Migraine in Chiari 1 Malformation: a cross-sectional, single centre study

DOI: https://doi.org/10.1007/s13760- 021- 01716- z

2.3 Comorbidities

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Abstract-Summary In Chiari 1 Malformation (CM1) the most frequent symptom is exertional headache, but other headache types have been reported, such as migraine.

This cross-sectional study is aimed to examine the prevalence of migraine in a group of CM1 headache patients and to compare clinical-demographic characteris- tics between migraine and non-migraine patients.

The association between CM1 diagnosis and headache characteristics (indepen- dent variables) and migraine diagnosis (dependent variable) was estimated by logis- tic regression models.

Seventy-eight patients (67 females) out of 230 presenting headache had a migraine (34%), 44/78 (56%) with aura; in 58/78 (74%) migraine was comorbid with secondary headache attributable to CM1.

Migraine prevalence in patients with isolated CM1 (52/120, 43.3%) was higher

(p = 0.0016) than in all the other patients (26/110, 23.6%).

This study shows a high prevalence of migraine in CM1 patients and a significant

association between migraine and isolated CM1.

Extended: Future larger studies are required to address these issues.

Introduction One of the most frequent symptoms in CM1 is exertional headache, occurring in 50–81% of CM1 patients [447, 448].

Pathognomonic characteristics of headache for CM1 are lacking and many other types of primary headaches have been reported in CM1 patients, such as migraine, tension-type headache, and cluster headache [449, 450].

The question, therefore, arises as to the possible co-occurrence of a primary and secondary headache in CM1 patients, where caution is required not to over- diagnose migraine as a headache attributed to CM1, which may affect the CM1 management. 57–93% of the headaches experienced by children and adults with CM1 have

been reported as improved after surgery [451–453].

In the management of these patients, other types of chronic headaches, not CM1

related, such as migraine, need to be diagnosed and pharmacologically treated.

Methods Between January 1, 2010 and December 2017, 427 adult patients with CM1 and syringomyelia were enrolled for a clinic, epidemiologic and genetic study, by the Centro Regionale Esperto Siringomielia e Sindrome di Chiari (CRESSC), Neuroscience Department, Turin.

Frequencies (absolute and percentage values) were calculated in the whole sam- ple for (a) migraine presence (with and without aura), (b) gender, (c) age, (d) diag- nosis (isolated CM1, other diagnosis).

Headache clinical characteristics (frequency, duration, quality, site, irradiation, symptoms, relieving and aggravating factors) were calculated as the frequency in the whole sample and isolated CM1 (CM1-type B) subgroup, both in migraine and non-migraine patients.

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2 Mechanisms

Logistic regression models to study the associations between CM1 diagnosis and headache characteristics (independent variables) and risk of migraine (dependent variable) were fitted, including variables that reached p < 0.1 at univariate analysis.

Results In this total sample of 427 patients, 78 (18%) had migraine, 44 (10%) with aura, 34 (8%) without aura.

230 patients, 78/230 (34%) had a migraine headache. Secondary headache attributable to CM1 was reported in 182/230 (79%) of

patients with headache, 43% of the total sample (427 patients).

Of the 78 patients who had migraine headaches, 58 (74%) also described second-

ary headache attributable to CM1.

Prevalence of migraine in patients with CM1 type B (52/120, 43.3%) was signifi-

cantly higher (p = 0.0016) compared to the other patients (26/110, 23.6%).

Discussion Both primary headache, such as migraine, and a secondary one, attributable to CM1, were reported in more than half of the sample; prevalence of migraine, mostly migraine with aura, was high in this adult CM cohort, both in the whole headache sample (34%) and even more in CM1 isolated subgroup (43%).

In 58 patients, migraine and secondary headache attributable to CM1 were in

comorbidity (74%).

Our results suggest that a primary headache, i.e. migraine, is highly prevalent in

CM1 patients, where it coexists with a secondary headache in 74% of the cases.

Is the fact that in this study no statistically significant difference was detected between the proportion of CM1 patients with and without migraine reporting each of the features required by the International Headache Society to diagnose headache attributable to CM1.

Conclusion This study quantified a significant prevalence of migraine in CM1 patients, often in comorbidity with headache secondary to the condition.

Future works would be welcome to evaluate the prevalence of CM1 in a large migraine cohort, to confirm if this linkage may be found also in migraineurs and to fully understand the relationships between headache, migraine and CM1.

Acknowledgement A machine generated summary based on the work of Ciaramitaro, Palma; Rota, Eugenia; Ferraris, Marilena; Stura, Ilaria; Migliaretti, Giuseppe; Cocito, Dario. 2021 in Acta Neurologica Belgica.

Headache in the course of multiple sclerosis: a prospective study