No comment

1. Nummular Headache test
Brian M. Grosberg, MD, Seymour Solomon, MD, and Richard B. Lipton, MD
Corresponding author
Brian M. Grosberg, MD
Montefiore Headache Center, 1575 Blondell Avenue, Suite 225, Bronx, NY 10461, USA.
E-mail: bgrosber@montefiore.org
Current Pain and Headache Reports 2007, 11:310-312 Current Medicine Group LLC ISSN 1531-
3433
Abstract
Nummular headache is a rare primary headache disorder characterized by focal and
well-circumscribed pain fixed within a round-, oval-, or elliptical-shaped region of the head. The
pain is usually mild to moderate in intensity but may be severe. Nummular headache is most
common in women in the fourth to fifth decade of life. The temporal pattern may be chronic
and continuous since onset, chronic evolved from episodic, or episodic. These headaches
typically are unilateral, side-locked, and fixed in location, commonly affecting the parietal
region. Many patients experience superimposed exacerbations of pain, lasting from seconds to
days. Sensory phenomena, such as parenthesis, allodynia, and dysesthesias, are frequently
reported in the region of the pain. Treatment with gabapentin, tricyclic antidepressants, or
botulinum toxin may be helpful.
Introduction
Derived from the Latin word for "coin," the term nummular headache was first
introduced in 2002, when Parejaet al. [1] described a series of 13 patients with well-
circumscribed pain confined to a round-, oval-, or elliptical-shaped region of the head in their
now classic paper in Neurology. Two years later, the same group reported a series of 14
additional patients [2.0]. Based largely on these reports by Parejaet al., nummular headache
was included in the appendix of the second edition of the International Classification of
Headache Disorders (ICHD-2) [3]. (Subjects included in the ICHD -2 appendix are entities
deemed to be in need of additional study.) Since the 2004 publication, more than 30 additional

2. cases have been reported in the English language literature [4-9].Almost a decade after the first
description more than 200 NH patients have been reported but population epidemiologic data
are still lacking. In two hospital-based series incidences of 6.4/100.000 [3] and 9/100.000 [4]
were estimated. In an outpatient neurologic service NH represented 0.25% of all consultations,
and 1.25% of the consultations were because of headache [5].
This article begins with a new case of nummular headache and summarizes the available
cases from the literature. We then review the diagnostic criteria proposed in ICHD-2. Lastly, we
present the available information on the epidemiology, clinical features, differential diagnosis,
and management of this disorder.
Case History
A 55-year-old man described an 8-month history of focal and well-circumscribed head
pain in the left occipital region. The affected area was perfectly circular, measuring 5 cm in
diameter. The pain was described as sharp, throbbing, and severe in intensity (8 of 10 on a 10-
point anchored scale). The pain was paroxysmal, with attacks lasting from 1 to 10 minutes and
occurring up to six times daily 10 to 12 days per month. The headaches rarely awakened him
from sleep. Associated features included mild phonophobia, allodynia, and tenderness of the
affected region. Exacerbating factors included high humidity and weather changes, specifically
from hot to cold weather. Prior treatment with oxcarbazepine and ibuprofen provided no relief.
The patient had a history of depression that was not being treated. Family history revealed that
his mother had migraine.
Outside of attacks, inspection and palpation of the cranium demonstrated no alopecia,
tenderness, hypoesthesia, allodynia, touch-evoked paresthesias, hyperalgesia, or lesions.
During bouts of pain, dysesthesias and allodynia were present within the affected region.
Neurologic examination was otherwise normal, and MRI of the brain was also normal.
Treatment with nortriptyline, 30 mg nightly, resulted in a reduction in the frequency and
intensity of headaches (two headaches per week, graded 1-2 of 10, lasting 1-3 minutes).
Diagnostic Criteria
The ICHD-2 criteria for nummular headache are provided in Table 1. The criteria require
mild to moderate head pain felt exclusively in a rounded or elliptical area, typically 2 to 6 cm in
diameter. In the case presented earlier, and in others from the literature, pain is sometimes
severe. The ICHD-2 criteria specify that the pain may be chronic and continuous or interrupted
by spontaneous remissions lasting weeks to months. In our case pain was intermittent. Other
causes of focal headache of fixed location must be excluded (see section regarding differential

