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SYRINGOMYELIA

Definition Pathology: Cavitation & gliosis Location Spinal cord: Usually cervical or thoracic Medulla Course: Chronic Clinical syndrome Epidemiology Onset: Most commonly between ages 25 to 40 Males somewhat > Females Clinical features: Variable depending on anatomical involvement Anterior horns Weakness & Wasting: Especially in hands & arms Fasciculations Posterior horns & Decussating sensory fibers Sensory loss: Pain & Temperature Distribution: "Suspended" sensory loss Involves: Arms & Trunk Spares: Legs Involvement of sacral areas may suggest association with neoplasm Pain: Occasional; Boring or lancinating Large fiber sensations: Usually preserved Tendon reflexes: Reduced or absent in arms Autonomic pathways Horner's syndrome Skin: Trophic changes Neurogenic bladder Corticospinal tracts: Variable involvement Spastic paraparesis: Legs > Arms Skeletal Scoliosis Cranial nerve involvement: With syringobulbia Most often unilateral XII: Tongue weakness & hemiatrophy IX - X: Dysphagia; Dysarthria XI: Weakness & wasting of sternomastoid & trapezius VII: Facial paresis Descending tract of V Reduced pain & temperature on lateral face "Onion skin" distribution Reduced corneal sensation & reflex Course: Very slow progression Radiology: A; B Surgical treatment: Posterior fossa decompression1 Variant syndrome: Presyrinx2 Spinal cord edema Caused by alterations in CSF flow at foramen magnum Location: Cervical or Thoracic Associated with disorders that disrupt CSF flow Chiari I & II malformations Trauma Subarachnoid hemorrhage Bacterial meningitis Cervical stenosis Posterior fossa arachnoid cyst Spinal arachnoiditis Neurosarcoidosis Clinical Onset age: 1 year to Adult; Childhood with Chiari malformation Course Onset may be acute or subacute Some with chronic progression MRI change may be asymptomatic Weakness: Arms or Quadriparesis Headache Bulbar: Dysphagia & Gagging MRI T2 prolongation T1 prolongation Cord enlargement without enhancement Treatment: Reversible with surgery Associated disorders Spina bifida (10%) ? Cervical rib Arnold-Chiari malformation Familial syringomyelia Prior trauma or hematomyelia Spinal cord gliomas & other neoplasms CHARGE syndrome Hydrocephalus Trichorhinophalangeal syndrome Type I Situs inversus

Bramwell