Syringomyelia is a disease in which fluid-filled cavities, often referred to as syrinxes, form within the spinal cord. These syrinxes typically persist at abnormally high pressure which causes progressive neurological symptoms as they expand and elongate over time, compressing the surrounding nerve fibres from within. Since the spinal cord connects the brain to nerves in the extremities, this damage may result in :
Typical:– 1) “central cord syndrome” with pain and sensory loss in upper extremities 2) sensory loss to patient feels like a cape has been placed on back of neck, shoulders, and arms
3) arreflexia in upper extremities 4) weakness in upper extremities
5) muscle loss in same distribution as sensory loss
6) bladder/bowel dysfunction 7) spasticity and weakness in legs
8) Horner’s syndrome If associated with Arnold-Chiari malformation
9) cough 10) headache If affects medulla and pons -
11) vocal cord paralysis 12) dysarthria 13) nystagmus
14) recurrent dizziness
15) tongue weakness If affects descending tract of trigeminal nerve -
16) facial numbness and sensory loss
17) and in severe cases, death.
Syringomyelia is comparatively rare with 8.4 cases per 100,000.
It occurs more frequently in men than in women, and it usually appears in the third or fourth decade of life.
About three quarters of all cases occur in combination with an Arnold-Chiari Malformation, an abnormality of the brain, in which the lower part of the cerrebellum protrudes into the upper cervical portion of spinal subarachnoid space, blocking the flow of cerebrospinal fluid (CSF).Severe trauma to the spinal cord often leads to scarring of the surrounding tissues and subsequent syrinx formation, although post-traumatic syringomyelia only accounts for about 10% of all cases.
At present treatment options are limited, with surgical intervention being the most common course of action. The aim is to alleviate the patient's symptoms by reducing the fluid pressure in the spinal cord and in the surrounding subarachnoid space. This is achieved by draining the syrinx, possibly inserting a shunt, and/or removing some of the surrounding bone (either from the vertebrae or the base of the skull, depending on the location of the syrinx).
