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

A 33-year-man with an encephalopathy of unknown aetiology, had an history of epilepsia for 30 years. Different types of seizures were seen, including grand mal and frontal attacks. Epilepsia was associated with mental retardation and behavioral disorders. At the age of 33, he was admitted for repetitive general convulsions. Epilepticus status lasted for two weeks and improved with vigabatrin et clonazepam. General seizures, frontal motor convulsions with arms and trunk antepulsion, and dacrystic attacks were seen. The latter seemed to be like normal crying because they were accompanied by lacrimation, contorted and mournful facies, and sobbing sounds. One year later, repetitive cardiac arrests occurred during a new epilepticus status. Cardiac arrests, observed on ECG holter lasted 10 to 24 seconds, without cardiac dysfunction. EEG patterns on ECG holter lasted 10 to 24 seconds, without cardiac dysfunction. EEG patterns included theta and delta activity with rhythmic slow wave epileptic activity, predominating on right side, in temporal areas. CT scan was normal. MRI showed right cerebral atrophy, prevailing in the temporo-mesial region, with right temporal horn enlargement. This case report of dacrystic seizures, the first one with MRI study, suggests that temporo-mesial structures of the non-dominant hemisphere may be involved in dacrystic and asystolic attacks.
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PMID:[Dacrystic and asystolic epileptic seizures]. 748 7

Recent effective treatments for subacute sclerosing panencephalitis (SSPE) are oral inosiplex and intrathecal interferon therapy. The former seems to be effective for life expectancy but not for neurological disability, and the latter is a debating antiviral treatment although favorable reports are increasing. A 12 year-10 month-old boy with SSPE was presented as a successful case of prolonged effectiveness in both neurological symptoms and life expectancy. There are no significant side effects by the 200 weeks treatment of intrathecal alpha-interferon of large dose (6 million unit/dose/week) and ordinary dose of inosiplex (100 mg/kg/day). Neurological disability index (Dyken) improved from 70% to 10%; speech and higher cerebral functions improved from aphasia and almost vegetative states to verbal communicable level; motor dysfunction from bedridden to wheel chair level; and myoclonic and other seizures were controlled. Laboratory data also improved; periodic synchronized discharge (PSD) and other paroxysmal discharges disappeared and alpha activity that had once disappeared in the background activity of active stages reappeared in the EEGs. Measles antibodies in serum and CSF improved, and oligoclonal bands disappeared. However diffuse brain atrophy remained on neuroimaging. Long-term intrathecal large dose treatment with alpha-interferon is effective and safe. It should be started as soon as the diagnosis is made, and a trial of large-dose and long-term therapy may be worthy even for more advanced cases irrespective of progress of neuroimages.
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PMID:[Successful treatment of subacute sclerosing panencephalitis with long-term intrathecal large dose of alpha-interferon--a case report]. 754 89

Over a 3-year-period (Dec. 1990-Nov. 1993) 12 children were found PCR-positive for CMV-DNA in CSF and brain biopsies. Three of the patients were immunologically compromised. During the same period CSF samples from 10 shunt-operated children and 143 virological routine CSF samples were PCR CMV negative. Clinical association with positive PCR-CMV reaction was considered likely in 6 patients: two boys developed prolonged fever and meningoencephalitis following neurosurgery, one infant girl had a course compatible with congenital inclusion disease, and three had prolonged fever following transplantation. Clinical association was deemed probable in 3 infant girls: one had neonatal infection, meningitis and intraventricular haemorrhage, one had neonatal encephalitis and failure to thrive, and one with neonatal seizures and encephalitis developed brain atrophy. Clinical association was judged possible in 3 patients: one infant girl with no signs of encephalitis developed brain atrophy, one had an Aicardi Type 1 syndrome and one 2 1/2-year-old boy had an acute encephalitis with insufficient serological support for CMV but was 12 months later PCR positive for CMV. We conclude that CMV may be an overlooked infectious agent of the CNS also in immunocompetent children. PCR aids in rapid diagnosis of CMV infection in the immunocompromised. CMV may occasionally be disclosed with PCR in other conditions as a probably non-relevant observation.
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PMID:Clinical, serological and PCR evidence of cytomegalovirus infection in the central nervous system in infancy and childhood. 777 Jan 29

