ABSTRACT

Syringomyelia is a progressive degenerative disease of spinal cord that is characterized with brachial amyotrophy and dissociative segmental sensorial deficit. It can occour only itself or occur with Chiari type 1 malformation.

A. A., 37 year-old, female, presented with weaness of arms and legs. In her neurologic examination; we obtained fibrillation of tongue, fasciculation under skin, atrophy at hand muscles, abdution deficiency at both arms, hyperactivity in tendon reflexes and extansor plantar reflex. The patient was hospitalized with the suspicion of amyotrophic lateral sclerosis. Her electromyography was normal. In her cervical magnetic resonance imaging; there was Chiari 1 malformation and syringomyelia in cervical region. The patient was conslutated with neurosurgeons. After suboccipital craniectomy; C1 total, C2 partial laminectomy was made with synthetic graft. There was a certain decrease in patient’s complaints postoperatively. We wanted to present this Chiari 1 malformation case with syringomyelia because of its similarity with amyotrophic lateral sclerosis (ALS) clinical course.

Keywords: Chiari 1 malformation, syringomyelia, ALS