Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Progressive multifocal leukoencephalopathy of the posterior fossa in an AIDS patient: clinical, radiographic and evoked potential findings. 322 20

Atypical forms of progressive multifocal leukoencephalopathy (PML) may simulate other disorders. A previously healthy 70-year-old female developed unsteadiness of gait, dysarthria, dementia and weakness leading to inanition and death from bronchopneumonia over a 43 month period. The diagnosis of PML was not suspected prior to death. Neuropathologic examination of the brain disclosed characteristic findings of PML-deep bilateral cerebral demyelinative foci with enlarged gemistocytic astrocytes and swollen oligodendrocytes containing intranuclear inclusions. Electron microscopy identified papova virus particles within these inclusions. An underlying source of immunosuppression was not identified either premortem nor at the time of autopsy. The prolonged clinical course, simulating that of a primary degenerative disease, and the lack of apparent immunocompromise are unusual features of PML and lend credence to the suggestions that variations in its expression and course are to be expected.
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PMID:Prolonged progressive multifocal leukoencephalopathy without immunosuppression. 369 Apr 32

We reviewed the clinical, radiographic, and pathologic features of 15 patients with the acquired immune deficiency syndrome (AIDS) and progressive multifocal leukoencephalopathy (PML). Brain tissue from 10 autopsy and 6 biopsy specimens was studied using: in situ hybridization (ISH) for JC virus (JCV), immunohistochemistry for human immunodeficiency virus (HIV) p24 antigen, and electron microscopy. Thirteen patients presented with focal neurologic deficits, while 2 presented with a rapid decline in mental status. PML was commonly the initial opportunistic infection of AIDS and produced hemiparesis, dementia, dysarthria, cerebellar abnormalities, and seizures. Magnetic resonance imaging was more sensitive than computed tomography in detecting lesions, and often showed multifocal areas of PML. CD4+ T-cell counts were uniformly low (mean 84/mm3), except in 1 patient who improved on 3'-azido-3'-deoxythymidine (AZT). PML involved the cerebral hemispheres, brain stem, cerebellum, and cervical spinal cord. The distribution of brain involvement was consistent with hematogenous dissemination of the virus. In 2 brain specimens, multiple HIV-type giant cells were present within the regions involved by PML. When co-infection by HIV and papovavirus was present, PML dominated the pathological picture. ISH for JCV showed virus in the nuclei of oligodendrocytes and astrocytes. Occasionally there was staining for JCV in the cytoplasm of glial cells and in the neuropil, the latter possibly a correlate of papovavirus spread between myelin sheaths, as seen by electron microscopy. ISH demonstrated more extensive foci of PML than did routine light microscopy.
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PMID:Progressive multifocal leukoencephalopathy in AIDS: a clinicopathologic study and review of the literature. 841 79

Cerebellar disorders associated with HIV infection are typically the result of discrete cerebellar lesions resulting from opportunistic infections such as toxoplasmosis and progressive multifocal leukoencephalopathy or primary CNS lymphoma. Clinical symptoms and pathologic abnormalities related to the cerebellum may also be observed with HIV dementia. A primary cerebellar degeneration with HIV has not previously been reported. Ten patients were identified over an 8-year period at five medical centers. All patients had clinical, laboratory, and radiologic evaluations, and three had neuropathologic examinations. Patients presented with progressively unsteady gait, slurred speech, and limb clumsiness. Examination revealed gait ataxia, impaired limb coordination, dysarthria, and abnormal eye movements. Cognition, strength, and sensory function remained normal. CD4 lymphocyte counts varied between 10 and 437 cells/mm3. Neuroimaging studies showed prominent cerebellar atrophy. Neuropathology showed focal degeneration of the cerebellar granular cell layer and unusual focal axonal swellings in the brainstem and spinal cord. Cultures, histopathology, and immunochemical studies showed no conclusive evidence of infection. We report a syndrome of unexplained degeneration of the cerebellum occurring in association with HIV infection.
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PMID:Cerebellar degeneration associated with human immunodeficiency virus infection. 1069 Oct 12

We experienced 8 cases of progressive multifocal leukoencephalopathy (PML) complicated by human immunodeficiency virus (HIV) type-1 infection from 1985 to 1999. These cases showed dementia, bradykinesia, dysarthria, hemiparesis, and so on. All of the cases were severely immunocompromised hosts, because none had more than 150/mm3 CD4 + lymphocytes; indeed, 5 of the cases were below 20/mm3. Other neurological complications except PML were primary CNS lymphoma, HIV encephalitis, and CMV encephalitis. The mean life durations was 7.6 months after the first symptom appeared, for all but one of the patients; the exceptional patient lived for 24 months after. Autopsy studies of the central nervous systems were performed for 7 cases, all of which showed extensive demyelinating lesions of the white matter, and in some cases these extended into the spinal cord. In contrast to Western countries, in Japan there have been few reports of AIDS-associated PML. Thus, this report was thought to be important here.
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PMID:[Clinical investigation of the 8 cases with AIDS (acquired immunodeficiency syndrome)-associated progressive multifocal leukoencephalopathy (PML)]. 1578 5

