Syringomyelia

Dark pool without drain
Fingers burned, lost sense of pain
Cored out spinal cord
Named for nymph turned hollow reed
Neurosurgery in need       Link to author

About

• Shawl like distribution of spinothalamic sensory loss with pain loss

Aetiology

• Developmental abnormality with an enlarged cavity in the cord begins in adolescence
• May be part of other developmental structural abnormalities that affect CSF drainage.
• Lower cervical and high thoracic longitudinal cyst or syrinx in the cervical cord anterior to the central canal
• Expanding cyst damages area around anterior commissure where the spinothalamic tracts cross
• There is a gradual enlargement of Syrinx with time
• Blockage of outflow from IVth ventricle with Arnold-Chiari malformation

Clinical

• Clinically a central cord syndrome with dissociated sensory loss
• Classically burns on fingers due to loss of pain sensation.
• Central sensory impairment has a cape-like distribution over the patient's shoulders
• Preservation of the dorsal columns proprioception and vibration.
• Hand and arm muscle LMN weakness and wasting as cord grey matter damaged as cavity expands.
• Upper motor neurone lesions below the lesion
• Bladder and bowels usually spared
• Horner's syndrome due to cervical cord damage
• Facial numbness (Trigeminal nerve nucleus down to C2)
• Extension into medulla causes syringobulbia with brainstem and even cerebellar signs
• Can be asymmetrical

Investigations

• Diagnosis by MRI scan of brainstem and cord

Management

• Surgical decompression and aspiration of cysts
• Surgery to correct Chiari defect may improve matters such as removal of posterior margin of the foramen magnum.