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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
  • 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


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References
1. Childs Nerv Syst 2009;25:453-459
2. Neurology 2008;71:351-356
3. Neuropediatrics 2014;45:39

2/2/2014