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

We report on symptoms, clinical course and prognostic factors of eight children (6 girls and 2 boys) with Sturge-Weber syndrome. The period of observation was 1 to 17 years (mean 8.8 years). All patients had the classical symptoms with facial port-wine stain nevus and intracranial calcifications. In two cases a bilateral distribution of the nevus was found. All patients but one had epileptic seizures which were followed by Todd's palsies in five. Four children additionally suffered from hydrophthalmia. Another patient had an angiomatosis of the retina and cataract. Neurological findings were normal in three patients, minimal signs were found in three, one patient had a spastic hemiplegia and another one hypotone tetraplegia. The mental development was normal in three patients, however severe dyscalculia occurred in one of them. Five patients were mentally retarded, three of them severely. The prognosis was the worse the earlier the cerebral seizures appeared.
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PMID:[Clinical aspects and course of Sturge-Weber syndrome in childhood]. 372 61

Developmental dyscalculia (DC) is a primary cognitive disorder of childhood manifested by disturbance of arithmetic ability. As an isolated learning disability (LD), it is usually treated by remedial education and not referred for further medical evaluation. We examined a group of 7 third-grade children with DC attending a mainstream school who had not progressed academically in spite of specific special education intervention. We were able to identify in all 7 children neurological conditions that had direct bearing on the children's cognitive disabilities and remedial programs. One child had petit mal seizures, another developmental Gerstmann syndrome, a third had dyslexia for numbers, and 4 children had attention deficit disorders without hyperactivity. Based on this experience, we suggest that the indications for medical or neurological assessment be broadened to include children who are not improving academically in spite of appropriate professional intervention.
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PMID:Developmental dyscalculia and medical assessment. 768 63

The clinical features of three affected members of a British pedigree with familial Alzheimer's disease are presented. This pedigree is one of six included in an earlier study which demonstrated linkage to chromosome 14. The individuals were investigated clinically and neuropsychologically, using both PET and MRI over a 4-year period. Further information from three deceased individuals was obtained, including histopathological confirmation of Alzheimer's disease in one case which came to autopsy. The mean age at onset for this family was 43 years. Neurological examination revealed myoclonic jerks in all cases, and one patient was documented to have seizures. Strikingly similar neuropsychological profiles were observed, characterized by an initial memory deficit with early dyscalculia and an impairment in speech production with relative absence of anomia. All individuals showed mild degrees of cerebral atrophy and two individuals had periventricular white matter lesions. PET scanning using [18F]fluorodeoxyglucose showed parieto-temporal hypometabolism in all cases and the two severely affected patients with speech production changes had additional left-sided frontal hypometabolism involving Broca's area. The least affected case initially had a more asymmetrical reduction in metabolism in the left inferior temporal and supramarginal gyri; a follow-up scan showed that this deficit had become bilateral and more severe. These clinical and neuroimaging features have not been previously reported in chromosome 14 linked pedigrees; the phenotypic variability between families suggests allelic heterogeneity at the chromosome 14 locus.
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PMID:Chromosome 14 linked familial Alzheimer's disease. A clinico-pathological study of a single pedigree. 789 4

