Krupina N E
Zh Nevrol Psikhiatr Im S S Korsakova. 2003;103(7):4-9.
Three hundred and sixty four patients with Chiari malformation (CM) type I were examined: 263--with isolated CM and 101--with concomitant syringomyelia (SM). To study clinical features of the disease, neurological examination, craniography and magnetic resonance imaging of the brain and spinal cord were used. Similar predisposing factors and groups of objective neurological symptoms were found. Differences in the first subjective appearance and age at the disease onset were revealed. In patients with isolated CM, brain stem and cerebella symptoms dominated in the clinical picture, with spinal disturbances and asymptomatic cases being sometimes found. In patients with CM + SM, brain stem and spinal disturbances were more frequent and severe, comparing to those with single CM. Similar clinical features are due probably to common disease pathogenesis and the different ones are the result of the cerebrospinal fluid disturbances, causing intracranial hypertension, hydrocephaly and SM.
对364例I型Chiari畸形(CM)患者进行了检查:263例为单纯CM,101例伴有脊髓空洞症(SM)。为研究该疾病的临床特征,采用了神经学检查、颅骨造影以及脑和脊髓的磁共振成像。发现了相似的诱发因素和客观神经症状群。揭示了疾病首次主观出现和发病年龄的差异。在单纯CM患者中,脑干和小脑症状在临床表现中占主导,有时会发现脊髓功能障碍和无症状病例。与单纯CM患者相比,CM + SM患者的脑干和脊髓功能障碍更频繁、更严重。相似的临床特征可能归因于共同的疾病发病机制,而不同的特征则是脑脊液紊乱导致颅内高压、脑积水和脊髓空洞症的结果。