SYRINGOMYELIA

disease of the spinal cord and medulla oblongata of the brain (syringobulbia). The condition is chronic and progressive, with pathological changes in the neural tissue, characterized principally by excessive development and cavitation. There is gradual dissociated sensory loss, muscle wasting, and spasticity. The disease is of unknown cause but is thought to be a developmental defect, frequently associated with other developmental anomalies. Symptoms ordinarily appear between the ages of 10 and 30; frequency is higher among males than females. Onset of the disease is slow. First noticeable signs may be weakness of the small hand muscles or burns or other injuries with lack of pain. This early loss of pain and temperature sense (but not that of touch) is brought about by pathology of pain and temperature fibres as they cross in the gray commissure of the spinal cord. This loss of sensation has a peculiar shawllike distribution over the arms and shoulders. The disease is slowly progressive; spasticity and hyperactive reflexes increase. Scoliosis, a lateral curvature of the spine, is frequent. Syringobulbia is manifested by tongue atrophy, dysphagia, loss of pain and temperature sensation in the face, and a variety of other neurological disorders. There is, at present, no treatment and no cure for the disease; but, since it progresses slowly, patients may live as long as 40 years after its onset.