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.