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- •Foreword
- •Contents
- •1.1 Introduction
- •1.2 Pathophysiology
- •1.3 Case Presentation
- •1.4 Case Discussion
- •1.5 Clinical Characteristics
- •1.6 Diagnostic Algorithm
- •1.8 Management
- •1.9 Conclusion
- •References
- •2.1 Introduction
- •2.2 Pathophysiology
- •2.3 Case Presentation
- •2.4 Case Discussion
- •2.5 Clinical Characteristics
- •1.7 Differential Diagnosis
- •2.6 Diagnostic Algorithm
- •2.7 Management
- •2.8 Conclusion
- •References
- •3.1 Introduction
- •3.2 Pathophysiology
- •3.3 Case Presentation
- •3.4 Case Discussion
- •3.5 Clinical Characteristics
- •3.6 Diagnostic Algorithm
- •3.7 Management
- •3.8 Conclusion
- •References
- •4.1 Introduction
- •4.2 Pathophysiology
- •4.3 Case Presentation
- •4.4 Case Discussion
- •4.6 Diagnostic Algorithm
- •4.7 Management
- •4.8 Conclusion
- •References
- •5.1 Introduction
- •5.2 Pathophysiology
- •5.3 Case Presentation
- •5.4 Case Discussion
- •5.5 Diagnostic Algorithm
- •5.6 Management
- •5.7 Conclusion
- •References
- •6.1 Introduction
- •6.2 Pathogenesis
- •6.3 Case Presentation
- •6.4 Case Discussion
- •6.5 Diagnostic Algorithm
- •6.6 Management
- •6.7 Conclusion
- •References
- •7.1 Introduction
- •7.2 Pathophysiology
- •7.3 Case Presentation
- •7.5 Differential Diagnosis
- •7.7 The Following Strategies Are Essential
- •7.7.1 Acute Symptom Relief
- •7.7.1.1 Pharmacological Treatment
- •7.7.2.1 Pharmacologic Prophylaxis
- •7.8 Conclusion
- •References
- •8.1 Introduction
- •8.3 Case Study
- •8.4 Case Discussion
- •8.5 Clinical Management
- •8.7 Diagnosis
- •8.8 Treatment
- •8.9 Conclusion
- •References
- •9.1 Introduction
- •9.2 Case Presentation
- •9.4 Diagnosis Algorithm
- •9.5 Secondary SUNCT
- •9.6 Management
- •9.8 Conclusion
- •References
- •10.1 Introduction
- •10.2 Pathophysiology
- •10.3 Case Presentation
- •10.4 Case Discussion
- •10.5 Clinical Characteristics
- •10.6 Diagnostic Algorithm
- •10.7 Management
- •10.8 Conclusion
- •References
- •11.1 Introduction
- •11.2 Pathophysiology
- •11.3 Case Presentation
- •11.4 Case Discussion
- •11.5 Clinical Characteristics
- •11.6 Diagnostic Algorithm
- •11.6.1 Step 1: Detailed Patient History
- •11.8 Management
- •11.9 Conclusions
- •12.2 Pathophysiology
- •12.3 Case Presentation
- •12.4 Case Discussion
- •12.6 Treatment
- •12.7 Conclusion
- •References
- •References
- •12.1 Introduction
- •13.1 Introduction
- •13.2 Pathophysiology
- •13.3 Case Presentation
- •13.4 Case Discussion
- •13.5 Clinical Characteristics
- •13.6 Diagnostic Algorithm
- •13.7 Management
- •13.8 Conclusion
- •References
- •14.1 Introduction
- •14.2 Pathophysiology
- •14.3 Case Presentation
- •14.3.1 Clinical Case 1
- •14.3.2 Clinical Case 2
- •14.4 Case Discussion
- •14.5 Clinical Characteristics
- •14.7 Treatment/Management
- •14.8 Conclusion
- •References
- •15.1 Introduction
- •15.2 Case Presentation
- •15.3 Case Discussion
- •15.4 Diagnostic Algorithm
- •15.5 Pathophysiology
- •15.6 Clinical Presentation
- •15.6.1 External-Compression Headache (ECH)
- •15.6.2 External-Traction Headache (ETH)
- •15.7 Management
- •15.7.1 Nonpharmacological Strategies
- •15.7.2 Pharmacological Strategies
- •15.7.3 Patient Education and Awareness
- •15.8 Conclusion
- •References
- •16.1 Introduction
- •16.2 Pathophysiology
- •16.3 Case Presentation
- •16.4 Case Discussion
- •16.6 Diagnostic Algorithm
- •16.7 Management
- •16.8 Conclusion
- •References
- •17.1 Introduction
- •17.2 Pathophysiology
- •17.3 Case Presentation
- •17.4 Case Discussion
- •17.5 Clinical Characteristics
- •17.6 Diagnosis
