NIH Clinical Research Studies

Protocol Number: 01-N-0085

Active Accrual, Protocols Recruiting New Patients

Title:
Establishing the Pathophysiology of Primary Spinal Syringomyelia
Number:
01-N-0085
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 pathophysiology of syringomyelia, we will also study patients with presyringomyelia in this protocol. After a syrinx is formed, the enlarged subarachnoid pressure waves compress the external surface of the spinal cord, propel the syrinx fluid, and promote syrinx progression.

Many neurosurgeons at prominent academic centers routinely use syrinx shunts to treat primarily spinal syringomyelia. This study should provide data that a surgical procedure that opens the spinal subarachnoid space corrects the underlying pathophysiology and resolves the syrinx and that invasion of the spinal cord is unnecessary.

Sponsoring Institute:
National Institute of Neurological Disorders and Stroke (NINDS)
Recruitment Detail
Type: Active Accrual Of New Subjects
Gender: Male & Female
Referral Letter Required: No
Population Exclusion(s): Children

Eligibility Criteria:
INCLUSION CRITERIA:

To be eligible to participate in this research study, the subject must :

Be 18 years of age or older.

Have syringomyelia or "presyringomyelia" (swelling of the spinal cord that precedes syringomyelia), documented by MRI.

Have evidence of neurological deterioration related to syringomyelia, "presyringomyelia", or spinal cord tumor. Prior surgery for syringomyelia does not result in exclusion from study.

Be able to comprehend the risks of testing and therapy and to give informed consent.

EXCLUSION CRITERIA:

Subjects will not eligible to participate in this research study if:

They are pregnant (because X-rays might injure a fetus).

They cannot have an MRI scan as determined by a radiologist .

They have a problem with bleeding that cannot be corrected.

They are unable to understand the risks of the testing and surgical therapy.

Their blood test for HIV (the virus that causes AIDS) is positive, because a positive HIV test would increase your risk of infection from research testing.

Special Instructions: Currently Not Provided
Keywords:
Arachnoiditis
Myelography
Intraoperative Ultrasonography
Magnetic Resonance
CSF Pressure
Trauma
Meningitis
Paralysis
Spondylosis
Recruitment Keywords:
Spinal Syringomyelia
Arachnoiditis
Presyringomyelia
Spinal Cord Edema
Magnetic Resonance Imaging (MRI)
Intraoperative Radiation
Ultrasonography
Trauma
Spondylosis
Meningitis
Paralysis
Conditions:
Syringomyelia
Investigational Drug(s):
None
Investigational Device(s):
None

Contacts:
Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citations:
Elucidating the pathophysiology of syringomyelia

Spinal cord swelling preceding syrinx development: case report

Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils Implications for diagnosis and treatment

Active Accrual, Protocols Recruiting New Patients

If you have:


Command Menu Bar

Search The Studies | Help | Questions |
Clinical Center Home | NIH Home


Clinical Center LogoWarren Grant Magnuson Clinical Center (CC)
National Institutes of Health (NIH)
Bethesda, Maryland 20892. Last update: 10/19/2004

Search The Studies Help Questions