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End-stage syringomyelia, natural history

Sagittal MRI scan from a nonsurgical 70-year-old male patient with slowly progressive syringomyelia starting at 30 years of age. Neurologic exam: short neck, rotary nystagmus. Wasting and fasciculations of the left side of the tongue. Left Horner syndrome. Widespread weakness and wasting in upper limbs. Spastic paraparesis. Dissociated sensory loss in the face (onion-bulb distribution), neck, and trunk down to T4. There is marked atrophy of the spinal cord and medulla, and a collapsed syrinx. (Contributed by Dr. Martín Nogués.)

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Associated Disorders

  • Arnold-Chiari malformation
  • Chiari I malformation
  • Ependymomas
  • Hemangioblastomas
  • Horner syndrome
  • Morvan syndrome