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

The author has operated on 40 patients with giant intracranial aneurysms, using various surgical approaches. Giant aneurysms predominated in females (3:1) and were most common in the age group 30 to 60 years. Patients presented with subarachnoid hemorrhage (17), visual disturbance (18), chronic headache (14), transient or progressive hemispheric deficit (6), seizure (2), dementia (2), and cerebrospinal fluid rhinorrhea (1). Giant aneurysms were located at the carotid artery (25), the basovertebral artery (8), the anterior communicating artery (5), and the middle cerebral artery (2). Eight of 40 patients had one or more other aneurysms and/or associated arteriovenous malformations. Aneurysms were treated with intramural thrombosis (21), neck occlusion (7), trapping (10), proximal parent artery ligation (1), and aneurysmorrhaphy (1). After as much as 8 years of follow-up, 32 patients (80%) showed complete or marked improvement in signs and symptoms; two patients (5%) had a poor recovery. There were six surgical mortalities (15%). Giant aneurysms can be treated with respectable results if the surgeon selects the technique best suited to the particular aneurysm. In general, neck occlusion, trapping, and aneurysmorrhaphy are best for giant aneurysms of the anterior circulation, and intramural thrombosis is best for those of the posterior circulation. Extra- and intracranial vascular anastomotic techniques are also of value. For success, a flexible approach is essential.
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PMID:Direct surgical treatment of giant intracranial aneurysms. 50 18

CT, MRI and neurological features of 27 children with tuberous sclerosis were prospectively compared. Imaging studies were positive in 92.5% of cases. CT was more useful in detecting subependymal nodules, while MRI showed the number and location of cerebral cortical and subcortical lesions more accurately. Cortical lesions in the cerebellar hemispheres were present in 26% of patients. Gadolinium-DTPA used in 10 patients showed slight enhancement of the subependymal lesions in 80% of cases, probably representing active lesions with alteration of the blood-brain barrier. None of the cortical and subcortical lesions enhanced. Giant-cell astrocytomas were detected in 5 patients, the postcontrast CT and MRI studies improving their assessment. Unlike subependymal nodules, all tumors showed marked enhancement regardless of their size. Seizures were present in 96% of patients, mostly beginning before 1 year of age. There was no clear relationship between the radiological features and the neurological evolution of these patients. Therefore, it is not possible to establish a clinical prognosis based on the radiological findings. MRI is the procedure of choice in the diagnosis of tuberous sclerosis. When pre and postcontrast MRI are negative, CT is used to exclude small calcified subependymal nodules. MRI follow-up is required only when tumoral development is clinically suspected.
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PMID:Neuroimaging in tuberous sclerosis: a clinicoradiological evaluation in pediatric patients. 149 3

An outbreak of food poisoning resulting in 13 deaths in children occurred in Malaysia during the Chinese Festival of the Nine-Emperor Gods in 1988. The offending food was a Chinese noodle called 'Loh See Fun' (LSF). The source was traced to a factory where a banned food preservative was added to make the LSF. The food poisoning was attributable to aflatoxins and boric acid. The clinical features included vomiting, pyrexia, diarrhoea, abdominal pain, anorexia, giddiness, seizures, and eventual coma. Initially, many presented with a Reye-like syndrome. Eleven post-mortem examinations were performed. The pathological findings included extensive coagulative necrosis of the liver with proliferative 'ductal/ductular metaplasia of the hepatocytes'. Giant cell formation, central vein sclerosis, bile stasis, and steatosis were also noted. There was presence of acute tubular necrosis, superficial upper gastrointestinal erosions, and ensuing encephalopathy. The eventual cause of death is acute hepatic and renal failure.
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PMID:An outbreak of aflatoxicosis and boric acid poisoning in Malaysia: a clinicopathological study. 189 May 47

Pathological findings in a case of hemimegalencephaly are presented. Hemispherectomy, performed because of intractable seizures, allowed an electron microscopic and Golgi study. Glial abnormalities consisted of hyperplasia of glia cells with giant astrocytes often containing several nuclei and proliferation of numerous Rosenthal fibers. Golgi stain showed many giant neurons with a perikaryon covered by perisomatic processes, and a complex dendritic tree. Glial abnormalities could be correlated with the firmness of the hemisphere and intense hypersignal on magnetic resonance imaging. Giant neurons were associated with an increase in size of the perikaryon and dendritic tree; this pattern suggests a polyploidy.
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PMID:Electron microscopic and Golgi study in a case of hemimegalencephaly. 275 Apr 82

The authors report a case of a six-year-old boy with seizures, mental retardation and some classic features of tuberous sclerosis, i.e., cutaneous hypomelanotic macules, poliosis, cyst-like lesions of the right upper limb and cerebral calcifications. From birth he showed partial gigantism of the first two fingers of the right hand associated with an arteriovenous malformation of the ulnar artery and agenesis of the radial artery. The EEG and CT scan studies are also reported. The occurrence of two dysgenetic disorders in one patient as a manifestation of disturbed embryogenesis at a very early stage of development is discussed.
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PMID:Tuberous sclerosis associated with partial gigantism in a child. 340 55

Giant aneurysms have rarely been reported in association with intractable complex partial seizures (CPS). We report a 30-year-old man with intractable CPS since age 18 years. Seizure onset was electrically localized to right temporal lobe. Preoperative neuroimaging studies showed a partially thrombosed giant aneurysm of the right posterior cerebral artery. Selective amygdalohippocampectomy and occlusion of the posterior cerebral artery did not cause deficits. The patient has been seizure-free for 15 months after operation. We review the relevant literature on aneurysms as a cause of epilepsy.
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PMID:Intractable complex partial seizures associated with posterior cerebral artery giant aneurysm: a case report. 798 26

