Chiari I malformation with syrinx.
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.
Division of Pediatric Neurology, Department of Pediatrics, University of Michigan, Ann Arbor, 48109-0202, USA.
Martha D
MD
Carlson
Karin M
KM
Muraszko
D000293:Adolescent; D001139:Arnold-Chiari Malformation / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D001259:Ataxia / Q000209:etiology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D013595:Syringomyelia / Q000150:complications / Q000175:diagnosis / Q000473:pathology*