OC-07 | A retrospective analysis of headache and CADASIL association

Danilo Antonio Montisano,¹ Giulia Regonesi,¹ Licia Grazzi,¹ Alessandra Parisi,¹ Alberto Raggi,² Elisabetta Soldini,² Giorgia Camarda,² Matilde Leonardi,² I. Canavero,³ E.M. Zacarias,³ Anna Bersano,³ Nicola Rifino³ | Fondazione IRCCS Istituto Neurologico Carlo Besta: ¹SSD Centro Cefalee; ²SC Neurologia Salute Pubblica e Disabilità, ³SC Malattie Cerebrovascolari, Milan, Italy

Background: Literature has shown that migraine, especially with aura, is a common comorbidity of CADASIL. However, the extent to which clinical and risk-related aspects of CADASIL are associated to migraine and tension-type headache (TTH) is unknown. In this study we addressed such associations based on retrospective data.

Methods: We conducted a cross-sectional analysis of data from the prospectively collected cohort of patients diagnosed with CADASIL at a specialized center. Patients were screened with the Migraine Screening Questionnaire (MS_Q; positive if >=4), and diagnoses for suspect cases (also with MS_Q >=3) were confirmed by neurologists. Associations between Fazekas score (3 is indicative of severe microvascular damage), EGFr risk score (3 is indicative of high risk), lacune count (0, 1-4, 5+ lacunes), NOTCH3–SVD Staging (score 0-4, higher is worse), and headache-related variables were analyzed using chi-square tests for categorical variables and t-test for quantitative variables.

Results: We included 108 patients, 64 (59%) females, mean age 53±14; 50 out of 108 (46%) were MS-Q positive and 59 (55%) fulfilled ICHD-3 criteria for a primary headache, of whom 48 for migraine (44%), and 30 for TTH (25%); 19 fulfilled the criteria for both migraine and TTH. Fazekas score was available for 98 patients (51% scored 3) and EGFr for all patients (53% scored 3). Neither Fazekas, nor EGFr or vasculare events were associated to MS_Q>=4, to any headache diagnosis and to monthly migraine/headache days and intake of medications. Lacune counts was associated to any migraine (p =.007) and TTH diagnosis (p=.003), with patients with established diagnosis having lower lacunes; NOTCH3–SVD score was associated to MS_Q>=4 (p =.029), diagnosis of any migraine (p =.006) and TTH diagnosis (p =.001), with patients with higher risk or stablished diagnosis having lower NOTCH3–SVD scores.

Conclusion: More than half of patients with CADASIL have a comorbidity with a headache disorder, and 44% with migraine, a rate that is three times that of the general population. Fazekas score and EGFr were not associated to headache diagnoses, whereas headache diagnoses, particularly TTH, were associated to lower lacune counts and NOTCH3–SVD score.