• Klin Monbl Augenheilkd · Mar 2005

    Case Reports

    [Arnold-Chiari I malformation: three unusual manifestations].

    • N Pellanda and D Mojon.
    • Abteilung für Orthoptik und Neuroophthalmologie, Kantonsspital Sankt Gallen, Schweiz.
    • Klin Monbl Augenheilkd. 2005 Mar 1; 222 (3): 218-21.

    BackgroundArnold-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 MethodsWe present three patients with unusual symptomatology.ResultsA 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.ConclusionsArnold-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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