- •17.7 Differential Diagnosis
- •17.8 Treatment
- •17.9 Conclusion
- •References
- •18.1 Introduction
- •18.2 Pathophysiology
- •18.3 Case Presentation
- •18.4 Case Discussion
- •18.5 Clinical Presentation
- •18.6 Diagnosis
- •18.7 Differential Diagnosis
- •18.8 Treatment
- •18.9 Conclusion
- •References
- •19.1 Introduction
- •19.2 Pathophysiology
- •19.3 Case Presentation
- •19.4 Case Discussion
- •19.5 Diagnostic Approach
- •19.6 Management
- •19.7 Conclusion
- •References
- •20.1 Introduction
- •20.3 Case Report
- •20.4 Case Discussion
- •20.6 Clinical Presentation
- •20.7 Diagnostic Algorithm
- •20.8 Conclusion
- •References
- •21.1 Introduction
- •21.2 Case Presentation
- •21.3 Clinical Characteristics
- •21.4 Diagnosis
- •21.5 Treatment
- •References
- •22.1 Introduction
- •22.3 Case Presentation 1
- •22.4 Case Discussion
- •22.5 Case Presentation 2
- •22.6 Case Discussion 2
- •22.7 Clinical Characteristics
- •22.8 Diagnostic Workup
- •22.9 Treatment
- •22.10 Prognosis
- •References
- •23.1 Introduction
- •23.2 Pathophysiology
- •23.3 Case Presentation
- •23.4 Case Discussion
- •23.6 Diagnostic Algorithm
- •23.7 Management
- •23.8 Conclusion
- •References
- •24.1 Introduction
- •24.2 Case Presentation
- •24.3 Case Discussion
- •24.4 Pathophysiology
- •24.6 Clinical Characteristics
- •24.8 Treatment Approaches
- •24.10 Conclusion
- •References
- •25.1 Introduction
- •25.2 Case Presentation
- •25.3 Case Discussion
- •25.4 Conclusion
- •References
- •26.1 Introduction
- •26.2 Pathophysiology
- •26.3 Case Presentation
- •26.4 Case Discussion
- •26.5 Clinical Characteristics
- •26.6 Diagnostic Algorithm
- •26.7 Management
- •26.8 Conclusion
- •References
- •27.1 Introduction
- •27.2 Case Presentations
- •27.3 Clinical Characteristics
- •27.4 Discussion
- •27.5 Conclusion
- •References
- •28.1 Introduction
- •28.2 Case Presentation
- •28.3 Case Discussion
- •28.4 Clinical Characteristics
- •28.5 Diagnosis
- •28.6 Conclusion
- •28.7 Key Messages
- •References
- •29.1 Introduction
- •29.2 Pathophysiology
- •29.3 Case Presentation
- •29.4 Clinical Presentation
- •29.5 Diagnosis
- •29.6 Treatment
- •29.7 Conclusion
- •References
- •30.1 Introduction
- •30.2 Clinical Case
- •30.3 Clinical Presentation
- •30.4 Differential Diagnosis
- •30.5 Diagnosis
- •30.6 Treatment
- •30.7 Conclusion
- •References
- •31.1 Introduction
- •31.2 Pathophysiology
- •31.3 Case Presentation
- •31.4 Case Discussion
- •31.5 Clinical Presentation
- •31.7 Conclusion
- •References
- •32.1 Introduction
- •32.2 Pathophysiology
- •32.3 Case Presentation
- •32.4 Case Discussion
- •32.6 Diagnosis
- •32.7 Additional Diagnostic Evaluations
- •32.8 Apply ICHD-3 Diagnostic Criteria [9]
- •32.10 Management
- •32.11 Conclusion
- •References
- •33.1 Introduction
- •33.2 Pathophysiology
- •33.3 Case Presentation
- •33.4 Clinical Characteristics
- •33.5 Diagnostic Algorithm
- •33.6 Treatment
- •33.7 Conclusion
- •References
- •34.1 Introduction
- •34.2 Pathophysiology
- •34.3 Case Presentation
- •34.4 Case Discussion
- •34.6 Diagnostic Algorithm
- •34.7 Treatment
- •34.8 Conclusion
- •References
- •35.1 Introduction
- •35.3 Case Presentation
- •35.4 Case Discussion
- •35.7 Treatment
- •35.7.1 Oxygen Therapy (100% Oxygen)
- •35.8 Conclusion
- •References
- •36.1 Introduction
- •36.2 Pathophysiology
- •36.3 Case Presentation
- •36.5 Diagnostic Algorithm
- •36.6 Treatment
- •36.7 Conclusion
- •References
- •37.1 Introduction
- •37.2 Pathophysiology
- •37.3 Case Presentation
- •37.4 Headache Characteristics
- •37.5 Case Discussion
- •37.6 Treatment
- •37.7 Conclusion