3. diagnosis). In our review of the English language medical literature, we identified 60 cases of
nummular headache. Herein, we summarize the available information from these somewhat
variable case reports.
Epidemiology, Demographic Features, and Comorbidity
Nummular headache is thought to be a rare disorder, but its true prevalence and
incidence are uncertain. In one hospital series, the incidence of nummular headache was
6.4/100,000/year [2.0]. Nummular headache has a female predominance, with a gender ratio of
2.3:1. The age at onset ranges from 13 to 72 years, with a mean age of 41 years. The duration
of symptoms before diagnosis ranged from 1 month to 50 years. According to the data on hand
the female/male ratio is 1.5:1, and the mean age of onset is 43.6 years (range, 4 –79 years). The
duration of symptoms before diagnosis range from less than 1 month to 50 years [6].In most
cases there was no specific precipitant identified, although in 7% headache began in close
relation to mild head trauma. Remote head trauma was reported in four patients (9%). A few
patients (< 7%) may report a possible relationship with a minor head trauma but hardly ever
report a link between the trauma site and the area where the pain was experienced [1, 3, 5, 11,
14, 20, 23 •• , 33+. One patient related onset of symptoms after an insect bite in the affected
region [23 •• +. Another patient developed an NH after surgical treatment of a hypophyseal
adenoma, but in the opposite hemicranium [30]. A history of concurrent headache was found in
13 patients (28%). Of the 13 patients, 10 had migraine, two had tension-type headache, one
had trigeminal neuralgia, and one had cervicogenic headache. Comorbid epilepsy or anxiety
was documented in 4%. There was no specific information on comorbidities or onset of
concomitant headache disorders relative to onset of nummular headache.In a recent extensive
review, 55 patients out of 200 (27.5%) reported previous headaches, such as migraine (n = 40),
tension-type headache (n = 8), migraine and tension-type headache (n =1), chronic daily
headache (n =1), orgasmic headache (n =1), primary stabbing headache (n =1), and trigeminal
neuralgia (n =1) [6]. Another recently published series of NH found medication overuse
headache in 25% patients [14].
Clinical Features
In most cases of nummular headache, the pain was mild to moderate in
intensity,although some patients experience severe pain. Fifty percent of the patients
experienced superimposed exacerbations of pain, lasting from seconds to days.Lancinating
exacerbations, lasting from several seconds to minutes — up to 2 hours, may superimpose the
baseline pain, or may occasionally be the prevailing pain profile *1, 3, 5, 7, 11, 13, 14, 15 •• ,23

4. •• , 24, 25, 27, 28, 29 •• , 30 – 32] Symptoms presented during daytime in all but a few
patients, who reported nocturnal pain with frequent or occasional awakenings.The pain
predominates during daytime and hardly ever awakens patients from sleep [3, 21, 27]. A few
patients reported that exacerbations could be precipitated by touching the symptomatic area
(9%) or by brushing the hair within the affected region (9%); this may have represented
allodynia. A distortion of sensation (paresthesia, hypoesthesia, dysesthesia, or allodynia) was
reported in the region of pain in 46% of patients, as exemplified by our case. In exceptional
cases the pain was precipitated by sexual intercourse (n =1), coughing and Valsalva maneuvers
(n =2) [34], menstruation, or sleep deprivation (n =1) [35].
The quality of the pain was most commonly described as pressure-like, sharp, or
stabbing. The attacks were strictly unilateral and without side shift in most of the patients,
although some (6%) experienced bilateral pain. The pain was present more often on the right
side (67%) than the left in patients with side-locked attacks. In most instances the headache is
strictly unilateral with the right side being slightly more affected than the left [6, 7].The pain
was usually localized to the parietal region (56%) and less often involved the occipital (11%),
temporal (19%), or frontal (8%) region; the remaining patients had pain that affected the vertex
or two contiguous regions of the head. The site and size of the affected area were typically
discrete and fixed within a rounded (75%) or oval/elliptical-shaped (25%) region of the head.
The symptomatic area is rounded (3/4) or elliptical (1/4), typically 1 to 6 cm in diameter (range,
0.6 – 10 cm) [6]. As a result, the patient could often delineate the outline of the affected region
with a finger. There is generally a good concordance between patient ’s description and
physician ’ s mapping of the symptomatic area *3].Rarely, the disorder may be bifocal or
multifocal, each symptomatic area keeping all the characteristics of NH [7, 15 •• , 16 • , 17 –
19+. Development of multifocal NH may occur with “ consecutive ” (appearance of a new focal
area following the extinction of the previous one), “ additive ” (appearance of a new focal area
in addition to the first one), or “ simultaneous ” (synchronous evolution of two focal areas)
sequences. The temporal pattern of pain was chronic and continuous since onset (unremitting;
57%), chronic 15 days/month but not continuous; 13%), or episodic (< 15 days/month; 30%).
Rarely, the chronic course evolves from anepisodic pattern [3, 14, 28].Short-lived periods of
spontaneous remission with return to the previous pattern were observed in one series of
patients.
Associated features were very rare. With the exception of one patient who reported
rhinorrhea specifically with exacerbations of pain, photophobia, phonophobia, nausea,
vomiting, or other autonomic features did not accompany the headaches. One patient
reported bilateral lacrimation and rhinorrhea during exacerbations [11], and phonophobia has
been described in two patients [21, 35]. Alopecia was noted within the area of pain in one
patientIn addition, a minority of patients may develop trophic changes such as a patch of skin

