What is the role of medication in the treatment of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)?

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

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.


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