- •References
- •38.1 Introduction
- •38.2 Pathophysiology
- •38.3 Case Presentation
- •38.4 Clinical Presentation
- •38.5 Diagnostic Algorithm
- •38.6 Treatment
- •38.7 Conclusion
- •References
- •39.1 Introduction
- •39.3 Case Presentation
- •39.4 Case Discussion
- •39.6 ICHD-3 Diagnostic Criteria [28]
- •39.6.1 Diagnostic Criteria
- •39.7 Diagnostic Algorithm
- •39.9 Conclusion
- •References
- •40.1 Introduction
- •40.3 Case Presentation
- •40.4 Case Discussion
- •40.5.1 Diagnostic Algorithm
- •40.6 Treatment
- •40.7 Conclusion
- •References
- •41.1 Introduction
- •41.3 Case Presentation
- •41.4 Clinical Presentation
- •41.5 Differential Diagnosis
- •41.6 Conclusion
- •41.7 Key Messages
- •References
- •42.1 Introduction
- •42.2 Pathophysiology
- •42.3 Case Presentation
- •42.5 Case Discussion
- •42.6 Clinical Presentation
- •42.7 Diagnostic Algorithm [9]
- •42.8 Preeclampsia
- •42.9 Eclampsia
- •42.10 Fetal Assessment
- •42.11 Treatment
- •42.12 Antihypertensive Management [8]
- •42.14 Conclusion
- •References
- •43.1 Introduction
- •43.2 Pathophysiology
- •43.3 Case Presentation
- •43.4 Case Discussion
- •43.5 Clinical Manifestations
- •43.6 Diagnosis
- •43.7 Treatment
- •43.8 Conclusion
- •References
- •44.1 Introduction
- •44.2 Pathophysiology
- •44.3 Case Presentation
- •44.4 Case Discussion
- •44.6 Diagnostic Approach
- •44.7 Management
- •44.8 Conclusion
- •References
- •45.1 Introduction
- •45.2 Pathophysiology
- •45.3 Case Presentation
- •45.6 Treatment
- •45.7 Conclusion
- •References
- •46.1 Introduction
- •46.2 Pathophysiology
- •46.3 Case Presentation
- •46.4 Clinical Characteristics
- •46.5 Differential Diagnosis
- •46.6 Treatment
- •46.7 Conclusion
- •References
- •47.1 Introduction
- •47.2 Pathophysiology
- •47.3 Case Presentation
- •47.4 Case Discussion
- •47.5 Clinical Presentations
- •47.6 Diagnostic Algorithm
- •47.7 Differential Diagnosis
- •47.8 Treatment
- •47.9 Conclusion
- •References
- •48.1 Introduction
- •48.2 Pathophysiology
- •48.3 Case Presentation
- •48.4 Case Discussion
- •48.5 Clinical Characteristics
- •48.7 Treatment
- •48.8 Conclusion
- •References
- •49.1 Introduction
- •49.2 Pathophysiology
- •49.3 Case Presentation
- •49.4 Clinical Presentation
- •49.5 Diagnosis
- •49.6 Treatment
- •49.7 Conclusion
- •References
- •50.1 Introduction
- •50.2 Pathophysiology
- •50.3 Case Presentation
- •50.4 Case Discussion
- •50.5 Clinical Characteristics
- •50.6 Diagnosis
- •50.7 Treatment
- •50.8 Conclusion
- •References
- •51.1 Introduction
- •51.2 Case Presentation
- •51.3 Clinical Characteristics
- •51.4 Diagnosis
- •51.5 Treatment
- •51.6 Conclusion
- •References
- •52.1 Introduction
- •52.2 Pathophysiology
- •52.3 Case Presentation
- •52.4 Case Discussion
- •52.5 Clinical Characteristics
- •52.6 Diagnosis
- •52.6.1 Cervicogenic Headache
- •52.6.2 Migraine
- •52.6.3 Neck Pain
- •52.6.4 Demyelinating Lesions
- •52.6.5 Cervical Myelitis
- •52.6.6 Occipital Allodynia
- •52.6.7 Cervical Muscle Spasms
- •52.7 Treatment
- •52.7.2 Acupuncture
- •52.7.3 Transcutaneous Electrical Nerve Stimulations (TENS)
- •52.8 Minimally Invasive Treatment
- •52.8.1 Nerve Blocks
- •52.8.2 Botulinum Toxin A
- •52.8.3 Radio Frequency
- •52.8.4 Occipital Nerve Stimulation
- •52.9 Surgical Treatments
- •52.10 Conclusions
- •References
- •53.1 Introduction
- •53.2 Pathophysiology
- •53.3 Characteristics of Pain
- •53.4 Case Presentation
- •53.5 Case Discussion
- •53.6 Clinical Characteristics
- •53.8 Treatment
- •53.9 Conclusion
- •References
- •54.1 Introduction
- •54.2 Pathophysiology
- •54.3 Case Presentation
- •54.4 Case Discussion
