J Headache Pain (2003) 4:125–131 DOI 10.1007/s10194-003-0046-5 REVIEW Juan A. Pareja Nummular headache, trochleitis, supraorbital Julia Pareja Julio Yangüela neuralgia, and other epicranial headaches and neuralgias: the epicranias Received: 7 April 2003 Abstract Nummular headache is char- accompaniments, tenderness on the Accepted in revised form: 28 August 2003 acterized by mild to moderate, pres- emergence or course of a pericranial sure-like head pain exclusively in a nerve or on the tissues where pain orig- small, rounded or oval area without inates, and possible presence of symp- underlying structural lesions. Either toms and signs (including effective during symptomatic periods or interic- treatment with locally injected anes- tally, the affected area shows a variable thetics or corticosteroids) of nerve dys- combination of hypoesthesia, dysesthe- function. These observations led to the sia, paresthesia, tenderness or discom- emergence of a conceptual model of fort. The particular topography and head pain with an epicranial origin that signs of sensory dysfunction suggest we propose to group under the appella- that nummular headache is an extracra- tion of epicranias (headaches and peri- nial headache probably stemming from cranial neuralgias stemming from epi- J.A. Pareja (౧) epicranial tissues such as the terminal cranial tissues). Nummular headache is Department of Neurology, branches of sensory nerves. Apart from the paradigm of epicranias. Epicranias Alcorcón Hospital Foundation, nummular headache, other headaches essentially differ from other primary Budapest 1, 28922 Alcorcón, Madrid, Spain and neuralgias such as idiopathic stab- headaches with an intracranial origin e-mail: [email protected] bing headache, trochleitis, supraorbital and features of visceral pain, i.e. Tel.: +34-91-6219513 neuralgia, external compression splanchnocranias that are characterized Fax: +34-91-6219975 headache, nasociliary neuralgia, occipi- by a painful area wider than that of J. Pareja tal neuralgias, and auriculotemporal epicranias, no clear borders, presence Department of Pediatrics, neuralgia have temporal or spatial fea- of autonomic features, regional muscle Santa Bárbara Hospital, tures that suggest a peripheral tension, and driving of the process Puertollano, Ciudad Real, Spain (extracranial) origin, i.e. stemming from the brain and brainstem. J. Yangüela from the bone, scalp, or pericranial Department of Ophthalmology, nerves. Common to these disorders is a Key words Nummular headache • Alcorcón Hospital Foundation, focal localization or a multidirectional Trochleitis • Supraorbital neuralgia • Alcorcón, Madrid, Spain sequence of paroxysms, paucity of Epicranias • Splanchnocranias Introduction there are a few phenotypes a headache can express [1]. These are migraine, tension-type headache, cluster headache, increased and decreased intracranial pressure, local lesion type, In the secondary headaches section of the 1988 classification vasodilator type and stabbing type. Theoretically, all such types of the International Headache Society (IHS), it was stated that of headache may have a primary and a symptomatic form. 126 Local lesion-type headache was described as continu- painful area does not seem to change in either shape or ous pain having a distinct maximum in a circumscribed size with time. area of 5 cm or less, but eventually spreading to the sur- Either during symptomatic periods or interictally, the roundings or referring to more distant areas. Pain from affected area may show a variable combination of hypoes- cranial bone metastasis was noted as the prototype of this thesia, dysesthesia, paresthesia, tenderness or discomfort. symptomatic focal headache. A primary circumscribed The pain is usually continuous with a chronic or remitting headache has recently been described as nummular temporal pattern. Pseudoremissions may be observed when headache (NH) [2]. It is characterized by mild to moder- the pain reaches a very low grade or when only discomfort ate, pressure-like, rather continuous pain, exclusively felt (not pain) in the affected area is reported. At times discom- in a rounded or elliptical area typically 2–6 cm in diame- fort may prevail. Lancinating exacerbations lasting for sev- ter, and neither attributed to local lesion nor systemic dis- eral seconds or gradually increasing from 10 minutes to 2 order [2]. hours may superimpose the baseline pain. Autonomic symp- Head pain is mostly conveyed by the trigeminal nerve, toms are typically absent. but the clinical manifestations may essentially differ Most patients worried that a serious underlying disease depending on the level of trigeminal lesion or dysfunction. could be the cause of their focal cranial pain. Since the pain Intracranial activation