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Pediatr Neurol. 2003 Aug;29(2):167-9.

Chiari I malformation with syrinx.

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  • 1Division of Pediatric Neurology, Department of Pediatrics, University of Michigan, Ann Arbor, 48109-0202, USA.


The Chiari I congenital malformation is characterized by caudal displacement of the cerebellar tonsils through the cervical canal. Although this malformation is often asymptomatic, coexisting syringomyelia can result in neurologic symptoms. We report a case of progressive ataxia with brainstem dysfunction in an adolescent female manifesting a severe Chiari I malformation with syrinx. Chiari decompression 4 years after initial presentation led to rapid improvement in most of her long-term symptoms. This case demonstrates the importance of consideration of Chiari I with syringomyelia in the differential diagnosis of progressive ataxia and brainstem symptoms.

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