The Frequent Unusual Headache Syndromes: A Proposed Classification Based on Lifetime Prevalence

There is no agreement on a single cutoff point or prevalence for regarding a given disease as rare. The concept of what is a rare headache disorder is even less clear and the spectrum from a very frequent, frequent, occasional to rare headache syndrome is yet to be established.

An attempt has been made to estimate the lifetime prevalence of each of the headache subtypes classified in the ICHD-II.

Using the ICHD-II, 199 different headache subtypes were identified. The following classification was made according to the estimated lifetime prevalence of each headache disorder: very frequent (prevalence >10%); frequent (between 1 and 10%); occasional (between 0.07 and 1%); and unusual or rare (<0.07%).

One hundred and fifty-four of 199 (77%) were categorized as unusual headache disorders, 7/199 (4%) as very frequent, 9/199 (5%) as frequent, and 29/199 (15%) as occasional forms of headache disorder.

The unusual headache syndromes do not appear to be as infrequent in clinical practice as has been generally believed. About three-fourths of the classified headache disorders found in the ICHD-II can be considered as rare. This narrative review article may be regarded as an introduction to the concept of unusual headaches and a proposed classification of all headaches (at least those listed in the ICHD-II).