Pellanda N, Mojon D
Abteilung für Orthoptik und Neuroophthalmologie, Kantonsspital Sankt Gallen, Schweiz.
Klin Monbl Augenheilkd. 2005 Mar;222(3):218-21. doi: 10.1055/s-2005-858019.
Arnold-Chiari Syndrome I is a malformation of the cervicomedullary junction, manifesting usually with downbeat nystagmus, palsy of the caudal cerebral nerves, headache, and vertigo.
We present three patients with unusual symptomatology.
A two-year-old child with isolated non-ocular torticollis, a 52-year-old male patient, and a 42-year-old female patient, both with gaze-evoked nystagmus, underwent a cerebral MRI examination. The findings of the first two patients were typical for an Arnold-Chiari syndrome. The malformation in the third patient was found only by reviewing the initial MRI.
Arnold-Chiari malformation may manifest atypically. An important step in the work-up of these patients is to ask the neuroradiologist to include the cervicomedullary junction in his evaluation.
I型阿诺德-奇亚里综合征是一种延髓颈髓交界处的畸形,通常表现为下跳性眼球震颤、低位脑神经麻痹、头痛和眩晕。
我们报告了三名有不寻常症状的患者。
一名两岁孤立性非眼性斜颈患儿、一名52岁男性患者和一名42岁女性患者,均有凝视诱发性眼球震颤,接受了脑部MRI检查。前两名患者的检查结果为典型的阿诺德-奇亚里综合征。第三名患者的畸形仅在复查初始MRI时发现。
阿诺德-奇亚里畸形可能表现不典型。对这些患者进行检查的一个重要步骤是要求神经放射科医生在评估中包括延髓颈髓交界处。