In this study, 78 patients with aqueductal stenosis were submitted to detailed neurodevelopmental assessment with a follow-up of 5-25 years. Sixty-eight percent of patients were categorized as normal; they either attended normal school courses or had regular jobs. Among these, 34% had some motor abnormalities (ataxia, mild hemiparesis, visual disturbances). Twenty-four percent (19 cases) were moderately disabled (trainable retardation) and 8% (6 cases) were severely handicapped. Epilepsy was observed in 13% of the cases. Incidence of recurrent and generalized seizures paralleled neurodevelopmental outcome (5% in normal, 16% in moderately disabled and 50% in severely disabled patients). Endocrine dysfunctions were evident in 28% of the cases and were characterized by precocious or delayed puberty, amenorrhea and somatic underdevelopment. No patient with ventricular enlargement and a cortical mantle width below 20 mm showed a good outcome. Large ventricles were compatible with normal mental development when compensated with a corresponding cranial vault enlargement. In patients with normal mental status and motor abnormalities, long-term CT scan findings revealed the presence of focal brain abnormalities (poroencephaly, brain atrophy, calcifications, extracerebral collections).
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PMID:Long-term outcome in aqueductal stenosis. 777 81

Computed tomographic (CT) brain scan was performed on 142 consecutive epileptic patients of Saudi Arab extraction without clinical features of acute illness or a progressive neurological disorder at presentation. CT brain scan was normal in 56 patients (39%). CT scan was abnormal in 57 (73%) of 78 patients over 20 years of age compared with 29 (45%) of 64 younger patients (x2 = 11.3; p < 0.001); and in 25 (75%) of 36 patients presumed to suffer from symptomatic epilepsy on clinical grounds compared with 36 (46%) of 76 others with idiopathic epilepsy (x2 = 8.2; p < 0.01). Potentially treatable lesions were detected in 11 (8%) patients and congenital lesions in 9 (6%). Multiple lesions were present in the CT scan of 20 patients. Cerebral atrophy (CA), the commonest lesion detected, was present in 52 (37%) of 142 patients and 60% of 86 patients with abnormal CT scans. Diffuse CA was present in 21% of 78 patients over 20 years of age compared with 14% of 64 younger patients. Similarly, diffuse CA occurred in 25% of 48 patients with late-onset epilepsy compared with 14% of 94 patients in whom seizures began before the age of 20 years. Our findings are in accord with those previously reported and underscore the need for selective and judicious use of CT scan facilities in epileptic patients.
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PMID:Computed tomographic brain scan findings in Saudi epileptic patients. 787 89

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

A study of 72 alcoholics, hospitalized for alcohol withdrawal syndrome, was undertaken to determine the incidence of seizures, their relationship with other withdrawal symptoms, the presence of brain atrophy and the relationship of this last with withdrawal intensity severity. Sixty-seven (93%) were male and the mean age was 44.9 +/- 1.3 (mean +/- SEM) years. Thirty-three (46%) of the 72 patients had seizures at admission, 10 of these developed minor withdrawal symptoms, in 18 delirium tremens ensued and 5 showed no symptoms of withdrawal. Thirty-nine (54%) had withdrawal syndrome without seizures. Twenty-one of these developed minor withdrawal syndrome and 18 delirium tremens. Seizures showed no relationship with the other withdrawal manifestations, and in all the cases preceded them. Our findings also show that alcoholics with seizures due to withdrawal are more prone to suffer seizures in their future withdrawal episodes, and that alcoholics who suffer morning withdrawal symptoms are prone to develop delirium tremens. In 46 patients a CT scan was performed. Though the alcoholics showed ventricular and sulcal enlargement, brain atrophy was similar when the seizure and non-seizure groups or those with and without delirium tremens were compared. However, cortical and ventricular atrophy were related to the existence of previous episodes of withdrawal syndrome [corrected].
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PMID:Alcoholic withdrawal syndrome and seizures. 794 73

