CSF beta-endorphin levels in patients with infantile autism.

We measured CSF levels of beta-endorphin, an opioid hormone, in 19 patients with infantile autism and in 3 patients with Rett syndrome, and compared them with control values. In infantile autism, CSF levels of beta-endorphin did not differ significantly from those of age-matched controls. There was no significant correlation between CSF levels and clinical symptoms, including self-injurious behavior, pain insensitivity, and stereotyped movement. However, CSF levels of beta-endorphin were significantly higher in the patients with Rett syndrome than in the control (p < .05). Data suggest that neurons containing beta-endorphin may not be involved in patients with infantile autism. Thus, there is no relationship between dysfunction of brain opioid and autism.

Bilateral opercular syndrome: an unusual complication of perinatal difficulties.

We describe an 8-year old boy with pseudobulbar palsy and speech disturbance associated with fetal distress, mild birth asphyxia and probably intracranial hemorrhage. There is a remarkable dissociation between orofacial voluntary movements and emotional expression. MRI revealed cortical atrophy and subcortical gliosis of both opercula. The clinical and neuroradiological features correspond to bilateral opercular syndrome. We assume that a reduction of cerebral blood flow involving both opercula induced pseudobulbar palsy with severe expressive speech disturbance.

Disseminated intravascular coagulation syndrome in anorexia nervosa.

A 15 year old boy with anorexia nervosa developed disseminated intravascular coagulation syndrome (DIC). Because of severe cachexia he had been admitted to the Shimane Prefectural Central Hospital. During his hospitalization he developed generalized massive ecchymosis. Laboratory data revealed not only DIC but also multiple organ complications. The patient was treated intravenously with FOY (gabexate mesilate, a protease inhibitor), heparin, a transfusion of fresh frozen plasma, antithrombin III concentrates and platelets. Intravenous hyperalimentation was also administered. The laboratory data, the general condition and the emotional state of the patient improved remarkably. We emphasize the importance of keeping in mind coagulopathy as a complication in anorexia nervosa.

[CT findings and prognoses of anoxic brain damage due to near-drowning in children].

We investigated the relationship between serial cranial CT findings and prognoses in 11 children after near-drowning. These patients were rescued after heart arrest for more than 10 minutes and all comatose on admission. CT scans were performed within 2 weeks, at 3 weeks-1 month, 2-4 months and more than 5 months after admission. Characteristics of CT findings and prognoses were classified into four groups. Group 1: low density areas in thalami, basal ganglia and cortical white matters within 2 weeks (three cases; one died, two became vegetative). Group 2: enlargement of the third ventricle at 3 weeks-1 month, and atrophy of pons at 2-4 months (three cases; severe quadriplegia and mental retardation). Group 3: enlargement of the third ventricle at 3 weeks-1 month, but atrophy of pons not observed at 2-4 months (three cases; mild motor disabilities and mild mental retardation). Group 4: enlargement of third ventricle not observed at 3 weeks-1 month (two cases; neither paralysis nor mental retardation).

Varicella with delayed hemiplegia.

We report 4 children who developed acute hemiplegia 7 weeks to 4 months after varicella infection. In 2 patients, carotid angiography demonstrated segmental narrowing and occlusion of the middle cerebral artery. Their clinical and angiographic features were similar to those associated with contralateral hemiplegia after herpes zoster ophthalmicus, the pathogenesis of which comprises cerebral angiitis due to varicella zoster viral infection. We believe that our patients had the same pathogenesis. In a survey of infectious diseases in our region, the frequency of varicella with delayed hemiparesis was roughly 1:6,500 varicella patients.

Multiple system atrophy with retinal degeneration in a young child.

A 4-year-old girl with multiple system degeneration and retinal degeneration was presented. There was onset of an ataxic gait at two years and rapid progression of retinal degeneration, myoclonus and cranial nerve palsy. Neuropathological examination revealed severe degeneration of the cerebellar cortex and the pathways of auditory and deep sensation, as well as degeneration of the cerebellar efferent fibers, the striatonigral system, the cerebellar afferent fiber system and lower motor neurons. Cases of young children with spinocerebellar degeneration have been reported in several families of olivopontocerebellar atrophy (OPCA), but degenerative changes in our case were more widespread than those in OPCA cases. The multiple system lesions in the central nervous system and retina of this child are different from those of any other previously reported cases.

A clinical trial of interferon therapy in a case of rapidly progressive SSPE.

A 6-year-old Japanese boy with rapidly progressive SSPE was reported, who received interferon therapy and recovered from stage III-A to stage II-B according to the criteria of Freeman. This is the first report of the beneficial therapeutic effect of interferon therapy on rapidly progressive SSPE, which is lethal and poorly responded to any kind of previous therapeutic trials.

A morphometric CT study of Down's syndrome showing small posterior fossa and calcification of basal ganglia.

We report characteristic and morphometric changes of cranial computed tomography (CT) with increasing age in 56 patients with Down's syndrome aged from 0 month to 37 years. Patients were compared with 142 normal controls aged 0 to 59 years. Width of ventricles, Sylvian fissures, posterior fossa, pons and cisterna magna were measured on CT. The incidences of the cavum septi pellucidi, cavum vergae and cavum veli interpositi and high density in the basal ganglia were examined. There was high incidence (10.7%) of bilateral calcification of basal ganglia in Down's syndrome, although that of pineal body and choroid plexus calcification was similar in Down's syndrome and controls. Basal ganglia calcification is more frequently seen in young Down's syndrome and may be related to the premature aging characteristic of Down's syndrome. The CT in Down's syndrome showed relatively small posterior fossa, small cerebellum, small brain stem and relatively large Sylvian fissures in those under one year of age. There was a high frequency of midline cava and large cisterna magna. There were no significant atrophic changes on CT except after the fifth decade comparing with controls.