title: Cerebellar-dependent associative learning is preserved in Duchenne muscular dystrophy: a study using delay eyeblink conditioning.
abstract: OBJECTIVE
  Besides progressive muscle weakness cognitive deficits have been reported in patients with Duchenne muscular dystrophy (DMD). Cerebellar dysfunction has been proposed to explain cognitive deficits at least in part. In animal models of DMD disturbed Purkinje cell function has been shown following loss of dystrophin. Furthermore there is increasing evidence that the lateral cerebellum contributes to cognitive processing. In the present study cerebellar-dependent delay eyeblink conditioning, a form of associative learning, was used to assess cerebellar function in DMD children.
  
  
  METHODS
  Delay eyeblink conditioning was examined in eight genetically defined male patients with DMD and in ten age-matched control subjects. Acquisition, timing and extinction of conditioned eyeblink responses (CR) were assessed during a single conditioning session.
  
  
  RESULTS
  Both groups showed a significant increase of CRs during the course of learning (block effect p < 0.001). CR acquisition was not impaired in DMD patients (mean total CR incidence 37.4 ± 17.6%) as compared to control subjects (36.2 ± 17.3%; group effect p = 0.89; group by block effect p = 0.38; ANOVA with repeated measures). In addition, CR timing and extinction was not different from controls.
  
  
  CONCLUSIONS
  Delay eyeblink conditioning was preserved in the present DMD patients. Because eyeblink conditioning depends on the integrity of the intermediate cerebellum, this older part of the cerebellum may be relatively preserved in DMD. The present findings agree with animal model data showing that the newer, lateral cerebellum is primarily affected in DMD.
authors.affiliation: Department of Neuropediatrics, Developmental Neurology and Social Pediatrics,University of Duisburg-Essen, Essen, Germany.
authors.forename: Ulrike
authors.initials: U
authors.lastname: Schara
authors.affiliation: Department of Neuropediatrics, Developmental Neurology and Social Pediatrics,University of Duisburg-Essen, Essen, Germany.
authors.forename: Melanie
authors.initials: M
authors.lastname: Busse
authors.affiliation: Department of Neurology, University of Duisburg-Essen, Essen, Germany.
authors.forename: Dagmar
authors.initials: D
authors.lastname: Timmann
authors.affiliation: Department of Neurology, University of Duisburg-Essen, Essen, Germany.
authors.forename: Marcus
authors.initials: M
authors.lastname: Gerwig
mesh_terms: D000293:Adolescent; D001767:Blinking; D002526:Cerebellar Diseases / Q000235:genetics / Q000503:physiopathology*; D002531:Cerebellum / Q000378:metabolism / Q000503:physiopathology*; D002648:Child; D003214:Conditioning, Classical; D003215:Conditioning, Eyelid; D005260:Female; D006801:Humans; D007858:Learning; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000235:genetics / Q000503:physiopathology*