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

Ghosh, Ritwik; Dubey, Souvik; Ray, Alok K.; Roy, Dipayan; De, Kaustav; Mandal, Arpan; Naga, Dinobandhu; Swaika, Bikash Chandra; Pandit, Alak; Benito‐León, Julián

doi:10.1111/cen3.12661

Cited by 2 publications

(2 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Two prior case reports have described the development of Wernicke's encephalopathy following the diagnosis of NMOSD. 6,7 However, in our case the thiamine level drawn prior to supplementation was elevated, and the patient did not improve with high dose thiamine. Three prior case reports and a case series have raised the possibility of NMOSD mimicking the features of Wernicke's encephalopathy.…”

Section: Discussioncontrasting

confidence: 59%

Neuromyelitis Optica Spectrum Disorder Resembling Wernicke’s Encephalopathy: A Case Report and Review of the Literature

Lynch,

Saez Calveras,

Amin

2024

The Neurohospitalist

View full text Add to dashboard Cite

We describe a case of Neuromyelitis Optica Spectrum Disorder (NMOSD) mimicking Wernicke’s Encephalopathy (WE) to highlight an atypical presentation of NMOSD. A 39-year-old female presented with subacute encephalopathy and progressive ophthalmoplegia. Her MRI revealed T2 hyperintensities involving the mammillary bodies, periaqueductal grey matter, medial thalami, third ventricle, and area postrema. Whole blood thiamine levels were elevated and she did not improve with IV thiamine. CSF was notable for lymphocytic pleocytosis and elevated protein. She tested positive for serum Aquaporin-4 (AQP4) antibody. Subsequent imaging revealed multilevel lesions in the cervical and thoracic spinal cord. Her CSF GFAP antibody also came back positive. She steadily and significantly improved after high-dose IV steroids and plasmapheresis. She later started on chronic rituximab therapy. This represents a unique case of NMOSD presenting with the classical clinical and imaging features of WE, as opposed to the typical presenting symptoms of NMOSD. As such, demyelinating disorders should be considered when there is concern for diencephalic and midline pathologies, particularly without classic WE risk factors. Conversely, clinicians should be aware of secondary nutritional complications arising from severe area postrema syndrome.

show abstract

Section: Discussioncontrasting

confidence: 59%

Neuromyelitis Optica Spectrum Disorder Resembling Wernicke’s Encephalopathy: A Case Report and Review of the Literature

Lynch,

Saez Calveras,

Amin

2024

The Neurohospitalist

View full text Add to dashboard Cite

show abstract

“…“Double-doughnut,” a radiological biomarker of dengue encephalitis, can help identify this life-threatening otherwise treatable condition to prompt and adequately identify and treat these patients, preventing further clinical and neurological sequelae. However, similar bilateral symmetrical thalamic involvement with clinical presentation of acute encephalitis syndrome or comatose state can also be seen particularly in Japanese B encephalitis (which is quite prevalent in India) 21,22 and rarely in autoimmune encephalitis, 23 Wernicke’s encephalopathy, 24 subacute sclerosing pan-encephalitis, 25 bilateral thalamic infarction caused by top of basilar/Percheron’s artery occlusion and deep cerebral venous sinus thrombosis, 26,27 and also in neuromyelitis optica spectrum disorders (NMOSD) 28…”

Section: Discussionmentioning

confidence: 99%

An Intriguing Case of Expanded Dengue Syndrome With Co-existing Encephalitis, Pancreatitis, and Hepatitis: The Classic Thalamic “Double-Doughnut” Sign Revisited

Biswas,

León-Ruiz,

Ghosh

et al. 2024

The Neurohospitalist

Self Cite

View full text Add to dashboard Cite

Background Dengue neuro-infection can present with symptoms ranging from mild to severe. Atypical presentations, such as expanded dengue syndrome, pose diagnostic and therapeutic challenges. Neuroimaging findings, particularly the “double-doughnut” sign on brain magnetic resonance imaging (MRI), have emerged as one of the most valuable aids in diagnosing complex cases of central nervous system infection by dengue virus. Case Presentation We report the case of a 35-year-old female from rural West Bengal, India, with expanded dengue syndrome. The patient presented with fever, headaches, body aches, and sudden disorientation over minutes, which progressed to a coma. Neurological examination revealed profound unconsciousness and nuchal rigidity. Laboratory findings were consistent with dengue infection, including altered liver and pancreatic enzyme levels. The diagnosis was facilitated by identifying the “double-doughnut” sign on the brain MRI, which suggested dengue encephalitis. This finding and clinical and serological evidence guided the treatment strategy. Discussion The “double-doughnut” sign, though not exclusive to dengue encephalitis, proved crucial in this case, aiding in differentiating from other causes of encephalitis. Recognition of this sign can be pivotal in diagnosing expanded dengue syndrome, facilitating timely and appropriate intervention, and improving patient outcomes. This case also underscores the importance of considering dengue in the differential diagnosis of encephalitis, especially in endemic areas. Also, this case’s excellent outcome (both clinically and radiologically) was noteworthy.

show abstract

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

Cited by 2 publications

References 23 publications

Neuromyelitis Optica Spectrum Disorder Resembling Wernicke’s Encephalopathy: A Case Report and Review of the Literature

Neuromyelitis Optica Spectrum Disorder Resembling Wernicke’s Encephalopathy: A Case Report and Review of the Literature

An Intriguing Case of Expanded Dengue Syndrome With Co-existing Encephalitis, Pancreatitis, and Hepatitis: The Classic Thalamic “Double-Doughnut” Sign Revisited

Contact Info

Product

Resources

About