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Results for “Leukoencephalopathy, Progressive Multifocal”

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PubMed1984

Progressive multifocal leukoencephalopathy in a renal transplant recipient. Increased diagnostic sensitivity of computed tomographic scanning by double-dose contrast with delayed films.

A progressive, deteriorating neurologic disorder developed in a 28-year-old white man 10 years after he successfully received a living related donor kidney transplant. An extensive neurologic evaluation was unrevealing, including normal results of computed tomographic scanning of the brain with and without contrast medium. Repeated computed tomographic scanning after a double dose of radiocontrast medium in conjunction with delayed imaging revealed multiple areas of abnormal enhancement. This technique helped to direct brain biopsy, which led to the early diagnosis of progressive multifocal leukoencephalopathy and the institution of specific therapy.

Adult↗

PubMed2005

Successful cidofovir therapy of progressive multifocal leukoencephalopathy preceding angioimmunoblastic T-cell lymphoma.

Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating infectious disease, caused by the human polyomavirus JC (JCV), that usually occurs in immunocompromised patients. In this setting, PML has been observed in increasing numbers of patients with hematological malignancies, mostly lymphoproliferative B-cell disorders. Despite attempts with various drugs, PML has generally remained unresponsive to treatment. We report the successful use of cidofovir in a patient who developed PML 6 months before angioimmunoblastic T-cell lymphoma (AITL) was diagnosed. To the best of our knowledge, this is the first case of PML in AITL. Our case demonstrates the expanding clinical importance of PML in hematological conditions, and neurological symptoms and/or white matter changes on central nervous system imaging should arouse the suspicion of PML and lead to rapid cidofovir introduction. Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating infectious disease, caused by the human polyomavirus JC (JCV), that usually occurs in immunocompromised patients. In this setting, PML has been observed in increasing numbers of patients with hematological malignancies, mostly lymphoproliferative B-cell disorders. Despite attempts with various drugs, PML has generally remained unresponsive to treatment. We report the successful use of cidofovir in a patient who developed PML 6 months before angioimmunoblastic T-cell lymphoma (AITL) was diagnosed. To the best of our knowledge, this is the first case of PML in AITL. Our case demonstrates the expanding clinical importance of PML in hematological conditions, and neurological symptoms and/or white matter changes on central nervous system imaging should arouse the suspicion of PML and lead to rapid cidofovir introduction.

Cidofovir↗

PubMed1998

Survival prolongation in HIV-associated progressive multifocal leukoencephalopathy treated with alpha-interferon: an observational study.

UNLABELLED: A retrospective chart review was conducted to determine the effect of alpha-Interferon (alpha-IFN) on disease progression, symptom palliation, and survival in HIV-associated Progressive Multifocal Leukoencephalopathy (PML). METHODS: Subjects were HIV seropositive patients diagnosed with PML at the Johns Hopkins Hospital between 1985 and July of 1986. Diagnostic criteria for PML included both clinical symptomatology and histologic or radiographic confirmation. All patients with concomitant CNS infections were excluded. Patients receiving a minimum treatment of 3 weeks of 3 million units of alpha-IFN daily were compared to untreated historical controls. From 104 PML cases reviewed, 77 met the defined criteria for PML. Twenty-one patients had received open-label alpha-IFN treatment in a non-randomized manner for at least 3 weeks, and 32 met criteria for inclusion in the untreated group as historical controls. Deceased treated patients were comparable to deceased untreated patients with respect to age, gender, race, HIV risk factors, AIDS-defining illnesses, and CD4+ counts. CD4+ counts and use of anti-retroviral medications within 6 months of PML onset were higher among those who were living at the time of the study. RESULTS: Among deceased patients, median survival of treated patients was 127.5 days longer than that of untreated patients (Chi-square=4.21, P=0.04). When living and deceased treated patients were combined, the median survival was 325 days (range 35 - 1634) versus 121 days (range 46 - 176) in untreated patients (Chi-square=13.47, P < 0.001). When survival times in untreated patients were left-censored to account for possible survivorship bias in treated patients, survival in treated patients remained significantly prolonged (325 days versus 175.5 days, Chi-square=4.65, P=0.03). In addition, use of alpha-IFN was associated with a significant delay in the onset of memory loss (Chi-square=8.59, P < 0.01). Seven alpha-IFN treated patients showed sustained remissions of several months to over a year, with documented improvements in mental status, aphasia, dysarthria, dysphagia, paresis, and dyscoordination. Moreover, four IFN-treated patients had evidence of MRI lesion regression, although this was not always correlated with clinical remission. Four of 32 untreated patients also reported transient symptomatic improvements. CONCLUSION: This open-label study suggests that alpha-IFN may delay progression, palliate symptoms, and significantly prolong survival in HIV-associated PML, and we therefore suggest that a controlled clinical trial is warranted.

