title: Long-term outcome of patients with multiple cerebral cavernous malformations.
abstract: OBJECTIVE
  Multiple cerebral cavernous malformations (MCCMs) typically occur in patients with a family history of these lesions. Literature on MCCMs is scarce, and little is known about their natural history.
  
  
  METHODS
  Of 264 consecutive patients with cerebral cavernomas treated at the Department of Neurosurgery, Helsinki University Central Hospital, in the past 27 years, 33 patients had MCCMs. Lesions were categorized according to the Zabramski classification scale. Follow-up questionnaires were sent to all patients. Outcome was assessed using the Glasgow Outcome Scale, and amelioration of epilepsy was assessed using the Engel scale. All clinical data were analyzed retrospectively.
  
  
  RESULTS
  The mean age of patients at diagnosis was 44 years. Sex presentation was almost equal. Nine percent of all patients had a family history of the disease. Patients presented with epilepsy, acute headache, and focal neurological deficits. MCCMs were incidental findings in 2 patients. Altogether, 416 cavernomas were found: 70% supratentorial and 30% infratentorial. Fifteen patients had symptomatic hemorrhage before admission to our department. Surgery was performed on 18 patients. In most cases, the largest cavernoma was removed. Postoperatively, 1 patient experienced temporary hemiparesis, and another developed permanent motor dysphasia. No mortalities occurred. The mean follow-up time was 7.7 years. Twenty-six patients (79%) were in good condition. Among patients with epilepsy who underwent lesionectomy, 70% had an Engel class I outcome. On follow-up magnetic resonance imaging, 52 de novo cavernomas were found.
  
  
  CONCLUSIONS
  Surgical treatment of patients with MCCMs is safe. An extirpation of the clinically active cavernoma prevents further bleedings and improves outcome of epilepsy.
authors.affiliation: Department of Neurosurgery, Helsinki University Central Hospital, Helsinki, Finland. juri.kivelev@hus.fi
authors.forename: Juri
authors.initials: J
authors.lastname: Kivelev
authors.forename: Mika
authors.initials: M
authors.lastname: Niemelä
authors.forename: Riku
authors.initials: R
authors.lastname: Kivisaari
authors.forename: Reza
authors.initials: R
authors.lastname: Dashti
authors.forename: Aki
authors.initials: A
authors.lastname: Laakso
authors.forename: Juha
authors.initials: J
authors.lastname: Hernesniemi
mesh_terms: D000293:Adolescent; D000328:Adult; D020785:Central Nervous System Vascular Malformations / Q000175:diagnosis / Q000503:physiopathology* / Q000601:surgery*; D002648:Child; D005260:Female; D023261:Glasgow Outcome Scale; D006801:Humans; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019635:Neurosurgical Procedures / Q000379:methods*; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D014057:Tomography, X-Ray Computed / Q000379:methods