PMID- 22277475 OWN - NLM STAT- MEDLINE DCOM- 20120918 LR - 20190918 IS - 1882-0654 (Electronic) IS - 0009-918X (Linking) VI - 51 IP - 11 DP - 2011 Nov TI - [Progressive multifocal leukoencephalopathy (PML)]. PG - 1051-7 AB - Progressive multifocal leukoencephalopathy (PML) is caused by reactivation of latently infected JCV when hosts' immune system is impaired by HIV infection, hematologic diseases, collagen diseases, immunemodulatory therapy and so on. PML was rare but HIV infection and Natalizumab have made it much more common while the prognosis is much better than other PML. PML patients present with various signs and symptoms including hemiparesis, dementia, aphasia, visual disturbance, cranial nerve paresis, cerebellar signs and bladder bowel disturbance. Brain MRI reveals characteristic demyelinating lesions in the CNS white matter and CSF mild increase of protein with or without mild mononuclear pleocytosis. Detection of JCV genome from CSF is crucial for the clinical diagnosis of PML. PML was once thought to be fatal but some HIV infected PML patients showed halting progression or even recovery after introduction of HAART. In addition, anti-malarial drug mefloquine was found to be effective. Recovery of immunity may provoke some inflammatory responses known as immune reconstruction inflammatory syndrome (IRIS) which requires high dose corticosteroid. In Japan, we are providing free test of CSF-JCV genome and organized a unique system for surveillance and clinical research of PML. Using this system we hope to improve diagnosis and therapy of PML in Japan. FAU - Mizusawa, Hidehiro AU - Mizusawa H AD - Department of Neurology, Tokyo Medical and Dental University, Graduate School of Medical and Dental Sciences. FAU - Kishida, Shuji AU - Kishida S FAU - Saijo, Masayuki AU - Saijo M FAU - Yukishita, Motohiro AU - Yukishita M FAU - Shishido-Hara, Yukiko AU - Shishido-Hara Y FAU - Sawa, Hirohumi AU - Sawa H FAU - Nagashima, Kazuo AU - Nagashima K FAU - Nukuzuma, Souichi AU - Nukuzuma S FAU - Yamada, Masahito AU - Yamada M LA - jpn PT - English Abstract PT - Journal Article PL - Japan TA - Rinsho Shinkeigaku JT - Rinsho shinkeigaku = Clinical neurology JID - 0417466 SB - IM MH - Humans MH - *Leukoencephalopathy, Progressive Multifocal/drug therapy/pathology EDAT- 2012/01/27 06:00 MHDA- 2012/09/19 06:00 CRDT- 2012/01/27 06:00 PHST- 2012/01/27 06:00 [entrez] PHST- 2012/01/27 06:00 [pubmed] PHST- 2012/09/19 06:00 [medline] AID - JST.JSTAGE/clinicalneurol/51.1051 [pii] AID - 10.5692/clinicalneurol.51.1051 [doi] PST - ppublish SO - Rinsho Shinkeigaku. 2011 Nov;51(11):1051-7. doi: 10.5692/clinicalneurol.51.1051.