• Internal medicine · Sep 2024

    Cryptogenic New-onset Refractory Status Epilepticus with Hyperintensity of T1-weighted Magnetic Resonance Imaging in the Bilateral Basal Ganglia: An Autopsy Report.

    • Sunao Takahashi, Daisuke Ono, Hiroshi Shintaku, Jun Oyama, Yoichiro Nishida, Kinya Ishikawa, and Takanori Yokota.
    • Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Japan.
    • Intern. Med. 2024 Sep 27.

    AbstractWe present a 76-year-old man with cryptogenic new-onset refractory status epilepticus (C-NORSE) with an initial abnormal signal in the nucleus accumbens and a remarkable hyperintense signal on T1-weighted magnetic resonance imaging in the bilateral basal ganglia (BG). His status epilepticus did not respond to most anti-epileptic therapies or immunotherapies, and he died of sepsis. An autopsy revealed severe neuronal loss and hypertrophic astrocytes in the BG and limbic system, with no signs of inflammation or malignancy. This case suggests that lesions in the BG may reflect secondary degeneration and predict poor outcomes in C-NORSE.

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