Post syringomyelia progressive muscular atrophy: A late sequel to syringomyelia?

Abstract

We review eight cases of syringomyelia with late sequelae of progressive muscular weakness that suggested the diagnosis of amyotrophic lateral sclerosis (ALS). We propose that this is not ALS, but rather a novel syndrome with distinguishing clinical features; sensory deficits from the initial syrinx, relative sparing of the cranial region, and a slower rate of progression. Imaging studies did not reveal re-expansion of the syrinx, nor any other structural pathology to explain the progressive motor degeneration. Spinal cord regions (cervical, thoracic, lumbosacral) involved on initial presentation predicted the regions of late progression of motor neuron loss, suggesting that pathogenesis may involve an initial loss of motor neurons due to the syrinx, and then age related loss as seen in post-poliomyelitis progressive muscular atrophy. We conclude that these cases are examples of a novel syndrome of post-syringomyelia progressive muscular atrophy.

Keywords

• syringomyelia
• chiari malformation
• amyotrophic lateral sclerosis
• post polio progressive motor atrophy