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190
E. Ya n ık and D. Vurallı
– Surgical Intervention: It is indicated in cases in which endovascular treat-
ment is not feasible and involves microsurgical interruption of the stula
connection.
– Headache management: Analgesics may be helpful. In addition, treatment of
secondary factors such as intracranial hypertension is effective in
management.
19.7 Conclusion
These rare vascular malformations are essential causes of secondary headaches
because of their potential complications. Headaches due to DAVFs can often be
mistaken for primary headaches. Understanding the relationship between headaches
and DAVFs, along with a high index of suspicion, is essential for early diagnosis
and effective management. Appropriate imaging and multidisciplinary management
are required. Future research should focus on identifying headache phenotypes and
rening treatment algorithms for DAVF-associated headaches.
Acknowledgement We would like to sincerely thank Prof. Dr. Bijen Nazlıel for her counselling
on the case.
References
1. Reynolds MR, Lanzino G, Zipfel GJ. Intracranial dural arteriovenous stulae. Stroke.
2017;48(5):1424–31. https://doi.org/10.1161/STROKEAHA.116.012784.
2. Gupta A, Periakaruppan A. Intracranial dural arteriovenous stulas: a review. Indian
J Radiol Imaging. 2009;19(1):43–8.
PMID:19774139
3. Corbelli I, De Maria F, Eusebi P, Romoli M, Cardaioli G, Hamam M, Floridi P, Cupini LM,
Sarchielli P, Calabresi P.Dural arteriovenous stulas and headache features: an observational
study. J Headache Pain. 2020;21(1):6.
4. Padilha IG, Pacheco FT, Araujo AIR, Nunes RH, Baccin CE, Conti MLM, Maia ACM
Jr, Rocha AJD. Tips and tricks in the diagnosis of intracranial dural arteriovenous stulas: a pictorial review. J Neuroradiol. 2020;47(5):369–81. https://doi.org/10.1016/j.
neurad.2019.06.004.
5. Brown RD Jr, Wiebers DO, Nichols DA. Intracranial dural arteriovenous stulae: angiographic predictors of intracranial hemorrhage and clinical outcome in nonsurgical patients. J
Neurosurg. 1994;81(4):531–8. https://doi.org/10.3171/jns.1994.81.4.0531.
6. van Rooij WJ, Sluzewski M, Beute GN. Dural arteriovenous stulas with cortical venous
drainage: incidence, clinical presentation, and treatment. AJNR Am J Neuroradiol.
2007;28(4):651–5.
https://doi.org/10.1186/s10194- 020- 1073- 1.
https://doi.org/10.4103/0971- 3026.45344.
19 Dural Arteriovenous Fistula (DAVF) Attributed Headache
7. Kim MS, Han DH, Kwon OK, Oh CW, Han MH.Clinical characteristics of dural arteriovenous stula. J Clin Neurosci. 2002;9(2):147–55. https://doi.org/10.1054/jocn.2001.1029.
8. Miller TR, Gandhi D. Intracranial dural arteriovenous stulae: clinical presentation and management strategies. Stroke. 2015;46(7):2017–25. https://doi.org/10.1161/
STROKEAHA.115.008228.
9. Headache Classication Committee of the International Headache Society (IHS). The international classication of headache disorders, 3rd edition. Cephalalgia. 2018;38(1):1–211. https://
doi.org/10.1177/0333102417738202.
10. Gandhi D, Chen J, Pearl M, Huang J, Gemmete JJ, Kathuria S.Intracranial dural arteriovenous stulas: classication, imaging ndings, and treatment. AJNR Am J Neuroradiol.
2012;33(6):1007–13. https://doi.org/10.3174/ajnr.A2798.
11. Noguchi K, Melhem ER, Kanazawa T, Kubo M, Kuwayama N, Seto H. Intracranial dural
arteriovenous stulas: evaluation with combined 3D time-of-ight MR angiography and MR
digital subtraction angiography. AJR Am J Roentgenol. 2004;182(1):183–90. https://doi.
org/10.2214/ajr.182.1.1820183.
12. Alatakis S, Koulouris G, Stuckey S.CT-demonstrated transcalvarial channels diagnostic of
dural arteriovenous stula. AJNR Am J Neuroradiol. 2005;26(9):2393–6.
