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

• Published in: Internal Medicine Vol. 63; no. 2; pp. 333 - 336
• Main Authors: Uchigami, Hirokazu, Hamada, Masashi, Maekawa, Hirotaka, Ishiura, Hiroyuki, Kodama, Satoshi, Shirota, Yuichiro, Takahashi, Miwako, Momose, Toshimitsu, Toda, Tatsushi
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 15.01.2024; Japan Science and Technology Agency
• Subjects: Blood flow; Case Report; Cerebrovascular Circulation; Computed tomography; Consciousness; Dementia - complications; Dementia disorders; disturbance of consciousness; encephalitic episode; Encephalitis - complications; Humans; Intranuclear Inclusion Bodies; Neurodegenerative Diseases - complications; Neurodegenerative Diseases - diagnostic imaging; neuronal intranuclear inclusion disease; single-photon emission computed tomography; encephalitic episode; disturbance of consciousness; single-photon emission computed tomography; neuronal intranuclear inclusion disease
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.1015-22

Encephalitic 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.