Objective:

We systematically evaluate the clinical features and management of migraine in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) in a multi-center health system. 

Background:

Migraine is common in patients with CADASIL and may be the earliest symptom of the disease.

Design/Methods:

Clinical data was extracted from the electronic medical records of patients diagnosed with CADASIL and managed at Mayo Clinic Enterprise. Diagnosis was confirmed by a pathogenic NOTCH3 mutation or granular osmiophilic material on skin biopsy. 

Results:

Seventy-five patients with CADASIL were identified. Fifty-one patients (68.0%) reported headaches, with a mean age of onset of 36.3 years (SD = 15.6). Thirty patients (40.0%) had a diagnosis of migraine with aura and ten patients (13.3%) had a diagnosis of migraine without aura. The most common type of aura was visual (83.3%). Male sex was associated with lower odds of diagnosis of migraine (OR = 0.1, 95% CI = 0.0 - 0.6, P = 0.007). Twenty-seven patients (67.5%) required at least one form of acute therapy, and twenty-three patients (57.5%) required at least one form of preventive therapy. Acute therapy helped in twenty-five cases (92.6%) and preventive therapy helped in sixteen cases (69.6%). The presence of visual aura was associated with higher odds of need for acute therapy (OR = 3.6, 95% CI = 1.9 - 6.7, P = 0.003). Also, the presence of any type of aura was associated with higher odds of response to preventive therapy (OR = 75.0, 95% CI = 3.9 - 1434.1, P = 0.004).

Conclusions:

More than half of patients with CADASIL had a diagnosis of migraine, most commonly female patients. Around two thirds of them tried at least one form of acute or preventive therapy. Resistance to preventive therapy was seen in around 30% of the cases, highlighting the need for advances in migraine management in CADASIL.