13. Alkhaibary A, Alnefaie N, Alharbi A, Alammar H, Arishy AM, Alshaya W, Khairy
S.Intracranial dural arteriovenous stula: a comprehensive review of the history, management, and future prospective. Acta Neurol Belg. 2023;123(2):359–66. https://doi.org/10.1007/
s13760- 022- 02133- 6.
191
Chapter 20
Headache Attributed toCavernous
Angioma
MarceloValença andLuizSeveroBemJunior
20.1 Introduction
Secondary headaches pose a signicant diagnostic challenge, particularly when
they mimic common primary headache patterns or are initially attributed to functional or psychiatric causes. Among these, headache attributed to a cavernous angioma (also known as a cerebral cavernous malformation [CCM]) is rare but clinically
signicant [1, 2]. It is classied under code 6.3.4in the International Classication
of Headache Disorders, third edition (ICHD-3). Although cavernous angiomas are
frequently asymptomatic and detected incidentally on brain imaging, they can occasionally manifest with focal neurological symptoms, epileptic seizures, and, less
commonly, headache.
The causal association between cavernous angiomas and headache remains a
subject of debate, due to the limited number of systematic clinical studies and the
potential overlap with seizures or minor hemorrhagic events. However, when there
is a clear temporal relationship between headache onset and lesion detection, signicant improvement following surgical resection, and an anatomically consistent
location, the headache may be directly attributed to the cavernous angioma, in line
with ICHD-3 diagnostic criteria.
In this chapter, we present and discuss a clinical case of a young male patient
with a new-onset headache of an unusual pattern, initially misinterpreted as a
M. Valença
Federal University of Pernambuco, Recife, Pernambuco, Brazil
Center of Excellence in the Treatment of Migraine and Other Headache Disorders, Hospital
Esperança, Recife, Pernambuco, Brazil
L. S. B. Junior (
Neuroequillibrium-neuromodulation center, Recife, Pernambuco, Brazil
Paraiba Pain Center, Campina Grande, Paraiba, 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_20
*)
193© The Author(s), under exclusive license to Springer Nature
194
manifestation of panic disorder. The subsequent diagnosis of a left temporal lobe
cavernous angioma explained both his headache and seizure-like episodes. This
case highlights the importance of neuroimaging in evaluating new-onset headaches.
It illustrates the practical application of ICHD-3 criteria, emphasizing the need to
differentiate between primary headache disorders, epileptic events, and headaches
caused by structural brain lesions.
M. Valença and L. S. B. Junior
20.2 Pathophysiology ofHeadache inCavernous Angioma
Headache associated with cerebral cavernous angiomas is a multifactorial phenomenon, often reecting the complex interplay between structural, vascular, inammatory, and epileptogenic mechanisms. These low-ow vascular malformations can
provoke headache through several pathways. One key contributor is recurrent
microhemorrhage, which is common in cavernous angiomas and can result inlocal
hemosiderin deposition, reactive gliosis, and perilesional inammation—factors
that sensitize surrounding neural tissue and may trigger nociceptive pathways.
Additionally, vasogenic edema due to the inammatory response around the lesion
can cause increased local intracranial pressure and further stimulate pain-sensitive
structures.
In the present case, a relatively large cavernous angioma located in the left temporal lobe exemplies the convergence of these mechanisms. The mass effect
exerted by the lesion may stretch or compress adjacent cortical areas, further contributing to pain generation. Moreover, temporal lobe cavernomas are frequently
associated with epileptic activity, and seizures themselves—or the peri-ictal state—
may present with or be followed by headache. Therefore, in such patients, headache
may represent not only a primary symptom of the lesion itself but also a secondary
manifestation of seizure activity or local cortical irritation.
Taken together, headache in patients with cavernous angiomas, particularly when
located in eloquent regions such as the temporal lobe, should be evaluated in the
broader context of microbleeds, inammation, epileptogenic potential, and mass
effect. Understanding these overlapping mechanisms is essential for guiding both
diagnostic workup and therapeutic strategies.
