The clinicopathological features of a previously unrecognised type of acute encephalopathy prevalent among Japanese children is described by reviewing the records of 13 consecutive patients treated and 28 previously reported cases.

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Owing to improved technology and care for patients who need mechanical ventilation, the quality of life as well as the prognosis for long‐term ventilator‐assisted patients has improved significantly in recent years. However, the increased number of these patients has raised economic, ethical and medical problems. In order to assess the magnitude of these problems, we conducted the first nationwide survey on the status of long‐term ventilator‐assisted children in Japan. Questionnaires were mailed to 2524 pediatric departments at hospitals in Japan with more than 100 beds. At the time of the survey, 282 hospitals had 567 patients who had been ventilated for more than a month. Among these patients, 434 were younger than 20 years and had been ventilated for more than 3 months. The most common basic disorders were: various myopathies (n = 65), hypoxicischemic encephalopathy (n = 60), spinal muscular atrophy type 1 (Werdnig‐Hoffmann disease, n = 55), chronic lung disorders of prematurity (n = 21), Ondine's curse (n = 22), drowning (n = 17) and congenital heart diseases (n = 16). Of these 434 patients, only 61 were ventilated at home. Although home care was considered suitable for chronic ventilator patients by many pediatricians who responded to the survey, its realization has been hampered by the lack of a system and regulations to support it. The fact that many pediatricians in Japan have actively prolonged the life of Werdnig‐Hoffmann patients, from whom aggressive life saving measures have been withheld in most Western countries, has raised ethical as well as medical issues.

Profound abnormalities of the brain were noted in a 6-year-old Japanese boy with congenital muscular dystrophy (CMD). Pathological alterations included diffuse cerebral and cerebellar micropolygyria, with bilateral temporal agyria, and abnormal fusion of gray matter in the basal portions of both frontal hemispheres. Microscopically, the architecture of both cerebral and cerebellar cortices was severely distorted, with irregular arrangement of neurons and increased vascularization. Skeletal muscles showed dystrophic changes rather than neurogenic atrophy. Eight autopsy cases of CMD with similar pathologic findings have been reported in Japan, although the lesions in the brain are quantitatively different from case to case. The findings indicate that CMD is a dysplastic disease of the central nervous system, with dystrophic involvement of skeletal muscles.