A machine generated summary based on the work of Göbel, Carl H.; Karstedt, Sarah C.; Münte, Thomas F.; Göbel, Hartmut; Wolfrum, Sebastian; Lebedeva, Elena R.; Olesen, Jes; Royl, Georg. 2020 in The Journal of Headache and Pain.

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Primary Stabbing Headache

DOI: https://doi.org/10.1007/s11910- 019- 0955- 6

Abstract-Summary To provide a comprehensive and updated review of the literature on primary stab- bing headache.

Changes to the ICHD-3 criteria have resulted in increased sensitivity to capture

primary stabbing headache (PSH).

According to the ICHD-3, the sharp stabbing pain is no longer restricted to the

first division of the trigeminal nerve.

Secondary etiologies for stabbing headaches are part of the differential diagno- sis of primary stabbing headache; therefore, it is reasonable to perform neuroimaging.

Introduction Primary stabbing headache (PSH) is a primary headache disorder that was first described by Lansche in 1964 as “ophthalmodynia periodica” [12].

The latest International Classification of Headache Disorders, Third Edition (ICHD-3) criteria was published in 2018 and describes PSH as “transient and local- ized stabs of pain in the head that occur spontaneously in the absence of organic disease of underlying structures or of the cranial nerves.”

Epidemiological of Primary Stabbing Headache Age, gender, referral bias, definition of PSH, and co-morbidity with other headache disorders appear to affect this data.

This difference may be due to referral bias and reporting bias since PSH is com-

monly associated with other headache disorders.

Epidemiological studies have consistently shown that in the adult population, PSH occurs more commonly in females with a female to male ratio of 1.49–6.6:1 [13, 14].

A review of PSH in children found the mean age of onset is ages 4.5–9 and unlike the female predominance in adults, there appears to be no gender predomi- nance in children [15].

Diagnostic Criteria of Primary Stabbing Headache The ICHD-3 diagnostic criteria for Primary Stabbing Headache require all of A to E: data from [16] Headache Classification Committee of the International Headache Society (IHS).

No cranial autonomic symptoms C. Not fulfilling ICHD-3 criteria for any other headache disorder D. Not better accounted for by another ICHD-3 diagnosis Due to changes in the diagnostic criteria and our understanding of primary stabbing head- ache over the years, previously labeled PSH or its equivalents may be excluded or other variations of headache may be included in the current ICHD-3 criteria for PSH.

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The first edition of ICHD, published in 1988, referred to PSH as a idiopathic

stabbing headache [17].

The new 2018 ICHD-3 diagnostic criteria are the most sensitive to capture only

primary headaches causing stabbing pain.

ICHD-3 does not comment on response to indomethacin and does not limit the

location of the stabbing pain to the first division of the trigeminal nerve [16].

Clinical Features of Primary Stabbing Headache The clinical features of primary stabbing headache include the type of pain, dura- tion, frequency, location, and lack of associated symptoms.

The mean duration of the pain was 1.42 s in a group of 280 migraine patients

with distinct co-morbid primary stabbing headaches [18].

Previous studies on primary/idiopathic stabbing headache may have excluded patients who would now be included in the ICHD-3 criteria such as those with stab- bing pain outside of the first division of the trigeminal nerve.

Triggers for PSH are not common, but a few case reports have described some potential triggers for paroxysmal stabbing pain particularly in the patients with co- morbid migraine.

Head motion, rapid alterations in posture, physical exertion, and bright lights in patients during a migraine attack appear to trigger stabbing pain in the same loca- tion as the migraine [19]; however, these triggers are an unlikely culprit in true pri- mary stabbing headache.

Pediatric PSH A recent review of ICHD-3 beta highlights that headache disorders in children have bio-psycho-social aspects that distinguish clinical presentation and management, although these specific aspects have not been elucidated for PSH [20].

Pain from PSH in children can be located in many regions, including more occip-

ital predominance in comparison with adults [21, 22].

Pediatric PSH is less often associated with other headache types, including migraine [15, 23], but may be associated with extracephalic symptoms such as abdominal pain [24].

Proposed Mechanism Current theories include irritation of trigeminal and extratrigeminal nerves and/or intermittent impairment of central pain processing leading to hyper-excitability of neurons or spontaneous synchronous discharge of neurons.

Ephaptic impulses are presumed to travel to the corresponding peripheral nerve

distribution with the perception of stabbing pain [25, 26].

Other theories include dural sinus stenosis [27] and brainstem inflammation or focal demyelination [28, 29]; although these theories would suggest a secondary etiology for the stabbing headache.

Differential Diagnosis The different diagnosis for PSH includes short-lasting, stab-like primary and sec- ondary headaches, and may provisionally include probable PSH.

The diagnosis of all primary headaches must exclude secondary etiologies.

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3 Diagnosis

After the secondary etiologies have been considered and ruled out, the differen-

tial diagnosis of stabbing headaches is limited to primary headaches disorders.

The duration, frequency, location, presence or absence of cranial autonomic fea-

tures, and triggers are used to determine the primary headache disorder.

Although PSH, like paroxysmal hemicrania and hemicrania continua, is responsive to indomethacin, these latter headache disorders have longer duration of pain and presence of cranial autonomic features, which differentiates them from PSH.

Investigations Recurrent stabbing headaches could be due to secondary aetiologies; therefore, neu- roimaging is reasonable.

Blood work including ESR is also reasonable in patients over the age of 50 who present with stabbing pain particularly if they have additional features of giant cell arteritis [30].

Treatment Responsiveness to indomethacin is not specific to PSH.

Response to indomethacin is now known to vary, and some experts estimate up

to 60% of patients with PSH may respond to indomethacin treatment [31]. The mechanism of indomethacin for PSH may be anti-inflammatory. For patients with inadequate response, contraindications or intolerance to indo- methacin, alternative treatment options suggested from small observational studies include other NSAIDs, such as selective COX-2 inhibitors, etoricoxib [32] and cele- coxib [33], melatonin [34–38], onabotulinumtoxin A (BoNTA) [39], gabapentin [40], topiramate [27], acetazolamide [41], and nifedipine [42].

Similar to indomethacin, the mechanism of benefit from acetazolamide and topi- ramate may be from their effect to lower of intracranial pressure, although this remains to be studied in PSH [43, 44].

No adverse effects were reported and therefore BoNTA may be a logical treat-

ment option for PSH.

Indomethacin is not often used in children less than age 15, but there have been some case reports of effective indomethacin treatment for two patients ages 2.5 and 5 [21].

Conclusion Epidemiological data on PSH will need to update to reflect this change.

Although indomethacin continues to be the main therapy for PSH, other treat- ment options including selective COX-2 inhibitors, melatonin, and onabotulinum- toxin A may be considered.

Acknowledgement A machine generated summary based on the work of Murray, Danielle; Dilli, Esma 2019 in Current Neurology and Neuroscience Reports.

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Automatic migraine classification via feature selection committee and machine learning techniques over imaging and questionnaire data