Twenty-one cases (12 males, 9 females) of Lafora's disease in 16 families were studied at the National Institute of Mental Health and Neuro Sciences (NIMHANS), Bangalore, India, from 1982 to 1990. Mean age of onset was 13.5 years (range 9.5-18 years). First symptom was generalized tonic-clonic seizure (17), myoclonus (3), or dementia (1). All patients eventually developed the classical triad, except 1 who has had only myoclonus. Seven had occipital seizures. Other signs included behavioral changes (9), brisk tendon reflexes (11), cerebellar signs (8), and visual impairment (4). Patients from 14 of the 16 families (85%) were products of consanguineous marriage. More than 1 sibling was affected in 6 families. Scalp EEGs showed diffuse background slowing with epileptiform discharges in all and progressive slowing as the disease progressed in 3. Photosensitivity occurred in 4 of the 17 cases studied (23.5%). EEG abnormalities were documented in the presymptomatic stage in 2 cases 6 months and 6 years before clinical symptom onset. Visual evoked responses were abnormal in 4 of the 6 cases studied. Giant somatosensory evoked potentials (SSEP) were observed in all 8 cases studied. Lafora bodies were demonstrated in axillary skin in 14 of 17 (82.4%), in liver in 4 of 10 (40%), and in both brain biopsy specimens. In 2 cases, liver biopsy was positive while axillary skin biopsy was negative. In the brain, inclusions were evident in glial and capillary endothelial cells in addition to neurons. Although our cases were similar to those described earlier, the relative rarity of visual phenomena is emphasized. The clinical pattern was consistent with autosomal recessive inheritance. The high frequency of consanguinity in the South Indian population may be responsible for the many cases observed at our center.
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PMID:Lafora's disease in south India: a clinical, electrophysiologic, and pathologic study. 838 90

Giant interhemispheric cysts in association with dysgenesis of the corpus callosum are rare. Clinical and developmental data concerning affected patients are limited, the pathologic basis for these cysts has not been established, and prognosis is uncertain. The purpose of our study was to describe the clinical and developmental findings for 11 children with giant interhemispheric cysts, dysgenesis of the corpus callosum, and ventricular dilatation. Eight of the children required ventriculoperitoneal shunting, eight had normal neurologic examinations, and seven had experienced seizures. Seizure control appeared to correlate with neurodevelopmental function. Cognitively, children ranged from the average to mildly/moderately retarded level. Language delays were frequent, particularly in children with predominantly left hemispheric cysts. Adaptive behavior was mildly delayed with weakness observed in motor skills. Despite the striking neuroimaging abnormalities, our findings suggest the possibility of only mildly delayed neurodevelopmental outcome is good for children with this constellation of neurologic anomalies.
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PMID:Clinical and developmental findings in children with giant interhemispheric cysts and dysgenesis of the corpus callosum. 853 76

We describe a characteristic pattern of immunoreactivity for synaptophysin in tuberous sclerosis. We analyzed cortical tubers from surgical specimens taken from six patients with tuberous sclerosis, which were obtained by surgical resections for the treatment of intractable seizures. The cortical tubers were characterized by blurred lamination of the cerebral cortex, hypercellularity, and gliotic changes. Neuropil in the cortex of cortical tubers showed reduced immunoreactivity for synaptophysin in all patients. 'Giant cells' were investigated in the cortex and white matter regions of cortical tubers. Some 'giant cells' had neuronal characteristics such as Nissl substance, a centrally placed chromatin-marginated nucleus, prominent nucleolus, positive immunoreactivity for microtubule-associated protein 2, and negative immunoreactivity for glial fibrillary acidic protein. Other 'giant cells' were indeterminate in cell type because they lacked Nissl bodies, distinct nucleolus, consistent immunoreactivity for microtubule-associated protein 2 and glial fibrillary acidic protein. Almost all 'neuronal giant cells' and some of the 'indeterminate giant cells' in the white matter showed intense immunoreactivity for synaptophysin: cell borders were surrounded by an intense immunoreactive halo. In conclusion, these immunohistochemical patterns for synaptophysin assist in characterizing these abnormal cells in the cortical tubers of patients with tuberous sclerosis.
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PMID:Giant cells in cortical tubers in tuberous sclerosis showing synaptophysin-immunoreactive halos. 907 86

We report the extraordinary association of hemimegalencephaly with chronic encephalitis and cytomegalovirus (CMV) positivity in a 5-month-old infant with intractable seizures and a left hemisphere resection. Microscopy revealed a severe neuronal migration disorder (NMD) with fusion of gyri, marked disarray of neuronal lamination, neuronal gigantism and extensive neuronal heterotopias. Also widespread were microglial nodules, gliosis and nodular calcifications and some foci of frank necrosis with calcification. Occasional perivascular and leptomentingeal lymphocytic infiltrates were present. No viral inclusions were identifiable. Polymerase chain reaction on multiple specimens showed unequivocal CMV positivity. In intrauterine CMV infection. NMDs such as polymicrogyria are well recognized, but the association of hemimegalencephaly with CMV infection has not previously been described. Our finding of chronic encephalitis with CMV positivity and hemimegalencephaly in the same patient raises questions about the role of CMV in the etiopathogenesis of the NMD.
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PMID:Coexistence of hemimegalencephaly and chronic encephalitis. Detection of cytomegalovirus by the polymerase chain reaction. 908


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