Primary stabbing headache: a new dural sinus stenosis-associated primary headache?

Abstract

Primary stabbing headache (PSH) is a primary syndrome of unknown aetiology, characterised by brief, jabbing stabs predominantly felt in the orbital, temporal and parietal areas, whose frequency may vary from one to many per day, usually responding to indomethacin. PSH frequency in the general population is not well defined, but recent evidence suggests it could be more frequent than previously thought. In clinical series, PSH incidence was 33/100,000 per year, while in a population study 35.2 % prevalence was found. PSH was previously described as isolated or associated to other headache syndromes, most frequently with migraine. There is evidence that an idiopathic intracranial hypertension without papilledema, a condition usually associated to significant stenosis of dural sinuses (93 % sensitivity and specificity), is much more prevalent than believed and may run asymptomatically in up to 11 % of otherwise healthy individuals. In migrainous prone people, a sinus stenosis-associated intracranial hypertension without papilledema (ss-IHWOP) comorbidity may represent a powerful risk factor for progression of pain. Besides migraine, significant sinus stenosis has been found overrepresented also in chronic tension type headache as well as in exertional, cough, sexual activity-associated headaches (all indomethacin responsive primary headaches) and in altitude headache (an acetazolamide responsive condition). To explore the possible association between venous outflow disturbances and PSH, we retrospectively investigated the co-occurrence of sinus venous stenosis in patients referring to our headache centre since 2004 diagnosed with PSH who completed the diagnostic protocol. Out of 50 consecutive patients reporting PSH as the main or as accessory complaint, 8 (6 females, 2 males) performed MR venography (MRV). All MRV revealed significant unilateral or bilateral sinus stenosis. Mean age at PSH onset was 35.3 ± 18.9 years (range 11-67 years). Duration of attacks ranged 1-3 s. Median daily frequency of attacks was 4 (range 2-20); median number of days per month with PSH presentation was 14 (range 4-30). Six patients described attacks in temporal or parietal areas, one at the top of the head, and one in the occipital area. Only one patient had isolated PSH; all the others were diagnosed also with migraine without aura. Seven out of eight patients responded to indomethacin 75 mg/die, and one to topiramate 100 mg/die. Interestingly, both drugs share with acetazolamide a CSF pressure lowering effect. Our findings indicate that PSH is associated with central sinus stenosis and suggest that an undiagnosed ss-IHWOP might be involved in PSH pathogenesis.

Primary stabbing headache in children and adolescents

“…cough headaches, nummular headaches, cold stimulus headaches, or headaches associated with sexual activities) are unlikely to account for the pathogenesis of the stabs among our patients as they were not precipitated or triggered by cough, cold, sex, posture, or other manoeuvres. Transient stabbing headache has been reported among patients with underlying sinister causes . In our cohort, patients were typically developing individuals with typical neurological examination and their brain imaging showed no intracranial abnormalities.…”

“…The decision regarding whether or not to perform brain imaging in PSH is a dilemma. A review of the literature reveals that organic causes such as brain tumour may present with new‐onset stabbing headaches . The ICHD‐3 beta undoubtedly suggests stabs that are strictly localized to one region of the cranium may indicate underlying organic causes that must be excluded .…”

“…A review of the literature reveals that organic causes such as brain tumour may present with new-onset stabbing headaches. 5,[11][12][13][14][15][16][17][18] The ICHD-3 beta undoubtedly suggests stabs that are strictly localized to one region of the cranium may indicate underlying organic causes that must be excluded. 1 Therefore, we have arranged, after parental agreement, brain imaging for patients with stabs.…”

Intracranial pressure in unresponsive chronic migraine

“…Notably, the prevalence of intracranial venous sinus stenosis at magnetic resonance venography (MRV) was found to be much higher than that previously expected in both IIHWOP and CM [7, 8]. Sinus stenosis has been recently reported to be highly prevalent also in other primary headaches [9–11]. Sinus stenosis is considered a reliable marker of idiopathic intracranial hypertension (IIH) with a sensitivity and specificity of 93 % [12].…”

Primary and Secondary Stabbing Headache

“…It is not clear if a similar mechanism on a smaller scale would be relevant to the occurrence of primary stabbing headache in the general population. Finally, one case series described 8 patients with primary stabbing headache, 7 of whom also had migraine, who on magnetic resonance venography had unilateral or bilateral transverse sinus stenosis . The authors posited that since this finding is also occasionally associated with idiopathic intracranial hypertension without papilledema, a similar pathophysiology could be relevant.…”