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A Cross-Sectional Analysis of Migraine-Related Disability in CADASIL

A Mayo Clinic Cohort

Goldstein, Eric D. MD*; Badi, Mohammed K. MD*; Klaas, James P. MD; Glover, Patrick BS*; Rozen, Todd D. MD*; Huang, Josephine F. MD*; Lin, Michelle P. MD*; Meschia, James F. MD*

doi: 10.1097/NRL.0000000000000253
Original Articles

Objectives: Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is inherited microangiopathy characterized by recurrent subcortical infarcts. A majority of those with CADASIL report coexistent migraine with aura. The authors aim to quantitatively describe migraine-related disability within a CADASIL cohort.

Methods: A cross-sectional analysis was performed in a cohort of CADASIL. The Migraine Disability Assessment (MIDAS) quantified migraine-related disability. Further metrics obtained include first and last migraine, aura semiology, and therapeutic measures.

Results: Twenty-four individuals were included [63% (15/24) female individuals; mean age, 56 y; range, 34 to 81 y]. Fifty-four percent (13/24) reported migraine, whereas 46% (11/24) reporting varying degrees of migraine-related disability. MIDAS Questionnaire scores appeared bimodal: 58% (14/24) scored 0 to 5, 7% (1/24) scored 6 to 10, 7% (1/24) scored 11 to 20 and 33% (8/24) scored over 20. Severe disability was associated with the multiplicity of aura semiologies and poor response to pharmacologic prophylaxis.

Conclusions: A bimodal distribution of migraine-related disability was observed. A third of individuals had a severe disability and appeared medically refractory to medical migraine prophylactic measures. This study may serve as a reference point for future trials quantitatively gauging response of novel migraine treatment strategies within this unique population.

*Department of Neurology, Mayo Clinic, Jacksonville, FL

Department of Neurology, Mayo Clinic, Rochester, MN

Supported by Clinician Investigator funds from Dr James Meschia with financial support from the following donor funds: the Earl and Nyda Swanson Neurosciences Research Fund and Harley N. and Rebecca N. Hotchkiss Endowed Fund in Neuroscience Research honoring Ken and Marietta.

M.K.B. is a research fellow enrolled in the CORPS program at Mayo Clinic in Florida with J.F.M. serving as the mentor. The remaining authors declare no conflict of interest.

Correspondence to: Mohammed K. Badi, MD, 4500 San Pablo Rd, Jacksonville, FL 32224. E-mail:

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