A 44-year-old woman presented with dysarthria, visual disturbances, ataxia and cognitive impairment. There was a rapid progression of her neurological disease, and she died 8 months later. She was previously treated for a low-grade follicular B-cell lymphoma; complete remission was achieved by conventional radiotherapy and chemotherapy, including rituximab. Two years later, the neurological symptoms and signs started. MRI revealed a cerebral demyelinating process. Serology was negative. Autopsy disclosed areas in cerebral white matter with grey discoloration. Microscopy revealed demyelination, oligodendroglial viral inclusions and gliosis with bizarre astrocytes. Polymerase chain reaction (PCR) was positive for JC virus. These findings were consistent with progressive multifocal leukoencephalopathy (PML). This is one of recent reports on PML occurring in a patient treated with the anti-20 monoclonal antibody rituximab.
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PMID:Progressive multifocal leukoencephalopathy in a lymphoma patient with complete remission after treatment with cytostatics and rituximab: case report and review of the literature. 1741 5

We describe a patient with a giant MS plaque developing during natalizumab administration whose clinical presentations mimicked PML. This 27year-old man developed new onset confusion, altered behavior, left hemianesthesia and worsening dysarthria, gait and balance with a new, large, rim-enhancing, temporoparietal subcortical lesion after four infusions of natalizumab. Cerebrospinal JC virus polymerase chain reaction was negative. No neutralizing antibody to natalizumab was detected. Following discontinuation of natalizumab, plasma exchange, and a single dose of 1000mg of methylprednisolone, he demonstrated clinical and radiographic improvement. Distinguishing PML from MS may be very difficult in some instances. The frequency with which an aggressive demyelinating disorder due to MS occurs during treatment with natalizumab remains to be determined.
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PMID:A giant MS plaque mimicking PML during natalizumab treatment. 2014 66

The symptoms associated with progressive multifocal leukoencephalopathy (PML) reflect the location of pathologic brain lesions. These symptoms include visual deficits, cognitive impairment, and motor weakness; in patients with acquired immunodeficiency syndrome (AIDS), presenting signs can also include gait disturbance, dysarthria, dysphasia, and ocular palsy. Recently, PML has been observed in patients treated with biologic agents; natalizumab recipients currently represent the second largest group of patients with PML (behind patients with AIDS). Although brain biopsy is the most accurate and reliable method for diagnosing PML, it is rarely used today. Diagnosis is usually based on detection of JC virus in the cerebrospinal fluid by polymerase chain reaction, the clinical presentation, and demonstration of PML brain lesions on magnetic resonance imaging. With immune reconstitution, the prognosis of PML has improved markedly.
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PMID:The clinical features of PML. 2212 35

A 69-year-old Caucasian woman with a 15-year history of refractory chronic lymphocytic B-cell leukaemia (CLL), treated with alemtuzumab in the past 10 months presented with a subacute right foot drop. Initial evaluation with a brain CT scan, lumbosacral MRI, nerve conduction studies and LP was negative. In the following months, progressive right hemibody weakness and dysarthria developed. Brain MRI showed a bilateral parasagittal frontal lesion. Alemtuzumab treatment was withdrawn. Progressive multifocal leukoencephalopathy (PML) was confirmed by PCR. Attempted antiviral therapies proved fruitless. Inexorable clinical deterioration ensued and the patient passed away 10 months after the presentation. This case report intends to call attention for PML as a potential fatal complication of severe immunosuppression, including the possible role of new monoclonal antibodies (such as alemtuzumab) in its pathogenesis.
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PMID:Progressive multifocal leukoencephalopathy in a patient with chronic lymphocytic leukaemia treated with alemtuzumab. 2440 83

Progressive multifocal leukoencephalopathy is a rare, demyelinating disease of the central nervous system caused by JC virus. Fewer than 30 cases have been reported in HIV- and non-infected children. We report the case of a 15-year-old girl with progressive multifocal leukoencephalopathy and AIDS who presented with nystagmus, dysarthria and ataxia. Following combined antiretroviral therapy, she developed immune reconstitution inflammatory syndrome, which proved fatal.
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PMID:Progressive multifocal leukoencephalopathy in pediatric patients: case report and literature review. 2463 69


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