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

We studied offspring of Vietnamese mothers and French fathers who had been born in Vietnam and brought to France to be raised. All but 72 of 3,451 such individuals completed background and neurological questionnaires. Those with positive answers on the latter were examined neurologically. The median year of birth for the 3,379 respondents was 1948, the median year of arrival in France was 1957, and they had resided in France a median of 18 years before interview in 1975. Twenty-five persons had neurological symptoms, and 6 others reported a history of seizures alone. A total of 16 reported seizures: 7 with infantile febrile fits and 9 with afebrile seizure disorders. Three cases of exacerbating-remitting multiple sclerosis (MS) were discovered as well as 3 persons in whom MS was suspected: 1 with cervical myelopathy, 1 with progressive paraparesis, and 1 with bilateral optic neuropathy. The 3 MS cases provided a crude prevalence rate (or 18-year cumulative risk) of 89 per 100,000. Their age-specific prevalence rate was 169 per 100,000 population aged 20 through 29 years. Each measure was similar to such rates for Denmark and had lower 95% confidence limits that clearly exceeded expectations for Vietnam. The results provide further evidence that the risk of MS can be increased by migration in childhood from low- to high-risk regions.
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PMID:Multiple sclerosis in a migrant population: 2. Half-orientals immigrating in childhood. 743 68

Cavernous angiomas are vascular malformations affecting any part of the central nervous system (CNS). The management of asymptomatic cavernous angiomas is still debated due to their poorly understood natural history, although more data are now available regarding results of surgical treatment in symptomatic cases. The authors report their surgical experience with 18 pediatric patients operated on for symptomatic CNS cavernous angiomas. The children ranged in age from 10 months to 17 years, without a relevant sex difference. Cavernous angiomas were intracranial in 17 cases: 15 being in the supratentorial compartments and two in the cerebellum. Clinical manifestations were as follows: seizures in 11 cases, focal neurologic deficits in five, and headache in one. The 18th case was observed in a girl showing paraparesis in the spinal subdural-extramedullary space at T8-T9 level. Excision of four deep cerebral lesions was performed after stereotactic localization through non-eloquent cortex. Pathologic confirmation of cavernous angiomas was obtained in all patients. Mortality from surgical procedures was absent in this series. The follow-up period ranged from 1 to 16 years. All 11 epileptic patients obtained seizure control; improvement or stabilization of neurologic symptoms was observed in the remaining seven patients.
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PMID:Surgical management of cavernous angiomas in children. 794 Jan 4

Neurological involvement in Wegener's granulomatosis was studied by reviewing the charts of 324 consecutive patients in whom the diagnosis was made at the Mayo Clinic. One hundred nine patients (33.6%) had neurological involvement. Peripheral neuropathy occurred in 53; cranial neuropathy, in 21; external ophthalmoplegia, in 16; cerebrovascular events, in 13; seizures, in 10; cerebritis, in 5; and miscellaneous involvement, in 25. The mean age and sex ratio were similar in the patients with and those without neurological involvement. Among the patients with peripheral neuropathy, 42 had mononeuropathy multiplex; 6, distal symmetrical polyneuropathy; and 5, unclassified peripheral neuropathy. Multiple mononeuropathy was a major presenting symptom in 8 patients. A significantly higher percentage of patients with peripheral neuropathy, compared to those without peripheral neuropathy, had kidney involvement (p < 0.001). The second, sixth, and seventh cranial nerves were most frequently affected. Multiple cranial nerves were affected in 8 patients. Unusual neurological manifestations in the miscellaneous group were spastic paraparesis, temporal arteritis, Horner's syndrome, and papilledema.
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PMID:Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. 838 87

In 1926, Foix, Chavany and Marie described an acquired syndrome of fasciopharyngoglossomasticatory diplegia resulting from bilateral infarction of the anterior operculum. Clinical features consisted of facial diplegia, dysarthria, pseudobulbar palsy, mild to severe mental retardation, and seizures. A developmental form, similar in presentation in adults with MRI findings consisting of bilateral perisylvian cortical malformation consistent with polymicrogyria involving the sylvian fissure and opercular cortex, has been recognized; but few pediatric cases of congenital bilateral perisylvian syndrome (CBPS) have been reported. Over the past four years, we have encountered 12 cases of CBPS presenting in childhood. Age ranges were from 1 week to 11 years with a median of 2.25 years; six were less than two years of age. Seven were male and five female. Ten had bilateral perisylvian polymicrogyria on MRI; two had unilateral perisylvian schizencephaly with contralateral perisylvian polymicrogyria. Clinical manifestations included developmental delay in 7; poor palatal function in 5; hypotonia in 4; arthrogryposis in 4; hemiparesis in 3; apnea in 3; paraparesis in 2; micrognathia in 2; pectus excavatum in 2; quadriparesis in 1; and hearing loss in 1. Seizures occurred in seven (58%) and consisted of infantile spasms (n = 1), generalized tonic-clonic (n = 1), complex partial (n = 2), partial motor (n = 2; 1 with secondary generalization), and febrile convulsions (n = 1). CBPS has different manifestations in the pediatric population than in adults. CBPS is more common than previously thought, is recognizable by MRI and should be suspected clinically in any infant or child presenting with oromotor dysfunction/pseudobulbar signs and developmental delay, especially if there are associated congenital malformations. Epilepsy is not a constant feature in the pediatric presentation and is variable in type and severity.
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PMID:Pediatric congenital bilateral perisylvian syndrome: clinical and MRI features in 12 patients. 930 9

