Chiari 2 without spinal dysraphism: Does it blow a hole in the pathogenesis?

Valentina Citton, Irene Toldo, Laura Balao, Andrea Pettenazzo, Enzo Emanuelli, Stefano Sartori, Milena Calderone, Renzo Manara

Research output: Contribution to journalArticlepeer-review


Chiari malformation type 2 is characterized by hindbrain protrusion and a constellation of supratentorial malformations. Chiari malformation type 2 is thought to be causally related to myelomeningocele due to intrauterine cerebrospinal fluid overdrainage. This relationship is so strong that it has become a rule. A 14-year-old girl affected by mental retardation, spastic triparesis, and epilepsy is presented. Brain magnetic resonance imaging disclosed a severe Chiari malformation type 2, whereas spine magnetic resonance imaging was unremarkable. The authors discuss previous literature describing rare cases of Chiari malformation type 2 without open spinal dysraphism and the relevance of concomitant, sometimes overlooked, neuroimaging findings, underlying how exceptions might be hurdles but might also eventually strengthen the rules.

Original languageEnglish
Pages (from-to)536-539
Number of pages4
JournalJournal of Child Neurology
Issue number4
Publication statusPublished - Apr 2012


  • Chiari malformation type 2
  • epilepsy
  • myelomeningocele
  • spinal dysraphism

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health


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