Friede R L, Roessmann U
Acta Neuropathol. 1976 Mar 30;34(3):219-35. doi: 10.1007/BF00688677.
A system is presented for the classification of chronic herniations of the cerebellar tonsils in the absence of space-occupying intracranial lesions, based on a survey of the literature and 13 own cases. The Arnold-Chiari malformation in adults typically involves herniation of the cerebellar tonsils instead of herniation of the vermis as is typical when it occurs in infancy. Identification of chronic tonsillar herniation with the Arnold-Chiari malformation in adults was thought to require at least one other sign of the Arnold-Chiari complex, e.g. a medullary deformity. Cases for which chronic herniation and sclerosis of the cerebellar tonsils present as the only nervous lesion are classified as "chronic tonsillar herniation". Such cases may manifest with neurological symptoms during adult life, or they may be found incidentally at autopsy. Attention is drawn to the occurrence of chronic tonsillar herniation in 7 infants and children where it apparently represents a cause of sudden unexpected death. The overlap between chronic tonsillar herniation and the Arnold-Chiari malformation of adults is discussed in regard to the frequence of associated osseous anomalies, hydrocephalus and syringomyelia.
基于文献综述和13例自身病例，本文提出了一种在无颅内占位性病变情况下对小脑扁桃体慢性疝进行分类的系统。成人的阿诺德-奇阿利畸形通常涉及小脑扁桃体疝，而非婴儿期典型的小脑蚓部疝。成人阿诺德-奇阿利畸形伴慢性扁桃体疝的诊断被认为至少需要阿诺德-奇阿利复合体的另一征象，如延髓畸形。小脑扁桃体慢性疝和硬化作为唯一神经病变的病例被归类为“慢性扁桃体疝”。此类病例在成年期可能出现神经症状，或在尸检时偶然发现。本文提请注意7例婴儿和儿童发生慢性扁桃体疝的情况，其显然是意外猝死的原因。本文还讨论了慢性扁桃体疝与成人阿诺德-奇阿利畸形在相关骨异常、脑积水和脊髓空洞症发生率方面的重叠情况。
