Mai Hamaguchi1, Keisuke Suzuki2, Hiroaki Fujita1, Takeo Uzuka3, Hadzki Matsuda3, Yukiko Shishido-Hara4, Satoko Arai5, Toshiki Nakamura6, Shigeru Kikuchi3, Kazuo Nakamichi7, Masayuki Saijo7, Koichi Hirata1. 1. Department of Neurology, Dokkyo Medical University, Shimotsuga, 321-0293, Tochigi, Japan. 2. Department of Neurology, Dokkyo Medical University, Shimotsuga, 321-0293, Tochigi, Japan. keisuke@dokkyomed.ac.jp. 3. Department of Neurosurgery, Dokkyo Medical University, Shimotsuga, Japan. 4. Department of Pathology and Applied Neurobiology, Kyoto Prefectural University of Medicine, Kyoto, Japan. 5. Department of Rheumatology, Dokkyo Medical University, Shimotsuga, Japan. 6. Department of Neurology, Rehabilitation Amakusa Hospital, Saitama, Japan. 7. Department of Virology 1, National Institute of Infectious Diseases, Tokyo, Japan.
Abstract
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a subacute onset demyelinating disease caused by JC virus and characterized by multifocal involvement of the subcortical white matter and cerebellar hemispheres or peduncles on magnetic resonance imaging (MRI). However, non-HIV PML patients with brain lesions limited to the cerebellum and brainstem have not been well characterized. METHODS: We report a 68-year-old man with systemic lupus erythematosus under treatment with immunosuppressants who developed non-HIV PML with brain lesions limited to the cerebellum and brainstem and successfully treated with a combination of mefloquine and mirtazapine. We performed a literature review to characterize patients with non-HIV PML with brain lesions limited to the cerebellum and brainstem. RESULTS: Eight cases with non-HIV brainstem/cerebellar form PML were identified including our case. All cases had compromised status related underlying diseases. Four (50%) had a good prognosis. Five cases were treated, including 3 with favourable outcomes. Between the good prognosis group (n = 4) and the poor prognosis group (n = 4), treatment status for PML and the interval between the initial manifestation and diagnosis did not differ. Among those who performed contrast-enhanced brain imaging, lesion enhancement was related to good prognosis (good prognosis group vs. poor prognosis group; 100% vs. 0%). CONCLUSION: PML should be considered in the differential diagnosis of brain lesions limited to the cerebellum and brainstem in immunocompromised patients. The presence of immune response against JC virus and inflammatory reactions may indicate good prognosis in non-HIV brainstem/cerebellar form PML.
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a subacute onset demyelinating disease caused by JC virus and characterized by multifocal involvement of the subcortical white matter and cerebellar hemispheres or peduncles on magnetic resonance imaging (MRI). However, non-HIV PML patients with brain lesions limited to the cerebellum and brainstem have not been well characterized. METHODS: We report a 68-year-old man with systemic lupus erythematosus under treatment with immunosuppressants who developed non-HIV PML with brain lesions limited to the cerebellum and brainstem and successfully treated with a combination of mefloquine and mirtazapine. We performed a literature review to characterize patients with non-HIV PML with brain lesions limited to the cerebellum and brainstem. RESULTS: Eight cases with non-HIV brainstem/cerebellar form PML were identified including our case. All cases had compromised status related underlying diseases. Four (50%) had a good prognosis. Five cases were treated, including 3 with favourable outcomes. Between the good prognosis group (n = 4) and the poor prognosis group (n = 4), treatment status for PML and the interval between the initial manifestation and diagnosis did not differ. Among those who performed contrast-enhanced brain imaging, lesion enhancement was related to good prognosis (good prognosis group vs. poor prognosis group; 100% vs. 0%). CONCLUSION: PML should be considered in the differential diagnosis of brain lesions limited to the cerebellum and brainstem in immunocompromised patients. The presence of immune response against JC virus and inflammatory reactions may indicate good prognosis in non-HIV brainstem/cerebellar form PML.
Authors: Antônio José da Rocha; Ingrid Aguiar Littig; Renato Hoffmann Nunes; Charles Peter Tilbery Journal: Arq Neuropsiquiatr Date: 2013-09 Impact factor: 1.420
Authors: M A Rueger; H Miletic; K Dorries; C Wyen; C Eggers; M Deckert; G Faetkenheuer; A H Jacobs Journal: Clin Infect Dis Date: 2006-02-13 Impact factor: 9.079