Chiari malformation comprises a spectrum of congenital malformations characterized by a herniation of the cerebellar tonsils below the foramen magnum. Chiari malformation type I (CM-I) is the most prevalent subtype seen in clinical practice. This condition variably compresses the cerebellum and medulla-spinal cord junction secondary to malformation of the posterior fossa. Most neurologists and neurosurgeons recognize the sensorimotor and lower brainstem manifestations that result in the clinical picture of CM-I. The effects of CM-I on cognitive functioning, however, and their impact on neuropsychological performance are poorly understood, despite having long been recognized. This article reviews neuropsychological deficits demonstrated by individuals with CM-I, and explores cerebellocortical neuroanatomic pathways to provide possible rationale for the neurocognitive impairments present in affected individuals.
