Rinsho Shinkeigaku (Clinical Neurology)

Symposium 20

Progressive multifocal leukoencephalopathyiPMLj

Hidehiro Mizusawa, M.D., Ph.D.1), Shuji Kishida, M.D., Ph.D.2), Masayuki Saijo, M.D., Ph.D.3), Motohiro Yukishita, M.D., Ph.D.4), Yukiko Shishido-Hara, M.D., Ph.D.5), Hirohumi Sawa, M.D., Ph.D.6), Kazuo Nagashima, M.D., Ph.D.7), Souichi Nukuzuma, Ph.D.8) and Masahito Yamada, M.D., Ph.D.9)

1)Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Graduate School of Medical and Dental Sciences
2)Department of Neurology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital
3)Department of Virology, National Institute of Infectious Diseases
4)Division of Neurology, Department of Internal Medicine, Faculty of Medicine, Saga University
5)Department of Pathology, Kyorin University School of Medicine
6)Division of Molecular Pathobiology, Research Center for Zoonosis Control, Global COE Program, Hokkaido University
7)Department of Pathology, Sapporo Higashi-Tokushukai Hospital
8)Department of Microbiology, Kobe Institute of Health
9)Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science

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.
Full Text of this Article in Japanese PDF (803K)

(CLINICA NEUROL, 51: 1051|1057, 2011)
key words: Progressive multifocal leukoencephalopathy (PML), JC virus (JCV), Human immunodeficiency virus (HIV), Natalizumab, Mefloquine

(Received: 20-May-11)