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   J Child Neurol. 2010 May;25(5):623-7. doi: 10.1177/0883073810361382. Epub 2010   
   Mar 1.   
   Childhood-onset CADASIL: clinical, imaging, and neurocognitive features.   
   Hartley J1, Westmacott R, Decker J, Shroff M, Yoon G.   
   Author information   
   Abstract   
   CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts   
   and leukoencephalopathy) is a progressive neurodegenerative condition,   
   associated with mutations in the notch3 gene. Symptoms include migraine with   
   aura, mood disorders,    
   progressive cognitive decline, subcortical ischemic strokes, dementia, and   
   premature death. We present an 8-year-old boy with attention and behavioral   
   difficulties, as well as a family history of the condition. Magnetic resonance   
   imaging revealed    
   subcortical foci of increased T2 hyperintensity, and sequencing of the notch3   
   gene revealed 1 previously reported mutation and 1 novel sequence variant.   
   Neurocognitive assessment revealed deficits in several aspects of executive   
   functioning and in verbal    
   learning. To our knowledge, this is the youngest reported patient with this   
   condition, and it prompts reconsideration of CADASIL as an adult-onset disease.   
   PMID: 20197270 [PubMed - indexed for MEDLINE]   
      
      
   http://www.ncbi.nlm.nih.gov/pubmed/20197270   
      
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