[55, 56] There is no effective disease-specific treatment for CADASIL and current therapy addresses symptoms. For migraine with aura, conventional prophylactic medications are recommended if attack frequency warrants treatment. Acetazolamide has been anecdotally reported to be effective in the prophylaxis of migraine in CADASIL, but randomized, controlled trials are lacking.57-59 Acetazolamide has

also been suggested to improve overall cerebral hemodynamics buy Staurosporine in CADASIL, perhaps suggesting a protective effect, but this has also not been proven in controlled trials.[60, 61] For acute treatment of migraine attacks, triptans and ergot derivatives should generally be avoided due to the high risk of stroke in these patients, and simple analgesics and non-steroidal anti-inflammatory drugs are preferred.[22] For secondary stroke prevention, antiplatelet agents should be used over anticoagulants due to high

prevalence of cerebral microbleeds, which may suggest an increased risk of symptomatic intracerebral hemorrhage.[43, 62] Cerebrovascular risk factors should be tightly controlled, including appropriate use of antihypertensive agents and statins.[22] The efficacy of donepezil in the treatment of cognitive impairment in CADASIL patients was studied in a randomized, controlled trial of 168 patients. There was no significant difference between donepezil and placebo in the primary end-point, which was defined as a change from baseline in the score buy ABT-199 on the vascular Alzheimer’s disease assessment scale cognitive subscale at 18 weeks. Improvement was noted, however, on several secondary end-points that were measures of executive function,

but its clinical significance remains unclear.[63] An important aspect of care in CADASIL patients is supportive and involves rehabilitation, physiotherapy, psychiatric and psychological support, and nursing care. Genetic counseling is also important for these patients and for their at risk asymptomatic family members.[22] Acute head pain in the postpartum period should raise concern, especially if “red flags” are MCE公司 present, as Dr. Robbins points out. The differential diagnosis of sudden severe headache is long, and even when diagnostic testing is negative, a high suspicion level should persist before diagnosing sudden acute (“crash”) migraine or benign thunderclap headache. In this case, initial work-up might have yielded the diagnosis of CADASIL, but the patient was lost to follow-up and further evaluation was halted. It is peculiar that this genetic disease produces in most patients characteristic migraine auras and headaches.