Abstract
Ultrashort cephalic paroxysms are well known. In the parish of Vågå, Norway, 35.2% of the 18-65-year-old subjects (n = 1779) were recently found to have such jabs. In the present work, a search has been made for extracephalic ‘jabs’. A questionnaire was in its entirety administered by the same investigator (O.S.) in a ‘semistructured’ way. Facial jabs were present in three women, and in one of them the pain spread to the head. Four subjects had jabs occurring at random throughout the body, also including the cephalic area. Pure nuchal jabs were present in 12 subjects, 10 of whom were males. This sex preponderance difference differs significantly from that in jabs in general (with 40.2% males). The characteristics of the extracephalic jabs, i.e. the duration and temporal pattern, do not seem to differentiate them essentially from jabs in general. The subjects were not asked specific questions regarding extracranial jabs. Most of the affected individuals gave information spontaneously about their jabs. For these reasons, this study is not a proper prevalence study. It does show, however, that extracranial jabs exist, and it gives some indications as to their frequency.
Introduction
Idiopathic stabbing headache (ISH) (1) and ‘jabs and jolts syndrome’ (jabs) (2) are the terms used by the International Headache Society (IHS) and the International Association for the Study of Pain (IASP), respectively, to describe ultrashort, cephalic pain paroxysms. These two terms are in all probability synonymous and may therefore probably be used interchangeably.
The IASP description states (2) that the syndrome is ‘probably common, since it appears both on its own and in many combinations’. Such combinations have been specified (3–7). Paroxysms of this sort were recently demonstrated to occur frequently, i.e. in 35.2% of the population at large (8).
Jabs have been considered mainly to be located in the trigeminal area: ‘exclusively or predominantly – first division of the trigeminal nerve’ (1). Cases of such paroxysms in the occipital/retroaural areas (9) and ‘occipital/nuchal areas’ (10) have, nevertheless, been described more recently. In our study, the ‘occipital/nuchal area’ group was used since it was hard both for the subject and the investigator to locate the paroxysms exactly (10). In the exceptional case in our series, however, the subject pointed out the neck as the precise localization: the pain paroxysm was primarily located in the neck without any co-location in the occipital area.
In other rarely occurring cases, paroxysms, seemingly of the same nature, were located in the face or at various sites in the entire body.
Various kinds of extracephalic ultrashort paroxysms will be dealt with in the present study.
Patients
In the Vågå study of headache epidemiology, 1779 subjects in the age group 18–65 years were asked about short-lasting paroxysms, i.e. 85.7% of the target group. A structured interview based on a questionnaire was carried out by O.S. A detailed overview of the design of this study has been published previously (11).
Some subjects responded in a positive way to the general questions regarding short-lasting paroxysms, but provided information on several variants as far as localization was concerned.
Such variants were: facial jabs, which were present in three women; the mean present age was 50 years (range 39–62 years); whole body jabs, present in two women and two men; the mean present age was 40 years (range 32–49 years); nuchal jabs, which were present in 12 subjects (two women and 10 men). These jabs were only localized in the neck, i.e. without any spreading of pain to the head. The mean present age was 33 years (range 21–49 years).
None of the subjects belonged in more than one category; in other words, there was a total of 19 subjects in the whole series.
Specific questions regarding extracranial location were not asked; information about these variants was forwarded spontaneously by the subjects.
Results
Facial jabs
In two of the three individuals, there were short-lasting paroxysms; in one (no. 2), the somewhat protracted paroxysms seemed to consist of multiple spikes (Table 1). None of them had experienced jolts or vocalization. One reason for this may simply be the relative rarity of such adjunct phenomena; even jolts are present in only approximately one out of three cases of jabs (10). ‘Features indicating cervical abnormalities’ were present in all three subjects with facial jabs, and in two of them (nos 1 and 3) to a more than minimal extent (Table 1). The average level of such ‘features’ in the entire material in the Vågå study was 0.79+ (see Table 1). Two of them had migraine. The pain attacks spread to the head (temporal area) in one case (no. 3). In this case, jabs appeared both before migraine attacks, and without any temporal relation to such attacks. When antedating a migraine attack, there could be volleys of jabs on the migraine side, which seemed to shift. In these situations, the intensity of the jabs seemed stronger, and the character seemed to change, to an ‘icing’/numbness feeling, locally. This subject, who seemed to have jabs of regular duration, is particularly interesting in connection with observations on the long-lasting jabs/migraine-like headache combination to be reported elsewhere (12).