AIDS-Related Opportunistic Infections↗

PubMed2002

Progressive multifocal leukoencephalopathy in chronic lymphocytic leukemia after treatment with fludarabine.

A 65-year-old man with chronic lymphocytic leukemia (CLL) diagnosed 11 years ago and treated with standard dose of fludarabine developed a rapidly fatal progressive neurological syndrome. Differential diagnoses included brain infiltration by CLL as opposed to progressive multifocal leukoencephalopathy (PML). A magnetic resonance imaging (MRI) scan showed a hyperintense T2-weighted signal in the left frontal region. Cerebro-spinal fluid polymerase chain reaction (PCR) was positive for virus JC (JCV). These findings were compatible with the diagnosis of PML. Fludarabine has been used to treat acute leukemias, CLL and follicular lymphomas. Its toxicity includes myelosuppression, immunosuppression and sporadic life-threatening neurotoxicity, although standard doses of it are considered safe. Late-onset fatal cerebral dysfunction caused by JCV after standard-dose fludarabine has been described previously. The widespread and increasing use of fludarabine makes it interesting to define the potential of standard doses of fludarabine for causing severe neurological side-effects such as PML.

Aged↗

PubMed1998

Progressive multifocal leukoencephalopathy in a patient with hypogammaglobulinemia.

We describe a child with congenital hypogammaglobulinemia that was diagnosed at 13 months of age. When he was 4 years old, gait disturbances began. The main neurological manifestations were progressive spastic tetraparesis and intellectual and speech deterioration. No infectious agent was identified. A magnetic resonance imaging scan of the central nervous system revealed periventricular demyelinating areas in the frontal, temporal, and parietal lobes with cortical atrophy. Stereotactic brain biopsy confirmed the diagnosis of progressive multifocal leukoencephalopathy caused by JC virus. He was treated with intravenous and intraventricular cytarabine and interferon-alpha, and there was clinical improvement. We emphasize the need for brain biopsy as soon as a neurological complication is suspected in patients with congenital hypogammaglobulinemia for whom cerebrospinal cultures or polymerase chain reaction analyses are negative.

Agammaglobulinemia↗

PubMed2005

Progressive multifocal leukoencephalopathy: clinical description and demonstration of the causal agent.

A 40 year old man with AIDS had memory deficit and visual impairment. The brain magnetic resonance images showed abnormalities in the white matter not enhanced with gadolinium. JC-virus DNA was detected in the cerebrospinal fluid. He died after a few weeks of progressive neurologic deterioration. Histologic examination showed changes compatible with progressive multifocal leukoencephalopathy. Polyoma virus etiology was confirmed by immunostaining of brain tissue sections and electron microscopy of infected oligodendrocytes.

Acquired Immunodeficiency Syndrome↗

PubMed2006

Natalizumab and progressive multifocal leukoencephalopathy: migrating towards safe adhesion molecule therapy in multiple sclerosis.

Natalizumab, a humanized monoclonal antibody against alpha4beta1 integrin, was shown in clinical trials to dramatically reduce the relapse rate, development of new magnetic resonance imaging (MRI) lesions and progression of disability in patients with relapsing multiple sclerosis. Following its expedited approval, sales of the drug were discontinued owing to the emergence of two cases of progressive multifocal leukoencephalopathy (PML), a rare but deadly viral infection of the central nervous system (CNS) associated with immunosuppression. Owing to the effect of natalizumab on central nervous system leukocyte recruitment, the emergence of PML has been attributed to diminished immunosurveillance. The lack of additional opportunistic or CNS infections among natalizumab-treated patients, however, suggests that alternate mechanisms may contribute to the infectious risk. This review examines how the inhibition of alpha4beta1-mediated adhesion might establish a unique milieu for the development of PML and how future approaches to selective adhesion molecule therapy in multiple sclerosis might avoid a similar fate.

