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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5526_Библиотеки_им_академика_М_И_Перельмана.pdf
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T. Yılmaz and B. Baykan
examination, and appropriate blood tests and neuro-imaging studies, preferably
MRI with contrast enhancement, are crucial in the diagnostic process.
This case is signicant as it highlights that both family physicians and patients
may not adequately recognize this type of primary headache, leading to high concern. Studies have shown that primary care physicians often encounter patients with
headache complaints but may lack sufcient knowledge in this area, underscoring
the need for regular educational programs.
In this case, the patient’s long-standing history of migraine suggests that NH
may coexist with migraine, as reported in studies dealing with other primary headaches. Their high rate of co-existence with migraine may indicate shared predisposing factors, hinting at a “eadache continuum” concept for primary headaches [5].
The resolution of her symptoms with NSAIDs supports that symptomatic treatment may be sufcient in mild cases. However, if the symptoms are refractory to
NSAIDs and become chronic, gabapentin, which is commonly used in the treatment
of neuropathic pain, could be considered [18].
17.5 Clinical Characteristics
Pain is typically localized to a distinct cranial area, clearly outlined by the patient,
and is most commonly round (80%) or oval-shaped (20%) (Fig.17.1). The diameter
of the painful region, as per diagnostic criteria, generally ranges between 1 and 6cm
[12]. However, cases with larger areas (up to 10cm in diameter) have been reported
[15]. Notably, the shape and size of the painful area remain consistent over time [12].
Pain is most frequently localized to the parietal region (44%) and less commonly
to the occipital region (22%), with involvement of other regions being rare. It typically presents as a single focus, with bifocal cases being rare and multifocal presentations extremely rare. The pain is described as pressing, stabbing, burning, or
throbbing [6]. Abnormal sensations, such as allodynia, dysesthesia, paresthesia, and
hypoesthesia, may accompany pain episodes in the affected area. Pain intensity is
typically mild to moderate; however, severe pain was reported in 25% of cases and
very severe pain in 2%, highlighting that high-intensity pain is not uncommon. In a
cohort of 102 patients, the mean score on the visual analog scale (VAS), used to
assess pain intensity, was 5 [6]. In the Head-MENA-A study on other primary headache disorders, the mean VAS score for NH was found to be 6.6 (4–8) [5]. Typically,
there were no associated symptoms, such as photophobia, phonophobia, or nausea.
However, it has been reported that these symptoms are associated with some cases
[5, 11].
The temporal pattern of NH is heterogeneous. NH consists of continuous pain
with periods of exacerbations or remissions. Approximately three-quarters of the
patients experience pain on more than half of the days in a month, while one-quarter
17 Nummular Headache
Fig 17.1 Nummular
headache is most
commonly localized in the
parietal region. The pain is
typically described within
well-dened, round or
oval-shaped areas
169
report pain on fewer than 15days per month. On symptomatic days, the pain may
be continuous or intermittent, with durations ranging from seconds to minutes,
hours, or even days [2, 19].
17.6 Diagnosis
According to the ICHD-3 classication, NH is categorized under “The Primary
Headaches” within the section of “4. Other Primary Headache Disorders” as 4.8
Nummular Headache and 4.8.1 Probable Nummular Headache. NH diagnosis is
established according to the well-dened ICHD-3 criteria [2] listed below:
4.8 Nummular Headache
A: Continuous or intermittent head pain fullling criterion B
B: Felt exclusively in an area of the scalp with all of the following four
characteristics:
1. Sharply contoured,
2. Fixed in size and shape,
3. Round or elliptical,
4. 1–6cm in diameter
C: Not better accounted for by another ICHD-3 diagnosis
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T. Yılmaz and B. Baykan
If only three of the B criteria are fullled, and it does not fulll the ICHD-3 criteria for any other headache disorder and is not better accounted for by another
ICHD-3 diagnosis, a diagnosis of probable NH can be made [2].
4.8.1 Probable Nummular Headache
A. Continuous or intermittent head pain fullling criterion B
B. Felt exclusively in an area of the scalp, with three only of the following
four characteristics:
1. Sharply contoured
2. Fixed in size and shape
3. Round or elliptical
4. 1–6cm in diameter
C. Not fullling ICHD-3 criteria for any other headache disorder
D. Not better accounted for by another ICHD-3 diagnosis
Other causes, particularly structural and dermatologic lesions, should have been
ruled out through a thorough patient history, physical examination (including
examination of the scalp, pericranial muscles, nerves, and arteries), and cranial
MRI or computed tomography (CT) scan. Laboratory tests that may be conducted
include a complete blood count, evaluations of metabolic and liver functions, thyroid function analysis, erythrocyte sedimentation rate, alkaline phosphatase levels,
and screenings for antinuclear antibodies, rheumatoid factor, and other individualized tests [3, 20].