20.3 Case Report
A man, 32years old, presented to the emergency department three times over the
past 30days with continuous, dull, occasionally pulsatile left frontotemporal headache, with intermittent exacerbations. The pain improved with common analgesics
such as dipyrone and tramadol.
He worked in elevator repair and maintenance, reporting high levels of stress. He
had been evaluated by neurologists and was thought to be experiencing panic attacks
20 Headache Attributed toCavernous Angioma
195
while inside elevators, with headache exacerbations attributed to stress and possible
panic disorder. No neuroimaging was initially requested.
On his most recent emergency visit, an otorhinolaryngology consultation was
requested. A computed tomography (CT) scan of the paranasal sinuses was performed, and a temporal lesion was incidentally revealed. The patient denied any
history of headaches in the past 30days.
Subsequent brain magnetic resonance imaging (MRI) revealed a lesion consistent with a cavernous angioma in the left temporal lobe. The axial T2-weighted MRI
demonstrates a well-dened, heterogeneous lesion in the left temporal lobe
(2.2×2.3×2.2cm) located near the mesial region. The lesion exhibits the classic
“popcorn” or “mulberry” appearance typical of a cavernous angioma (cavernoma),
with a mixed signal intensity core reecting blood products at different stages of
degradation and a hypointense rim consistent with hemosiderin deposition. There is
no evident surrounding edema or mass effect, suggesting a chronic lesion without
recent hemorrhage. The hypointense rim suggests hemosiderin deposition, indicating prior microhemorrhages. The lesion is adjacent to the hippocampus and parahippocampal structures, consistent with mesial temporal involvement (Fig.20.1),
which may explain the patient’s seizure-like episodes and headache.
The patient underwent surgical resection of the lesion with complete removal
(Fig.20.2). Postoperatively, he had no neurological decits and reported complete
resolution of the headaches.
The presumed panic attacks were later interpreted as epileptic seizures, consistent with mesial temporal lobe epilepsy. He was started on carbamazepine (200mg
every 8hours) and continues treatment.
Fig. 20.1 MRI ndings. Typical magnetic resonance imaging appearance of a cavernous angioma
located in the left temporal lobe, near the mesial border of the temporal lobe, shown in the axial
(left panel) and coronal (right panel) views
196
M. Valença and L. S. B. Junior
abc
Fig. 20.2 Microsurgical procedure. (a) Left temporal craniotomy showing the lateral surface of
the left temporal lobe with a corticotomy over the middle temporal gyrus. (b) With the aid of a
temporal retractor, the dark-colored cavernoma is visualized. (c) The cavernoma is being removed
using a surgical aspirator
20.4 Case Discussion
20.4.1 ICHD-3 Diagnostic Criteria—6.3.4 Headache
Attributed toCavernous Angioma
A. Any new headache fullling criterion C
B. A cavernous angioma has been diagnosed
C. Evidence of causation demonstrated by at least two of the following:
1. The headache has developed in close temporal relation to other symptoms
and/or clinical signs of the cavernous angioma, or led to its discovery.
2. One or both of the following:
• The headache has signicantly worsened in parallel with other symptoms
or clinical or radiological signs of growth of the cavernous angioma.
• The headache has signicantly improved or resolved after removal of the
cavernous angioma.
3. The headache is localized to the site of the cavernous angioma
D. The headache is not better accounted for by another ICHD-3 diagnosis
Note: Intracerebral hemorrhage has been excluded by appropriate
investigations.
20.5 Commentary onthePresented Clinical Case
Criterion A—New headache:
The patient presented with a new, continuous headache that began approximately
30days prior, thus fullling criterion A.
20 Headache Attributed toCavernous Angioma
Criterion B—Diagnosis of cavernous angioma:
MRI revealed a lesion consistent with a cavernous angioma in the left tempo-
ral lobe.
Criterion C—Evidence of causality (at least two must be fullled):
• C1 (present): The headache led to the discovery of the cavernous angioma
after imaging was requested by an otorhinolaryngologist.
• C2b (present): There was complete resolution of the headache following surgical removal of the lesion.
• C3 (present): The pain was localized to a region compatible with the lesion’s
location (left temporal lobe → left frontotemporal headache).
• Therefore, criterion C is fully satised.
