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  • Title: [Arnold-Chiari I malformation: three unusual manifestations].
    Author: Pellanda N, Mojon D.
    Journal: Klin Monbl Augenheilkd; 2005 Mar; 222(3):218-21. PubMed ID: 15785984.
    Abstract:
    BACKGROUND: 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. PATIENTS AND METHODS: We present three patients with unusual symptomatology. RESULTS: 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. CONCLUSIONS: 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.
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