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[Brain stem dysfunction in Arnold-Chiari II syndrome]
A M Holschneider1, J A Bliesener, M Abel
1Kinderchirurgische Klinik, Städtischen Kinderkrankenhauses Köln.
Insights
Arnold-Chiari II syndrome can cause brain stem issues in myelomeningocele patients. Early intervention for symptoms like stridor is crucial for better outcomes.
Area of Science:
- Neurology
- Pediatrics
- Developmental Biology
Context:
- Myelomeningocele is a severe birth defect impacting the spinal cord and brain.
- Arnold-Chiari II malformation is a common complication associated with myelomeningocele.
- Brain stem dysfunction can arise as a sequel to Arnold-Chiari II malformation.
Purpose:
- To analyze the clinical presentation and outcomes of brain stem signs in pediatric patients with myelomeningocele and Arnold-Chiari II malformation.
- To propose a refined classification system for brain stem signs based on observed symptom combinations.
Summary:
- This study reviewed 76 myelomeningocele patients, identifying 12 with brain stem signs linked to Arnold-Chiari II syndrome.
- Observed signs included stridor, apnea, and dysphagia, occurring in various combinations.
- A proposed three-grade classification highlights that the presence of all three signs (Grade III) indicates a poor prognosis, with 6 of 12 affected patients dying.
Impact:
- The findings suggest that stridor in myelomeningocele patients warrants consideration of Arnold-Chiari malformation.
- Early diagnosis and surgical intervention, such as shunt revision, may improve patient prognosis.
- This research contributes to a better understanding of brain stem dysfunction in pediatric neurological conditions.
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.