Early onset Familial Alzheimer's Disease (FAD) is an autosomal dominant disease with apparent complete penetrance. It is genetically heterogeneous with some families carrying mutations in the amyloid precursor protein (APP) gene which segregate with the disease. In addition, there is allelic heterogeneity with four mutations associated with FAD. Three mutations have been reported at APP 717, just distal to the C-terminus of the beta-amyloid domain, APP 717 val-ile, APP 717 val-phe, and APP 717 val-gly, which are associated with autopsy-proven Alzheimer's disease (AD). APP 670/671 lies at the N terminus of the beta-amyloid domain and is associated with clinically diagnosed FAD in two Swedish families. FAD tends to have prominent myoclonus and this is shared by the cases with APP mutations. In two unrelated UK families with APP 717 val-ile mutations there was early prominent memory impairment with dyscalculia proceeding to generalized cognitive impairment with a lack of insight. There was a late development of a gait disturbance with extrapyramidal features in some members. Positron emission tomography (PET) with fluorodeoxyglucose demonstrated posterior bitemporal biparietal hypometabolism in one case. Magnetic resonance imaging (MRI) showed generalized cerebral atrophy particularly affecting the temporal lobes and hippocampus. At autopsy, a single case showed extensive beta-amyloid deposition with congophilic angiopathy and widespread senile plaques and neurofibrillary tangles. The cytoskeletal pathology associated with abnormally phosphorylated tau was similar to cases of sporadic AD. In addition, there were widespread cortical and subcortical Lewy bodies. A single family with the APP 717 val-gly mutation also showed prominent myoclonus, lack of insight, and seizures, PET, in a single case, showed classical biparietal bitemporal hypometabolism. Autopsy, in a single case, showed diffuse deposits of beta-amyloid throughout the cortex with frequent neuritic plaques and neurofibrillary tangles. No other inclusion bodies were seen. There was severe congophilic angiopathy. The age at onset of APP mutations is around 50 years of age by contrast to other early onset FAD pedigrees.
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PMID:Alzheimer's disease families with amyloid precursor protein mutations. 823 83

Surgery for an area of focal cortical dysplasia in a critical region is reported in a right-handed female manifesting intractable focal epilepsy and verbal cognitive deterioration. She developed the first seizure at 2 years of age and was treated with phenytoin and zonisamide, with good control until 10 years of age. Although seizures did not occur at 9 years of age, she manifested dyscalculia, right-left disorientation, and finger agnosia, and N-isopropyl-p-iodoamphetamine single-photon emission computed tomography (SPECT) revealed focal hypoperfusion in the left parietal lobe. At 11 years of age, she developed regular nocturnal seizures and gradually lost the ability to understand the meaning of sentences. Verbal IQ declined from 94 to 63, and the area of hypoperfusion detected by interictal N-isopropyl-p-iodoamphetamine SPECT spread over the left parietotemporal lobes. Magnetic resonance imaging revealed focal cortical dysplasia mainly in the left parietal lobe, and ictal technetium-99m-ethyl cysteinate dimer SPECT images demonstrated an area of hyperperfusion around the focal cortical dysplasia, including the left precentral gyrus. Because of the overlap between the epileptogenic and functional cortex, the authors concluded that cortical resection, including focal cortical dysplasia, was inappropriate in this patient.
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PMID:Cognitive deterioration associated with focal cortical dysplasia. 1002 67

To clarify the relationship between epileptic attacks and cognitive dysfunction, we examined the serial findings of 123I-IMP single photon emission computed tomography (SPECT) in relation to the intelligence quotient (IQ), assessed by Wechsler Intelligence Scale of Children-Revised, in two female patients with focal cortical dysplasia (FCD) over a 10-year period. The age of patient 1 at the initial assessment was 2 years, and the age of patient 2 was 9 months. They developed complex partial epilepsy in infancy, and were treated with antiepileptic drugs, which remained effective until 11 years of age, when their epileptic attacks recurred. Patient 1, a 14-year-old girl with FCD of the left parietal lobe suffered from dyscalculia, right-left disorientation, and finger agnosia even when she was free of epileptic attacks. Following the recurrence of seizures which occurred every night, she became unable to understand what was said to her. A hypoperfusion area detected by 123I-IMP SPECT was restricted to the left parietal lobe during the seizure-free period, but spread to the temporo-parietal lobes following the recurrence. Her verbal IQ declined from 94 (at 9 years of age) to 63 (at 11 years and 8 months). After her seizures were controlled again (at 14 years and 4 months), the 123I-IMP SPECT findings improved. Patient 2, a 12-year-old girl with FCD of the left frontal lobe, showed cognitive dysfunction. Her verbal IQ declined from 91 (at 7 years and 5 months) to 76 (at 11 years and 8 months) following a recurrence of epileptic attacks. 123I-IMP SPECT showed hypoperfusion in the left frontal lobe, where the accumulation count ratio (left/right ratio) declined from 0.86 (at 3 years) to 0.64 (at 11 years). These findings suggest that epileptic attacks are related to cognitive dysfunction in FCD patients. This cognitive dysfunction appears to correlate with the appearance of hypoperfusion areas, as detected by 123I-IMP SPECT.
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PMID:[Longitudinal study of cognitive function in two patients with focal cortical dysplasia]. 1100 34

