Sudden onset of Chiari malformation Type I in previously asymptomatic patients

Chiari malformation Type I (CM-I) is usually suspected in patients with slowly progressing neurological symptoms. However, in some instances, especially if syringomyelia is associated, an abrupt clinical onset is reported and is accompanied by an acknowledged risk of potentially severe clinical signs or even sudden death. Little is known about such a critical course in CM-I/syringomyelia complex. The authors describe 3 challenging cases of the abrupt onset of CM-I/ syringomyelia to reveal more information on the clinical presentation and pathogenetic mechanisms of this sudden and potentially severe clinical phenomenon: a 38-year-old man experienced acute respiratory failure requiring intubation following acute decompensation of hydrocephalus associated with Noonan syndrome, a 1-year-old boy had sudden hemiparesis and Horner syndrome after a minor head/ neck injury, and a 2.5-year-old boy presented with quickly progressing tetraplegia and dyspnea after a mild flexion and extension neck injury a few hours before. All 3 patients showed a CM-I/ syringomyelia complex at diagnosis, and all of them had a good neurological outcome after surgery despite the ominous clinical presentation. (DOI: 10.3171/2011.8.PEDS11160)