Antibodies, Monoclonal↗

PubMed1988

Progressive multifocal leukoencephalopathy of the posterior fossa in an AIDS patient: clinical, radiographic and evoked potential findings.

A 39-year-old intravenous drug user presented with dysarthria and a syndrome of the left cerebellar hemisphere. While in hospital, he developed progressive brainstem findings. Repeated CT scans revealed a lucency in the white matter of the left cerebellar hemisphere. Brainstem auditory and short-latency somatosensory evoked potentials provided evidence of brainstem dysfunction without corresponding lesions on CT. Biopsy of the cerebellum established the diagnosis of progressive multifocal leukoencephalopathy (PML) and the acquired immunodeficiency syndrome (AIDS). Postmortem examination revealed brainstem lesions appropriate to the evoked potential findings and a radiographically inapparent lesion in the right internal capsule. Based on this case and a review of the literature we conclude that: (1) PML occurs with significant prevalence in AIDS patients and may involve the posterior fossa; (2) the diagnosis of posterior fossa PML is suggested by certain clinical and radiographic criteria and may be confirmed by brain biopsy; (3) evoked potentials may be abnormal in PML and can reveal functional abnormalities of white matter without apparent CT abnormalities.

Acquired Immunodeficiency Syndrome↗

PubMed1998

Human immunodeficiency virus-related progressive multifocal leukoencephalopathy with a CD4+ of 444 per mm3: a case report.

A 47-year-old homosexual man with human immunodeficiency virus infection and CD4+ cell count of 444 per mm3, and with a viral load of 95,720 copies per mL, developed a rapidly progressive cerebellar lesion one month after undergoing coronary artery bypass graft. Diagnostic data were unremarkable with the exception of the magnetic resonance imaging that revealed multiple subcortical and cerebellar lesions consistent with a demyelinating process. The patient died within two months. Postmortem examination of the cerebellum revealed acute phase progressive multifocal leukoencephalopathy. This case demonstrates that multifocal leukoencephalopathy must be considered in HIV+ patients in the setting of neurologic deterioration with radiographic evidence of demyelination regardless of CD4+ count.

AIDS-Related Opportunistic Infections↗

PubMed1989

[Progressive multifocal leukoencephalopathy in Wegener's granulomatosis in relation to therapy with cyclosporin A].

A female patient with Wegener's granulomatosis developed severe bone marrow depression after two years treatment with cyclophosphamide. Corticosteroids alone could not sufficiently suppress disease activity, therefore additive therapy with Cyclosporin A was started. Four weeks later the patient developed a central nervous system disorder with affective disturbances and progressive somnolence. However, inspite of intensive diagnostic procedures, no definite diagnosis could be established. After another two months she died. Post-mortem-examination showed progressive multifocal leukoencephalopathy. An association between immunosuppressive therapy and reactivation of JC-Virus is suggested.

Brain↗

PubMed1987

[Progressive multifocal leukoencephalopathy. Late complication in chronic lymphatic leukemia].

In a 44 year old patient with chronic lymphatic leukemia and secondary antibody deficiency syndrome, a disorder of articulation and a left hemiparesis developed during a thrombocytopenic phase. Computer tomography of the cranium, CSF diagnostics and the electroencephalogram did not provide any indication for the cause of the rapidly progressive cerebral symptoms. In the NMR tomogram, a diffusedly increasing intensity in the T2-weighted tomograms were shown around the central region in the right brain. The patient died of a Pseudomonas septicemia. At autopsy, a typical finding of progressive multifocal leukoencephalopathy was found in the areas altered in the NMR tomography. Papova-like virions could be demonstrated in glial cells by electron microscopy.

Adult↗

PubMedDate not supplied

[Progressive multifocal leukoencephalopathy in elderly immunocompetent patients. Report of 2 cases].