Facial jabs
Migraine ± A, Migraine with (+) or without (–) aura.
∗‘Icing’ feeling and numbness prior to jabs.
Facial jabs generally seem to have the same qualities as cephalic jabs, only with another localization. The face seems to be a rare localization for jabs. However, facial jabs may not necessarily be as rare as depicted here: jabs were asked about in a general manner, and jabs with a facial localization were not specifically asked about.
Jabs occurring at random throughout the body
In four subjects there had during adulthood been regular jabs of 1–3 s duration, but all over the body. Cephalic jabs were in other words also present in these individuals. There were apparently only solitary jabs, no volleys. The interval between paroxysms was usually long, even of months duration. Jabs occurred in one area, once, or repetitively at one time and then occurring in an entirely different area the next time.
The prevalence figures obtained for this type of jab would seem to be in another category of reliability from those obtained for jabs in the other areas, such as the head, and possibly also the face. The main reason for this is that the principal investigator (O.S.) may not have been so persistent and systematic in trying to unravel such jabs as was the case with ordinary jabs. There is good reason to believe that unless direct questions are posed concerning jabs occurring at random throughout the body, not all such cases will be discovered. One is inclined to believe, however, that whole body jabs do not occur nearly as frequently as cephalic jabs.
Nuchal jabs
A number of subjects had jabs in the occipital area, and in some of them the jabs could also occasionally be co-located in the nuchal area: ‘occipital/nuchal jabs’ (10). However, 12 subjects indicated that they had jabs only in the nuchal area, and not in the back of the head (Table 2). These subjects were not included among those in the ordinary series of jabs (8), because ‘regular’ jabs were defined as being cephalic. The prevalence of nuchal jabs (0.7% in the entire material (12 out of 1779)) is rather low when compared with the prevalence of jabs/ISH in the entire series in the Vågå study, i.e. 35.2%.
Nuchal jabs only – no spreading to occipital area
Case 7: the pain could spread towards the shoulder.
Case 12: dizziness with the jabs. EEG-negative.
In those who had other headaches, there is no positive information as to dependence upon the coexisting headache from a temporal point of view (possible exception no. 11).
M ± A, Migraine with (+) or without (–) aura; T-TH, tension-type headache.
∗Scale from 0 to 5+.
The mean present age was 33 years; and all except two were in the thirties or less. Only three subjects could give quite definite information as to the age of onset: 18–20 years of age. These individuals were thus generally well below the age of degenerative processes. It is striking that 10 of the 12 cases (83%) were males (Table 2). When compared with jabs in general, i.e. with 40.2% males (375 females and 252 males), a significant difference was found (P = 0.009, Fisher's exact test). Could this male preponderance be spurious due to the fact that a question concerning nuchal jabs was not posed to everyone? This is highly unlikely: the general impression from the Vågå study is that the ‘female proneness’ to mention minor complaints greatly exceeds that of males. If the sex preponderance were to be distorted in any way in this series, an artificial under-representation of males would seem more likely.
It is also striking that many of the males had hard physical work, which could also cause extra strain on the neck: eight of 10 males worked as electricians, carpenters, farmers, car mechanics, factory workers, or truck drivers. This may have played a predisposing role. These special circumstances are not a complete explanation, however: there were also female cases, probably with less physically demanding work (Table 2). Traits indicating an abnormal function in the neck were, however, within control limits (mean 0.25; range 0–1.0; mean level in the entire Vågå series 0.79) (Table 2). No particular precipitation mechanisms seemed to be present. Nor had these parishioners been exposed to particular head/neck traumas.
In all except one, the duration seemed to be 1–3 s. The intensity of pain and the long-term pattern did not seem to differ from those of jabs in general. There was apparently no clearly increased prevalence of exertional headache among these subjects (Table 2), the prevalence of exertional headache among the population in general being 12.3%.
In particularly long-lasting jabs (attacks lasting 10–120 s (12)), four of six had migraine, i.e. 67%. The latter migraine frequency does not differ significantly from that in the present nuchal jabs (in four of 12 cases, i.e. 33%; P = 0.075), mainly due to the small numbers in the series.
