[Syringomyelia as a rare cause of respiratory insufficiency requiring ventilation]

For 17 years a now 45-year-old man had suffered from progressively more severe flaccid paresis of the arms and thoracic muscles, spastic paralysis of the legs and kyphoscoliosis. Artificial ventilation was required when he contracted pneumonia. Although it was being treated with antibiotics, frequent bronchoalveolar lavage had to be done because of repeated atelectases. After 6 weeks clonuses developed in the legs, predominantly on the right, stretch synergisms and opisthotonos. The pupils were small with sluggish reaction to light, and there was a positive "doll's head" phenomenon. The level of consciousness alternated between somnolence and sleepiness. Magnetic resonance imaging demonstrated cavities in cervical and thoracic spinal cord, supporting the diagnosis of an abnormal cerebrospinal circulation due to gliosis in syringomyelia. To secure cerebrospinal fluid drainage, the cerebellar tonsils were resected, together with a duraplasty and partial resection of the atlas. Following this he became fully conscious and the spastic state improved. During the following 8 weeks it became possible gradually to wean him from the artificial ventilation and achieve satisfactory mobilization so that he could be discharged to domiciliary care.