title: Abnormal sensorimotor plasticity in CADASIL correlates with neuropsychological impairment.
abstract: OBJECTIVE
  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a small vessel disease of the brain caused by mutations in the NOTCH3 gene. CADASIL progresses, in some cases, to subcortical dementia with a particular cognitive impairment. Different diseases in the dementia spectrum share a central cholinergic and sensorimotor plasticity alteration. We aimed to study different intracortical circuits and sensorimotor plasticity in CADASIL patients using transcranial magnetic stimulation protocols, and to determine whether these characteristics correlated with the results of clinical neuropsychological evaluation.
  
  
  METHODS
  Ten CADASIL patients and 10 healthy subjects were included in the study. All subjects underwent a transcranial magnetic stimulation study examining different intracortical circuits. Sensorimotor plasticity was also assessed using a paired associative stimulation and extensive neuropsychological tests.
  
  
  RESULTS
  CADASIL patients showed a lack of intracortical facilitation, short latency afferent inhibition and sensorimotor plasticity when compared with control subjects. CADASIL patients also showed an altered neuropsychological profile. Correlation between sensorimotor plasticity and neuropsychological alterations was observed in CADASIL patients.
  
  
  CONCLUSIONS
  These results suggest that acetylcholine and glutamate could be involved in the dementia process in CADASIL and that abnormal sensorimotor plasticity correlates with the neuropsychological profile in CADASIL patients.
authors: Unidad de Trastornos del Movimiento, Servicio de Neurología y Neurofisiología Clínica, Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Rocío/CSIC/Universidad de Sevilla, Seville, Spain.
authors: Francisco J
authors: FJ
authors: Palomar
authors: Aida
authors: A
authors: Suárez
authors: Emilio
authors: E
authors: Franco
authors: Fátima
authors: F
authors: Carrillo
authors: Eulogio
authors: E
authors: Gil-Néciga
authors: Pablo
authors: P
authors: Mir
mesh_terms: D000328:Adult; D000368:Aged; D046589:CADASIL / Q000503:physiopathology* / Q000523:psychology*; D016022:Case-Control Studies; D002540:Cerebral Cortex / Q000503:physiopathology*; D004576:Electromyography / Q000379:methods / Q000523:psychology; D019054:Evoked Potentials, Motor / Q000502:physiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009434:Neural Pathways / Q000503:physiopathology; D009473:Neuronal Plasticity / Q000502:physiology*; D009483:Neuropsychological Tests / Q000706:statistics & numerical data*; D050781:Transcranial Magnetic Stimulation / Q000379:methods / Q000523:psychology