Discussion
The nuchal jabs
A sizeable number of extracephalic jabs seemed to manifest themselves in the neck only. There are various striking features in those with nuchal localization of jabs, for instance the preponderance of males; also, many of them had an occupation that might seem to entail periodic strain upon the neck. One reason why the jabs manifested themselves in the neck might be increased strain in the nuchal area. However, the fact that the area of subjective complaints coincides with that of cervicogenic headache (CEH) by no means indicates a mutual pathogenesis. There are actually no particular indications that these jabs were even forerunners of a CEH headache. Thus, traits indicating an abnormal function in the neck did not seem to dominate in this group. The age of onset (33 years) may seem to have been somewhat lower than the average age of onset in CEH, i.e. 35.1 ± 17.5 years (13). The neck might still play a role in cephalic jabs in general in a way that we do not grasp at present.
Among the few short-lasting headache forms with a male preponderance is SUNCT syndrome. In the present cases, however, there were no autonomic accompaniments, and there was no definite information as to a unilaterality without side alternation, as in SUNCT syndrome. Moreover, attacks could not be readily precipitated mechanically. Actually, the duration of the solitary paroxysms was also too short for SUNCT to be a real alternative, in that SUNCT attacks have not been observed to last <5 s (14). Another decisive feature is that we were faced with a neck ache, and not a headache in these cases. Possibly, these were jabs of the regular variety, only with a differing location.
The putative nature of jabs
It would seem logical that such short-lasting, apparently rather homogeneous paroxysms have the same background and the same pathogenesis, irrespective of whether there is an anterior or posterior cephalic localization of the jabs. Since the jabs are ‘spread’ over extensive areas, the ‘underlying pathology’ is also likely to be ‘spread’.
It is more likely that there is a fundamental difference between jabs that are and that are not stereotyped as regards localization. Those that have a stereotyped appearance might have a localized, underlying dysfunction. Those with a steady shift of location could originate in the periphery (‘nerve irritation’?).
Any theory that is going to explain the pathogenesis of jabs to the full must explain why jabs seem to be mainly in the cephalic area and also why they appear more frequently anteriorly than posteriorly in the head.
Some reservations concerning this type of research
We have previously been privileged to observe the first few patients with certain headaches, e.g. chronic paroxysmal hemicrania (CPH) and SUNCT syndrome over and over again over years, prior to describing these headaches. We could witness repetitive attacks, study clinical features in detail, and we could convince ourselves of the reproducibility of various features. We could in other words be fairly certain as to various pathological phenomena of a forthcoming attack and also as to the sequence of the various symptoms and signs. We even in the exceptional case had a fair hunch as to when the next attack would appear (in some cases of CPH and, during exacerbations, in SUNCT).
The repeated observations compensated for the low ‘n’ (which actually was only 2–3) in the original communications. Furthermore, and importantly, the patients were suffering considerably and, for that reason, were as eager as we were to find the right answers, hoping for relief. We felt confident with that type of clinical research in spite of the small number of cases.
The situation with the ordinary subject in the present study was entirely different. He/she had been ‘commandeered’ to participate in the study, was not a patient, at least did not feel to be such, and was not personally interested in any deeper exploration of the headache. To the best of our knowledge, not one of the subjects examined had ever consulted a physician because of the jabs/stabs. The allocated time at the examination was limited. If any extraordinary traits were uncovered, there would be only a limited time for details and for control of findings. Admittedly, there were additional telephone interviews with a number of the parishioners to obtain more accurate information and compensate for the original shortage of time, and, in some cases, another appointment was arranged. It remains the case, however, that there were more cases in the present subgroups, namely 3–12, than in the original CPH/SUNCT/hemicrania continua series. Even so, the relative brevity and shallowness of the contact with the subjects created a situation almost diametrically opposite to that which antedated the description of, for example, CPH. The situation during the present investigation was not as well suited to a detailed, entirely correct description of somewhat unusual clinical features.
The present descriptions will therefore naturally lack some precision and detail. The results should accordingly be taken with caution.
Footnotes
Acknowledgements
We are indebted to GlaxoSmithKline of Norway, Pharmacia & Upjohn, and the Alf Harborg Foundation, Department of Neurology, St Olavs. Hospital, Trondheim, Trondheim University Hospital, for generous support during the various phases of the investigation. The authors are also grateful to the personnel at the Vågå Health Centre at Vågåmo for their aid. Last, but not least, we thank the inhabitants of the Vågå commune for their collaboration.