis optica spectrum disorder (NMOSD), an autoimmune astrocytopathy, may share common clinico‐radiological features with Wernicke's encephalopathy (WE). A variant of NMOSD, known as area postrema syndrome (APS), that presents with intractable hiccups and associated vomiting, might lead to the depletion of nutrients if not detected and treated early. Autoimmune thyroid disorders (i.e., Graves’ disease) may be associated with NMOSD. Rarely, thyrotoxicosis can give rise to thiamine depletion and WE. Case presentation Here, we present a case of untreated hyperthyroidism in an Indian female who presented with thyrotoxicosis and later developed WE, possibly also contributed by NMOSD (APS)‐induced recurrent vomiting. The patient recovered with antithyroid drugs, parenteral thiamine, and immunomodulatory therapy. The possible pathogenic mechanisms have been discussed. Conclusion Our case establishes the importance of considering NMOSD variants in metabolic encephalopathy, especially if neuroimaging is suggestive and in the backdrop of another autoimmune disorder. Neuromyelitis Optica Spectrum Disorder may share common clinico‐radiological features with Wernicke’s encephalopathy. We present a case of untreated hyperthyroidism in a female who presented with thyrotoxicosis and later developed Wernicke’s encephalopathy, possibly contributed by NMOSD‐induced recurrent vomiting. This case re‐establishes the importance of considering cerebral syndrome, diencephalon syndrome and area postrema syndrome variants of NMOSD while managing a case of metabolic encephalopathy, particularly if neuroimaging features are suggestive.
ISSN:1759-1961
1759-1961
DOI:10.1111/cen3.12661