Infantile spasms: etiological factors, clinical aspects, and long term prognosis in 200 cases.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to T Negoro.
Explore the source record for details and available documents.
We investigated the prognostic factors for mental and physical development and seizure control by dividing the subjects into various etiologic groups in 200 patients with infantile spasms, all of whom (except 48 who died) were aged six years or older. The results were as follows: 1) Intermediate (4-12 mos) onset was found to be a favorable prognostic factor for seizure control in cryptogenic cases, although there was no relation between the age of onset and prognosis in other etiologic groups. 2) There was a significant correlation between the treatment lag and long-term prognosis for mental and physical development only in cryptogenic cases. A short treatment lag (0-2 mos) was associated with a good prognosis. All cryptogenic patients who had no relapse after ACTH therapy developed normally, although in symptomatic cases, there was no correlation between the relapse and the outcome.
A follow-up study was made on 200 children (115 boys, 85 girls) who had had infantile spasms, in order to compare their present condition over the age of six years with various prognostic factors. 48 of the children (30 males and 18 females) had died, and all the rest were aged six years or older at the time of final follow-up. 139 of the children had received ACTH therapy: at final follow-up, spasms had ceased in 43.5 per cent, and about the same proportion showed normal physical development; 23 per cent had normal mental development and 15.4 per cent were attending ordinary schools. Complete recovery (normal mental and physical development and attending ordinary schools) was achieved in only 19 cases (9.5 per cent). Of the cryptogenic cases, 44.4 per cent had made a full recovery. The poor prognostic factors for continuing seizures were evolution into other types of fits, relapse of seizures after ACTH therapy, seizures concomitant with spasms, and convulsions before the onset of spasms. Poor prognostic factors for physical development were delayed development before the onset of spasms, neurological abnormalities, PEG abnormality, symptomatic aetiology, neonatal convulsions, low birthweight, perinatal asphyxia and being female. Poor prognostic factors for mental development were delayed development before the onset of spasms, neurological abnormalities, PEG abnormality, prenatal and perinatal aetiology, relapse after initial ACTH therapy, laughing attacks, and evolution into other types of fits. Only in the cryptogenic cases was there significant correlation between the delay in treatment and the long-term prognosis for mental development. Poor prognostic factors for educability were very similar to those for mental development. In spite of conflicting views as to the long-term effects of ACTH, prompt treatment seems to be mandatory, at least in cryptogenic cases of infantile spasms.
radioimmunoassays have been developed which enable accurate and sensitive determination of gliclazide in human serum. Antisera A and B against gliclazide were obtained from guinea pigs immunized with conjugates A and B prepared by coupling gliclazide homologues, 1-(p-toluenesulfonyl)-3-(4'-carboxypiperidino)urea and 1-(4-methyl-3-carboxybenzenesulfonyl)-3-(3-azabicyclo[3,3,0]oct-3-yl)urea, to bovine serum albumin. 3H-Gliclazide was used as a tracer. Dextran-coated charcoal was used to separate bound and free 3H-gliclazide in the reaction mixture. The assays of gliclazide in serum were possible over a concentration range from 0.25 to 20 microgram/ml with the antiserum A and from 0.1 to 10 microgram/ml with the antiserum B, respectively, using 0.01 ml of human serum without the need for an extraction procedure. The antisera used for the assays were specific for gliclazide. Data obtained by the radioimmunoassay with the antiserum A are in good agreement with those by the radioimmunoassay with the antiserum B and gas-liquid chromatography. Serum levels of gliclazide in healthy volunteers receiving single oral dosing (40 mg/subject) have also been determined.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The influence on pregnancy of sensitized T lymphocytes against male histocompatibility antigens was investigated using inbred strain mice. Number of abortion, litter size, fetal weight and placental weight were studied on 18th day of pregnancy in the mice which were administered intravenously with T lymphocyte enriched fraction of sensitized spleen cells against allogeneic male histocompatibility antigens. The incidence of abortion in the experimental mice was higher than that in control mice. Mean fetal weight in the experimental mice was less than that in the control mice although mean placental weight in the experimental mice was unaffected. Those results show that immunologic interruption of pregnancy due to histoincompatibility between mother and fetus can occur and that the immunologic interruption of pregnancy is caused by sensitized T lymphocytes against paternal inherited histocompatibility antigens. Possible process of the immunologic interruption of pregnancy is discussed.