We investigated the features of children with spinal cord insults (SCI) occurring in the pre-, peri-, and neonatal periods by sending 340 questionnaires to all paediatric neurologists, paediatric urologists, and neonatologists in the UK and Ireland. We requested information about timing, nature, and level of SCI in their patients; family and maternal history; pregnancy, delivery, and neonatal period; clinical presentation, imaging, laboratory studies, and outcome. Two-hundred and sixty-one questionnaires were returned with data on 58 patients with SCI. Seven out of the 58 children with SCI had pure dysraphic cord syndromes and were excluded. Fifty-one patients (33 males, 17 females, one unknown), born between 1972 and 1996, remained. Clinical presentations included severe respiratory failure (N=20; five of whom died neonatally) and hypotonia or weakness recognized either during the neonatal period (N=12) or after 28 days (N=10). Data on clinical presentation were not given in nine cases. Lesions were cervical (N=22) and thoraco-lumbar (N=29). SCI was ascribed to ischaemia (N=12), trauma (N=4), and other associated underlying conditions (N=11), whilst the aetiology was unknown in 24 cases. Mean gestational age (36.2 weeks) and birthweight (2.6 kg) were lower than previously reported with the lowest figures associated with thoraco-lumbar and ischaemic lesions. More males were affected by lesions than females and the incidence of preterm delivery, multiple pregnancy, breech presentation, forceps delivery, and caesarean delivery were higher than average. Forceps delivery was associated with cervical lesions. Outcome data were given in 47 children, nine of whom died either neonatally or within the first 20 months of life. Motor disability ranged from a complete recovery in one out of 40 to paraparesis in 26 out of 40, and tetraparesis in 13 out of 40 patients: 17 out of 39 were ambulant. Sphincter dysfunction was present in 22 out of 38 patients and scoliosis in 16 out of 37. Learning difficulties were present in 10 out of 39, behavioural problems in five out of 39 and seizures in four out of 39 patients. SCI in the pre-, peri-, and neonatal periods are rare but probably under-diagnosed and are heterogeneous in aetiology, presentation, and outcome. Boys appear to be more susceptible than girls.
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PMID:Spinal cord insults in the prenatal, perinatal, and neonatal periods. 1037 52

Chronic subdural haematomas are mainly related to slight or moderate head trauma with consecutive lesion of bridge or cortical veins and bleeding in the subdural space. Further predisposing factors are known impairment of coagulation (coagulopathies, treatment with anticoagulants, alcohol abuse), risk factors for degenerative disease of the arteries (diabetes mellitus, arterial hypertension), and development of pressure gradients (hydrocephalus, epileptic seizures, lumbar puncture, CSF drainage and cerebral atrophy). Chronic subdural haematomas appear bilaterally in 20 to 25% of cases. We report on a 69-year-old male with a 4-day history of intermittent, proximal, painless paraparesis (BMA grade M2-5) without a trigger event. Sensibility was normal in all qualities and vigilance was not disturbed. Computed tomography of the neurocranium revealed a bitemporally located chronic subdural haematoma with extension to parietal on both sides. Trepanation was performed over the tuber parietale and temporoparietally on both sides, with release of 150 ml fluid. The neurologic deficits regressed totally within 12 hours postoperatively. To the best of our knowledge, we are the first to describe the clinical paradox of intermittent, painless paraparesis with preserved sensibility and without disturbances of vigilance, as manifestation of a chronic subdural haematoma possibly leading to impairment of cerebral blood flow in the area of the middle cerebral artery. Small changes in systemic blood pressure lead to changes in cerebral perfusion pressure due to vessel compression by the haematoma, thus explaining the intermittent character of the clinical presentation.
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PMID:[Intermittent paraparesis as manifestation of a bilateral chronic subdural hematoma]. 1046 9

