Progressive Multifocal Leukoencephalopathy: Electron Microscope Study of Four Cases

Woodhouse, M. A.; Dayan, A. D.; Burston, J.; Caldwell, I.W.; Adams, J. Hume; Melcher, D.H.; Urich, H.

doi:10.1093/brain/90.4.863

Cited by 38 publications

(8 citation statements)

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“…Another case is cited (Brain and Wilkinson, 1965) of a male patient, aged 40 years, with cerebellar dysfunction, parkinsonian facies and tremor associated with bronchial carcinoma; death occurred after 14 months and the neuropathological findings included the typical loss of Purkinje cells with astrocytosis of the cerebellar cortex and demyelination and perivascular lacunae affecting the globus pallidus. A further case, a male aged 45 years, developed parkinsonian facies, tremor and other neurological features 11 years after the onset of lymphoma; death occurred after 2 months, and demyelination and microglial proliferation involving the basal ganglia, and demyelination with loss of Purkinje cells and astrocytosis of the cerebellum were found (Woodhouse et al, 1967). In the present case, despite the clinical features of extrapyramidal disease, the basal ganglia were histologically normal.…”

Section: Discussionmentioning

confidence: 41%

“…The older immunological literature contains references to cross-reactivity between components of brain, other tissues, and corpus luteum, testicle and kidney (Wilkinson, 1964;Wilkinson and Zeromski, 1965) and, in the midsixties, complement-fixing autoantibodies to brain tissue in serum and CSF were observed in patients with paraneoplastic neurological diseases (Wilkinson, 1964). These autoantibodies were present only in patients with sensory carcinomatous neuropathy (Wilkinson and Zeromski, 1965), and further studies suggested reactivity with a neuronal antigen which was organ-specific but not speciesspecific, and was probably a protein present in the microsome-rich fraction of brain homogenates (Zeromski and Wilkinson, 1966). Moreover, experimental studies (Coates and Carnegie, 1975;Flavell et al, 1979) in guinea-pigs have shown that injection of acid extracts of cancer tissue may induce cell-mediated immune responses to basic protein of myelin, demonstrable by in vitro assays and cutaneous reactivity.…”

Section: Discussionmentioning

confidence: 99%

“…In one detailed case study (Smith, Gonda and Malamud, 1958) including postmortem on a male aged 63 years, severe degeneration and cell loss from the cerebellum and its centrifugal connections together with demyelination, cell loss and gliosis of the corpus Luysi and globus pallidus in the extra-pyramidal system were found; there was no family history of cerebellar ataxia nor was tumour apparent at post-mortem. Another case is cited (Brain and Wilkinson, 1965) (Woodhouse et al, 1967 (Brain and Wilkinson, 1965) (Paone and Jeyasingham, 1980). The aetiology of the paraneoplastic neurological syndromes remains unknown, but several mechanisms, not mutually exclusive, have been suggested (Joynt, 1974;Henson, 1970).…”

Section: Discussionmentioning

confidence: 99%

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Cerebellar cortical degeneration with ovarian carcinoma

Steven

Mackay

Carnegie

et al. 1982

Postgraduate Medical Journal

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A case is described of progressive cerebellar degeneration in association with ovarian carcinoma, with nearly 4 years elapsing between the onset of neurological illness and the discovery of the carcinoma. Neither treatment with prednisolone nor surgical removal of the tumour effected any improvement, CAT scanning late in the illness showed marked cerebellar atrophy. Histology at post-mortem showed complete loss of cerebellar Purkinje cells. The cerebrospinal fluid gave weak binding to multiple neural components in a solid-phase radio-immuno-assay.

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Section: Discussionmentioning

confidence: 41%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Cerebellar cortical degeneration with ovarian carcinoma

Steven

Mackay

Carnegie

et al. 1982

Postgraduate Medical Journal

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“…PML, which was first described as an entity by Astrom et a1 (19), is associated with papovavirus infection (20,21), usually of the JC type (5,20) but occasionally SV40-like virus (22)(23)(24). The majority of patients affected by the disease are immunocompromised either by primary diseases such as lymphoma, leukemia, tuberculosis, sarcoidosis, and acquired immunodeficiency syndrome or by immunosuppressive therapy.…”

Section: Discussionmentioning

confidence: 99%

Progressive multifocal leukoencephalopathy complicating systemic lupus erythematosus

