CADASIL (Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy) Follow-up

Updated: Oct 09, 2018
  • Author: Reza Behrouz, DO, FACP; Chief Editor: Helmi L Lutsep, MD  more...
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Follow-up

Further Outpatient Care

Patients with CADASIL should be followed routinely by a neurologist or a vascular neurologist to monitor their degree of disability and rate of progression. Management is primarily symptom-based and addressing rehabilitation needs is paramount. Routine psychiatric follow-up is also reasonable if the patient suffers from a complex mental disorder.

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Further Inpatient Care

Inpatient care of patients with CADASIL depends on the reason for admission. Inpatient care of patients with CADASIL who present with a stroke is not entirely different than patients without CADASIL who have a stroke (see Acute Stroke Management). However, safety and efficacy of recombinant tissue plasminogen activator (rtPA) in patients with CADASIL who present with an acute cerebral infarction within the therapeutic window has not been systematically assessed.

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Inpatient & Outpatient Medications

See Medication.

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Prognosis

Exact mortality rate in patients with CADASIL is unknown.

Age at onset for stroke is 45-50 years.

Mean age at death has been reported to be 61 years after a mean disease duration of approximately 23 years. [6]

Close to 80% of patients are completely dependent immediately before death. [7]

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Patient Education

Genetic counseling

Because CADASIL has an autosomal dominant inheritance, patients and their families should be counseled on the nature of the disorder and the probability of developing or transmitting it.

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