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It is considered the most common clinical manifestation of John Cunningham virus JC virus infection in the brain 7 , and is seen in three clinical contexts:. Progressive multifocal leukoencephalopathy is strongly associated with immunosuppressed states, and primary PML developing in an immunocompetent patient is very rare.
Patients with progressive multifocal leukoencephalopathy present with various neurological symptoms. It typically spares the optic nerve and the spinal cord. The most frequently encountered symptoms include:. Lesions tend to have a confluent, bilateral but asymmetrical supratentorial white matter and thalamic involvement 7.
However, basal ganglia, brainstem and cerebellum also can be involved. Subcortical frontal and parieto-occipital regions are common locations. However, isolated posterior fossa involvement has also been reported Histology reveals demyelinating plaques involving the white matter and subcortical U-fibers. Other findings include infected oligodendrocytes with enlarged amphophilic nuclei located at the periphery of the lesions, macrophages containing phagocytosed cellular debris and myelin, and reactive gliosis with enlarged astrocytes Asymmetric focal zones of low attenuation involving the periventricular and subcortical white matter.
This is in contradistinction to more symmetrical hypoattenuation seen in HIV encephalopathy. Typically seen as multifocal, asymmetric periventricular and subcortical involvement. There is little, or no mass effect or enhancement 10 and the subcortical U-fibers are commonly involved with a predilection for the parieto-occipital regions In fact, even though subcortical U-fiber involvement is an important feature of lesions of multiple sclerosis, these are relatively uncommon and if new lesions have a subcortical U-fiber distribution, this favors PML Corpus callosum may be involved If untreated, PML is usually fatal within one year, often within 2 to 6 months Some reports also state some benefit with cytarabine or mirtazapine, the latter especially in natalizumab-associated PML.
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Progressive multifocal leukoencephalopathy
Gonzalo Bustamante F. Among patients infected with HIV has also been described the lysis of the granullar cells of the cerebellum and cerebellar atrophy, attributed to a variant of the JCV. We present 37 years old HIV infected men, with postural dizziness, followed by gait disturbances, and a cerebellar syndrome, scanned speech, hyperreflexia, pendular reflexes, Babinski sign and mild cognitive impairment were present. Brain MRI showed hyperintense areas of the white matter in the cerebral hemispheres, thalamus and brainstem, associated with incipient atrophy of the cerebellum.
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It is considered the most common clinical manifestation of John Cunningham virus JC virus infection in the brain 7 , and is seen in three clinical contexts:. Progressive multifocal leukoencephalopathy is strongly associated with immunosuppressed states, and primary PML developing in an immunocompetent patient is very rare. Patients with progressive multifocal leukoencephalopathy present with various neurological symptoms. It typically spares the optic nerve and the spinal cord. The most frequently encountered symptoms include:. Lesions tend to have a confluent, bilateral but asymmetrical supratentorial white matter and thalamic involvement 7.
Progressive multifocal leukoencephalopathy PML is a rare and often fatal viral disease characterized by progressive damage -pathy or inflammation of the white matter leuko- of the brain -encephalo- at multiple locations multifocal. It is caused by the JC virus , which is normally present and kept under control by the immune system. The JC virus is harmless except in cases of weakened immune systems. PML occurs almost exclusively in patients with severe immune deficiency , most commonly among patients with acquired immune deficiency syndrome AIDS , but people on chronic immunosuppressive medications including chemotherapy are also at increased risk of PML, such as patients with transplants, Hodgkin's lymphoma , multiple sclerosis , psoriasis , and other autoimmune diseases.
Aim: To analyse the clinical findings, complementary examinations and prognosis of patients with progressive multifocal leukoencephalopathy PML treated in our institution, comparing populations with and without associated human immunodeficiency virus HIV. Patients and methods: A retrospective study of the medical records of patients with probable or definite PML was carried out. Clinical variables, complementary studies cerebrospinal fluid, magnetic resonance imaging of the brain and prognostic variables were analysed. Results: Fourteen patients with definite and one probable diagnosis of PML were included.