Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014070 (encephalomyelitis)
 13,017 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cortical demyelinated lesions are frequent and widespread in chronic multiple sclerosis (MS) patients, and may contribute to disease progression. Inflammation and related oxidative stress have been proposed as central mediators of cortical damage, yet meningeal and cortical inflammation is not specific to MS, but also occurs in other diseases. The first aim of this study was to test whether cortical demyelination was specific for demyelinating CNS diseases compared to other CNS disorders with prominent meningeal and cortical inflammation. The second aim was to assess whether oxidative tissue damage was associated with the extent of neuroaxonal damage. We studied a large cohort of patients diagnosed with demyelinating CNS diseases and non-demyelinating diseases of autoimmune, infectious, neoplastic or metabolic origin affecting the meninges and the cortex. Included were patients with MS, acute disseminated encephalomyelitis (ADEM), neuromyelitis optica (NMO), viral and bacterial meningoencephalitis, progressive multifocal leukoencephalopathy (PML), subacute sclerosing panencephalitis (SSPE), carcinomatous and lymphomatous meningitis and metabolic disorders such as extrapontine myelinolysis, thus encompassing a wide range of adaptive and innate cytokine signatures. Using myelin protein immunohistochemistry, we found cortical demyelination in MS, ADEM, PML and extrapontine myelinolysis, whereby each condition showed a disease-specific histopathological pattern. Remarkably, extensive ribbon-like subpial demyelination was only observed in MS, thus providing an important pathogenetic and diagnostic cue. Cortical oxidative injury was detected in both demyelinating and non-demyelinating CNS disorders. Our data demonstrate that meningeal and cortical inflammation alone accompanied by oxidative stress are not sufficient to generate the extensive subpial cortical demyelination found in MS, but require other MS-specific factors.
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PMID:Extensive subpial cortical demyelination is specific to multiple sclerosis. 3191 98

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an autoimmune neurological disorder associated with the presence of anti-GFAP IgG. Meningoencephalitis is the predominant clinical presentation of autoimmune GFAP astrocytopathy in published case series. We report a case of autoimmune GFAP astrocytopathy with the unusual feature of radiculoneuritis in addition to encephalomyelitis, resulting in flaccid paralysis unresponsive to immunotherapy. Imaging data confirmed involvement of brain, spinal cord and nerve roots. Electrodiagnostic testing showed changes consistent with a severe sensorimotor neuropathy with active denervation. The results of this case suggest the need for future studies to assess the impact of peripheral nerve involvement on the outcome of autoimmune GFAP astrocytopathy.
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PMID:Autoimmune glial fibrillary acidic protein astrocytopathy resulting in treatment-refractory flaccid paralysis. 3192 53

Chikungunya, a viral infection that presents with fever, rash and polyarthritis, is usually an acute febrile illness. Uncommon neurological manifestations include meningoencephalitis, encephalitis, myelitis, Guillain-Barre syndrome, myelopathy and neuropathy. During an outbreak of the disease in La Reunion Island, abnormalities were observed in the magnetic resonance imaging (MRI) of patients with encephalitis and acute disseminated encephalomyelitis, showing bilateral, frontoparietal, white matter lesions with restricted diffusion, similar to our case. We report a 57-year-old male patient with comorbidities, admitted with high fever, arthralgia, asthenia, vomiting, psychomotor agitation, behavioral changes and seizures. Cerebrospinal fluid (CSF) values revealed pleocytosis (98 cells/mm3 with 68% lymphocytes and 12% monocytes) and high levels of protein (161 mg%). Brain MRI showed hyperintense lesions in the temporal and frontal lobes and bilaterally in the posterior thalamus. CSF serology was positive for IgM antibodies to Chikungunya virus. Encephalitis due to an acute viral infection by Chikungunya was diagnosed. The patient's clinical condition worsened and he died on the twenty-fourth day of admission to our hospital.
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PMID:Chikungunya encephalitis: report of a fatal case in Northeastern Brazil. 3257 26

Glial fibrillary acidic protein is a recently identified rare cause of autoimmune encephalomyelitis, in which the cerebrospinal fluid shows lymphocytic pleocytosis accompanied by linear perivascular radial gadolinium enhancement in the brain. We report a 19-year-old man admitted to the intensive care unit with suspected viral meningoencephalitis. Magnetic resonance imaging showed mild encephalopathy with a reversible splenial lesion. He quickly developed a coma and acute respiratory failure. Glial fibrillary acidic protein antibodies and human parainfluenza virus were detected by cerebrospinal fluid exams. He was treated with intravenous immunoglobulin, methylprednisolone pulses, plasma exchange and then six infusions of cyclophosphamide plus two of rituximab, which resulted in a total recovery.
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PMID:Severe anti-GFAP meningo-encephalomyelitis following viral infection. 3282 72

An outbreak of corona virus disease 2019 (COVID-19) began in China in December 2019, and rapidly spread to become a worldwide pandemic. Neurological complications encountered in hospitalized patients include acute arterial ischemic cerebrovascular stroke, cerebral venous thrombosis, critical illness-associated cerebral microbleeds, hypertensive hemorrhagic posterior reversible encephalopathy, meningoencephalitis/flare up of infections, flare up of multiple sclerosis, acute disseminated encephalomyelitis, cerebral hemodynamic/hypoxic changes such as watershed ischemic changes and hypoxic ischemic encephalopathy, and spine manifestations of Guillain Barre syndrome and viral myelitis. The purpose of our study is to illustrate the different neuroimaging features in critically ill hospitalized COVID-19 positive patients in the State of Qatar.
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PMID:Spectrum of neuroimaging findings in COVID-19. 3330 96


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