[A sudden-death in a case of Arnold-Chiari malformation (type I) with sleep apnea]

We presented a sudden-death case of Arnold-Chiari malformation (type 1) accompanied with spina bifida and closed meningomyelocele. Polysomnography revealed the increase of both central and mixed type apneas to compare with the findings of typical Pickwikian syndrome.

30-year-old female without obese or obstruction of upper air way. Spina bifida and closed meningomyelocele at sacral portion were found at her birth. She had no treatment and had not been able to walk because of paralysis at low extremities. Since she was 25-year-old, she had had insomnia which accompanied by choked feelings, palpitations, clumsiness of hands and anxiety. Snoring was light, and she had neither respiratory disturbances nor hypersomnia during awake. She was admitted to our hospital for treatment of decubitus. 2nd June, 1977, she was found acrocyanosis during sleep and immediately she was resurrected. Physical examinations revealed there was no accounting for sudden respiratory arrest: the cardio-pulmonary system was normal during awake and laboratory findings also failed to disclose the episode. But she had slight dysfunctions of lower cranial nerves: fine nystagmus according to the head-position, decreased gag reflex, and paresis in the recurrence nerve of N.XII and etc. Angiography showed communicating hydrocephalus. Though ventriculoperitoneal shunt operation was performed at 8th June, sleep apnea could not be improved. Therefore we examined in order to clarify her sleep apnea. She was not obese, we could not find any obstructions of upper air way. Nocturnal polygraphy was performed at 8th July. The results were summarized as follows: (1) Total sleep time was 293 minutes and numbers of sleep apnea were 134 times.(ABSTRACT TRUNCATED AT 250 WORDS)