title: Surgical Treatment for Patients with Moyamoya Syndrome and Type 1 Neurofibromatosis.
abstract: BACKGROUND
  The current study describes the impact of surgery in preventing follow-up ipsilateral transient ischemic attacks (TIAs)/strokes in an East Coast North American cohort of patients with both moyamoya syndrome (MMS) and neurofibromatosis type 1 (NF1) (MMS-NF1).
  
  
  METHODS
  We retrospectively reviewed records of patients with MMS and NF1 at the Johns Hopkins Medical Institutions from 1990-2014. Baseline characteristics and follow-up results including subsequent ipsilateral strokes were collected and compared between a revascularization group (group 1) and a conservatively managed group (group 2) on a per-hemisphere basis.
  
  
  RESULTS
  A total of 9 patients (14 hemispheres) were included in our study. The average age of all patients at NF1 diagnosis was 2.1 ± 7.7 years, with 6 being female (66.7%). The average age of all patients at MMS diagnosis was 10.4 ± 16.6 years with the median age being 7.7 years (range: 4.1-27.0 years). Race distribution was: White (n = 4, 44.4%), Black (n = 3, 33.3%), and Asian (n = 2, 22.2%). Four patients (44.5%) experienced cerebrovascular manifestations of MMS before MMS diagnosis. Group 1 was younger at MMS diagnosis (P = 0.009), likely with a more acute symptom onset (P = 0.077). Management strategies were: pial synangiosis (n = 3, 21.4%) and conservative (n = 11, 78.6%). During an average follow-up period of 6.28 ± 2.0 years, no ipsilateral TIAs/strokes were observed for group 1; conversely, 2 ipsilateral TIAs (18.2%) and 2 ipsilateral strokes (18.2%) occurred in group 2.
  
  
  CONCLUSIONS
  In our study of non-Asian patients with MMS-NF1, revascularization reduced stroke recurrence and deterioration of symptoms. However, more studies are warranted to further explore the role of revascularization procedures given the rarity of this disease combination.
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Jose L
authors.initials: JL
authors.lastname: Porras
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Wuyang
authors.initials: W
authors.lastname: Yang
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Tomas
authors.initials: T
authors.lastname: Garzon-Muvdi
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Risheng
authors.initials: R
authors.lastname: Xu
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Jaishri
authors.initials: J
authors.lastname: Blakeley
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Allan
authors.initials: A
authors.lastname: Belzberg
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Justin M
authors.initials: JM
authors.lastname: Caplan
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Syed
authors.initials: S
authors.lastname: Khalid
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Geoffrey P
authors.initials: GP
authors.lastname: Colby
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Alexander L
authors.initials: AL
authors.lastname: Coon
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Rafael J
authors.initials: RJ
authors.lastname: Tamargo
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Edward S
authors.initials: ES
authors.lastname: Ahn
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. Electronic address: jhuang24@jhmi.edu.
authors.forename: Judy
authors.initials: J
authors.lastname: Huang
mesh_terms: D000293:Adolescent; D000328:Adult; D002548:Cerebral Revascularization / Q000009:adverse effects* / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D002546:Ischemic Attack, Transient / Q000175:diagnosis / Q000209:etiology / Q000517:prevention & control*; D008297:Male; D009072:Moyamoya Disease / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D009456:Neurofibromatosis 1 / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Moyamoya; Neurofibromatosis 1; Revascularization; Stroke