Fatal status migrainosus in Chiari 1 malformation

BACKGROUND: Headaches are common in Chiari Type 1 malformation (CM-1). The prevalence of migraine headaches in CM-1 is similar to that of the general population. However, when migraine headaches occur with CM-1, they tend to have an earlier age of onset, are more frequent and certainly more severe than when they occur without CM-1 association. The exact role or impact of CM-1 in migraine headaches has not been fully elucidated. CASE DESCRIPTION: We report a fatal case of status migrainosus in 7 years old with CM-1 and review the literature on the possible associations. CONCLUSION: Migraines occurring in association with CM-1 pose a management challenge and can be potentially fatal especially if associated with autonomic symptoms. The exact pathophysiological interaction between these two conditions when they occur simultaneously needs to be further elucidated.