title: Motor and cognitive improvements in patients with Huntington's disease after neural transplantation.
abstract: BACKGROUND
  Huntington's disease is a neurodegenerative disease of genetic origin that mainly affects the striatum. It has severe motor and cognitive consequences and, up to now, no treatment. Motor and cognitive functions can be restored in experimental animal models by means of intrastriatal transplantation of fetal striatal neuroblasts. We explored whether grafts of human fetal striatal tissue could survive and have detectable effects in five patients with mild to moderate Huntington's disease.
  
  
  METHODS
  After 2 years of preoperative assessment, patients were grafted with human fetal neuroblasts into the right striatum then, after a year, the left striatum. Final results were assessed 1 year later on the basis of neurological, neuropsychological, neurophysiological, and psychiatric tests. The results obtained were compared with those of a cohort of 22 untreated patients at similar stages of the disease who were followed up in parallel. Repeated magnetic resonance imaging (MRI) and positron emission tomography (PET) scanning with fluorine-18-labelled fluorodeoxyglucose was also done to assess metabolic activity.
  
  
  RESULTS
  The final PET-scan assessment showed increased metabolic activity in various subnuclei of the striatum in three of five patients, contrasting with the progressive decline recorded in the two other patients in the series, as seen in patients with untreated Huntington's disease. Small areas of even higher metabolic activity, coregistering with spherical hyposignals on MRI were also present in the same three patients, suggesting that grafts were functional. Accordingly, motor and cognitive functions were improved or maintained within the normal range, and functional benefits were seen in daily-life activities in these three patients, but not in the other two.
  
  
  CONCLUSIONS
  Fetal neural allografts could be associated with functional, motor, and cognitive improvements in patients with Huntington's disease.
authors.affiliation: INSERM U 421/IM3 and CHU Henri Mondor-AP/HP, Créteil, France.
authors.forename: A C
authors.initials: AC
authors.lastname: Bachoud-Lévi
authors.forename: P
authors.initials: P
authors.lastname: Rémy
authors.forename: J P
authors.initials: JP
authors.lastname: Nguyen
authors.forename: P
authors.initials: P
authors.lastname: Brugières
authors.forename: J P
authors.initials: JP
authors.lastname: Lefaucheur
authors.forename: C
authors.initials: C
authors.lastname: Bourdet
authors.forename: S
authors.initials: S
authors.lastname: Baudic
authors.forename: V
authors.initials: V
authors.lastname: Gaura
authors.forename: P
authors.initials: P
authors.lastname: Maison
authors.forename: B
authors.initials: B
authors.lastname: Haddad
authors.forename: M F
authors.initials: MF
authors.lastname: Boissé
authors.forename: T
authors.initials: T
authors.lastname: Grandmougin
authors.forename: R
authors.initials: R
authors.lastname: Jény
authors.forename: P
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authors.lastname: Bartolomeo
authors.forename: G
authors.initials: G
authors.lastname: Dalla Barba
authors.forename: J D
authors.initials: JD
authors.lastname: Degos
authors.forename: F
authors.initials: F
authors.lastname: Lisovoski
authors.forename: A M
authors.initials: AM
authors.lastname: Ergis
authors.forename: E
authors.initials: E
authors.lastname: Pailhous
authors.forename: P
authors.initials: P
authors.lastname: Cesaro
authors.forename: P
authors.initials: P
authors.lastname: Hantraye
authors.forename: M
authors.initials: M
authors.lastname: Peschanski
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D016380:Brain Tissue Transplantation; D003071:Cognition; D003342:Corpus Striatum / Q000637:transplantation; D005073:Evoked Potentials, Somatosensory; D016332:Fetal Tissue Transplantation; D005500:Follow-Up Studies; D006801:Humans; D006816:Huntington Disease / Q000503:physiopathology / Q000523:psychology / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D009043:Motor Activity; D009483:Neuropsychological Tests; D014055:Tomography, Emission-Computed; D016896:Treatment Outcome