17.7 Differential Diagnosis
Linear headache (LH) is a newly described type of headache in recent years, characterized by persistent pain along a xed linear trajectory and presenting in either
episodic or chronic form. This headache type shares some features with NH, but
signicant differences also exist. Whether LH represents a variant of NH or a distinct headache type remains a topic of debate. Both types of headaches can exhibit
symptoms of sensory dysfunction in the painful area. In both LH and NH, the pain
is localized to a specic region, and its location remains xed. While NH is typically conned to a circular or oval area, LH is characterized by a xed, linear pathway, which distinguishes it from NH [21].
In the context of differential diagnosis, ruling out secondary cases is crucial, as
indicated above. To exclude the presence of systemic or structural diseases, a thorough examination of the scalp and skull, as well as systemic and neurological
17 Nummular Headache
171
assessments, neuroimaging, and blood screening, is essential. The differential diagnosis should carefully investigate potential causes such as intracranial lesions, vascular abnormalities, infections, and other secondary conditions [4].
Psychogenic headaches should also be considered in the differential diagnosis of
NH.Initially, some authors hypothesized a psychogenic origin for NH, but current
evidence does not support this view. A recent study comparing NH patients and
healthy controls found no signicant differences in depression or anxiety levels
between the two groups. Additionally, depression and anxiety were not associated
with key pain parameters such as intensity, exacerbations, pain area size, or frequency [22]. These ndings indicate that NH occurs independently of anxiety and
depression, distinguishing it clearly from psychogenic headaches.
17.8 Treatment
When no underlying cause is identied, primary NH typically follows a benign
course. In such cases, reassuring patients about the non-serious nature of their condition often sufces, and additional treatment may not be necessary [4, 18].
Spontaneous remissions, either temporary or permanent, have been reported in the
natural course of NH in up to 19.4% of patients [23]. However, for those experiencing severe or refractory pain that does not respond to analgesics, prophylactic treatment may be warranted.
Currently, no standardized treatment guidelines exist for NH; however, a limited
number of drugs have been utilized in its management, with treatment suggestions
primarily derived from clinical experience and observations [4, 18].
The managements used for NH management include NSAIDs, gabapentin, carbamazepine, botulinum toxin type A (BoNT-A), triptans, tricyclic antidepressants
(TCA), and nerve block techniques. NSAIDs have been a mainstay in medium-term
and acute exacerbation therapies, with variable effectiveness reported across studies, ranging from no response to partial or excellent results. Overall, analgesics and
NSAIDs have been effective in over 60% of cases. Therefore, their as-needed use
can be considered for patients with mild continuous pain, intermittent pain requiring occasional treatment, or as an add-on to preventive therapies [4].
Gabapentin was identied as the most commonly used treatment for NH, with a
response rate of 67% [18]. It is recommended at a dose of 800 mg/day for its efcacy and tolerability [23]. Although there are no controlled clinical trials, various
case reports and series have demonstrated that BoNT-A injections are highly effective and well-tolerated, with minimal side effects [4, 18, 24]. In the extensive series
of 53 patients by García-Azorín etal., patients were administered 25 U of BoNT-A,
distributed across one central and four peripheral points within the painful area. It
was shown to reduce the monthly headache frequency signicantly between weeks
8–12 and 20–24. Additionally, it decreased the number of intense headache days
and acute medication days during the same periods. Approximately two-thirds of
the patients achieved a 50% response rate, while half of them achieved a 75%
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T. Yılmaz and B. Baykan
response rate [24]. In the study by Zhu etal., it was suggested that NH can be effectively treated with acupuncture or a combination of amitriptyline with indomethacin, ibuprofen, or carbamazepine [25]. At the case level, triptans have demonstrated
effectiveness in patients with migrainous features [11, 26].
Subcutaneous anesthetic injections have been attempted in several patients; however, they have generally shown little to no therapeutic effect, with only rare
instances of partial or temporary relief reported [12]. Dach etal. demonstrated that
blocking the greater occipital nerve provided pain relief in their NH cases [27]. The
combination of topiramate and palmitoylethanolamide has been reported as effective at the case level [28].
In secondary NH patients, symptom relief has been reported with specic treatments targeting the cause. For example, in a case related to varicella-zoster infection, antiviral therapy was effective; in a case associated with meningioma, surgical
intervention was performed; and in cases related to supercial aneurysm and
Langerhans histiocytosis, symptoms improved following surgical excision [4].
17.9 Conclusion
NH is a benign primary headache disorder that can coexist with other primary headache types. A thorough diagnostic approach is essential to exclude secondary
causes. NSAIDs can be an effective treatment option, while prophylactic treatment
may be required in refractory cases.
