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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Strokes in children and adolescents, although rare, are serious when they occur. A practical clinical approach is to differentiate between those events that occur in the presence of systemic disease and those that are restricted conditions involving just the intracranial circulation. Division on the basis of the presence or absence of intracranial hemorrhage further narrows down the differential diagnosis and dictates the urgency with which angiography should be performed. Treatment depends on the underlying condition and on the development of seizures, increased intracranial pressure, or subarachnoid hemorrhage. Prognostic statements are difficult and must be highly individualized.
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PMID:Strokes in children and adolescents. 64 11

Certain diseases do not consistently affect a single area of the nervous system and, in fact, may cause concomitant multifocal involvement. Depending on the area of the nervous system affected, these diseases may cause a wide spectrum of neurologic problems and therefore must be included in the differential diagnosis for most problems discussed previously. Diseases causing multifocal lesions should especially be suspected when there are concomitant neurologic deficits that cannot be localized to a single site. An example would be a cat with seizures and paraparesis without thoracic limb involvement, in which case separate lesions affecting the forebrain and thoracolumbar spinal cord would be suspected. Inflammatory neurologic diseases most commonly cause lesions at multiple sites and will be discussed here. These conditions frequently cause concomitant systemic disease. Additional disease categories that may cause multifocal lesions, and are somewhat more likely to result in strict neurologic disease, are discussed in Multiple Neurologic Deficits: Non-infectious Diseases (page 440).
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PMID:Multiple neurologic deficits. Inflammatory diseases. 180 64

Sneddon's syndrome consists of livedo reticularis and cerebral vascular accidents with no evidence of systemic disease responsible for the livedo. The syndrome has been assimilated to a subgroup of systemic lupus erythematosus (SLE) with presence of antibodies directed against phospholipids. Recently, a significant increase in the frequency of cardiac valve diseases has been demonstrated in some SLE patients with livedo reticularis, cerebral vascular accidents and antiphospholipid antibodies. We report the case of a 26-year old woman who had been presenting for 6 years with idiopathic livedo reticularis. Her history was remarkable for the occurrence of 2 cerebral ischaemic accidents at the ages of 23 and 26 years, generalized convulsive seizures at 22 years, and hypertension of pregnancy with 2 miscarriages. Biopsy of the livedo showed normal histological patterns, but electron microscopy detected an obliterating endothelial proliferation and endothelial cells with numerous Weibel-Palade bodies. Laboratory signs of SLE, as well as antiphospholipid antibodies were absent. At the age of 26 years, cardiac abnormalities were heard at auscultation for the first time, and echocardiography showed that they were due to a fairly loose mitral stenosis. According to Burton's criteria our patient had all the typical features of Sneddon's syndrome. The finding of mitral stenosis--an emboligenic cardiopathy that is potentially responsible for cerebral vascular accidents--raises the problem of its relationship with Sneddon's syndrome. The association does not seem to be fortuitous, since our case is very similar to the cases of SLE or antiphospholipid antibody syndrome associated with cardiac valve lesions. However, this case is particular in that 6 years after the onset of the disease there was still no sign of SLE and of antiphospholipid antibodies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Livedo reticularis, cerebrovascular disorders and mitral disease: a new cause of Sneddon's syndrome?]. 208 86

In extracerebral systemic lupus erythematosus (SLE), the complement system plays a prominent pathogenic role, and decreased serum concentration of the 4th component (C4) is a reliable indicator of systemic disease activity. In diffuse CNS-SLE, however, the pathogenic role of complement is less clear. In 12 patients with active diffuse CNS-SLE presenting with delirium (4), organic personality syndrome (3), or generalized seizures (5), we determined the CSF indexes of the complement components C3, C4, and factor B, and of IgG, IgA, and IgM. There was a significant increase of the C4 index in these patients compared with controls and a significantly higher CSF C4 index in patients with an increased IgM index. We conclude that intrathecal C4 is being produced in diffuse CNS-SLE.
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PMID:Diffuse CNS involvement in systemic lupus erythematosus: intrathecal synthesis of the 4th component of complement. 221 52

