Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Antibodies against a glutamate receptor in the central nervous system are reported to be an important autoimmune factor in infantile or childhood refractory epilepsy and in adult-onset paraneoplasstic cerebellar ataxia. We here reported a patient with glutamate receptor antibodies-positive elderly-onset intractable epilepsy and advanced-stage stomach cancer. A 77-year-old man with a history of resected bladder cancer suddenly developed an attack of epilepsia partialis continua in the right arm, followed by a generalized epileptic status. The ictal EEG showed spikes in the left parietal region with diffuse slow wave backgrounds. The brain MRI demonstrated a focal high signal intensity in the left postcentral gyrus on the T2-weighted and FLAIR images. The SPECT showed a high uptake in the focal area corresponding to the MRI lesion, suggesting a seizure focus. The routine analysis in the CSF was normal, but the glutamate receptor antibodies (IgG, IgM and C-terminal) were all positive. The status epilepticus was refractory to anticonvalsants, and the barbiturate anesthesia was introduced. Subsequently, the epilepsy has been fairly controlled by valproic acid and phenobarbital. The post ictal MRI demonstrated a focal atrophy in the left postcentral gyrus. The systemic evaluations revealed an advanced-stage stomach cancer. The glutamate receptor antibodies may play a role in late-onset refractory epilepsy, and may be an autoimmune factor in paraneoplstic neurologic syndrome presenting with epilepsy.
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PMID:[Intrathecal glutamate receptor antibodies in a patient with elderly-onset refractory epilepsy]. 1450 54

Onconeural antibodies are associated with cancer and paraneoplastic encephalitis. While their pathogenic role is still largely unknown, their high diagnostic value is undisputed. In this study we describe the discovery of a novel target of autoimmunity in an index case of paraneoplastic encephalitis associated with urogenital cancer.A 75-year-old man with a history of invasive bladder carcinoma 6 years ago with multiple recurrences and a newly discovered renal cell carcinoma presented with seizures and progressive cognitive decline followed by super-refractory status epilepticus. Clinical and ancillary findings including brain biopsy suggested paraneoplastic encephalitis. Immunohistochemistry of the brain biopsy was used to characterize the inflammatory response. Indirect immunofluorescence assay (IFA) was used for autoantibody screening. The autoantigen was identified by histo-immunoprecipitation and mass spectrometry and was validated by expressing the recombinant antigen in HEK293 cells and neutralization tests. Sera from 125 control patients were screened using IFA to test for the novel autoantibodies.IFA analysis of serum revealed a novel autoantibody against brain tissue. An intracellular enzyme, Rho-associated protein kinase 2 (ROCK2), was identified as target-antigen. ROCK2 was expressed in affected brain tissue and archival bladder tumor samples of this patient. Brain histopathology revealed appositions of cytotoxic CD8+ T cells on ROCK2-positive neurons. ROCK2 antibodies were not found in the sera of 20 patients with bladder cancer and 17 with renal cancer, both without neurological symptoms, 49 healthy controls, and 39 patients with other antineuronal autoantibodies. In conclusion, novel onconeural antibodies targeting ROCK2 are associated with paraneoplastic encephalitis and should be screened for when paraneoplastic neurological syndromes, especially in patients with urogenital cancers, occur.
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PMID:Rho-associated protein kinase 2 (ROCK2): a new target of autoimmunity in paraneoplastic encephalitis. 2855 30