CADASIL: clinical features

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

CADASIL: clinical features. Stroke develops in 85% of patients. It typically occurs in the 4th decade. Fully referenced neurology checklist.

Stroke

  • Stroke develops in 85% of patients
  • It typically occurs in the 4th decade
  • It is almost always lacunar
  • The risk is increased by hypertension, smoking, and microbleeds
  • Smoking leads to an earlier onset age of stroke

Migraine

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Neuropsychiatric features

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Other features

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Atypical manifestations

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Severity scoring system

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Poor prognostic features

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Acronym

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References

  1. Adib-Samii P, Brice G, Martin RJ, Markus HS. Clinical spectrum of CADASIL and the effect of cardiovascular risk factors on phenotype study in 200 consecutively recruited individuals. Stroke 2010; 41:630-634.
  2. Razvi SSM, Muir KW. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Pract Neurol 2004; 4:50-55.
  3. Puy L, De Guio F, Godin O, et al. Cerebral microbleeds and the risk of incident ischemic stroke in CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy). Stroke 2017; 48:2699-2703. 
  4. Reyes S, Viswanathan A, Godin O, et al. Apathy: a major symptom in CADASIL. Neurology 2009; 72:905-910.
  5. Schon F, Martin RJ, Prevett M, Clough C, Enevoldson TP, Markus HS. “CADASIL coma”: an underdiagnosed acute encephalopathy. JNNP 2003; 74:249-252.
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