Three cases are presented, in two of which the CNS lesions revealed the presence of systemic lupus erythematosus (SLE). The diagnosis of SLE was certain according to the criteria of the ARA, and it was further confirmed by results of renal needle puncture biopsy. Case 1: A 16-year-old adolescent developed choreic movements followed, one month later, by psychotic symptoms suggesting a mixed hebephrenic-catatonic schizophrenic affection. Cutaneous lesions and signs of renal insufficiency 3 months later established that these disorders were related to SLE. A favourable outcome was observed rapidly for the systemic signs, recovery from neuropsychic symptoms being obtained after 3 months only but then in a few days. This course suggests the diagnosis of a "functional psychosis" of lupus origin. Case 2: A 24-year-old woman developed left hemiparesis followed by febrile coma. The slowly favourable course of the disease led to the appearance of a progressive dementia, with numerous epileptic seizures. Although tests for antinuclear antibodies were negative and the ESR was normal, several minor biological anomalies were suggestive of a systemic disease and the diagnosis of SLE was finally established. Corticotherapy produced only slight transient improvement. This progression towards dementia with progressive cerebral atrophy is most probably related to cerebral lupus lesions, the initial coma in the absence of any other apparent cause possibly being the first sign. Case 3: A 47-year-old woman developed simultaneously or separately episodes of arthralgia and uveitis of unknown origin over a 12-year period, and attacks of regressive multilocular neurological deficiency over a 15-year period.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Central nervous system involvement in systemic lupus erythematosus]. 671 10

Between December 1981 and May 1991, 44 infants and children with congenital toxoplasmosis were referred to our study group. A uniform approach to evaluation and therapy was developed and is described herein along with the clinical characteristics of these infants and children. In addition, case histories that illustrate especially important clinical features or previously undescribed findings are presented. Factors that contributed to the more severe disabilities included delayed diagnosis and initiation of therapy; prolonged, concomitant neonatal hypoxia and hypoglycemia; profound visual impairment; and prolonged, uncorrected increased intracranial pressure with hydrocephalus and compression of the brain. Years after therapy was discontinued, three children developed new retinal lesions (without loss of visual acuity when therapy for Toxoplasma gondii was initiated promptly), and three children experienced a new onset of afebrile seizures. Most remarkable were the normal developmental, neurological, and ophthalmologic findings at the early follow-up evaluations of many--but not all--of the treated children despite severe manifestations, such as substantial systemic disease, hydrocephalus, microcephalus, multiple intracranial calcifications, and extensive macular destruction detected at birth. These favorable outcomes contrast markedly with outcomes reported previously for children with congenital toxoplasmosis who were untreated or treated for only 1 month.
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PMID:Early and longitudinal evaluations of treated infants and children and untreated historical patients with congenital toxoplasmosis: the Chicago Collaborative Treatment Trial. 805 36

Cerebral angiotropic large cell lymphoma is a rare fatal neurologic disorder characterized by multifocal intravascular proliferation of large pleomorphic cells within vessels of all caliber, predominantly skin and nervous system. Clinical manifestations in previously reported cases were dominated by focal neurologic signs, epilepsia and progressive dementia. We report a case of a 70 year-old man with subacute dementia, epileptic seizures and cerebrovascular events. There was no evidence of a systemic disease outside the nervous system. Cerebrospinal fluid contained 13 leukocytes/mm3 (49% of lymphocytic cells) and more than 100 mg/dl of protein. Cytology was negative. Cranial MRI demonstrated cerebral atrophy and an increased paraventricular signal in T 2 weighted images. A frontal brain biopsy revealed only neuronal dystrophy and astrocytic gliosis. Despite treatment with corticosteroids the patient died 18 months after the onset of the first symptoms. Autopsy was performed and revealed B cell lymphoma.
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PMID:[An anatomo-clinical case of dementia in endovascular large-cell lymphoma]. 859 51

