[Brain stem dysfunction in Arnold-Chiari II syndrome]

Abstract

Among 76 patients suffering from myelomeningocele treated during 1978 to 1987 there were 12 children with brain stem signs as a sequel to Arnold-Chiari II syndrome. In 2 of these patients only stridor was seen, in 4 stridor with attacks of apnoea, in 2 attacks of apnoea with dysphagia, and in 4 children stridor, attacks of apnoea and dysphagia. Hence, it will be necessary to modify the classification given by Charney et al (4) in respect of brain stem patterns of signs according to three grades, since the signs of stridor, apnoea and dysphagia can be combined with each other in different ways. The prognosis is infaust if all 3 signs and hence grade III of brain stem lesions are present. On the whole, 6 out of 12 patients with brain stem signs died. For this reason, a possible Arnold-Chiari malformation should always be considered if stridor is observed, and, if necessary, early decompression treatment by means of a shunt revision should be performed.