Syringomyelia

Syringomyelia is a chronic neurological disorder characterized by the formation of a fluid-filled cavity (syrinx) within the spinal cord. This cavity can expand over time, damaging the spinal cord and disrupting the transmission of nerve signals. The condition primarily affects the central gray matter of the spinal cord, most commonly in the cervical (neck) region, and can extend into the brainstem (syringobulbia) or thoracic segments. Syringomyelia may occur in association with Chiari malformation type I (the most common cause), spinal cord injury, tumors, meningitis, or tethered spinal cord, or it may be idiopathic with no identifiable cause. Key symptoms typically develop gradually and include dissociated sensory loss (loss of pain and temperature sensation with preserved touch), particularly in a "cape-like" distribution across the shoulders, arms, and upper back. Patients may experience chronic neuropathic pain, muscle weakness and atrophy (especially in the hands and arms), stiffness, and spasticity in the lower extremities. Scoliosis may develop, particularly in younger patients. As the syrinx enlarges, autonomic dysfunction, bladder and bowel disturbances, and sexual dysfunction may occur. Some patients experience Horner syndrome or headaches, particularly if an associated Chiari malformation is present. Treatment depends on the underlying cause and severity of symptoms. In cases associated with Chiari malformation, posterior fossa decompression surgery is often the primary intervention. Direct surgical drainage of the syrinx via shunting procedures may be considered when the cavity is large or progressive. For post-traumatic or tumor-related syringomyelia, addressing the underlying cause is essential. Conservative management includes pain management, physical therapy, and monitoring with serial MRI imaging. There is currently no cure, and the clinical course is variable — some patients remain stable for years while others experience progressive neurological deterioration. Early diagnosis and appropriate intervention can help prevent irreversible spinal cord damage.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Syringomyelia