Case series
Stabbing headache in patients with autoimmune disorders

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Abstract

Stabbing headache is a relatively rare type of chronic “primary” headache with distinctive features with respect to more common forms of paroxysmal headache, such as cluster headache and trigeminal neuralgia. Drug treatment is empirical because of the lack of knowledge on the pathophysiology of stabbing headache. We examined 26 patients recruited over 10 years, who met the diagnostic criteria for stabbing headache. Interestingly, more than half of these patients had autoimmune disorders, including multiple sclerosis, Sjögren's disease, Systemic Lupus Erythematosus, Behçet's disease, autoimmune vasculitis, and antiphospholipid antibody syndrome. We speculate that stabbing headache may develop as a result of neuroinflammation and, at least in some cases, may be an epiphenomenon of focal demyelinating lesions of the upper or lower brain stem.

Introduction

Primary stabbing headache, previously indicated as “ice-pick” pain, jabs and jolts, or ophthalmodynia periodica, is characterized by “transient and localized stabs of pain in the head that occur spontaneously in the absence of organic disease of underlying structures or of the cranial nerves” (International Headache Society; ICHD-II, code 4.1). Stabs last for up to a few seconds, and recur with irregular frequency ranging from one to many per day, without any identifiable cause [1], [2], [3], [4]. Stabbing headache is generally considered as a primary headache, with onset in a young age, greater prevalence in women, and possible association with other forms of headache. The aetiology is unknown. Here, we carried out a detailed analysis of 26 patients who met the diagnostic criteria for stabbing headache. Remarkably, more than half of these patients had demyelinating disorders or disorders that may be associated with demyelination. We speculate that at least some patients with stabbing headache have underlying demyelinating lesions of the CNS.

Section snippets

Methods

In this retrospective study of 20,534 outpatients who visited the Neuropathophysiology Unit of Azienda Policlinico of the University of Catania (Italy) in 10 years (1996–2007), 735 reported various types of headache, and 26 patients (9 men; 17 women) met the standard ICHD-II criteria for stabbing headache. A physician interviewed all patients following a specific protocol for headaches, which included a familiar and personal medical history, time and age at onset, duration, intensity, frequency,

Results

The age at onset in stabbing headache in this study ranged from 18 and 75 years. Symptoms had begun less than 1 year before the visit in 4 patients, less than 5 years in 15 patients, and more than 5 years in 7 patients.

Pain attacks had typically a short duration (a few seconds) and the number of clusters was highly variable (Fig. 1). Pain showed always a high intensity (VAS score, 7–10) and was described as a nail or a needle penetrating the head or as a single stab or series of stabs lasting for

Discussion

Our study suggests that the question raised by the International Classification of Headache Disorders in relation to point 4 of ICHD-II (“primary or secondary headaches or both “?) is particularly appropriate for stabbing headache. This question synthesizes all doubts and uncertainties on the clinical classification and pathophysiology of stabbing headache. Stabbing headache is currently classified in the group of “other primary headaches” (code 4.1 of ICHD-II), but the real nature of this type

Conflict of interest

The authors declare that they have no conflict of interest related to the publication of this manuscript.

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