Intradural extramedullar leptomeningeal hemangioblastomatosis and paraneoplastic limbic encephalitis diagnosed at autopsy: An unlikely pair

Rageshree Ramachandran, Han Sung Lee, Brandy Matthews, Alan Shatzel, Tarik Tihan

Research output: Contribution to journalArticle

5 Scopus citations

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 extramedullar 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.

Original languageEnglish (US)
Pages (from-to)104-108
Number of pages5
JournalArchives of Pathology and Laboratory Medicine
Volume132
Issue number1
StatePublished - Jan 1 2008
Externally publishedYes

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ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology

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