Newton

Aldridge

Lessells

et al. 1986

Arthritis & Rheumatism

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Two patients with systemic lupus grythematosus who developed progressive multifocal leukoencephalopathy (PML) are described. Although the time of PML onset could not be determined with certainty, it probably developed after steroid-induced immunosuppression. However, high-dose corticosteroids appeared to produce transient amelioration in the neurologic disorder. We suggest that PML is an important addition to the list of conditions to be considered in the differential diagnosis when a patient with systemic lupus erythematosus develops neurologic abnormalities.The neurologic manifestations of systemic lupus erythematosus (SLE) are well-recognized, though their pathologic basis is still poorly understood. Various pathogenetic mechanisms have been suggested, including cerebral vascuhtis and antineuronal antibodies (1) and, recently, anticardialipin antibodies (2). There are no accepted criteria for the diagnosis of cerebral lupus and, consequently, no agreement among investigators regarding its incidence, which may be as high as 75% of patients at some stage in the illness (3,4). CASE REPORTSPatient 1. In 1960, an anemic 36-year-old woman with a history of Raynaud's phenomenon since childhood was found to have a renal calculus associated with hypercalciuria, impaired urinary concentration, and a mild compensated renal tubular acidosis. The Westergren erythrocyte sedimentation rate (ESR) was 120 mm/hour, serum gamma globulin levels were increased, and a test for antinuclear factor (ANF) was weakly positive.In 1965, during the sixteenth week of her second pregnancy, she developed acidosis and a "butterfly" facial eruption; lupus erythematosus (LE) cell preparations and ANF were strongly positive. On medical advice she submitted to pregnancy termination by hysterectomy, after which her metabolic disturbances reverted to normal levels and her facial eruption regressed.In 1971, she complained of visual disturbance accompanied by left retroorbital pain and was found to have left eye visual acuity of 6/18 with a central scotoma. Optic atrophy was identified, and the episode was attributed to retrobulbar neuritis. In addition to the cutaneous facial changes typical of SLE, the skin of her hands manifested a bound-down appearance suggestive of early sclerodactyly. She had an ESR of 12 mm/hour, a normal value for creatinine clearance, and a normal chest radiograph. LE cells were identified, ANF and homogeneous antibodies were strongly positive. Mitochondria1 and smooth muscle antibodies and the Wassermann reaction were negative. Serum gamma globulins were increased. There was a mild but persistent proteinuria, and renal biopsy showed histo-

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“…From 1958 to 2004, a total of 46 patients (M/F 30/ 15, median age 54 years, range 23-79 years) with a definitive diagnosis of LPD-associated PML were reported in the literature [2,. Twenty-two of them (47.8%) were assigned to group A [2,[11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] and 24 (52.2%), to group B . The number of published cases of PML associated with LPDs began to increase after 1997, when purine analogues became widely used ( Fig.…”

Section: Demographic and Clinical Characteristicsmentioning

confidence: 99%

Changes in the natural history of progressive multifocal leukoencephalopathy in HIV-negative lymphoproliferative disorders: Impact of novel therapies

et al. 2005

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The aims of this study were to evaluate the clinical characteristics of HIV-negative patients affected by lymphoproliferative disorders (LPD) who developed progressive multifocal leukoencephalopathy (PML), to delineate the risk factors, and to analyze whether the new antineoplastic therapies are changing the natural history of this infectious disease. We retrospectively analyzed 46 cases with confirmed LPD-associated PML published from 1958 to 2004. Patients were stratified according to two different time periods: group A included patients diagnosed before 1989, and group B included patients diagnosed since 1990, after introduction of purine analogues. Group A patients (n = 22) had received alkylating agents and/or radiotherapy, and the majority (63.6%) had advanced Hodgkin disease. At univariate analysis, uncontrolled Hodgkin disease was the only risk factor for PML. In group B patients (n = 24), the most frequent treatments received were purine analogues (58.3%) and high-dose therapy with hematopoietic stem cell transplantation (33.3%; HDT/HSCT). B-cell chronic lymphocytic leukemia (45.8%) and aggressive non-Hodgkin lymphoma (24.9%) were the most frequent underlying LPDs. Patients treated with purine analogues were more likely to have active LPD, lower CD4 cell counts, and to be older and male than were HSCT recipients. The median interval from purine analogues or HDT/HSCT to PML was 11 months. In HDT/HSCT recipients, this interval was delayed for 10 months when peri-transplantation rituximab was used. Univariate analysis identified age >55 years, male sex, and CD4 cell counts £0.2 · 10 9 /L as risk factors for PML in patients treated with purine analogues. Mortality rates were 95.4% (group A patients), 90% (purine analogues), and 62.5% (HDT/HSCT recipients). At univariate analysis, the only factor that significantly correlated with recovery from infection was female sex. Our findings indicate (1) the possible reduction in reported cases associated with Hodgkin disease and the increasing number of published cases associated with the new antineoplastic therapies (purine analogues and HDT/ HSCT); (2) among patients treated with purine analogues, PML is more common in male patients with CD4 cell counts £0.2 · 10 9 /L; (3) the use of rituximab after HDT/ HSCT seems to delay the onset of PML; and (4) the prognosis is slightly better in transplant recipients. Am.

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Progressive Multifocal Leukoencephalopathy: Electron Microscope Study of Four Cases

Cited by 38 publications

References 0 publications

Cerebellar cortical degeneration with ovarian carcinoma

Cerebellar cortical degeneration with ovarian carcinoma

Progressive multifocal leukoencephalopathy complicating systemic lupus erythematosus

Changes in the natural history of progressive multifocal leukoencephalopathy in HIV-negative lymphoproliferative disorders: Impact of novel therapies

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