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

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 cons...

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Bibliographic Details
Published in:Internal Medicine 2024/01/15, Vol.63(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: Article
Language:English
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
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Summary: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.
ISSN:0918-2918
1349-7235
DOI:10.2169/internalmedicine.1015-22