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Alexander Disease
Joseph Junewick, MD FACR
over 9 years ago
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Chiari I with Syringomyelia

Case Detail

Anatomy: Brain-Spine
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 8 years ago
Updated: over 8 years ago
Tags: PEDS
Modality/Study Types: MR
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Teenager with headache and upper extremity weakness.

Case Images


Chiari I with Syringomyelia


MR – Sagittal T1 and T2 images show pegged tonsils and large beaded central syrninx.


Chiari I malformation is a congenital hindbrain dysgenesis characterized by downward displacement of cerebellar tonsils through the foramen magnum into the spinal canal. The exact criteria by which tonsillar ectopia should be considered abnormal has been a controversial issue but in general requires tonsillar herniation of at least 5mm. Patients with Chiari I malformations may remain asymptomatic often present with symptoms relating to hindbrain compression, such as headache, weakness, numbness, ataxia. When syringomyelia is present (seen in 30% – 56% in Chiari I patients), additional findings could include central cord syndrome, impaired sensation, scoliosis, spasticity, and torticollis. MRI is the imaging modality of choice to diagnose Chiari I malformation and associated impaired CSF flow and syringomyelia.


Elster AD, Chen MY. Chiari I Malformations: Clinical and Radiologic Reappraisal. Radiology (1992); 183:347-353.


Rekha Meesa, MD

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