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)

There were 17 survivors of severe neonatal respiratory distress treated with IPPV for more than 24 hours (36-520 hrs, mean duration of IPPV 6 days) followed into the second year of life. 11 of these young children were physically and neurologically normal. 3 were developmentally retarded (3 months or more), 2 had neurological defects without mental subnormality. These defects (Hemiplegia and Diplegia) were correlated with low gestational ages (32 and 33 weeks respectively) 1 child was severely defective. This one was treated because of apnea caused by seizures in postmaturity syndrome (44 weeks gestation). The overall incidence of defects was 3/17 (= 17%).
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PMID:[Development after respiratortreatment during the newborn period (author's transl)]. 94 49

The clinical pattern and etiology of 544 cases of cerebral palsy were studied retrospectively. Of these cases, 354 (65.1%) were males. Four hundred and ninety seven (91.4%) cases were of spastic type. Hypotonic, ataxic and athetoid cerebral palsy were observed in 5.5, 1.5 and 1.3% cases, respectively. There was one case each of tremor and mixed type. In the spastic group, quadriplegia comprised the maximum number of cases (34.9%). Hemiplegia (28.7%) and diplegia (21.9) were also common. Mental retardation was found in 47.2%, while speech impairment was observed in 37% cases. Other handicaps included visual (9%), seizures (8.8%), and auditory handicap (2.9%). The etiological factors were prenatal in 7.7% cases, natal in 43.8% cases and postnatal in 26.1% cases. More than one etiological factor was observed in 14.5% cases, while in 7.9% cases, no apparent cause could be found.
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PMID:Cerebral palsy. 807 18

In a child with left hemimegalencephaly and seizures, a reversible operculum syndrome developed when continuous epileptic discharges spread from the left hemisphere to the contralateral central regions. The operculum syndrome lasted for three months until left hemispherectomy was performed. Soon after surgery the seizures and the operculum syndrome resolved. The operculum syndrome is a facio-pharyngo-glosso-masticatory diplegia usually due to structural lesions in both opercular regions. The reversibility of the syndrome in the reported case demonstrates that the operculum syndrome is sometimes functional rather than lesional.
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PMID:Reversible operculum syndrome caused by progressive epilepsia partialis continua in a child with left hemimegalencephaly. 190 90

This article presents a 4-year-old boy who suffered from weakness of the right extremities since birth. Physical examination revealed mild mental retardation and right spastic hemiplegia. No seizures were noted. A brain CT scan showed bilateral clefts along Sylvian fissures, more marked on the left side, which communicated with the lateral ventricle. The septum pellucidum was absent. There was an evident squaring of the frontal horns. The CT findings were consistent with the diagnosis of schizencephaly. When a patient with mental retardation and spastic hemiplegia or diplegia fails to show a history of perinatal cerebral insult, the possibility of schizencephaly should be considered. In that case, a brain CT scan is a rapid and accurate diagnostic tool.
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PMID:[Schizencephaly: report of one case]. 263

Foix, Chavany, and Marie described a syndrome of faciopharyngoglossomasticatory diplegia resulting from bilateral anterior opercular infarction. We describe identical twins who have a developmental form of the syndrome. The twins, aged 41 years, were the product of a normal pregnancy and birth, but had subsequent delayed motor milestones, seizures, poor language development, mild mental retardation, drooling, absent gag reflexes, inability to protrude the tongue, brisk jaw jerks, impaired fine finger movements, symmetrical brisk reflexes, flexor plantar responses, and mildly spastic gait. Magnetic resonance imaging showed bilateral perisylvian cortical dysplasia compatible with polymicrogyria and incomplete opercular formation.
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PMID:Developmental Foix-Chavany-Marie syndrome in identical twins. 378 77

5,928 newborn infants were admitted between the years 1974 and 81 to the University of Innsbruck, Department of Pediatrics. 418 (7%) of them were premature infants with a birthweight of less than 1,500 g. 21 (18%) of the premature infants with a birthweight below 1,000 g survived (21 of 117). Among those 21 surviving infants only 7 (33%) had an appropriate birthweight, 14 (67%) were too small for their gestational age. Survival in the weight range between 1,000-1,500 g was 71% (213 of 301). The percentage of dystrophic infants was 23% (49 of 213). The place of birth has a significant influence on the survival of these very small immature infants. 24% (n = 84) of patients delivered in the nearby Clinic of Obstetrics survived in contrast to only 3% (n = 33) of those born in peripheral hospitals. This is reemphasized by the evaluation of psychomotor development of surviving infants; of 14 patients with normal psychomotor milestones 13 were delivered at our obstetric department. 157 patients were regularly followed up during their first year of life. 43% can be considered normal by all parameters. 57% show some abnormalities in their development during the first years of life. During their second year of life only 13% showed psychomotor problems and this number decreased to 5% in the third year. All these children show varying degrees of hemi- or diplegia and/or seizure activities. A routine check up at 12 month of life seems important as all patients with permanent retardation can be correctly diagnosed at this age.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Development of premature children below a birth weight of 1,500 g]. 398 26

