title: Intradural extramedullary leptomeningeal hemangioblastomatosis and paraneoplastic limbic encephalitis diagnosed at autopsy: an unlikely pair.
abstract: Typical cerebellar or spinal cord hemangioblastoma is often solitary and has a benign clinical course, whereas disseminated hemangioblastomatosis is extremely rare. We report a 75-year-old man with disseminated "leptomeningeal" hemangioblastomatosis and concurrent paraneoplastic limbic encephalitis who died of this disease. The patient presented with gait problems and cognitive deficits, and was diagnosed with a cervical spinal tumor. Surgical resection achieved an apparent gross total resection, but later the patient began experiencing severe cognitive decline. His condition progressively deteriorated during the next few months, and he died 15 months after his initial surgery. Autopsy revealed disseminated extramedullary intradural hemangioblastomatosis with extensive leptomeningeal nodules involving the entire spinal cord, medulla, pons, and midbrain, as well as histologic features of limbic encephalitis. There was no evidence of von Hippel-Lindau disease, and no specific etiology was identified for the dementia. To the best of our knowledge, this is the first case of limbic encephalitis occurring in the setting of disseminated hemangioblastomatosis.
authors.affiliation: Department of Pathology, University of California at San Francisco School of Medicine, San Francisco, CA, USA.
authors.forename: Rageshree
authors.initials: R
authors.lastname: Ramachandran
authors.forename: Han Sung
authors.initials: HS
authors.lastname: Lee
authors.forename: Brandy
authors.initials: B
authors.lastname: Matthews
authors.forename: Alan
authors.initials: A
authors.lastname: Shatzel
authors.forename: Tarik
authors.initials: T
authors.lastname: Tihan
mesh_terms: D000368:Aged; D003704:Dementia / Q000150:complications / Q000473:pathology; D003937:Diagnosis, Differential; D004388:Dura Mater / Q000473:pathology*; D017809:Fatal Outcome; D018325:Hemangioblastoma / Q000150:complications / Q000473:pathology* / Q000601:surgery; D006801:Humans; D020363:Limbic Encephalitis / Q000150:complications / Q000473:pathology* / Q000601:surgery; D008297:Male; D008577:Meningeal Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D013125:Spinal Neoplasms / Q000175:diagnosis