CADASIL (Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy) Medication

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

Aspirin and related medications are often used in an effort to prevent thrombotic occlusion of cerebral arteries. However, the benefit of antiplatelet agents for CADASIL has not been established. [5] Moreover, because of the potential presence of microhemorrhages, using these drugs in patients with CADASIL may increase their risk of intracerebral hemorrhage. [32] A study involving 168 patients with CADASIL showed that donepezil (Aricept) had no effect on the primary endpoint (the V-ADAS-cog score) in patients with CADASIL with cognitive impairment. [33] Two isolated case reports suggest the benefit of acetazolamide (ACZ) in migraine associated with CADASIL. [34, 35]

In fact, studies using transcranial Doppler and Tc-99m brain perfusion single-photon emission computed tomography (SPECT) scanning have reported increases in cerebral blood flow (CBF) and cerebrovascular reactivity (CVR) after the administration of ACZ. [36, 37] Further investigations evaluating the efficacy and benefit of this agent in CADASIL are needed.

The use of 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors (statins) has not proven any benefit in patients with CADASIL. [4] 5HT1B/1D agonists (triptans) are not routinely used in CADASIL-associated migraine due to a presumed potential to increase the risk of stroke. Medications for migraine prophylaxis are reasonable, although no study has clearly demonstrated their efficacy in CADASIL-related migraine.