Syringomyelia is a medical condition characterised by the development of a fluid-filled cyst, known as a syrinx, within the spinal cord. This complex disorder manifests through several distinct features:
- Cavitation of the Spinal Cord: Predominantly occurring in the cervical (neck) region, this involves the formation of cavities within the spinal cord.
- Sensory Loss: Individuals with syringomyelia often experience bilateral loss of pain and temperature sensations, usually starting at the level of the lesion.
- Muscle Weakness and Paralysis: Progressive muscle weakness can lead to flaccid paralysis and muscle atrophy, particularly in the upper limbs, due to the destruction of ventral horn cells in the spinal cord.
- The condition is often linked to developmental abnormalities in the formation of the spinal cord’s central canal. Syringomyelia primarily affects the brainstem (referred to as syringobulbia) and the cervical region of the spinal cord. The lesion’s presence leads to cavitation and gliosis, a form of scarring, in the central region of the spinal cord.
Signs and Symptoms of Syringomyelia
Syringomyelia presents a range of symptoms:
- Loss of Sensory Functions: Patients typically lose pain and temperature sensations in a shawl-like distribution across the body. This is due to the interruption of nerve tracts in the spinal cord.
- Preserved Sensations: Interestingly, vibratory sense, tactile discrimination, and proprioceptive sense remain normal, as the ascending tracts in the posterior column of the spinal cord are not affected.
- Motor Impairments: Bilateral spastic paralysis of the legs, exaggerated reflexes, and a positive Babinski sign are common. These occur when the lesion expands to affect the descending tracts.
- Hand Weakness: Often, there is lower motor neuron weakness in the small muscles of the hand, which can be bilateral or affect one hand more than the other.
- Additional Complications: Over time, joints in the elbow, wrist, and hand can become disorganised or dislocated due to a lack of sensation. Horner syndrome, characterised by drooping eyelids, pupil constriction, and lack of facial sweating, can also develop.
Syringomyelia is often associated with other neurological conditions like Hydrocephalus and Arnold-Chiari malformation.
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