Chiari malformation type 1 presenting as isolated unilateral foot drop with rapid recovery following posterior fossa decompression

Constantine L. Karras, Nikhil K. Murthy, Siting J. Trybula, Tord D. Alden*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Background: Chiari malformation (CM) type 1 is characterized by descent of the cerebellar tonsils resulting from crowding of the posterior fossa. In 30% of cases, it is associated with syringomyelia. When symptomatic, it may result in a classic constellation of symptoms. Case Presentation: Here we describe a case of a 16-year-old male who presented with isolated, unilateral foot drop due to CM type 1 and holosyrinx. This unique presentation is extremely rare, and we additionally present a review of all other reported cases in the literature. After undergoing posterior fossa decompression with C1 laminectomy and duraplasty, our patient made a complete neurological recovery within 2 weeks postoperatively and his MRI entire spine at 3 months postoperatively demonstrated a nearly complete resolution of the holosyrinx with significant decompression of the foramen magnum. Conclusion: This rare presentation highlights the importance of maintaining a broad differential, particularly in pediatric patients, and expediting the workup in order to offer a surgical decompression within 1–2 months of foot weakness to maximize the probability of a full neurological recovery.

Original languageEnglish (US)
Pages (from-to)821-825
Number of pages5
JournalChild's Nervous System
Volume38
Issue number4
DOIs
StatePublished - Apr 2022

Keywords

  • Chiari malformation
  • Craniectomy
  • Holocord syrinx
  • Holosyrinx
  • Suboccipital decompression
  • Weakness

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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