Inflammatory progressive multifocal leukoencephalopathy with human T-cell lymphotropic virus-1 coinfection. 2024

Sachiko Hasebe, and Kota Maekawa, and Yukiko Shishido-Hara, and Kazuo Nakamichi, and Nobuaki Funata, and Makio Takahashi
Department of Neurology, Kyoto University Graduate School of Medicine Faculty of Medicine, Kyoto, Japan sachiko_h@kuhp.kyoto-u.ac.jp.

A middle-aged man with progressive multifocal leukoencephalopathy (PML) in a human T-cell lymphotropic virus type-1 (HTLV-1) carrier on haemodialysis presented with mild dysarthria and ataxia. Brain MRI revealed asymmetric T2-hyperintense lesions in the cerebral white matter, cerebellum and brainstem. A small amount of JC virus (JCV) genome in cerebrospinal fluid was detected by PCR and cerebellar biopsy demonstrated JCV-DNA presence. Pathological findings showed demyelinating lesions and glial cells with mildly enlarged nuclei, accompanied by T-lymphocytes, neutrophils and plasma cell infiltration. The CD4+/CD8+ratio was 0.83. High-dose corticosteroid therapy was effective for inflammatory PML lesions, and the administration of mefloquine combined with mirtazapine led to favourable outcome. The encephalitis in this case is considered to have occurred secondarily to JCV infection in the presence of HTLV-1 infection. Therefore, it is crucial to investigate the presence of HTLV-1 in order to understand the aetiology of this brain inflammation.

UI MeSH Term Description Entries
D007577 JC Virus A species of POLYOMAVIRUS, originally isolated from the brain of a patient with progressive multifocal leukoencephalopathy. The patient's initials J.C. gave the virus its name. Infection is not accompanied by any apparent illness but serious demyelinating disease can appear later, probably following reactivation of latent virus. Human Polyomavirus JC,JC polyomavirus,Polyomavirus, JC,John Cunningham Virus,Polyomavirus hominis 2,Polyomavirus JC, Human,Virus, John Cunningham
D007968 Leukoencephalopathy, Progressive Multifocal An opportunistic viral infection of the central nervous system associated with conditions that impair cell-mediated immunity (e.g., ACQUIRED IMMUNODEFICIENCY SYNDROME and other IMMUNOLOGIC DEFICIENCY SYNDROMES; HEMATOLOGIC NEOPLASMS; IMMUNOSUPPRESSION; and COLLAGEN DISEASES). The causative organism is JC Polyomavirus (JC VIRUS) which primarily affects oligodendrocytes, resulting in multiple areas of demyelination. Clinical manifestations include DEMENTIA; ATAXIA; visual disturbances; and other focal neurologic deficits, generally progressing to a vegetative state within 6 months. (From Joynt, Clinical Neurology, 1996, Ch26, pp36-7) Encephalitis, JC Polyomavirus,Progressive Multifocal Leukoencephalopathy,JC Polyomavirus Encephalopathy,Encephalopathies, JC Polyomavirus,Encephalopathy, JC Polyomavirus,JC Polyomavirus Encephalitis,Leukoencephalopathies, Progressive Multifocal,Multifocal Leukoencephalopathies, Progressive,Multifocal Leukoencephalopathy, Progressive,Progressive Multifocal Leukoencephalopathies
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000078785 Mirtazapine A piperazinoazepine tetracyclic compound that enhances the release of NOREPINEPHRINE and SEROTONIN through blockage of presynaptic ALPHA-2 ADRENERGIC RECEPTORS. It also blocks both 5-HT2 and 5-HT3 serotonin receptors and is a potent HISTAMINE H1 RECEPTOR antagonist. It is used for the treatment of depression, and may also be useful for the treatment of anxiety disorders. (N-Methyl-11C)mirtazapine,(S)-Mirtazapine,6-Azamianserin,Esmirtazapine,Norset,ORG 3770,ORG-3770,Org 50081,Remergil,Remeron,Rexer,Zispin,6 Azamianserin,ORG3770
D015368 Human T-lymphotropic virus 1 A strain of PRIMATE T-LYMPHOTROPIC VIRUS 1 isolated from mature T4 cells in patients with T-lymphoproliferation malignancies. It causes adult T-cell leukemia (LEUKEMIA-LYMPHOMA, T-CELL, ACUTE, HTLV-I-ASSOCIATED), T-cell lymphoma (LYMPHOMA, T-CELL), and is involved in mycosis fungoides, SEZARY SYNDROME and tropical spastic paraparesis (PARAPARESIS, TROPICAL SPASTIC). ATLV,Adult T-Cell Leukemia-Lymphoma Virus I,HTLV-1,HTLV-I,Human T-Cell Leukemia Virus I,Leukemia Virus I, Human T-Cell,T-Cell Leukemia Virus I, Human,Adult T Cell Leukemia Lymphoma Virus I,Human T Cell Leukemia Virus I,Leukemia Lymphoma Virus I, Adult T Cell,Leukemia Virus I, Human T Cell,T Cell Leukemia Virus I, Human,Human T lymphotropic virus 1
D015490 HTLV-I Infections Diseases caused by HUMAN T-LYMPHOTROPIC VIRUS 1. Human T-lymphotropic Virus 1 Infection,Human T-lymphotropic Virus 1 Infections,Infections, HTLV-I,HTLV I Infections,HTLV-I Infection,Human T lymphotropic Virus 1 Infection,Human T lymphotropic Virus 1 Infections,Infection, HTLV-I,Infections, HTLV I
D015767 Mefloquine A phospholipid-interacting antimalarial drug (ANTIMALARIALS). It is very effective against PLASMODIUM FALCIPARUM with very few side effects. Lariam,Mefloquine Hydrochloride,Mephloquine,Ro-21-5998-001,WR-142,490,WR-177,602,Ro 21 5998 001,Ro215998001,WR 142,490,WR 177,602,WR142,490,WR177,602

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