Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The January Case of the Month (COM). A 56 year-old female presented with a 5-month history of dizziness, focal motor seizures and progressive immobility. CT scan showed a large frontal tumor with a meningioma-like pattern. The patient underwent right frontal craniotomy and complete resection of the tumor. Biopsy showed lymphoid infiltrates consisting predominantly of centrocyte-like cells admixed with plasma cells and lymphoid follicles. Immunohistochemistry revealed CD20-positive cells and light chain restriction with reactive T cells. These appearances are characteristic of the MALT-type lymphomas. There was no evidence of systemic lymphoma. The patient is symptom-free 18 months after complete resection. MALT lymphoma is rare but recognized subtype of primary meningeal lymphoma. MALT lymphoma of dura tends to be relatively indolent, with excellent prognosis. It is suggested that meningeal MALT-type lymphoma may follow on from a pre-existing inflammatory process.
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PMID:January 2003: 56-year-old female with right frontal tumor of the dura. 1294 31

A 47-year-old Hispanic male presented with visual field disturbances, memory impairment, and a seizure. CT and MRI were consistent with meningioma. Both neurologic exam and routine laboratory tests were within normal limits. The patient underwent craniotomy and subtotal resection of the tumor. On H&E, the lesion was composed of a lymphoid mass with well-defined irregularly shaped follicles surrounded by a monomorphic population of small lymphocytes. Marginal zones stained for B-cell markers, CD20 and CD79a, one T-cell marker, CD43, and kappa light chains. While other markers did not stain the majority of tumor cells, they did identify other lymphoid and plasma cell elements. A diagnosis of marginal zone B-cell lymphoma of dura, mucosa-associated lymphoid tissue (MALT)-type (extranodal) was made. MALT-type lymphomas are unusual in the nervous system; this is the first such case reported in a male and serves to emphasize the wide diversity of presentation of a neoplasm originally described in the GI tract and thus far described in the CNS only in females.
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PMID:Primary dural mucosa-associated lymphoid tissue-type lymphoma: case report and review of the literature. 1517 17

We report a case of extranodal marginal zone B-cell lymphoma (MZL) mimicking meningioma in a lateral ventricle and suggest its possible pathogenesis. The patient was a 63-year-old male with seizure attack. MRI showed a 3.5x1.7 cm sized homogeneously enhancing mass in the trigon of the right lateral ventricle, possibly arising from the choroid plexus. The provisional diagnosis was meningioma or metastasis. In the surgical findings, the mass was attached to the choroid plexus and totally removed. Pathologic examination revealed a monotonous population of medium-sized mononuclear lymphoma cells associated with meningothelial whorl formation and neoplastic cells which invaded the meningothelial cells. The tumor was immunopositive for CD79a, CD20, CD43 and bcl-2. We suggest that intracranial extranodal marginal zone B-cell lymphomas might mimic meningiomas, because of their relation with the arachnoid cells and that meningothelial cells serve as a substitute for the mucosal surface in intracranial MALT lymphomas.
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PMID:Extranodal marginal zone B-cell lymphoma mimicking meningioma in lateral ventricle: a case report and possible pathogenesis. 1662 74

In a 64-year-old woman presenting with rapidly progressive dementia, brain magnetic resonance imaging revealed a diffuse leukoencephalopathy without gadolinium enhancement, and the 14.3.3 protein was found to be positive in the cerebrospinal fluid. An electroencephalogram showed diffused slow waves and epileptic seizures without periodic paroxysmal activity. The patient died 3 months after onset of symptoms, and an autopsy restricted to the brain was performed. Gross examination was not informative, and only microscopic examination permitted identification of scattered lymphomatous cells on all sections from the brain hemispheres, brain stems and cerebellum. Immunopositivity of these tumor cells for CD20 attested their B phenotype. This observation illustrates "lymphomatosis cerebri," a recently described entity, which has to be considered in the differential diagnosis of rapidly progressive dementia in adults.
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PMID:A 64-year-old woman with progressive dementia and leukoencephalopathy. 1749 46

An acute to chronic idiopathic necrotizing meningoencephalitis was diagnosed in 5 Chihuahua dogs aged between 1.5 and 10 years. Presenting neurologic signs included seizures, blindness, mentation changes, and postural deficits occurring from 5 days to 5.5 months prior to presentation. Cerebrospinal fluid analyses from 2 of 3 dogs sampled were consistent with an inflammatory disease. Magnetic resonance imaging of the brain of 2 dogs demonstrated multifocal loss or collapse of cortical gray/white matter demarcation hypointense on T1-weighted images, with T2-weighted hyperintensity and slight postcontrast enhancement. Multifocal asymmetrical areas of necrosis or collapse in both gray and white matter of the cerebral hemispheres was seen grossly in 4 brains. Microscopically in all dogs, there was a severe, asymmetrical, intensely cellular, nonsuppurative meningoencephalitis usually with cystic necrosis in subcortical white matter. There were no lesions in the mesencephalon or metencephalon except in 1 dog. Immunophenotyping defined populations of CD3, CD11d, CD18, CD20, CD45, CD45 RA, and CD79a immunoreactive inflammatory cells varying in density and location but common to acute and chronic lesions. In fresh frozen lesions, both CD1b,c and CD11c immunoreactive dendritic antigen-presenting cells were also identified. Immunoreactivity for canine distemper viral (CDV) antigen was negative in all dogs. The clinical signs, distribution pattern, and histologic type of lesions bear close similarities to necrotizing meningoencephalitis as described in series of both Pug and Maltese breed dogs and less commonly in other breeds.
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PMID:Necrotizing meningoencephalitis in five Chihuahua dogs. 1848 90