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173
Part IV
Headache Attributed to Cranial or
Cervical Vascular Disorder
Chapter 18
Headache Attributed toArteriovenous
Malformation
CláudioManoelBrito, ElderMachadoSarmento, andLeonardoSarmento
18.1 Introduction
Headache is a common complaint, and it is most frequently classied as a primary
headache, such as migraine or tension-type headache. However, headaches can also
be secondary to a variety of underlying conditions, including cranial or cervical
vascular disorders. One such condition is arteriovenous malformation (AVM), an
abnormal connection between arteries and veins in the brain or spinal cord. AVMs
are characterized by a tangle of abnormal vessels, where arteries connect directly to
veins without the intervention of capillaries, which can lead to various neurological
complications. The presence of an AVM can alter cerebral hemodynamics, predisposing to events such as hemorrhage, seizures, headache, and neurological decits
[1, 2]. Unfortunately, due to the gravity of AVM rupture and cerebral hemorrhage,
research on headaches associated with brain AVMs is still scarce [3].
AVMs are rare vascular lesions, with an incidence rate of approximately 1.12 to
1.42 cases per 100,000 person-years [1, 4]. The estimated prevalence is 1in 10,000
individuals [2]. Although AVMs can become symptomatic at any age, the most
common presentation occurs in the third or fourth decade of life [4]. The prevalence
of headache in patients with AVMs varies widely, with estimates ranging from 6 to
52% [5]. This variation may reect different diagnostic criteria and patient populations studied. A study of 1289 patients with AVMs found that 14% presented with
chronic headache [5]. A review of 51 patients with IVM (intracranial vascular malformations) found that 47% suffered from migraine-type headaches [6].
C. M. Brito (*) · E. M. Sarmento
Headache, Sociedade Brasileira de Cefaleia, Barra Mansa, Brazil
L. Sarmento
Internal Medicine, Institution: Santa Casa de Barra Mansa, Barra Mansa, Brazil
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_18
177© The Author(s), under exclusive license to Springer Nature
178
C. M. Brito et al.
18.2 Pathophysiology
The underlying mechanisms of headache in patients with AVMs are not fully understood. However, several theories have been proposed:
1. Increased intracranial pressure: AVMs can increase intracranial blood volume,
leading to increased intracranial pressure and, consequently, headache.
2. Steal phenomenon: The high arteriovenous ow in AVMs can divert blood ow
from adjacent brain areas, causing ischemia and headache [7].
3. Activation of the trigeminovascular system: AVMs can activate the trigemino-
vascular system, a neural pathway involved in the pathogenesis of migraine,
resulting in headache [7].
4. Inammation: Inammation of blood vessels and surrounding tissues may con-
tribute to headache.
5. Cortical spreading depression (CSD): CSD, an electrophysiological phenome-
non underlying migraine aura, can be triggered by cortical ischemia or irritation [8].
6. Hemodynamic alterations: In rare cases, headache may be related to hemody-
namic alterations, such as ow inversion in the internal jugular vein, which can
lead to cerebral venous congestion [9].
7. Cortical lesions or microembolization: Symptomatic migraine with aura (MWA)
may occur due to cortical lesions or microembolization from carotid pathologies [8].
18.3 Case Presentation
A 15-year-old boy, presented with a history of headaches that started at the age of
13. His headache was initially infrequent (occurring less than once a month), throb-
bing, and of moderate intensity. The pain was always restricted to the right frontoparietal region and mainly brought about by physical activities, such as playing
soccer or running. Sometimes the headache was associated with nausea and dizziness. He reported relief with rest and/or sleep. In the last months, the pain became
more frequent and more intense, preventing the patient from going to school on
many occasions. Initially, he was treated with abortive medication, but lately, preventive medications were started (unarizine and propranolol). So far, no neurological symptoms have been reported. There was no familial history of headache. In
March 2024, he had dysesthesia in the left side of the body, during a headache crisis.
A computed tomography (CT) of the head without contrast showed a right frontoparietal AVM.Digital subtraction angiography (DSA) conrmed it to be a SpetzlerMartin grade IV AVM [10] (Fig.18.1). The patient was started on sodium divalproex
of extended release, which controlled the dysesthesias and, partially, the headache
(decreasing the frequency and intensity). The patient was offered interventional
18 Headache Attributed toArteriovenous Malformation
Fig. 18.1 DAS: Right frontoparietal AVM
179
treatment, but after a lengthy discussion of the risks and advantages of treatment,
the parents opted for conservative treatment.
18.4 Case Discussion
The present case illustrates how a secondary headache can be mistaken for a primary headache, initially presenting as episodic, throbbing, and triggered by physical exertion, with relief upon rest or sleep. Some factors should raise suspicion of a
secondary headache: the locked side, the progressive nature, and, nally, the focal
sensitive crises on the left side of the body. The head CT suggested an arteriovenous
malformation, later conrmed by magnetic resonance imaging (MRI) angiography.
The sodium divalproate controlled the crises and helped prevent the headaches.
Unfortunately, the family declined the proposed interventional treatment. The
patient has been followed up for a year now, with no alterations in the neurological status.