Rapidly progressive dementia with generalized myoclonus in an adult: Do not forget subacute sclerosing panencephalitis

Ghosh, Ritwik; Dubey, Souvik; Mukherjee, Arindam; Benito‐León, Julián

doi:10.1016/j.nrleng.2021.09.006

Cited by 2 publications

(1 citation statement)

References 12 publications

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“…"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 In closing, 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..…”

Section: Discussionmentioning

confidence: 90%

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

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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.

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Section: Discussionmentioning

confidence: 90%

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

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Subacute Sclerosing Panencephalitis in a 63-Year-Old Woman Presenting as Generalized Choreoathetosis

Mondal

Deb

Mahata³

et al. 2023

The Neurohospitalist

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The persistence of measles virus infection in childhood and early adolescence can rarely lead to a fatal progressive neurodegenerative disorder known as subacute sclerosing panencephalitis (SSPE), characterized by behavioral disturbances and intellectual disability followed by myoclonic jerks and occasional negative myoclonus. Movement disorders are rarely presenting manifestations in SSPE. We herein report a 63-year-old woman with generalized choreoathetosis as the presenting manifestation of stage-I SSPE. Our case was atypical for the patient's age and clinical presentation with generalized choreoathetosis and bilateral putaminal and caudate nucleus signal hyperintensity. Though highly uncommon, neurologists should keep SSPE as a differential diagnosis among patients with movement disorders. Measles-endemic countries should be more vigilant to the atypical and rare presentations of SSPE, such as generalized choreoathetosis.

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Rapidly progressive dementia with generalized myoclonus in an adult: Do not forget subacute sclerosing panencephalitis

Cited by 2 publications

References 12 publications

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

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

Subacute Sclerosing Panencephalitis in a 63-Year-Old Woman Presenting as Generalized Choreoathetosis

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