Rare Primary Headaches

Conclusion

Rare primary headaches are a heterogeneous group of headache entities. The underlying pathophysiology is more or less unknown in all headaches. Therapeutic recommendations are based on case reports, and small case series as controlled trials are missing due to the low prevalence of these diseases. A secondary cause of the presenting headache should always be excluded with appropriate neuroimaging, and in some cases a lumbar puncture is necessary.