Multiple meningiomas in different neuraxial compartments are quite rare. The authors report two new cases of association between cranial and spinal meningiomas, one of them in a patient operated upon for multiple intracranial meningiomas. The first case was a 60-year-old woman with progressive paraparesis who had been operated on 13 years earlier for multiple intracranial meningiomas. A myelo-CT scan showed a block of contrast medium at T1-T2; the lesion was removed via a standard laminectomy. The second patient was a 76-year-old woman with a 6-month history of spastic paraparesis. MRI detected an extramedullary intradural lesion at T6-T7. A cerebral MRI, performed because of the onset of seizures, showed a right parietal lesion. Removal of the thoracic lesion was followed, 6 months later, by removal of the cerebral one. Both patients presented a progressive improvement of the paraparesis and returned to normal daily activities.
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PMID:Multiple meningiomas in different neuraxial compartments. Report of two cases. 1081 90

Benign fibrous histiocytomas (BFHs) are tumors with fibroblastic and histiocytic components without histological anaplasia. Intracerebral lesions are exceptional and to our knowledge a spinal location was not yet described. We describe 2 cases of BFHs of the neural axis: the first, a 22-month-old boy with Down's syndrome, presented with a paraparesis and the magnetic resonance (MR) of the spine disclosed an intradural extramedullary, thoracic mass, totally resected; the second, a 13-year-old boy with left partial motor seizures, in whom the MR of the brain showed an intracerebral, right frontal tumor, also surgically removed. Both patients are free of recurrence, 6 years and 15 months after surgery, respectively. Histological examination and immunoreactivity for vimentin and histiocytic markers favored the diagnosis of BFH. It is likely that these tumors may originate from spinal dura mater mesenchymal stem cells and from the intracerebral perivascular pial sheath or the brain vessel walls themselves, respectively. Other benign, isolated, intracranial fibrohistiocytic neoplasms, namely the juvenile xanthogranuloma, can harbor a clinical, morphological and immunohistochemical profile overlapping the one of the BFH. Intracranial germ cell tumors may be associated with Down's syndrome, although harboring an unusual, non-pineal and non-chiasmatic location. One can speculate that a similar, still unknown genetic mechanism responsible for this association, could also induce the growth of other type of tumors in patients with this syndrome. BFHs should be added to the differential diagnosis of intracerebral or spinal dural attached tumors. Furthermore, we propose to name these intracranial tumors "benign isolated fibrohistiocytic tumors of the CNS".
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PMID:Benign isolated fibrohistiocytic tumor arising from the central nervous system. Considerations about two cases. 1204 82

A 5-year-old, castrated male mixed-breed dog was presented for paraparesis, ataxia, hyperesthesia, and thrombocytopenia of 5 months' duration and recurrent seizures during the preceding 2 weeks. Multifocal neurological, ophthalmological, pulmonary, and cardiac diseases were identified. Magnetic resonance imaging and cerebrospinal fluid analysis supported a tentative diagnosis of neoplastic or inflammatory disease. A computed tomography-guided biopsy provided both cytopathological and histopathological evidence of intravascular lymphoma. The disease progressed despite chemotherapy with prednisone, L-asparginase, and vincristine. Postmortem histopathological examinations suggested intravascular lymphoma in the central and peripheral nervous systems as well as in multiple other organ systems. This is the first description of an antemortem diagnosis and treatment of intravascular lymphoma involving the central nervous system of a dog.
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PMID:Intravascular lymphoma involving the central and peripheral nervous systems in a dog. 1254 21

We report a 66-year-old woman who developed mental deterioration in her school days, and progressive gait disturbance, dysarthria and bradykinesia in her 40 s. Her parents were consanguineous, and the two of her brothers were suspected to have the allied disorder. On physical examination, she was short-statured and high-arched palate was observed. Neurological examination revealed dementia, abnormal eye movement, dysarthria, spastic paraparesis with hyperreflexia, bilateral Babinski signs, cerebellar ataxia and dysuria. Brain MRI showed marked hypoplasia of corpus callosum with dilatation of lateral ventricles and cerebral sulci and significant cerebellar atrophy. Amino acid analyses showed significant elevation of glycine without ketosis in serum, cerebrospinal fluid, and urine, which lead us to the diagnosis of late-onset nonketotic hyperglycinemia(NKH). NKH is known to be a rare autosomal recessive metabolic disorder primarily caused by deficient activity of various components of the mitochondrial glycine cleavage system. Onset of the disease occurs most often in early infancy, however, later-onset variants have been described. Usually, late-onset NKH only manifests mild mental deterioration, character change, seizure, ataxia or spastic paraparesis, which sometimes makes difficulty in differentiating this disease from other hereditary cerebellar ataxia or spastic paraparesis. In addition, many structural brain abnormalities have been reported accompanied with NKH, and especially, agenesis or hypoplasia of corpus callosum is the most characteristic feature in this disease. Therefore, we emphasized that amino acid analyses should be considered in any patients who have cerebellar ataxia or spastic paraparesis of unknown cause with these neuroradiological findings.
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PMID:[Late-onset nonketotic hyperglycinemia: a case report]. 1259 24


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