A 64‐year‐old man presenting MRIs of expansive multiple high intensity areas in the cerebral white matter, cerebellum and brainstem

Takeda, Shingo; Yamazaki, Kazunori; Miyakawa, Takashi; Takahashi, Hitoshi; Ikuta, Fusahiro; Arai, Hiroyuki

doi:10.1111/j.1440-1789.2007.00814.x

Cited by 3 publications

(2 citation statements)

References 5 publications

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“…A highly intense area can usually be seen on T2-weighted MRI. 16 There is no known effective cure for PML but, in some cases, the disease slows or stops if the patient's immune system improves; some AIDS patients with PML have been able to survive for several years with the advent of highly active antiretroviral therapy (HAART). 17 Clinical physicians should consider PML in chronic psychiatric patients who are likely to develop opportunistic infection.…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological study of early progressive multifocal leukoencephalopathy incidentally found in a schizophrenia patient

et al. 2009

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A 58-year-old Japanese man developed psychomotor excitement and hallucinatory paranoia at age 53, which gradually developed to residual schizophrenia. He was administered various common tranquilizers until death. Myelodysplastic syndrome was noted 10 months before death. A routine autopsy was performed. The brain weighed 1365 g, and macroscopic observation revealed no remarkable findings. However, microscopic examination disclosed cells with enlarged and basophilic nuclei, and unusual astrocytes in the demyelinated foci, especially at the corticomedullary junctions in the temporal and occipital lobes. On the other hand, the white matter was relatively intact. Immunohistochemical analysis using anti-JC virus protein, VP-1 antibody, demonstrated JC virus-infected cells in not only abnormal glial cells and neurons but also normal-looking cells, which are suggestive of progressive multifocal leukoencephalopathy (PML). Immunostaining for GFAP revealed severe gliosis and some scattered abnormal enlarged nuclear cells in the lesions. Some clusters of CD8-positive lymphocytes were seen, which kill infected cells. PML could be considered a short-term disease preceding death, as "incidental PML" in this case. This is a rare autopsy case of early PML occurring in a schizophrenia patient with PML.

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Section: Discussionmentioning

confidence: 99%

Clinicopathological study of early progressive multifocal leukoencephalopathy incidentally found in a schizophrenia patient

et al. 2009

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“…Such widespread spinal cord involvement has been reported only rarely. Part of the clinical course and neuropathological findings of this case has been described elsewhere 6–8 …”

Section: Introductionmentioning

confidence: 99%

Progressive multifocal leukoencephalopathy showing extensive spinal cord involvement in a patient with lymphocytopenia

Takeda¹,

Yamazaki²,

Miyakawa³

et al. 2009

Neuropathology

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A 64-year-old Japanese man who was diagnosed as having cerebral infarcts at an early clinical stage was found to have progressive multifocal leukoencephalopathy (PML). A decrease of leukocytes and lymphocytes had been detected in the previous year. During a total clinical course of 11 months, he showed marked depletion of lymphocytes ranging from 264/microL to 459/microL. Autopsy disclosed no underlying diseases such as malignancies or tuberculosis. Extensive PML lesions were seen in the cerebral white matter. Small perivascular cuffs comprising many CD8+ T lymphocytes and a few CD4+ T cells were scattered in the PML lesions. CD20+ B cells were rarely evident. The subsets of the infiltrating lymphocytes differed from those of primary or spontaneous PML. Similar extensive PML lesions were observed not only in the cerebellum and brainstem but also in the spinal cord. All 26 segments of the spinal cord, especially the cervical, lumbar and sacral cord, showed extensive lesions involving the lateral and anterior columns. To our knowledge, only three cases of PML with such extensive spinal cord lesions have been reported previously. These three cases, and the present one, may represent a group of PML that shows extensive lesions in the spinal cord as well as the cerebrum, cerebellum and brainstem. The underlying disease in the present case was unclear. Because lymphocytopenia is not observed in primary or spontaneous PML, and the immunohistochemical findings of the infiltrating lymphocytes in the present case are different from primary or spontaneous PML, the decrease in his total blood lymphocytes may have played a significant role in his immunosuppressed condition as the underlying disease.

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Update on PML: Lessons from the HIV uninfected and new insights in pathogenesis and treatment

Hartman¹,

Huang

2008

Curr HIV/AIDS Rep

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Significant advances in our understanding of progressive multifocal leukoencephalopathy (PML) and its causative agent, JC virus, have been made since PML was first described 50 years ago. However, immune reconstitution remains the only proven, effective therapy in this devastating central nervous system disorder. Early diagnosis and adjustments of immune suppressants and modulator agents are critical in managing PML in HIV-negative patients. This review summarizes recent advances in our understanding of PML in HIV-uninfected patients in oncology, rheumatology, organ transplantation, and idiopathic immune deficiency and in association with novel therapeutics. Brain MRI data from our case series of brain biopsy-proven HIV-negative PML patients indicate the presence of an inflammatory/immune reaction in brain tissues, which was confirmed by immunocytologic analysis. Future studies to better understand PML pathogenesis in HIV-negative individuals may help uncover new potential therapeutic targets and improve PML outcomes.

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A 64‐year‐old man presenting MRIs of expansive multiple high intensity areas in the cerebral white matter, cerebellum and brainstem

Cited by 3 publications

References 5 publications

Clinicopathological study of early progressive multifocal leukoencephalopathy incidentally found in a schizophrenia patient

Clinicopathological study of early progressive multifocal leukoencephalopathy incidentally found in a schizophrenia patient

Progressive multifocal leukoencephalopathy showing extensive spinal cord involvement in a patient with lymphocytopenia

Update on PML: Lessons from the HIV uninfected and new insights in pathogenesis and treatment

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