Clinical and surgical management of holocervical spinal cord ependymomas.

Background:Spinal ependymomas are rare tumors of the central nervous system, and those spanning the entire cervical spine are atypical. Here, we present two unusual cases of holocervical (C1-C7) spinal ependymomas. Case Description:Two patients, a 32-year-old female and a 24-year-old male presented with neck pain, motor, and sensory deficits. Sagittal MRI confirmed hypointense lesions on T1 and hyperintense regions on T2 spanning the entire cervical spine. These were accompanied by cystic cavities extending caudally into the thoracic spine and rostrally to the cervicomedullary junction. Both patients underwent gross total resection of these lesions and sustained excellent recoveries. Conclusion:Two holocervical cord intramedullary ependymomas were safely and effectively surgically resected without incurring significant perioperative morbidity

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

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

Technical Report: Durable efficacy of an endoscope-assisted syringo-panventriculoatrial shunt for concurrent hydrocephalus and syrinx

The management of syringomyelia in association with hydrocephalus can be a challenging pathology to treat with durable results. We present a durable treatment of a patient with symptomatic, idiopathic hydrocephalus, Chiari type I malformation, and syringomyelia with an endoscope-assisted placement of a single syringo-panventricular multiperforated shunt catheter via a transfrontal transaqueductal approach with simultaneous endoscopic third ventriculostomy (ETV). We discuss the technical details required for this single-shunt approach to a complex pathology, and indications for appropriate use of this novel approach in experienced hands. The patient rapidly improved radiographically and symptomatically, with a functioning shunt twelve years postoperatively. For surgeons well-versed in endoscopic techniques, this approach can be safe, durable, and obviate the need for an otherwise complex shunting system with multiple catheters, or a more traditional invasive craniovertebral decompression, in a patient with idiopathic hydrocephalus and a syrinx