Department of Neurology, Graduate School of Medicine, The University of Tokyo, Japan.
Department of Clinical Laboratory, The University of Tokyo Hospital, Japan.
Intern Med. 2024 Jan 15;63(2):333-336. doi: 10.2169/internalmedicine.1015-22. Epub 2023 May 31.
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-[I] 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.
脑炎发作是神经元核内包涵体病（NIID）的一种临床表现，常表现为意识一过性障碍。本文报道了一例经基因确诊的 NIID 患者，其首发症状为进行性痴呆，且意识障碍呈迁延性，发病前有急性发作性头痛。在此期间，我们进行了两次 N-异丙基-p-[I]碘代安非他命单光子发射计算机断层扫描，发现不同区域的血流量增加。脑炎发作后继发迁延性意识障碍可能与线粒体功能障碍导致的不同区域反复高灌注有关。NIID 患者即使出现迁延性意识障碍，也可逐渐自发地恢复。
