***Please note that the contents of this page are being dynamically provided by, and is the property of, Washington University School of Medicine Website.  Christopher S. Burton Syringomyelia Foundation, Inc. does not endorse this organization, is not responsible for any content that this website is linked to and is not responsible for any loss or accuracy of any of this information.***

Home, Search, Index, Links, Pathology, Molecules, Syndromes, Muscle, NMJ, Nerve, Spinal, Ataxia, Antibody & Biopsy, Patient Info

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 T1                                  T2

Link to actual page on Washington University School of Medicine website:

 http://neuromuscular.wustl.edu/spinal/syrinx.htm