of the trigeminovascular system is a itself is, in most cases, not annoying, only reassurrance is final common pathway for many primary hedaches [3]. generally necessary. However, the extracranial, sensitive branches of the trigeminal nerve could be at either the origin of the pain or the conveyance of local (extracranial) nociceptive head pain. In such cases, the painful area would be restricted to Idiopathic stabbing headache either the cutaneous territory of a dysfunctioning pericra- nial nerve or the involved tissues within a focal area. In Idiopathic stabbing headache (ISH) is characterized by the addition, signs and symptoms of neuropathic pain, such as extreme brevity of the paroxysms, generally lasting 1–2 tenderness along the course of the nerve, hypoesthesia or seconds and rarely up to 10 seconds. The stabs can affect dysesthesia should be found, indicating nerve dysfunction. any area of the head, with erratic changes in the location of Otherwise, there may also be focal discomfort or tender- the pain between one paroxysm and the next, either in the ness on the tissues of the affected area. same or the opposite hemicranium. Stabs may even syn- Herein, we review a number of headaches and neuralgias chronously occur on either side of the hemicranium. The that we postulate share the common characteristics of being paroxysms may appear always in the same region, usually highly localized and supposedly stemming from extracranial the orbit [4–9]. structures. For the head pain with a presumed peripheral ori- The lack of topographic organization of ISH parox- gin, we propose the general term epicranias which includes ysms makes their occurrence unpredictable both tempo- epicranial headaches and epicranial neuralgias. rally and spatially. Such chaotic localization could be the expression of multiple presumed origins, most probably in the terminal sensory fibers of the pericranial nerves [9]. This is why we postulate that ISH may have a “peripher- Epicranial headaches al” origin. ISH is a benign entity, it does not associate with any Nummular headache structural lesion and it often coexists with other primary headaches, usually migraine, tension-type headache, and This recently described headache [2] has an unusual, dis- hemicrania continua. ISH either appears synchronously with tinct feature: it is characterized by mild to moderate, pres- the concurrent headache, or both headaches follow an inde- sure-like pain exclusively felt in a rounded or oval area pendent course. Only in those cases in which the stabs are typically 2–6 cm in diameter, without underlying structur- confined to the same area, it is highly recommended to al lesions. The disorder tends to start from the forth decade exclude an underlying structural process. of life, and it prevails in women. The frequency is highly variable, going from one to sev- Although any region of the head may be affected, the eral paroxysms a day. Rarely, it has been described a “stab- parietal area, particularly its most convex part (tuber pari- bing headache status”. Usually the clinical course is either etale) is the common localization of this circumscribed sporadic or recurrent, and only exceptionally can a chronic headache. The pain remains confined to the same sympto- temporal pattern be recognized. matic area without duplication or multiplication of the The paroxysms are of moderate intensity, but at the symptoms in other parts of the head. Moreover, the same time surprising and alarming to the patients, who 127 ask for a reasonable explanation. Therefore, the patients congenital restrictive ophthalmopathy with shortening must be reassurred and clearly informed of the benignity and fibrosis of the superior oblique muscle tendon. It of the process. Only on rare occasions, when the parox- causes strabismus but no pain. Acquired Brown’s syn- ysms become extremely frequent, treatment with drome (symptomatic trochleitis) [13–17] is produced by indomethacin is recommended. A dose of 75 mg/day has local inflammatory processes, such as rheumatoid arthri- proved to be partially or totally effective in two-thirds of tis, hyperthyroidism, lupus, psoriasis or enteropathic patients. arthropathy (inflammatory bowel disease: ulcerative coli- tis and Crohn’s disease). Exceptionally, it can be caused by sinusitis, trauma or metastasis. In symptomatic trochleitis there is a severe restriction of vertical eye External compression headache movements with diplopia. Blood tests (routine blood work, erythrocyte sedimentation rate, standard biochemi- External compression headache results from continued cal determinations, thyroid function, antinuclear antibod- stimulation of cutaneous nerves of the head by the appli- ies, rheumatoid factor) and urine analysis are essential to cation