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

The therapeutic responses of seven children with Wilson's disease who presented with neurological disease were evaluated. Neurological abnormalities comprised intellectual deterioration in 7, conduct disorder in five, dystonia in three, choreoathetosis in three, seizures in one and hemiparesis in one. Lethargy and weight loss were present for several months in 6 children. Four children had clinically demonstrable liver disease which was fatal in two. Electroencephalography performed in two children was normal. Computed tomography (CT) of the brain in three children showed cerebral atrophy in all and areas of low attenuation in the basal ganglia which resolved on treatment in one. All patients were treated with penicillamine but, in four, triethylene tetramine (TETA) was substituted because of adverse effects. Neurological abnormalities in these patients were reversible.
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PMID:Neurological abnormalities in Wilson's disease are reversible. 356 99

The diagnosis of the childhood forms of neuronal ceroid lipofuscinosis is considered when a child presents with seizures, dementia, and pigmentary change in the retina. A diagnosis is based on the result of skin or conjunctival biopsy. We report two children who had CTs obtained at the onset of seizures and prior to the occurrence of intellectual deterioration or retinal pigmentary changes. In both cases, the CT showed marked enlargement of the fourth ventricle and cerebellar atrophy without concomitant cerebral atrophy. We suggest that this cerebellar atrophy may be an early sign of ceroid lipofuscinosis and may be of particular note when seen on the CT scan of a child with a recent onset of seizures.
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PMID:CT in ceroid lipofuscinosis. 358 22

We reviewed the CT findings of 387 patients with new-onset seizures after the age of 50. Seizures were generalized in 212 patients, focal in 160, and indeterminant in 15. CT scanning revealed cerebral atrophy in 113 cases, ischemic lesions in 75, cerebral neoplasms in 20, and no abnormality in 177 cases. Tumours were found in only three patients with generalized seizures, and all three had focal neurological deficits at the time of CT diagnosis, while 17 neoplasms were discovered in patients with a focal seizure disorder. The majority of patients with late-onset epilepsy have a normal CT scan with cerebral atrophy being the most common abnormality detected. Cerebral vascular disease appears to be the most frequently identified cause of late-onset epilepsy, while cerebral neoplasms are uncommon.
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PMID:CT findings in late-onset epilepsy. 366 70

Xeroderma pigmentosum is an unusual neurocutaneous disorder. Recent studies have classified patients with xeroderma pigmentosum into 10 groups by somatic cell hybridization methods. In this report we describe 32 patients with Group A xeroderma pigmentosum, including 1 patient with an atypical case, who were assessed for neurological complications. Of these patients, 17 had microcephaly, 13 short stature, and 21 mental retardation. In patients over 7 years of age, sensorineural deafness and spinocerebellar signs such as nystagmus, dysarthria, tremor, and ataxia were frequently observed; no patients below 7 years of age had such neurological complications. Electroencephalographic studies revealed abnormal slow and low voltage background activity. Two patients had focal abnormal discharges, one of whom developed versive seizures. Cranial computed tomographic scans revealed abnormalities, including ventricular dilatation, cerebral atrophy, cerebellar and brainstem atrophy, and cranial bone thickening. A patient with an atypical case of Group A xeroderma pigmentosum had less skin and neurological involvement, and higher levels of postultraviolet colony-forming ability and host cell reactivation than did a typical Group A case. It is possible that these less severe cytological findings are responsible for the less severe skin lesions and neurological complications noted clinically.
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PMID:Neurological manifestations in xeroderma pigmentosum. 374 Aug 15

The consequences of alcoholism on the peripheral and central nervous system are discussed. Polyneuropathy is present in 30% of the alcoholics, whilst cranial nerve involvement is found in 5-25%. Alcoholic myopathy is only very rarely seen. Wernicke's encephalopathy is found at post mortem investigation in 1.8% of alcoholics, but is rarely clinically diagnosed. The Marchiafava-Bignamy syndrome and central pontine myelinolysis are rarely seen; alcoholic amblyopia which is seen in 0.5% of the hospitalised alcoholics is more frequent, but still a rare finding. Cerebral seizures are common in chronic alcoholics with an incidence varying from 5 to 37% according to the type of drinking habit and have, thus, to be categorised. Brain atrophy is a common finding and correlates with the duration and extent of the alcoholism.
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PMID:[Neurologic sequelae of alcohol]. 378 82

