[Intracranial calcification on computerized tomography in childhood: classification and its clinical value].
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Biomedical subjects
Publications and source records attributed to N Goya.
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At a follow-up study of 385 patients with epilepsy beginning under age 15, 22 (5.7%) had died during the first 10 years after the onset of epilepsy and another 11 (2.9%) between 11 and 24 years. Mortality was significantly high in cases with the following clinical features: (1) epilepsy with onset before the first birthday (mortality being 25.5%), (2) symptomatic epilepsy in etiology (17.2%), (3) infantile spasms (40.7%), tonic epilepsy (33.3%) or myoclonic epilepsy (33.3%) as compared with grand mal (5.9%) in seizure type and (4) developmental retardation at the first visit (25.5%). Seizures were not controlled in 31 out of 33 patients at the time of death. The causes of death were status epilepticus or convulsion in 10, pneumonia in 5, severe emaciation in 3, "cerebral palsy" in 5, and drowning, suffocation, traffic accidents or acute lymphocytic leukemia, in one each, and unknown in 6. Most of the patients died at home.
A 5-year-old female patient with a left facial strawberry nevus, ipsilateral optic atrophy and cerebellar signs was observed. Computed tomography and cerebral angiography confirmed left cerebellar hypoplasia and cerebrovascular abnormalities such as internal carotid arterial stenosis, basilar artery occlusion, and a saccular aneurysm of left carotid siphon, all of which are presumed to develop congenitally. This case may represent a new variety of neurocutaneous syndrome which is different from other syndromes associated with facial hemangioma, such as the Sturge-Weber syndrome and the Wyburn-Mason syndrome.
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We report what we believe to be the first known case of linear nevus sebaceus syndrome with infantile spasms followed by Lennox-Gastaut syndrome. The EEG revealed hemihypsarrhythmia and then asymmetrical slow spike-wave complexes. A pneumoencephalogram showed dilation and distortion of the lateral ventricle ipsilateral to the facial nevus on the left side. A computerized tomographic scan disclosed low density in the left parietotemporal region, a feature not reported previously. The findings indicated extensive lateralized cerebral lesions.
In a 2-month-old boy with congenital insensitivity to pain and anhidrosis, the initial symptom was recurrent fever of unknown origin. After eruption of the first teeth, self-mutilation of the tongue was limited by extraction of the teeth. Unmyelinated fibers were essentially lacking, and the number of small myelinated fibers was decreased in sural nerve. This case is the youngest to be diagnosed and the first one in which nerve biopsy clearly showed the characteristic findings.
Spleen cells of female C57BL/6 mice, preimmunized to male histocompatibility antigen, elicited splenomegaly in adult male recipients and caused mortality of the newborn recipients. These cells, upon stimulation in vitro with the male antigen, were cytotoxic to male target cells.
The West syndrome and the Lennox-Gastaut syndrome are characterized by their onset in infancy and early childhood, intractable seizures occurring almost daily, severe psychomotor retardation, and poor prognosis. Among handicapped children, they offer the most serious problems in daily care at home or in institutions because of frequent attacks and marked retardation. A nationwide survey in Japan was performed to elucidate the natural history of these two syndromes.
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Thirty cases of intractable epilepsy in children were investigated to observe the correlation between the suppression of seizure discharges to intravenous diazepam and the type of seizure or effects of subsequent therapy. Suppression of seizure discharges were bilaterally poor in 75% of the cases with infantile spasms and 89% of cases with Lennox syndrome and other generalized seizures while it was unilaterally poor in 80% of the cases with focal or unilateral seizures. ACTH or steroid therapy was clinically and electroencephalographically effective in 80% of the cases with good suppression responses and in none of cases with good suppression that was resistant to the therapy. On the other hand, clinical seizures were not controlled in 73% of the cases showing poor suppression and EEG did not reveal improvement in 82% of the cases with poor suppression. EEG with intravenous diazepam is valuable for understanding the pharmacophysiological mechanism of epilepsy and it may be possible to select cases as candidates for ACTH or steroid therapy using this technique.
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Fifty-four patients with previously positive tuberculin reactions were followed for tuberculin sensitivity, between the incubation period of natural rubella and the 40th day after the onset of rash. In many instances, tuberculin sensitivity was partially or completely depressed beginning in the incubation period and extending for up to 4 weeks after rash onset. Two of 3 cases of subclinical rubella infection also showed this phenomenon. The highest incidence of negative reactions was 83% in the first 3 days after rash onset. A few subjects had a larger skin reaction to tuberculin than the baseline reading during the 2 to 5 weeks after rash onset.
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A sensitive solid phase radioimmunoassay method was established for the specific quantitative determination of secretory IgA (sIgA) by taking advantage of the dual antigenicities of sIgA, one specific for alpha-chain and the other for secretory component (SC). The sIgA and IgA in the sample were first bound by anti-IgA antibodies coated on the polystyrene tube, then the amount of bound sIgA was quantified by the use of 125I-labeled anti-SC antibodies. This method is quite sensitive and allows us to distinguish sIgA from IgA and free SC which usually coexist in exocrine secretions. Linear relationship was observed between the bound radioactivity of radioiodinated anti-SC and the amount of sIgA in the range of 5 to 60 ng of sIgA. With this method, the urinary sIgA levels in normal children and adults were measured. Urinary sIgA was detected in half of infants within 7 days after birth, while it could be demonstrated in almost all of the infants by 14th day. Then, it gradually increased and reached about a half of the adult level by the age of puberty. This method will be useful for the sensitive and specific measurement of sIgA in various exocrine secretions.