MRI findings of bilateral central macrogyria allowed the diagnosis of a congenital variant of Foix-Chavany-Marie syndrome in four patients aged between 13 and 32 years, with facio-pharyngo-glosso-masticatory central diplegia, mental retardation and seizures.
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PMID:MRI of congenital Foix-Chavany-Marie syndrome. 756 51

The advent of MRI technique has enabled the diagnosis of neuronal migration disorders(NMD) and made it possible to make "in vivo" diagnosis. Congenital bilateral perisylvian syndrome(CBPS) is a recently described disease identify characterized by pseudobulbar palsy, epilepsy, mental retardation, and migration disorders in the bilateral perisylvian area. We have identified four CBPS patients based on neuroimaging and dysarthria patterns among the candidates for epilepsy surgery. All the patients had orofacial diplegia and variable degrees of mental retardation. In the spectrographic analysis of dysarthria, the loss of specific characteristics of formants of vowels and increment of noise in the high frequency formants were observed. Epilepsy was present in all, but only one patient showed intractable seizure requiring surgical intervention. MRI was most helpful in identifying NMD and polymicrogyria in both centroparietal areas in this context. Great alertness is needed to identify this disorder to determine the etiology of epilepsy and dysarthria of uncertain origin.
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PMID:Congenital bilateral perisylvian syndrome: analysis of the first four reported Korean patients. 784 82

Advances in neuroimaging techniques have enabled the recognition of developmental malformations of the brain during life. Careful correlation of clinical and imaging features has identified several new syndromes. We have studied 31 patients with a congenital neurological syndrome characterised by pseudobulbar palsy, cognitive deficits, and bilateral perisylvian abnormalities on imaging studies. All patients had diplegia of the facial pharyngeal, and masticatory muscles, of variable severity. Some patients had slight dysarthria, whereas others were unable to speak. 85% of patients had mental retardation, ranging from mild to severe. Epilepsy was present in 27 (87%) and commonly consisted of atypical absence, atonic/tonic, tonic-clonic seizures, and, less frequently, partial attacks. Seizures were poorly controlled in 55%. Magnetic resonance imaging showed bilateral perisylvian cortical malformations consistent with polymicrogyria, confirmed at necropsy. Division of the corpus callosum in several patients resulted in seizure improvement. This congenital bilateral perisylvian syndrome can be clinically diagnosed and confirmed by imaging studies. Further studies are necessary to elucidate its cause.
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PMID:Congenital bilateral perisylvian syndrome: study of 31 patients. The CBPS Multicenter Collaborative Study. 809 39

Records of 108 patients with lupus erythematosus beginning in childhood (1953-1990) were reviewed; 25 had recorded neurologic findings. This is the largest group of childhood lupus erythematosus patients with neurologic disease that has been reported. The average age of children at the time of diagnosis of lupus was 154 months. There were 22 girls and 3 boys in the group. All patients met at least four of the 1982 American Rheumatism Association criteria for the classification of systemic lupus erythematosus. Average age at onset of neurologic difficulties was 168 months. In 4 patients, the neurologic symptoms preceded the diagnosis: 1 month (spastic diplegia), 1 month (bilateral weakness and spasticity), 24 months (chorea), and 26 months (chorea), respectively. Four patients had neurologic symptoms coincident with the diagnosis of lupus erythematosus. In those patients whose symptoms followed the diagnosis of lupus erythematosus, the average elapsed time until symptoms appeared was 33 months; the single lowest and highest outliers were discounted. Most frequent findings were headache (16/25) and behavioral aberrations (10/25). All behavioral manifestations were depression except in 1 patient. Other prevalent findings included hemichorea or chorea (7/25), cerebrovascular accident with hemiplegia or diplegia (7/25), seizures (5/25), visual loss (3/25), and cranial neuropathy (2/25). Vertigo and myelopathy occurred in 1 patient each. All patients were treated primarily with corticosteroids and azathioprine; in the presence of active disease, the drug dosages were increased with significant improvement in neurologic symptoms. Resolution usually occurred from days to months; most improved in a few days to a few weeks; 3-4 months was the longest period until symptoms subsided.
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PMID:Neurologic characteristics of childhood lupus erythematosus. 855 56


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