• Internal medicine · Jun 2023

    Case Reports

    A Case of Acute Reversible Encephalopathy with Neuronal Intranuclear Inclusion Disease Diagnosed by a Brain Biopsy: Inferring the Mechanism of Encephalopathy from Radiological and Histological Findings.

    • Azusa Orihara, Natsuki Miyakoshi, Yoko Sunami, Hideki Kimura, Yasuhiro Nakata, Takashi Komori, Jun Sone, and Kazushi Takahashi.
    • Department of General Internal Medicine, Tokyo Metropolitan Tama Medical Center, Japan.
    • Intern. Med. 2023 Jun 15; 62 (12): 182118251821-1825.

    AbstractA 75-year-old man presented with headache and disturbance of consciousness. Magnetic resonance imaging revealed edema localized mainly in the cortex and linear contrast enhancement. A brain biopsy revealed numerous astrocytes with inclusion, and genetic testing demonstrated prolonged GGC repeats in NOTCH2NLC. The present case provided two novel insights into the mechanism underlying encephalopathy associated with neuronal intranuclear inclusion disease. First, the histological findings at a site with contrast enhancement on magnetic resonance imaging did not demonstrate any organic association, such as the presence of inflammation or ischemic changes. Second, the imaging and cerebrospinal fluid findings demonstrated increased cerebral blood flow and opening of the blood-brain barrier, indicating the cause of the cerebral swelling.

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