We describe a case with symptoms of transient diffuse right hemisphere dysfunction (hemispatial neglect, dyscalculia, and disturbance of both spatial construction and visuospatial perception) occurring after status epilepticus. The clinical picture of this case suggested to us that these features could be understood as a variant of Todd's paralysis.
Seizure 2001 Mar
PMID:Cognitive dysfunction of right hemisphere-like Todd's paralysis after status epilepticus: a case report. 1140 56

Rolandic epilepsy (RE) is the most common childhood epilepsy syndrome with a good, long-term outcome. Nevertheless, some studies indicate that children with RE have more scholastic and neuropsychological problems than controls. The purpose of this study was to describe neuropsychological findings in a small group of Italian children with RE, focusing on dyslexia and dyscalculia. Possible correlations between these findings and the age-at-onset of seizures, duration of active epilepsy, frequency, type and localization of epileptic discharges were examined. Children affected by RE, aged nine to eleven years were selected from patients admitted to the outpatient service of our Clinic. They underwent cognitive evaluation, specific evaluation for dyslexia and dyscalculia, and awake and sleep EEG recordings. We found two patients out of the ten with dyscalculia, one of whom also had characteristics of dyslexia. This small study suggests that dyscalculia and dyslexia might be more frequent than expected in children with RE. No significant correlations between this finding and EEG, seizure-frequency or age-at-onset of epilepsy were found in our patients.
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PMID:Are dyslexia and dyscalculia associated with Rolandic epilepsy? A short report on ten Italian patients. 1807 30

The purpose of this study was to evaluate children with benign rolandic epilepsy, a childhood epilepsy characterized by centrotemporal/rolandic spike-wave discharges with infrequent partial seizures that may secondarily generalize. Recently, some investigators have questioned whether benign rolandic epilepsy is indeed "benign" or whether long-term cognitive outcome may be adversely affected. We initiated an ongoing study to identify children with benign rolandic epilepsy. The children were evaluated in the Texas Comprehensive Epilepsy Program using outpatient or continuous video-electroencephalographic monitoring, brain magnetic resonance imaging, magnetoencephalography, and neuropsychological testing. Neuropsychological testing revealed fine motor dysfunction, visuomotor integration deficits, dyscalculia, and/or expressive language deficits in all of the 9 patients evaluated, reaffirming that benign rolandic epilepsy is not necessarily a benign disorder. Our study shows a high concordance of motor and cognitive deficits in benign rolandic epilepsy, as others have previously suggested. Furthermore, magnetic source imaging shows a higher resolution of dipole localization compared with conventional electroencephalography, which may ultimately improve prediction of deficits. This reaffirms that magnetoencephalography is a valuable diagnostic tool in the evaluation of children with benign rolandic epilepsy.
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PMID:Benign rolandic epilepsy -- perhaps not so benign: use of magnetic source imaging as a predictor of outcome. 1840 Oct 32

Epilepsy is one of the most prevalent chronic disorders of childhood which can threaten child development and mental health. Among cognitive disorders, dyscalculia is one of the most important. In this study, 39 children and adolescents with idiopathic epilepsy underwent clinical and neuropsychological assessment to determine the intellectual level, math skills, reading and writing performance and neuropsychological profile. It was observed that the mathematical ability was below schooling expectations in a higher frequency than expected. There were no significant differences in mathematical performance among groups divided by number of antiepileptic drugs used, duration of disease and types and frequency of seizures. There was a positive correlation with intelligence quotient and attentional and reading level. These results suggest the existence not only of dyscalculia, but the concurrence of attentional and reading problems for the poor mathematical performance in this population.
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PMID:Developmental dyscalculia in children and adolescents with idiopathic epilepsies in a Brazilian sample. 2476 92


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