Chronic alcoholism is associated with hypercortisolemia and low serum zinc (Zn). Hypercortisolemia could be responsible for alcoholic cerebral atrophy and is also associated with enhanced NMDA neurotoxicity. It is hypothesized that low brain Zn, noted in chronic alcoholics, enhances NMDA excitotoxicity and ethanol withdrawal seizure susceptibility. Also, Zn deficiency can produce neuronal damage through increased free radical formation. Clinically, Zn replacement therapy may be a rational approach to the treatment of alcohol withdrawal seizures and alcohol-related brain dysfunction.
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PMID:Zinc deficiency and corticosteroids in the pathogenesis of alcoholic brain dysfunction--a review. 797 2

Progressive encephalopathy, developmental delay, microcephaly, electroencephalogram (EEG) and computed tomographic (CT) scan abnormalities have been reported in 80% of children with chronic renal failure (CRF) in infancy. Malnutrition, aluminium intoxication and psychosocial deprivation are proposed as causes. In 15 children with CRF from infancy we evaluated the effect of no aluminium salts and early vigorous nutritional and psychosocial support, in addition to the standard therapy, on neurological development. Six patients underwent dialysis (2 at birth) and 3 received transplants. None of our patients were given aluminium therapy. The nutritional status of the patients in the first 2 years of life was assessed with the waterlow classification. At the end of the follow-up period (mean 50 months range 14-148 months), patients underwent neurodevelopmental assessment, head CT scan, EEG, nerve conduction velocity (NCV) and auditory brain stem evoked response (ABER). None of our patients developed progressive encephalopathy or recurrent seizures. All have a normal neurological examination apart from hypotonia. Microcephaly was present in 5 patients. There was a good correlation between malnutrition in the first 2 years of life and microcephaly. Developmental delay was present in 3 patients; all 3 were microcephalic. There was evidence of brain atrophy on CT scan in only 3 patients. EEG was abnormal in 6 patients, but only severe in 1 patient. Only 1 patient had diminished NCV; all patients had a normal ABER. We conclude that a policy of no oral aluminium therapy and early nutritional support leads to better neurological outcome in children with CRF from infancy.
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PMID:Improved neurological outcome in children with chronic renal disease from infancy. 801

Sixteen patients (8 female, 8 male) with primary angiitis of the CNS (PACNS), were followed prospectively in a vasculitis clinic. Diagnosis was by angiography in patients without underlying disease. Median age at diagnosis was 36.5 years, and median duration of follow-up was 28 months. Onset was acute in 14 patients (88%), with 3.5 weeks (median) from onset symptoms to diagnosis. Three women developed symptoms within 3 weeks postpartum. The most frequent symptoms were severe headaches (12, 75%), stroke (6, 30%), transient ischaemic attack (TIA) (4, 28%), seizures (7, 44%), visual aberration (3, 19%), and cognitive impairment (5, 31%). Laboratory data included high ESR (2, 13%), leucocytosis (8, 80%), thrombocytosis (1, 6%), positive antinuclear antibody titre (3, 15%), and high levels of complement (5, 31%). Lumbar puncture was performed in 12 patients (75%). CSF analysis was abnormal in five patients (42%). EEG was abnormal in 5/9 patients. The major CT/MRI scan findings were cerebral haemorrhage (4, 25%), brain infarcts (5, 31%), brain atrophy (2, 13%) and non-specific lesions (2, 13%). Four patients had normal studies. All patients received corticosteroids (CS), and five were treated with oral cyclophosphamide. Two patients relapsed despite CS and cyclophosphamide therapy. All patients are alive, and at the last assessment, eight had a permanent neurological deficit, which included paresis (3, 19%), neurocognitive abnormalities (2, 13%), visual loss (2, 13%) and seizure activity (5, 31%). Our data suggest a non-progressive, non-fatal course in those PACNS patients diagnosed angiographically and treated with CS with or without cyclophosphamide.
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PMID:Primary angiitis of the CNS diagnosed by angiography. 804 67


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