A ten months old male infant was found to have a de-novo-deletion of the long arm of chromosome 11 (q23----qter). With regard to previous reports the combination of the following signs seems to be pathognomonic for partial monosomy of the long arm of chromosome 11: trigonocephaly, abnormal palpebral fissure, epicanthus, broad and depressed nasal bridge, carp shaped mouth, low-set malformed ears and statomotoric retardation. Cerebral atrophy, BNS-seizures, hypsarrhythmia and persistent leukocytopenia have not yet been described. Three distinct types of severity and probably correlated prognosis seem to exist.
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PMID:[Clinical picture of partial monosomy of chromosome 11 q]. 380 20

The clinical and computed tomographic (CT) findings in 17 patients with neurological manifestations of systemic lupus erythematosus were analyzed. Two patients presented with stroke syndromes; they did not have systemic manifestations of SLE at the time the neurological disturbances developed. Of 15 patients with prominent systemic evidence of SLE, 9 presented with stroke syndromes or seizures. CT showed a hypodense lesions representing infarction (7 cases) or hyperdense lesions representing intracerebral hematomas (2 cases). Six patients with gradual onset of neurobehavioral symptoms showed cerebral atrophy without infarction or hemorrhage.
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PMID:The cranial computed tomographic findings in patients with neurologic manifestations of systemic lupus erythematosus. 395 92

We observed a sudden respiratory arrest in four term newborn infants after a clinically symptom-free period. There were no cardiac, pulmonary or metabolic changes responsible for these events. Signs of cerebral dysfunction existed (muscular hypotonia, jitterness, seizures). Cranial computerized tomographic scans were performed during the period of respirator treatment within the first week of life. The density of the brain structures was significantly decreased in all cases, three of the patients showed a complete compression of the lateral ventricles. These findings indicated severe brain swelling as a consequence of cerebral hypoxia. There was a history of umbilical cord occlusion in two cases. In the remaining patients we must assume an undetected hypoxic-ischemic episode prior to the onset of labor. We used hyperventilation, corticosteroids, phenobarbital, diuretics and fluid restriction for therapy. Later on the babies received special physiotherapy. Control CT-scans were performed during the fourth or fifth week of life. The findings were normal in one patient. Signs of mild focal brain atrophy developed in two babies. A more severe cortical atrophic lesion of both temporal lobes was found in one patient. He suffered from a slight cerebral palsy. No neurodevelopmental handicaps could be found in all the other patients on long term follow-up. The EEG examination was performed between the fifth and seventh month of life. No pathologic changes were observed. We conclude that severe generalized brain edema in the newborn is not necessarily followed by extensive brain damage. We think it important to develop more sensitive methods for detecting a hypoxic ischemic crisis preceding the birth.
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PMID:[Clinical and computed tomographic observations on newborn infants with generalized brain edema due to perinatal asphyxia]. 397 83

Blindness is a feature of the group of storage diseases of children known as the ceroid-lipofuscinoses. Sequential studies in the ovine model, which most resembles the juvenile form of human disease, showed clearly that blindness had two components, a central and a peripheral. Whereas the central component, attributable to neuronal death and atrophy of the cerebral cortex, was responsible for early loss of vision, retinal atrophy was also extremely advanced in terminal stages of the disease. The primary retinal change was one of dystrophy of photoreceptor outer segments which preceded degeneration and necrosis of the photoreceptors cells themselves. Electroretinography showed that there was a progressive loss of a- and b-waves during the course of the disease, but this was preceded by a diminished c-wave which was eventually replaced by a negative potential. However, the pigment epithelium remained functionally (azide responsive) and ultrastructurally intact throughout the study. Loss of brain weight with selective cerebral atrophy also correlated with abnormal behaviour and facial manoeuvres that were interpreted as partial seizures that did not become generalized.
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PMID:Ceroid-lipofuscinosis (Batten's disease): pathogenesis of blindness in the ovine model. 405 72

Using a specific radioimmunoassay we have measured somatostatin-like immunoreactivity (SLIR) of CSF in patients with brain atrophy, spinal spasticity, seizures, brain tumors and inflammatory disorders. Patients with marked brain atrophy had significantly decreased somatostatin levels in CSF. In patients with spinal spasticity significantly higher levels were observed. Seizure patients had reduced levels but the difference was not significant. In patients with inflammatory disorders and malignant brain tumors SLIR levels were significantly elevated but not in patients with benign brain tumors. A possible pathophysiologic meaning of SLIR in spasticity and seizures is discussed. The altered levels in brain atrophy, tumors and inflammatory disorders are probably indirect signs of altered somatostatin turnover or increased somatostatin leakage from damaged CNS.
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PMID:Somatostatin-like immunoreactivity in the cerebrospinal fluid of neurological patients. 612 89


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