Aseptic pachymeningitis is a rare and serious complication of rheumatoid arthritis (RA). Herein, we describe a patient with rheumatoid factor-positive and anti-cyclic citrullinated peptide-positive RA who experienced a focal seizure, with aphasia and convulsions of the right side of the body. The findings of magnetic resonance imaging and histologic analysis led to a diagnosis of rheumatoid pachymeningitis. Because the patient had a large number of CD20-expressing B lymphocytes, therapy with rituximab was started and has resulted in complete and sustained remission of both the pachymeningitis and the RA for >2 years. Despite a decrease in immunoglobulins, the patient has remained free of infections, which illustrates the favorable outcome that can result from therapeutic B cell depletion in this potentially lethal manifestation of RA.
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PMID:Induction of complete and sustained remission of rheumatoid pachymeningitis by rituximab. 1947 65

We report four cases of biopsy-proven B-cell-rich primary angiitis of the central nervous system (PACNS). The mean age of the patients was 29 years (range, 23-37 years). The patients suffered from unilateral weakness (n = 2), seizure (n = 1), and hypersomnia, anorexia and confusion (n = 1). The vital signs and the results of laboratory tests were within normal limits in all the four cases except erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). ESR was elevated in one patient and CRP was elevated in two patients. The magnetic resonance imaging (MRI) scans revealed single (n = 2) or multiple (n = 2) irregularly enhancing lesions. Radiological studies initially indicated tumors such as glioma (n = 2) or lymphoma (n = 1), except in one case, in which the radiological analysis indicated vasculitis or demyelinating disease. All the cases involved both medium-sized (50-250 microm in diameter) and small-sized vessels (20-49 microm in diameter). The vascular, perivascular and parenchymal lymphocytes were polymorphous; however, CD20-positive B-cells were predominated in blood vessels while the CD8-positive T-cells infiltrated predominantly in brain parenchyma. Therefore, our patients revealed B-cell dominant lymphocytic vasculitis. Two patients who underwent active treatment (corticosteroid alone or with cyclophosphamide) showed remarkable clinical and radiological improvement but two patients still have initial neurological symptoms, namely, confusion and newly developed seizures, respectively, during the 19-101-month follow-up periods; this effect can be attributed to irreversible brain damage. Therefore, although early brain biopsy may be associated with histopathologic diagnostic pitfalls, it is a mandatory procedure for obtaining a confirmative diagnosis as well initiating early therapy, thereby reducing brain damage.
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PMID:B-cell dominant lymphocytic primary angiitis of the central nervous system: four biopsy-proven cases. 1973 59

Rasmussen encephalitis (RE) is characterized by chronic inflammation of one cerebral hemisphere which causes intractable epileptic seizures and progressive neurological deficits. Since antiepileptic pharmacotherapy is often ineffective the traditional therapy for Rasmussen encephalitis is hemispherectomy in one of its modern variants which renders the patient seizure free but leads to a severe deficit. To escape this dilemma, immunomodulatory therapeutic approaches such as rituximab, a monoclonal anti-CD20 antibody, offer an alternative and bear promising therapeutic potentials in Rasmussen encephalitis.
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PMID:New hope for Rasmussen encephalitis? 1983 59

A 55-year-old woman presented with an extremely rare primary central nervous system mucosa-associated lymphoid tissue (MALT) lymphoma manifesting as seizure and was subsequently diagnosed with dural MALT lymphoma in the cranium. Magnetic resonance imaging revealed a left frontal dural mass with peritumoral edema. Histological examination of the dural mass confirmed MALT lymphoma and revealed infiltration of small cells with irregular nuclear borders and expression of a B cell marker (CD20) but absence of CD5, CD10, CD23, and cyclinD1. Reactive T-cell infiltration was also seen. Subsequently, local irradiation (40 Gy/20 fractions) was performed. Magnetic resonance imaging showed complete remission just after irradiation was completed. There was no evidence of systemic MALT lymphoma. There has been no recurrence for 3 years without additional therapy.
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PMID:Primary central nervous system mucosa-associated lymphoid tissue lymphoma--case report. 2178 50

Rheumatoid arthritis is well known for multiple extra-articular manifestations. Here, we present a case of chronic rheumatoid meningitis occurring during treatment with methotrexate and the tumour necrosis factor (TNF) alpha antibody adalimumab. Nine and seven months, respectively, into the course of these two treatments, a 59-year-old Caucasian lady with mild, early, seropositive rheumatoid arthritis developed headaches and psychomotor retardation followed by seizures. The diagnosis was confirmed by a brain biopsy showing a necrotizing granulomatous meningitis. Withdrawal of both drugs and high dose corticosteroids led to marked improvement. The addition of the anti-CD20 antibody rituximab allowed discontinuation of the corticosteroids. This is the fifth published case describing the occurrence of rheumatoid meningitis during treatment with TNF blockers. TNF blockers and methotrexate thus do not appear to prevent this complication, and may even contribute to its development.
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PMID:Rheumatoid meningitis occurring during adalimumab and methotrexate treatment. 2196 88


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