Criterion D—Exclusion of other diagnoses:
The initial diagnostic hypothesis of panic disorder did not account for the persis-
tent headache. After further investigation and denitive treatment of the
lesion, the headache resolved completely, excluding other more likely alternative diagnoses. Furthermore, no hemorrhage was documented.
20.6 Clinical Presentation
197
Although the ICHD-3 notes that primary headache directly attributed to cavernous
angiomas is rare—headache being more commonly secondary to seizures or hemorrhage—in this case there is strong temporal, anatomical, and therapeutic correlation. Thus, the most appropriate diagnosis is 6.3.4 Headache attributed to cavernous
angioma.
However, since the patient presented with symptoms initially interpreted as panic
attacks, which were retrospectively understood as epileptic seizures (mesial temporal lobe syndrome), it is also reasonable to consider coding part of the headache
episode as 7.6 Headache attributed to epileptic seizure, particularly if the headache
followed episodes of emotional discomfort (interpreted as epileptic equivalents).
20.7 Diagnostic Algorithm
1. Clinical Presentation
• Focal seizures (especially temporal lobe)
• Headache (persistent or intermittent)
• Focal neurological decits
• Incidental nding on neuroimaging
• Family history of cerebral cavernous malformations (CCMs)
198
M. Valença and L. S. B. Junior
2. Initial Imaging
• MRI of the brain with and without contrast is the imaging modality of choice
– Preferred sequences: T2, T1, T2-FLAIR (uid-attenuated inversion recov-
ery), Gradient Echo (T2)* or susceptibility-weighted imaging (SWI)
– Findings:
• “Popcorn” or “mulberry-like” appearance
• Mixed signal core due to hemorrhagic products
• Peripheral hypointense rim (hemosiderin)
3. Exclusion of Other Pathologies
• Rule out arteriovenous malformations (AVMs) with MR angiography (MRA)
or digital subtraction angiography (DSA) if atypical features are present
• Differentiate from tumors, hemorrhagic metastases, or vascular
malformations
4. Advanced Imaging (if needed)
• Functional MRI: for lesions near eloquent cortex (motor, speech, etc.)
• Perfusion MRI: to assess surrounding tissue edema or hemodynamic changes
• CT scan: limited value but may show calcications or acute hemorrhage
5. Genetic Testing (if indicated)
• For patients with:
– Multiple cavernomas
– Family history of cavernomas
– Early-onset or progressive lesions
• Genetic panel for KRIT1 (CCM1), CCM2, and PDCD10 (CCM3) mutations
6. Electroencephalography (EEG)
• If seizures are present or suspected
• Localizes seizure focus; useful for pre-surgical evaluation
7. Neurosurgical/Neurological Evaluation
• Assessment of surgical risk vs. benet based on:
– Location (e.g., brainstem, eloquent cortex)
– Symptoms (seizures, hemorrhage, neurologic decits)
– Lesion size and growth
– Hemorrhagic history
20 Headache Attributed toCavernous Angioma
199
20.8 Conclusion
We found few reports in the literature describing cases of cavernomas associated
with headache. One rare case involved an 18-year-old female presenting with severe
headaches localized to the left temporal and facial regions. Imaging revealed a small
dural-based parietal convexity mass, initially suggestive of a meningioma due to
homogeneous enhancement and the presence of a “dural tail sign” on MRI [3].
Surgical resection, however, conrmed the lesion to be a cavernous angioma without evidence of prior hemorrhage. Postoperatively, the patient experienced complete resolution of her facial pain. Another report described an adolescent with
atypical facial and head pain caused by a preexisting cerebellar cavernous angioma;
both symptoms resolved following surgical excision of the lesion [4].
Additionally, a case of a brainstem cavernoma inducing migraine-like attacks
has been documented [1]. There is also a report of a patient exhibiting a complex
focal seizure accompanied by migraine-like headache, both attributable to a small
cavernoma in the frontal lobe [5]. Furthermore, migraine-like headache was
described in a patient with a frontal lesion [6]. An intriguing case involved a patient
with an acute symptomatic migraine attack and chronic occipital neuralgia, both
resulting from hemorrhage of a bulbocervical cavernoma [7].
