Neurological Sciences

, Volume 25, Supplement 3, pp s77–s83

Rare primary headaches: clinical insights


DOI: 10.1007/s10072-004-0258-8

Cite this article as:
Casucci, G., d’Onofrio, F. & Torelli, P. Neurol Sci (2004) 25: s77. doi:10.1007/s10072-004-0258-8


So-called “rare” headaches, whose prevalence rate is lower than 1% or is not known at all and have been reported in only a few dozen cases to date, constitute a very heterogeneous group. Those that are best characterised from the clinical point of view can be classified into forms with prominent autonomic features and forms with sparse or no autonomic features. Among the former are trigeminal autonomic cephalalgias (TACs) and hemicrania continua, while the latter comprise classical trigeminal neuralgia, hypnic headache, primary thunderclap headache, and exploding head syndrome. The major clinical discriminating factor for the differential diagnosis of TACs is the relationship between duration and frequency of attacks: the forms in which pain is shorter lived are those with the higher frequency of daily attacks. Other aspects to be considered are the time pattern of symptoms, intensity and timing of attacks, the patient’s behaviour during the attacks, the presence of any triggering factors and of the refractory period after an induced attack, and response to therapy, especially with indomethacin. Often these are little known clinical entities, which are not easily detected in clinical practice. For some of them, e. g., thunderclap headache, it is always necessary to perform instrumental tests to exclude the presence of underlying organic diseases.

Key words

Clinical featuresDifferential diagnosis Trigeminal autonomic cephalalgiasClassical trigeminal neuralgiaHypnic headacheThunderclap headacheExploding head syndrome

Copyright information

© Springer-Verlag Italia 2004

Authors and Affiliations

  1. 1.U. O. di Medicina GeneraleCasa di Cura “San Francesco”Telese Terme (BN)Italy
  2. 2.U. O. NeurologiaAzienda Ospedaliera MoscatiAvellinoItaly
  3. 3.Sezione di Neurologia, Dipartimento di NeuroscienzeOspedale Maggiore Padiglione BarbieriParmaItaly