Syringomyelia
Syringomyelia is a fluid-filled cavity (syrinx) within the spinal cord, most often arising from Chiari I malformation, trauma, or a spinal cord tumor. It produces a classic cape-like loss of pain and temperature sensation as the syrinx damages the crossing spinothalamic fibers, diagnosed by MRI and treated surgically when progressive.
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What this article covers
- Overview
Definition: syringomyelia is a fluid-filled, gliosis-lined cavity (syrinx) that forms within the spinal cord, most often in the cervical or upper thoracic segments.
- Epidemiology
Age of symptom onset: congenital, Chiari-associated disease most often becomes symptomatic in adolescence or young adulthood, even though the malformation is present from birth.
- Risk factors
Chiari I malformation: the dominant risk factor.
- Pathophysiology
Cerebrospinal fluid flow disturbance: in Chiari-associated disease, tonsillar herniation through the foramen magnum obstructs normal cerebrospinal fluid pulsation at the craniocervical junction, and…
- Clinical features
There is no formal diagnostic criteria set; presentation is defined by the pattern of sensory and motor findings that localize to a central cord lesion.
- Differential diagnosis
Multiple sclerosis: deficits separated in space and time rather than a single progressive central cord pattern; affects women more than men roughly 2 to 1 in a younger age range, with MRI showing…
- Diagnosis
First step: obtain a detailed history and neurologic examination to localize the sensory and motor pattern to a central cord process and identify red flags (progressive weakness, new bowel/bladder…
- Management
First step for an asymptomatic or minimally symptomatic, stable syrinx: conservative management with observation and serial clinical and MRI follow-up, since many syrinxes remain stable for years without intervention.
- Complications
Progressive motor weakness and atrophy: untreated or progressive disease can lead to significant hand and arm weakness that impairs fine motor function.
- Prognosis
Variable natural history: many syrinxes remain stable for years and never require surgery, while others progress steadily without intervention.
- Prevention
No primary prevention exists for congenital Chiari-associated syringomyelia, since the underlying malformation is present from birth.
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