title: Intramedullary ependymoma associated with Lhermitte-Duclos disease and Cowden syndrome.
abstract: The authors describe the case of a 45-year-old man with progressive gait ataxia and sensorimotor deficits of the upper and lower extremities. The patient had been diagnosed earlier with Lhermitte-Duclos disease (LDD) in the left cerebellar hemisphere and Cowden syndrome (CS). MR imaging studies revealed an intraspinal tumor at C6-C7. Microsurgical gross total resection of the tumor was achieved. Histolopathological examination revealed an intramedullary ependymoma. Postoperatively, neurological deficits gradually improved. This is the first reported case of ependymoma in a patient with LDD and CD. Coexistence of an intraspinal ependymoma with cerebellar LDD and CS appears to be rare, but can lead to treatment failure if missed.
authors.affiliation: Department of Neurosurgery, Medical School Hannover, MHH, Carl-Neuberg street 1, 30625 Hannover, Germany.
authors.forename: Mohammad R
authors.initials: MR
authors.lastname: Farhadi
authors.forename: Marion
authors.initials: M
authors.lastname: Rittierodt
authors.forename: Alexandru
authors.initials: A
authors.lastname: Stan
authors.forename: Hans-Holger
authors.initials: HH
authors.lastname: Capelle
authors.forename: Barbara
authors.initials: B
authors.lastname: Tham-Mücke
authors.forename: Joachim K
authors.initials: JK
authors.lastname: Krauss
mesh_terms: D002574:Cervical Vertebrae; D004806:Ependymoma / Q000209:etiology* / Q000473:pathology* / Q000601:surgery; D006223:Hamartoma Syndrome, Multiple / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013120:Spinal Cord Neoplasms / Q000209:etiology* / Q000473:pathology* / Q000601:surgery