Clinical Trial: Study and Surgical Treatment of Syringomyelia

Study Status: Completed
Recruit Status: Completed
Study Type: Observational

Official Title: Establishing the Pathophysiology of Primary Spinal Syringomyelia

Brief Summary:

The goal of this study is to establish the mechanism(s) of progression of primarily spinal syringomyelia (PSS). Our preliminary study of syringomyelia emphasized syringomyelia associated with craniocervical junction abnormalities (CCJAS), such as the Chiari I malformation. This new protocol will expand the scope of our investigation to include primarily spinal syringomyelia (PSS), which is defined as syringomyelia not associated with craniocervical junction abnormalities (CCJAS). Etiologies of primarily spinal syringomyelia include 1) intradural scarring which is post-traumatic, post-inflammatory, or post-operative, 2) intradural-extramedullary masses such as arachnoid cysts or meningiomas, and 3) extramedullary-extradural spinal lesions such as cervical spondylosis or spinal deformity.

Our hypothesis is the following: Primarily spinal syringomyelia (PSS), results from obstruction of cerebrospinal fluid (CSF) flow within the spinal subarachnoid space; this obstruction affects spinal CSF dynamics because the spinal subarachnoid space accepts the fluid that is displaced from the intracranial subarachnoid space as the brain expands during cardiac systole; in the case of primarily spinal syringomyelia (PSS), a subarachnoid block effectively shortens the spinal subarachnoid space, reducing CSF compliance and the capacity of the spinal theca to dampen the subarachnoid CSF pressure waves produced by the brain expansion during cardiac systole; the exaggerated spinal subarachnoid pressure waves occur with every heartbeat and act on the spinal cord above the block to drive CSF into the spinal cord and create a syrinx. Presyringomyelia, a recently described state of spinal cord edema associated with progressive myelopathy and obstruction in CSF flow, is a precursor stage to syringomyelia that is consistent with this hypothesis. Because of the importance of this condition to the path

Detailed Summary:

Objective: The goal of this study is to establish the mechanism(s) of progression of primarily spinal syringomyelia (PSS), the type of syringomyelia that is associated with pathology in the spinal column and not at the craniocervical junction. Our hypothesis is that when a lesion obstructs the spinal subarachnoid space, it shortens the segment of spinal canal that dampens the CSF pressure waves that are produced with each heartbeat and creates enlarged spinal subarachnoid pressure waves that act on the spinal cord above the block to drive CSF into the spinal cord and create a syrinx. After a syrinx is formed, enlarged subarachnoid pressure waves compress the external surface of the spinal cord, propel the syrinx fluid, and promote syrinx progression.

Study Population: Subjects will have primary spinal syringomyelia associated with 1) intradural scarring which is post-traumatic, post-inflammatory, or post-operative, 2) intradural-extramedullary masses such as arachnoid cysts or meningiomas, and 3) extramedullary-extradural spinal lesions such as cervical spondylosis or spinal deformity, or 4) an intramedullary tumor.

Design: Subjects will have testing before and after standard surgical therapy of syringomyelia. Testing includes measurement of CSF pressure, neurologic examination, CT-myelography, and MR scanning. Results of CSF pressure measurements before surgery will be compared to measurements from normal controls that were previously studied. The effect of surgery on CSF pressure, neurologic examination, CT-myelography, and MRI scans will be evaluated.

Outcome Measures: The primary outcome measure is cervical CSF pulse pressure, which is the amplitude of the CSF pressure wave, compared to normal values. Secondary outcomes measures include change in CSF pulse pressure, neu
Sponsor: National Institute of Neurological Disorders and Stroke (NINDS)

Current Primary Outcome:

Original Primary Outcome:

Current Secondary Outcome:

Original Secondary Outcome:

Information By: National Institutes of Health Clinical Center (CC)

Dates:
Date Received: February 14, 2001
Date Started: February 8, 2001
Date Completion: May 18, 2011
Last Updated: January 24, 2017
Last Verified: May 18, 2011