• Internal medicine · Jan 2024

    Recovery after Prolonged Disturbance of Consciousness and Repeated Cerebral Perfusion Changes in Neuronal Intranuclear Inclusion Disease.

    • Hirokazu Uchigami, Masashi Hamada, Hirotaka Maekawa, Hiroyuki Ishiura, Satoshi Kodama, Yuichiro Shirota, Miwako Takahashi, Toshimitsu Momose, and Tatsushi Toda.
    • Department of Neurology, Graduate School of Medicine, The University of Tokyo, Japan.
    • Intern. Med. 2024 Jan 15; 63 (2): 333336333-336.

    AbstractEncephalitic episodes are a clinical manifestation of neuronal intranuclear inclusion disease (NIID) and often show transient disturbance of consciousness. We herein report a genetically confirmed patient with NIID who initially presented progressive dementia and showed prolonged disturbance of consciousness preceded by an acute-onset headache. During that time, we performed N-isopropyl-p-[123I] iodoamphetamine single-photon-emission computed tomography twice and found that the blood flow increased in different regions. Prolonged disturbance of consciousness following an encephalitic episode may be associated with repeated hyperperfusion in various regions resulting from mitochondrial dysfunction. NIID patients presenting with encephalitic episodes can recover gradually and spontaneously even after prolonged disturbances of consciousness.

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