- •54.5 Clinical Characteristics
- •54.6 Diagnostic Algorithm
- •54.7 Management
- •54.8 Conclusion
- •References
- •55.1 Introduction
- •55.2 Pathophysiology
- •55.3 Case Presentation

23 Post-endarterectomy Headache
223
23.4 Case Discussion
This case illustrates a typical situation of post-endarterectomy headache. Symptoms
appeared within the rst week after the procedure, and their characteristics were
cluster headache-like, according to the 3rd edition of the International Classication
of Headache Disorders (ICHD-3) [9]. Complementary tests were performed to rule
out complications such as cerebral hemorrhage or ischemia, arterial stenosis, or dissection; all of which were normal. According to the phenotype, treatment was successful with oxygen and verapamil.
23.5 Headache Characteristics ofPost-endarterectomy
The ICHD-3 describes three post-endarterectomy headache phenotypes: tensionlike, cluster-like, and severe headache [9].
The rst type was described in detail by Tehindrazanarivelo etal., and, in this
sample, approximately 60% of patients developed a headache after surgery. Most
individuals presented a headache that appeared in the rst 5days after surgery
(mainly in the rst 2days), which could be a continuous episode or several episodes
per day, lasting from a few minutes to several hours (5min to 12h), and its average
duration was 3days. Other characteristics of the headache are mainly bilateral location (72%), frontal (55%), of mild to moderate intensity (78%), pressure or heaviness (77%), without associated symptoms and without requiring treatment (77%) [4].
Some authors [3, 6, 10] have reported cases of cluster-like headache after endarterectomy. Still, it was De Marinis etal. [5] who showed the particularities of the
second type of headache, which occurred in 30% of individuals. The headache
began, on average, 48h after the procedure, with each attack lasting 2–3 h, and
occurring once or twice a day, during all the days it was active, which was generally
about 2weeks. Only two cases that lasted 3–4months were described. The pain had
a continuous-pulsating or pulsating characteristic of moderate-to-severe intensity,
in the frontal, retroocular, periocular, and temporoparietal regions, always ipsilateral to the surgery. It may or may not be associated with local autonomic manifestations (conjunctival hyperemia, lacrimation, nasal congestion, and rhinorrhea—25%)
and Horner’s syndrome (25%) [5].
The third form of headache presentation is part of cerebral hyperperfusion syndrome, which is a well-known complication that affects 1–3% of patients after endarterectomy. It’s a condition characterized by numerous symptoms and ndings that
include headache, seizures, hypertension, focal neurological decits, and intracerebral hemorrhage [7, 11]. Headache is usually one of the rst symptoms of the syndrome and begins 3days after surgery, abruptly or not, it can be unilateral (ipsilateral
to the procedure) or bilateral, pulsatile, of severe intensity, associated or not with
nausea, occurring in episodes over weeks to months [12–14].

224
23.6 Diagnostic Algorithm
Step 1: Clinical Suspicion
• Identify Key Symptoms:
– Headache initiating after carotid endarterectomy
Step 2: Clinical and Neurological Examination
• Identify complications signs:
– Arterial hypertension
– Focal neurological decits
Step 3: Diagnostic Workup
• Brain Computed Tomography Scan:
– Assess for intracranial hemorrhage
• Brain Magnetic Resonance Imaging:
– Look for signs of ischemic infarct
• Carotid artery duplex:
– Assess for restenosis or dissection.
