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SYRINGOMYELIA ³

General Epidemiology: Varied frequency 1.9 per 100,000 in Japan 8.4 per 100,000 in Western countries Pathology Cavitation: Fluid filled Surrounding tissue: Gliosis Location Spinal cord Usually cervical or thoracic: C2 to T9 Medulla Course: Chronic Children: Usually occurs with congenital anomalies Adults Onset: Most commonly between ages 25 to 40 Males > Females Causes Prior events: Meningitis; Spinal trauma Neoplasms: Intramedullary or Extramedullary Syndromes & Features 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 Sacral Involvement: May suggest 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: Late in disease course 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 Surgical treatment: Posterior fossa decompression 1 Variant syndrome: Neural tube defects Distribution: Legs Weakness Bowel & Bladder dysfunction Pain Association: Chiari II malformation Variant syndrome: Presyrinx 2 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 General: 1 year to Adult Chiari malformation: Childhood 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 Animals: Syrinx & Arnold-Chiari predisposition Cavalier King Charles Spaniel Brussels Griffon Syrinx: Associated disorders Childhood forms: General Embryonic Central canal of spinal cord: Abnormal dilatation Roof plate: Thin & Elongated Mesenchymal tissue Reduced between spinal cord & surface ectoderm Neural tube defect Open Gap in posterior vertebral arches Meneingomyelocele: Extensive syrinx to cervical levels Fetal Mesenchymal tissue: Normal Neural tube defect: Closed; Intact vertebral column Syrinx Located rostral to tethering lipoma or bony spur Stays localized to lower thoracic & lumbar levels Specific causes Spina bifida (10%) Arnold-Chiari malformation: Chiari I Neural tube defects Tethered cord Myelomeningocele CHARGE syndrome Familial syringomyelia Hydrocephalus Trichorhinophalangeal syndrome Type I Situs inversus Later onset Prior event: Trauma; Hematomyelia; Meningitis Neoplasms Intrinsic Spinal cord glioma Ependymoma Hemangioblastoma Extramedullary: Tumors; Arachnoid cysts Spinal canal stenosis ? Cervical rib Radiology: A; B

Oppenheim 1894 Oppenheim 1894 Oppenheim 1894 Bramwell T1                                  T2