INTRODUCTION: Progressive multifocal leukoencephalopathy is a disorder which is rare in immunocompetent patients. OBJECTIVES: We report the cases of two elderly patients with serology, in one case positive for hepatitis C, and in the other with anti-DNA antibodies, and discuss the part these might play in causing progressive multifocal leukoencephalopathy. CLINICAL CASES: Case 1. An 86 year old man had been found on serology investigations to be positive for hepatitis C virus. In November 1996 he complained of dysarthria and left hemi-negligence following an accidental fall. Since his clinical condition became worse he was admitted to hospital for further investigation. On neuroimaging studies the intracerebral lesions were increased. The only other finding confirmed was that of positive serology for hepatitis C virus. The patient deteriorated progressively and died 50 days after admission. Case 2. A 70 year old woman began to show progressive cognitive impairment and left hemiparesia in June 1996. Se was investigated in another centre and provisionally diagnosed as having vasculitis of the CNS, in view of her positive anti-DNA antibody and right frontoparietal hypodense lesion. Treatment had been started with corticosteroids. She was admitted to our hospital when her neurological deficits worsened. The immunological alterations were confirmed. On MRI the lesions in the white matter were seen to have progressed. The patient slowly improved. She was discharged from hospital in February 1997 in a semiconscious state, able to follow persons and things with her eyes, with global aphasia and with spastic tetraparesia which was mainly left-sided. She remains stable. CONCLUSION: Progressive multifocal leukoencephalopathy is a condition which should be remembered when dealing with immunocompetent patients.

Aged↗

PubMed1999

Progressive multifocal leukoencephalopathy after autologous bone marrow transplantation and alpha-interferon immunotherapy.

A patient with a stage IV mantle cell lymphoma (according to the REAL classification) was treated with high-dose chemotherapy and autologous bone marrow transplantation. One year later while on alpha-interferon immunotherapy she suffered from progressive loss of short-term memory and reported difficulties in recognizing objects. Magnetic resonance imaging (MRI) showed a vast ring-enhancing lesion of the left postcentral parietal area. Serial stereotactic biopsies disclosed progressive multifocal leukoencephalopathy without JC-virus in the cerebrospinal fluid. Therapy with subcutaneous interleukin-2 (IL-2) every other day and intrathecal cytarabine once a week was started. After 4 weeks the patient refused further treatment. Nevertheless her condition improved over the next 8 months and MRI scans showed a marked improvement in the lesions.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1984

Progressive multifocal leukoencephalopathy presenting as a single mass lesion.

Progressive multifocal leukoencephalopathy (PML) consisting anatomopathologically of multiple foci of white matter demyelination may present both clinically and at CT scanning as a single mass lesion. An additional similar case is reported. As antiviral therapy may prove useful, characteristics of additional examinations including CT scan are described to promote an early diagnosis.

Brain↗

PubMed1996

Progressive multifocal leukoencephalopathy: clinical and MR response to treatment.

Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease that occurs in immunocompromised hosts. We describe two patients with biopsy-proved PML who showed improvement clinically and radiologically after medical therapy. These cases reveal that interval improvement can in rare instances be consistent with a diagnosis of PML.

Adult↗

PubMed2006

Long-term remission in progressive multifocal leukoencephalopathy caused by idiopathic CD4+ T lymphocytopenia: a case report.

Progressive multifocal leukoencephalopathy is caused by JC virus, an opportunistic infection of the central nervous system. Antiretroviral treatment for progressive multifocal leukoencephalopathy in human immunodeficiency virus-infected patients is beneficial, but few data exist for patients who are not infected with human immunodeficiency virus. Idiopathic CD4+ T lymphocytopenia excludes human immunodeficiency virus infection. We describe a patient with progressive multifocal leukoencephalopathy with underlying idiopathic CD4+ T lymphocytopenia in whom functional recovery occurred without antiviral therapy.

Adult↗

PubMed2000

[Progressive multifocal leukoencephalopathy and pulmonary sarcoidosis].

An association of progressive multifocal leukoencephalopathy (PML) and sarcoidosis in a 47 year-old-woman is reported. This is the third case in which PML has been diagnosed by PCR. Clinical, biological and radiological features were in agreement with previous findings in immunologically suppressed patients. JC virus should be systematically detected by PCR in blood and cerebrospinal fluid (CSF) in patients with sarcoidosis presenting neurological and radiological PML manifestations.

Anti-Inflammatory Agents↗

PubMedDate not supplied

Progressive multifocal leukoencephalopathy: unusual MR findings.

A case of progressive multifocal leukoencephalopathy (PML) with a classic clinical presentation but with unusual pathological and radiographic findings is reported. The pathology revealed evidence of prior hemorrhage, and imaging studies revealed focal cerebral atrophy as well as contrast enhancement on MR scans. The contrast enhancement was visible only by utilizing magnetization transfer pulses on T1-weighted scans. The case report indicates that image criteria for PML may need to be redefined in the future.

Brain↗