ABSTRACT
Vertebral ankylosing hyperostosis is a systemic rheumatological abnormality of unknown etiology whose hallmark is luxurious flowing ossification of the anterior longitudinal ligament. It has been regarded as asymptomatic ankylosis requiring no medical or surgical treatment. However, dyspnea, dysphagia, spinal cord compression, and peripheral nerve entrapment have all been documented in association with this disorder. On the other hand, reports on spinal stenosis at lumbar level are extremely rare. We present a case of spinal stenosis assosiated with vertebral ankylosing hyperostosis.
Subject(s)
Ankylosis , Deglutition Disorders , Dyspnea , Hand , Hyperostosis, Diffuse Idiopathic Skeletal , Longitudinal Ligaments , Peripheral Nerves , Spinal Cord Compression , Spinal StenosisABSTRACT
Cerebellar hemangioblastoma comprises 2% of all brain tumors and 7% 10% of all posterior fossa tumors. It can arise in isolation("sporadic cases") or as a major manifestation of von Hippel-Lindau(VHL) disease, a well known autosomal dominant inherited tumor syndrome. Only 5-30% of these tumors are due to VHL disease. However, cerebellar hemangioblastoma occurs in younger patients, is often multiple and recurrent, and has a poorer prognosis than sporadic cases. We present a case of a 26-year-old woman with a right cerebellar hemangioblastoma, which recurred from a left cerebellar hemangioblastoma resected four years previously. Further evaluation established the diagnosis of VHL disease by demonstrating a cystadenoma in the pancreas and an omental cyst. Recently, the von Hippel-Lindau disease gene has been identified as a tumor suppressor gene and has been mapped to the short arm of chromosome 3(3p 25-26). Its absence or a defect in its structure is responsible for predisposition to the disease.