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Elucidating the pathophysiology of syringomyelia
J D Heiss1, N Patronas, H L DeVroom
1Surgical Neurology, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA. heissjd@codon.nih.gov
Journal of Neurosurgery
|October 3, 1999
Summary
Syringomyelia progression in Chiari I malformation is caused by cerebellar tonsil movement at the foramen magnum. Surgery to decompress the tonsils resolves this mechanism, halting syringomyelia progression and improving symptoms.
Area of Science:
- Neurosurgery
- Neurology
- Medical Imaging
Background:
- Syringomyelia, a condition causing progressive myelopathy, is frequently associated with Chiari I malformation.
- Understanding the mechanisms linking Chiari I malformation to syringomyelia progression is crucial for developing effective treatment strategies.
Purpose of the Study:
- To investigate the pathophysiological mechanisms underlying syringomyelia progression in patients with Chiari I malformation.
- To evaluate the impact of surgical decompression on syringomyelia and associated symptoms.
Main Methods:
- Prospective study of 20 adult patients with Chiari I malformation and symptomatic syringomyelia.
- Pre- and post-operative assessments included clinical examinations, MR imaging (including cine MR imaging for CSF flow), and cerebrospinal fluid (CSF) pressure measurements.
- Comparison with 18 healthy volunteers.
Main Results:
- Patients exhibited reduced CSF space diameters at the foramen magnum, increased CSF velocity, and reduced flow compared to controls.
- Cerebellar tonsils showed systolic descent and diastolic ascent, creating piston-like pressure waves that propagated syrinx fluid.
- Post-surgery, CSF pathways normalized, tonsil movement ceased, syrinx size decreased, and clinical symptoms improved.
Conclusions:
- Chiari I malformation acts as a piston, obstructing CSF flow at the foramen magnum and causing progressive syringomyelia.
- The observed resolution of tonsil abnormalities post-surgery suggests an acquired nature of the Chiari I malformation in these cases.
- Simple decompressive surgery effectively eliminates the syringomyelia progression mechanism without requiring more invasive procedures.