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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]