5. depression, hair loss, reddish color, and local increased temperature [17, 23 •• +. Skin biopsies
were performed in three patients with trophic changes, and were not specific for any particular
dermatologic disease [23 •• +. A 4-year-old child developed an NH in a circumscribed parietal
area with a congenital focal hair heterochromia [37].
Pathogenesis
The pathogenesis of nummular headache is uncertain. Some think that the pain stems
from a peripheral source, probably from any of the epicranial tissues, including the skull, all
layers of the scalp, vessels, and nerves [10]. We prefer to provisionally consider NH as an
epicrania (ie, a headache probably stemming from epicranial tissues, that is, internal and
external layers of the skull, and all the layers of the scalp, including epicranial nerves and
arteries) [47].Size and shape of the symptomatic area along with signs and symptoms of local
sensory dysfunction may suggest neuralgia of a terminal branch of a pericranial nerve.
Howevertwo features militate against such a concept: 1) anestheticblock of the symptomatic
area is usually of no avail;and 2) the occasional topography with an elliptical symptomaticarea
divided in half by the midline.Early studies designed to determine the extent and distribution of
pain-sensitive structures within the cranium [38, 39] showed that stimulation of the scalp and
galea produced sharply localized pain at the site of the stimulus, whereas stimulation of other
intracranial structures resulted in referred pain in a rather wide area. Recent evidence suggests
that nummular headache may be associated with a local increase of pain sensitivity to
mechanical stimulation within the symptomatic area [11]. Furthermore, pericranial tenderness
does not seem to be related to nummular headache [12].It has been demonstrated both in
migraineand tension-type headache that the pain threshold increases in different regions [53–
55], whereas in NH it is confined to the symptomatic area [1, 3, 36]. Furthermore, evidence of
increased mechanical pain sensitivity (lower pressure pain thresholds [PPTs]) restricted to the
symptomatic area in NH has been found [41].Patients with NH show similar topographic
pressure pain sensitivity maps of the head when compared with healthy controls, with local
decrease of PPT levels restricted to the symptomatic zone compared with the nonsymptomatic
symmetrical point (Fig. 1) [42].NH with trophic changes [23••+ might be considered a restricted
form of complex regionalpain syndrome, which would probably be related to nerveinjury.The
clinical observations do not suggest a psychogenic origin of NH, since the majority of patients
with NH have no previous diagnoses of another psychopathologic disease. Otherwise, it has
been documented that NH is not associated with depression and anxiety, since patients with
NH showed similar mood states to those of healthy controls [46••+.

6. Differential Diagnosis and Evaluation
A diagnosis of primary nummular headache is made only after alternative causes have
been excluded. To date, only one report of probable secondary nummular headache has been
described in a patient with Marfan's syndrome [13]. This patient presented with a 2-month
history of focal, circumscribed pain in the right frontal region. An angiogram of the right
external carotid artery revealed a fusiform aneurysm. The pain completely resolved after
surgical resection of the abnormal vessel. However, focal headache may result from a variety of
underlying causes, including metastatic disease or myeloma of the skull, focal intracranial
tumors involving the dura mater (meningiomas), local infection (osteomyelitis), Paget's disease,
or inflammatory diseases.Although NH is mostly regarded as a primary disorder, various focal
headaches with a nummular pattern have been related to local lesions of the scalp (fusiform
aneurysm of a branch of the superficial temporal artery) [48], the skull (fibrous dysplasia) [30],
or the adjacent intracranial structures (meningiomas, arachnoid cysts) [13, 34, 49].
An initial diagnostic work-up should include a complete neurologic examination
(including careful inspection and palpation of the scalp and the pericranial muscles, nerves, and
arteries), laboratory tests, and neuroimaging studies. Laboratory studies should include a
complete blood count, basic metabolic panel, liver function tests, thyroid function tests,
erythrocyte sedimentation rate, alkaline phosphatase, antinuclear antibodies, rheumatoid
factor, angiotensin-converting enzyme, and urine analysis. CT of the head or MRI of the brain
should be performed to exclude any underlying structural abnormality.
Management
There are no clear guidelines for the management of patients with nummular headache.
Initial reports of nummular headache suggested that the low level of pain associated with the
disorder did not necessitate treatment; if required, it was thought to be typically responsive to
conventional analgesics. However, contrary to these reports and the current ICHD -2 criteria for
nummular headache, patients may experience intense pain and require prophylactic therapy.
Treatment with subcutaneous injections of local anesthetics was without benefit. Recently,
several agents have been suggested to be partially or completely effective in some case reports,
including gabapentin [4,5] and botulinum toxin [7,8]. In such instances gabapentin (300–900 mg
daily) [8, 20, 33] proved to be effective in a substantial number of patients. Alternatively,
tricyclic antidepressants rendered satisfactory results in a small seriesof NH patients [27].Based
on the authors' clinical experience with patients who have nummular headache, tricyclic
antidepressants such as amitriptyline, nortriptyline, and protriptyline may also be
useful.Botulinum toxin type A has been tried in 24 cases [16•, 26, 29••, 59], with a generally