This clinical case fullls all ICHD-3 criteria for headache attributed to cavernous
angioma (6.3.4), with an additional possibility of coding as headache attributed to
epileptic seizure (7.6). The complete remission of the headache after resection of
the lesion strongly supports a causal relationship with the cavernous angioma.
References
1. Malik S, Young W. Midbrain cavernous malformation causing migraine-like headache.
Cephalalgia. 2006;26:1016–9.
2. Stellmann J-P, Kuhn M, Töpper R. Therapieresistenter Gesichtsschmerz durch ein
Hirnstammkavernom. Fortschritte Der Neurologie · Psychiatrie. 2007;75:552–4. https://doi.
org/10.1055/s- 2007- 980088.
3. Shen W, Chenn C, Hsue C, Lin T.Dural cavernous angioma mimicking a meningioma and
causing facial pain. J Neuroimaging. 2000;10:183–5. https://doi.org/10.1111/jon2000103183.
4. Epstein MA, Berman PH, Schut L.Cavernous angioma presenting as atypical facial and head
pain. J Child Neurol. 1990;5:27–30. https://doi.org/10.1177/088307389000500105.
5. Adhikari P, Nepali A, Shah A, Paudel S, Bhandari P, Nepali P.A small frontal lobe cavernoma
presenting with headache mimicking migraine and complex focal seizure: a case report. Clin
Case Rep. 2024;12 https://doi.org/10.1002/ccr3.8472.
6. Chirchiglia D, Della Torre A, Murrone D, Chirchiglia P, Marotta R.An unusual association
of headache, epilepsy, and late-onset Kleist’s pseudodepression syndrome in frontal lobe
cavernoma of the cerebral left hemisphere. Int Med Case Rep J. 2017;10:163–6. https://doi.
org/10.2147/IMCRJ.S133465.
7. Bruti G, Mostardini C, Pierallini A, Villani V, Modini C, Cerbo R. Neurovascular headache
and occipital neuralgia secondary to bleeding of Bulbocervical Cavernoma. Cephalalgia.
2007;27:1074–9.
https://doi.org/10.1111/j.1468- 2982.2007.01363.x.
https://doi.org/10.1111/j.1468- 2982.2006.01124.x.
Chapter 21
Headache Attributed toAngiitis
oftheCentral Nervous System: Giant Cell
Arteritis
LuizPauloQueiroz andAndressaMiozzoSoares
21.1 Introduction
Giant cell arteritis (GCA) is a chronic granulomatous inammatory vasculitis that
affects predominantly large- and medium-sized arteries in individuals older than 50.
GCA is the most common vasculitis in this age population [1].
There are three phenotypes of GCA: (1) cranial, (2) extracranial (large vessel),
and (3) mixed. The mixed phenotype may occur in up to 80% of cases. Cranial GCA
patients are at higher risk of visual and neurological complications; extracranial
GCA includes the development of stenosis and aneurysms of the aorta and its
branches, especially in the upper limbs (axillary arteries) [2]. There is also an association of GCA with polymyalgia rheumatica (PMR). Nearly 30–60% of patients
with GCA have concomitant PMR; and up to 25% of patients with PMR may have
subclinical GCA [1–3].
The incidence of GCA is approximately 20 new cases per 100,000 individuals
above 50years of age. The incidence is higher in Northern Europe and lower in
Africa, Asia, and Arabic countries. It is more common in females than males
(F2–3:1 M). Incidence increases with age, peaking in the 70’s (70–79 years
old) [4–7].
GCA is a medical emergency; if not diagnosed, it can lead to serious consequences for patients. The risk of permanent sight loss ranges from 8% to 30% [4].
The most affected cranial arteries are the temporal, ophthalmic, and posterior ciliary
arteries. Rarely are the vertebral and carotid arteries involved. Aortic involvement is
L. P. Queiroz (*)
Neurology Department, Universidade Federal de Santa Catarina, Florianópolis, Brazil
e-mail: lpqueiroz@oripa.com.br
A. M. Soares
Rheumatology Department, Universidade Federal de Santa Catarina, Florianópolis, 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_21
201© The Author(s), under exclusive license to Springer Nature