Our experience of an anaphylactic reaction to latex in a severely disabled patient led us to investigate latex allergy in 58 cases with severe motor and intellectual disabilities syndrome.
Latex specific IgE, total serum IgE, and eosinophil counts in peripheral blood were evaluated, as well as past history of treatment with medical latex materials, operation and allergic disorders.
Only one case who had been operated three times, had anaphylactic reaction and mild atopic dermatitis.
Fifteen cases (25.9%) had latex specific IgE of class 2 or more. In this latex positive group, past history of allergic disorders, such as bronchilal asthma, atopic dermatitis and drug eruptions, were the most prominent factor. Those without such a history were characterized by frequent use of medical latex materials and multiple operations.
In conclusion, the danger of an anaphylactic reaction to latex should be recognized, particularly in the medical care for disabled patients.

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On routine medical examination of one-month-old infants, general movements (GMs) were videorecorded for about 5 minutes of 252 infants born at Kyoto City Hospital or other hospitals, including the infants discharged from the NICU. Their ages ranged from 39 postmenstrual to 8 post-term weeks.
One of the experimenters assessed 181 infants among 252 available for assessment of their GMs twice at intervals of more than one month without being informed about their clinical histories. The subjects included 19 low-birthweight infants, and their average age was 3.5 post-term weeks. The assessment was carried out in comparison to the “gold standard” GMs depicted in a demonstration video produced by the GMs Trust. The Kappa value for intra-observer concordance was 0.85. Twenty (11.0%) out of the 181 infants were judged abnormal because of their poor repertoire of GMs in both the first and second assessment. Neither cramped synchronized GMs nor chaotic ones were observed. Meanwhile, 155 (85.6%) out of the 181 infants were found to be normal. The incidence of abnormal GMs was significantly higher among infants with history of asphyxia and/or respiratory distress than among low-risk infants. Three of the experimenters independently assessed GMs of 50 infants among 181 which had been video-recorded between June and September 1998. The inter-observer concordance was higher among the low-birth weight infants than among fullterm ones.
In conclusion, qualitative assessment of GMs by an experienced observer at routine medical examination of one-month old infants is a useful method for the follow-up of high-risk infants.

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In the past 10 years, we have treated 25 patients with chronic respiratory insufficiency due to a progressive or degenerative brain disorder. Ten patients died and the other 15 survived. Five of the former and 12 of the latter received life-sustaining mechanical ventilation care.
Even in the terminal stage of progressive or degenerative brain disorders, patients can survive for a longer period than previously, if life-sustaining mechanical ventilation care is given. In Japan we do not have a guideline for medical indication or decision-making for children with progressive or degenerative brain disorders. Whenever we see such patients, we have great difficulty in making a decision. It may therefore be necessary to discuss whether we should have such a guideline.

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We presented 3 cases who exhibited important clinical or histopathological findings relevant to the pathogenesis of acute encephalopathy with shock syndrome (AESS). Hyperpermeability of the blood vesseles in the central nervous system was revealed by autopsy in case 1 and by enhancement CT scan in case 2, which implicated that the primary lesion of AESS was vascular damage. Although other cytokines than interleukin (IL)-6 were not evaluated, high value of IL-6 in case 3 might suggest that the some highly activated cytokines caused AESS.

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We report here a nine-year-old girl with multiple sclerosis having consciousness disturbance at adm is-sion. Neurological examination revealed drowsiness, unstable emotion, decreased visual acuity, disturb ance of convergence, and clumsy coordination movements. Her cerebrospinal fluid IgG and myelin basic protein w e r e increased. Electroencephalogram showed intermittent, high voltage slow waves predominant in the frontal lobes. Magnetic resonance imaging (MRI) found multiple demyelinating plaques in the brainstem, thalamus, periventricular white matter. The brainstem reticular formation was involved. Since she had had bilateral acute optic neuritis and papillitis two years before the admission, the diagnosis of multiple sclerosis was made. Methylprednisolone pulse therapy improved her neurological symptoms and MRI findings. Multiplesclerosis in children, unlike that in adults, may present with symptoms mimicking an encephalopathy. Our case suggested that consciousness disturbance in childhood multiple sclerosis results from lesions in the brainstem activating reticular formation including the thalamus.

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Interleukin-6 (IL-6) levels in the cerebrospinal fluid (CSF) and serum were measured in two immunocompetent children with herpes zoster meningitis, who had vesicles, fever, headache and vomiting before admission. The causative agent was identified as varicella zoster virus (VZV) by detecting an increased antibody index in the serum and specific DNA (by polymerase chain reaction) in the CSF. Both patients fully recovered after treatment with acyclovir. The CSF IL-6 levels were high (260.1pg/ml, 106.1pg/ml) at the acute stage and thereafter showed a rapid recovery. The serum IL-6 levels were normal. The increased IL-6 level in the CSF may reflect intrathecal inflammatory response following invasion of VZV into the central nervous system.

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We report a 10-month-old female infant with Leigh encephalopathy caused by a T to G mutation at nucleotide 8993 of mitochondrial DNA. Initial manifestations were diarrhea and pyrexia, followed by disturbance of consciousness. Blood chemistry showed lactic acidosis, and cranial T₂ weighted magnetic resonance imaging demonstrated symmetric high-intensity areas in the basal ganglia, consistent with Leigh encephalopathy. Analysis of urinary organic acids revealed a increase of alpha-ketoglutamate. Derivatives of branched chain amino acids, which accumulate in maple syrup disease, were also increased. Lipoamide dehydrogenase (E3) deficiency was initially suspected; however, normal activity of pyruvate dehydrogenase complex excluded the diagnosis. The organic aciduria disappeared after two weeks. The CNS lesions in our case were observed more prominently in the floor of the bilateral frontal lobes than in the globus pallidus and putamen. In this case, mitochondrial DNA mutation may have caused organic aciduria and the atypical imaging findings.

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[in Japanese]

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[in Japanese]

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