7. good response. One series of 14 patients were treated with a dose of 10 U injected in the
symptomatic area resulting in substantial relief to all the patients treated [59]. A short series of
four patients with NH resistant to several treatments including NSAIDs, gabapentin, and local
anesthetic blocks were treated with botulinum toxin type A, 25 U, injected in several points
distributed in both the symptomatic and surrounding areas [29••]. With a latency of 6 to 10
days, all the patients improved, the benefit lasting for about 14 weeks. The procedure was
successfully repeated. Treatment with transcutaneous electrical nerve stimulation has been
reported as effective [25].
Conclusions
Nummular headache is a rare primary headache disorder of unknown etiology. The
diagnosis is one of both inclusion and exclusion. The hallmark of this disorder is a well-
circumscribed, focal headache that is usually round or oval in shape. Although usual pain is mild
or moderate, severe pain does not exclude the disorder. In patients with nummular headache,
the possibility of secondary disorders should be excluded by neurologic examination,
neuroimaging, and blood studies. Treatment is often difficult, although neuromodulators,
botulinum toxin, and tricyclic antidepressants have been helpful in some patients.

8. References and Recommended Reading
Papers of particular interest, published recently, have been highlighted as:
• Of importance
•• Of major importance
1.Pareja JA, Caminero AB, Serra J, et al.: Nummular headache: a coin-shaped cephalalgia.
Neurology 2002, 58:1678-1679.
2.0Pareja JA, Pareja J, Barriga FJ, et al.: Nummular headache: a prospective series of 14 new
cases. Headache 2004, 44:611-614. An excellent review of the demographic and clinical
features of nummular headache.
3. Headache Classification Subcommittee of the International Headache Society: The
International Classification of Headache Disorders 2nd Edition. Cephalalgia 2004, 24(Suppl 1):1-
160.
4. Evan RW, Pareja JA: Nummular headache. Headache 2005, 45:164-165.
5.Trucco M, Mainardi F, Perego G, Zanchin G: Nummular headache: first Italian case and
therapeutic proposal. Cephalalgia 2006, 26:354-356.
6. Cohen GL: Nummular headache: what denomination? Headache 2005, 45:1417-1418.
7.Dach F, Speciali J, Eckeli A, et al.: Nummular headache: three new cases. Cephalalgia 2006,
26:1234-1237.
8. Mathew NT, Kailasam J, Meadors L: Nummular headache responds to botulinum toxin
type a (BoNTA): experience in four cases. Cephalalgia 2006, 26:1378.
9.Seo M, Park S: Botulinum toxin in nummular headache. Cephalalgia 2005, 25:991.
10.Pareja JA: Nummular headache: what denomination? A rebuttal. Headache 2005, 45:1418.
11. Fernandez-de-las-Penas C, Cuadrado ML, Barriga FJ, Pareja JA: Local decrease of
pressure pain threshold in nummular headache. Headache 2006, 46:1195-1198.
12. Fernandez-de-las-Penas C, Cuadrado ML, Barriga FJ, Pareja JA: Pericranial tenderness is
not related to nummular headache. Cephalalgia 2007, 27:182-186.
13. Garcia-Pastor A, Guillem-Mesado A, Salinero-PaniaguaJ, Gimenez-Roldan S: Fusiform
aneurysm of the scalp: an unusual cause of focal headache in Marfan syndrome. Headache
2002, 42:908-910.

10. Table 1. 2004 International Headache Society criteria for the diagnosis of nummular
headache
A. Mild to moderate head pain fulfilling criteria B and C
B. Pain is felt exclusively in a rounded or elliptical area typically 2-6 cm in diameter
C. Pain is chronic and either continuous or interrupted by spontaneous remissions lasting weeks
to months
D. Not attributed to another disorder
(Data from Headache Classification Subcommittee of the International Headache Society
[3].)