Kon Tomoya, Funamizu Yukihisa, Suzuki Chieko, Sato Tsugumi, Kurotaki Hidekachi, Kurihara Aiichiro, Kurose Akira, Wakabayashi Koichi, Tomiyama Masahiko
Department of Neurology, Aomori Prefectural Central Hospital, Japan.
Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Japan.
Intern Med. 2019 May 15;58(10):1485-1489. doi: 10.2169/internalmedicine.1667-18. Epub 2018 Nov 19.
Primary central nervous system vasculitis (PCNSV) is an uncommon vasculitis restricted to the small- and medium-sized vessels in the brain and spinal cord. Previously, only 9 cases have been reported that initially manifested as an isolated spinal cord lesion with subsequent brain involvement, where the longest interval from the onset to brain involvement was 1 year and 11 months. We herein report the case of an isolated spinal cord lesion with subsequent brain involvement appearing seven years and five months later. This case shows that brain lesions can develop after an extended interval from spinal onset in PCNSV.
原发性中枢神经系统血管炎（PCNSV）是一种罕见的血管炎，局限于脑和脊髓的中小血管。此前，仅有9例报告最初表现为孤立的脊髓病变，随后累及脑部，从发病到脑部受累的最长间隔为1年11个月。我们在此报告1例孤立的脊髓病变，7年5个月后出现脑部受累的病例。该病例表明，在PCNSV中，脑部病变可在脊髓发病后的较长间隔时间后出现。
