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Table 1 Typical and atypical clinical manifestations of CADASIL

From: Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) as a model of small vessel disease: update on clinical, diagnostic, and management aspects

Typical manifestations  
 Migraine, usually with aura, as the first symptom in the third decade of life  
 Recurrent subcortical ischemic events (transient ischemic attack/stroke) in adulthood  
 Mood disturbances, apathy and depression among other psychiatric symptoms  
 Progressive cognitive decline, especially of executive functioning  
 Seizures, in a smaller but well-defined portion of patients  
Atypical manifestations  
 Pathological gambling [128]  
 Recurrent status epilecticus [129]  
 Schizopheniform organic psychosis [130, 131]  
 Neuropathy [132134]  
 Myopathy [100, 135138]  
 ‘CADASIL coma’ [25, 139]  
 Early onset [140, 141]  
 Late onset [39]  
 Bipolar disorder [142]  
 Inflammatory-like presentation [143]  
 Acute vestibular syndrome [144]  
 Spinal cord involvement [145, 146]  
 Acute confusional migraine [147]  
 Sporadic hemiplegic migraine with normal imaging [148]  
 Post-partum psychiatric disturbances [149]  
 Parkinsonism [150]  
 Recurrent transient global amnesia [151]