A retrospective analysis of six cases of central nervous system paracoccidioidomycosis, all but one proven by biopsy and surgery, was carried out to study the CT and clinical data and pathological correlation. Most of the patients were from the country. Headache, vomiting, seizures and hemiparesis were the most frequent symptoms. Papilloedema was present in four patients with raised intracranial pressure. Five patients had chronic lung disease and two with advanced systemic disease, skin and mucous membrane lesions were also observed. The neurological disturbance was sometimes the presenting features and the diagnosis was discovered incidentally after surgery. Both solitary and multiple parenchymal lesions were observed and the cerebral hemispheres were more commonly involved in four patients. Local meningeal involvement was observed in one with a single cortical granuloma. We emphasise the usefulness of CT, showing a rounded or lobulated mass with an isodense or radiolucent centre after contrast enhancement, surrounded by an irregular wall of varying thickness. There was always moderate oedema, extending peripherally. Other infections or neoplastic diseases may present similar findings. Preoperative diagnosis should rest on integration of clinical data, chest films, laboratory and neuroimaging studies.
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PMID:Paracoccidioidomycosis of the central nervous system: CT findings. 874 94

A 20-month-old boy had an 8-week history of vomiting, lethargy, generalized muscle weakness, and seizures. There was no history or clinical signs of an underlying systemic disease or an immunodeficiency. Cerebrospinal fluid (CSF) had 99 nucleated cells/cu mm, malignant cells, high protein and normal glucose. CT and MRI scans showed diffuse meningeal enhancement around the brain and spinal cord, but no parenchymal involvement. Biopsy of the leptomeninges showed malignant cells with marked nuclear pleomorphism and prominent clear to eosinophilic cytoplasm. The immunohistochemical studies were positive for histiocyte-macrophage markers and were negative with T and B cells, Ki-1, neural and glial cell antibodies. Multiple tests revealed no other site of disease. The patient died 3 months after onset of treatment despite intensive i.v. and intrathecal chemotherapy. We have not found any other reported case of primary histiocytic leptomeningeal lymphoma in a young child.
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PMID:Primary leptomeningeal histiocytic lymphoma in a young child. 888 15

We have retrospectively assessed the neurological manifestations in 34 patients with hemophagocytic lymphohistiocytosis (HLH) in a single center. Clinical, radiological, and cerebrospinal fluid (CSF) cytology data were analyzed according to treatment modalities. Twenty-five patients (73%) had evidence of central nervous system (CNS) disease at time of diagnosis, stressing the frequency of CNS involvement early in the time course of HLH. Four additional patients who did not have initial CNS disease, who did not die early from HLH complications, and who were not transplanted, also developed a specific CNS disease. Therefore, all surviving and nontransplanted patients had CNS involvement. Initially, CNS manifestations consisted of isolated lymphocytic meningitis in 20 patients and meningitis with clinical and radiological neurological symptoms in nine patients. For these nine patients, neurological symptoms consisted of seizures, coma, brain stem symptoms, or ataxia. The outcome of patients treated by systemic and intrathecal chemotherapy and/or immunosuppression exclusively (n = 16) was poor, as all died following occurrence of multiple relapses or CNS disease progression in most cases. Bone marrow transplantation (BMT) from either an HLA identical sibling (n = 6) or haplo identical parent (n = 3) was performed in nine patients, once first remission of CNS and systemic disease was achieved. Seven are long-term survivors including three who received an HLA partially identical marrow. All seven are off treatment with normal neurological function and cognitive development. In four other patients, BMT performed following CNS relapses was unsuccessful. Given the frequency and the poor outcome of CNS disease in HLH, BMT appears, therefore, to be the only available treatment procedure that is capable of preventing HLH CNS disease progression and that can result in cure when performed early enough after remission induction.
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PMID:Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis. 902 10


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