Syringobulbia is a neurological disorder characterized by a fluid-filled cavity (syrinx) within the spinal cord that extends to involve the brainstem (medulla). It usually occurs as a slit-like gap within the lower brainstem that may affect one or more of the cranial nerves, causing facial palsies of various kinds. In addition, sensory and motor nerve pathways may be affected by compression and/or interruption. This disorder is intimately associated with syringomyelia, in which the syrinx is limited to the spinal cord, and to the Chiari I malformation.
Usually, syringobulbia presents after syringomyelia, although isolated cases of syringobulbia have been documented.
Syringobulbia is a slowly progressive disorder that may cause dizziness (vertigo), involuntary rapid movement of the eyeball (nystagmus), and loss of feelings of pain and temperature in the face. Atrophy and small local involuntary contractions (fibrillation) of the tongue muscle may also occur, as well as stuttering (dysphonia), and a shrill or harsh voice. Symptoms may also include impaired vision, numbness, and an unsteady way of walking (gait instability).
Other symptoms may include hearing loss or ringing in the ears (tinnitus) and periodic limb movements. In rare cases, nausea, vomiting, and feeding difficulties may be early symptoms.
Syringobulbia can affect people of either sex. It usually is apparent before 30 years of age. In one study of a pediatric population, the average age of onset of symptoms was just under 15 years of age.
The diagnosis of syringobulbia is made by means of neuroimaging, typically magnetic resonance imaging (MRI).
Treatment of syringobulbia is almost invariably surgical and consists of efforts to reroute the flow of cerebrospinal fluid by the use of diversion tubes or shunts. The various surgical approaches for syringobulbia usually must be combine with treatment for syringomyelia.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
The National Institute of Neurological Disorders and Stroke (NINDS) is sponsoring a study that is designed to develop less invasive surgical techniques for the treatment of syringobulbia. For information about this study, visit the www.clinicaltrials.gov web site or contact the NIH Patient Recruitment office listed above. The identification number for this trial is NCT00011245.
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Saremi F, Zee C-S. Syringohydromyelia. emedicine. Last updated: July 8, 2003. 8pp.
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