Forty-six 1-cycloalkyl-4-(1,2-diphenylethyl)piperazines were synthesized. The influence of substituents on phenyl groups of 1-cycloalkyl-4-(1,2-diphenylethyl)piperazines 4a-c on the analgesic activity was investigated in experimental animals. The most active compounds, 5a-c, in this series had a m-hydroxyl group on the 2-phenyl group of 4a-c, while morphine has a phenolic hydroxyl group para to th- aminoethyl moiety. Their activities were 23-56 and 23-38 times those of their original compounds 4a-c and morphine, respectively, tested by the D'Amour-Smith method after subcutaneous administration.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The clinical symptoms and electroencephalographic (EEG) features of typical absence seizures, including transverse topographical analysis (TTA), were studied retrospectively using a simultaneous EEG-VTR system in 36 children aged between 3 and 17 years. Complex absences were more common than simple, and oral automatisms were the most frequent complex symptoms. Occurrence of complex symptoms was directly proportional to the length of seizures. There was no association between lateral TTA type and simple absences. Prognosis was favorable in patients with long seizure duration (P < 0.01), bilaterally synchronous monomorphic spike-waves throughout the seizure discharges (BSMTSD) (P < 0.005) and lateral TTA type (P < 0.05). Two different mechanisms of origin for BSMTSD/non-BSMTSD and lateral/non-lateral TTA types are probably responsible for the difference in prognosis. Favorable prognosis in patients with long seizures is discussed in relation to the duration of the illness. EEGs with BSMTSD and lateral TTA type may indicate a favorable prognosis in children with absence seizures.
Nine infants with benign complex partial seizures, diagnosed by simultaneous electroencephalogram and video recordings, are described. At mostly 3-10 months of age, these infants demonstrated clusters of seizures which consisted of motion arrest, decreased responsiveness, staring or blank eyes mostly with simple automatisms, and mild convulsive movements associated with focal paroxysmal discharges. The seizures were controlled easily with carbamazepine or phenobarbital and all patients remained seizure-free for more than 3 years. Four patients had family histories of benign types of infantile convulsions. Interictal electroencephalogram and psychomotor development were normal in all patients.
Three children with moyamoya disease are reported whose initial and predominant manifestations were choreic movements. Two of the patients presented with unsteady gait and the other with clumsiness. Choreic movements were recurrent and were often triggered by excitement, emotional tension, or crying. They occurred unilaterally or bilaterally and often alternated between the right and left. Moyamoya disease must be considered in the differential diagnosis of acquired chorea in children.
Positron emission tomography (PET) using 18F-labeled 2-deoxy-D-glucose was performed serially in 5 infants with idiopathic West syndrome. While tonic spasms persisted, 2 infants had hypometabolism in the bilateral temporo-parieto-occipital regions, which disappeared after cessation of spasms. In 2 other infants, PET revealed focal hypometabolism in the temporal region a few months after the disappearance of tonic spasms, but subsequent PET studies were normal. PET can detect transient metabolic abnormalities of the cerebral cortex which may be associated with the pathophysiology of West syndrome.
We describe the clinical and electroencephalographic (EEG) manifestations of four patients with simultaneous EEG-video-telemetry recording (VTR) documented partial seizures evolving to infantile spasms. Clinical manifestations of the partial seizures included cessation of activity, staring, automatisms, increased limb tone, and laughter. In each case, partial seizures were followed by a cluster of infantile spasms. Infantile spasms preceded by partial seizures have not been previously documented.
Seven infants with benign idiopathic partial epilepsy presented with apparently generalized tonic-clonic seizures (GTCs) which proved to be partial seizures evolving to secondarily generalized seizures. All were easily controlled with phenobarbital (PB) or carbamazepine (CBZ), and remained seizure-free > 3 years even after discontinuation of medication, and had a normal developmental outcome. The initial seizure manifestation consisting of motion arrest, staring or blank eyes, or crying associated with focal paroxysmal discharges was followed by GTCs in accordance with generalization of paroxysmal discharges. Four infants had a family history of benign type of childhood convulsions. The term "benign partial epilepsy in infancy" is proposed to combine this syndrome with the previously described benign infantile epilepsy with complex partial seizures.