P. S. FaroSantos
• Transcranial Doppler:
– Assess cerebral hemodynamic disturbances
Step 4: Apply ICHD-3 Diagnostic Criteria
A. Any new headache fullling criterion C
B. Carotid endarterectomy has been performed
C. Evidence of causation demonstrated by at least two of the following:
1. A headache develops within 1week of the carotid endarterectomy
2. The headache resolves within 1month after the carotid endarterectomy
3. Both of the following:
(a) The headache is unilateral, on the side of the carotid endarterectomy
(b) A headache has one of the following three distinct characteristics:
• Diffuse mild pain
• Cluster headache-like pain occurring once or twice a day in attacks
lasting 2–3h
• Pulsating severe pain
D. Not better accounted for by another ICHD-3 diagnosis.

23 Post-endarterectomy Headache
225
23.7 Management
Headache management should be based on its subtype, as each type probably has a
different underlying pathophysiological mechanism. Another way to establish treatment is according to the headache phenotype.
A headache that begins early and has tension-like characteristics can be treated
with analgesics and anti-inammatories alone, since it is usually mild and selflimiting. For the control of cluster-like headache, some authors have described a
good therapeutic response to conventional drugs (oxygen, verapamil, and lithium)
for the management of primary cluster headache [6, 10]. In addition, there is a
report of a good therapeutic response to sumatriptan in a patient with cluster headache secondary to carotid artery dissection [15].
Meanwhile, treatment of the third headache subtype is mainly preventive, since
cerebral hyperperfusion syndrome, the underlying cause of this headache, is a
potentially serious complication [7]. Lee et al. [11] observed that patients who
developed post-endarterectomy headache had greater intraoperative variability in
blood pressure and suggested strict control of intraoperative blood pressure parameters and limiting their variability to decrease the incidence of not only headache but
also cerebral hyperperfusion syndrome. Furthermore, Dolan and Mushlin [12]
described a case in which there was a dramatic improvement in post- endarterectomy
headache and arterial hypertension after the use of prazosin, which inhibits postsynaptic α1-adrenoceptors.
23.8 Conclusion
Headache secondary to carotid endarterectomy is common but underestimated. It is
essential to recognize its existence and understand its presentation so that proper
investigation and treatment can be carried out, since it varies according to its phenotype. Furthermore, it can precede serious complications, in the case of cerebral
hyperperfusion syndrome, and its recognition can help in the management of this
condition.
References
1. Abbott AL.Extra-cranial carotid artery stenosis: an objective analysis of the available evidence. Front Neurol. 2022;13:739999. https://doi.org/10.3389/fneur.2022.739999.
2. Leung YYR, Bera K, Urriza Rodriguez D, Dardik A, Mas JL, Simonte G, Rerkasem K,
Howard DPJ. Safety of carotid endarterectomy for symptomatic stenosis by age: metaanalysis with individual patient data. Stroke. 2023;54(2):457–67. https://doi.org/10.1161/
StrokeAHA.122.040819.

226
3. Messert B, Black JA.Cluster headache, hemicrania, and other head pains: morbidity of carotid
endarterectomy. Stroke. 1978;9(6):559–62. https://doi.org/10.1161/01.str.9.6.559.
4. Tehindrazanarivelo AD, Lutz G, PetitJean C, Bousser MG.Headache following carotid
endarterectomy: a prospective study. Cephalalgia. 1992;12(6):380–2. https://doi.
org/10.1111/j.1468- 2982.1992.00380.x.
5. De Marinis M, Zaccaria A, Faraglia V, Fiorani P, Maira G, Agnoli A.Post-endarterectomy
headache and the role of the oculosympathetic system. J Neurol Neurosurg Psychiatry.
1991;54(4):314–7. https://doi.org/10.1136/jnnp.54.4.314.
6. Björne A, Hindfelt B, Havelius U.Recurrence of cluster headache after carotid thrombendarterectomy. Headache. 1994;34(4):230–3.
hed3404230.x.
7. Lin Y-H, Liu H-M. Update on cerebral hyperperfusion syndrome. J NeuroIntervent Surg.
2020;12:788–93.
8. Ashina M, Hansen JM, Do TP, Melo-Carrillo A, Burstein R, Moskowitz MA.Migraine and the
trigeminovascular system-40 years and counting. Lancet Neurol. 2019;18(8):795–804. https://
doi.org/10.1016/S1474- 4422(19)30185- 1.
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. Dirkx THT, Koehler PJ.Post-operative cluster headache following carotid endarterectomy.
Eur Neurol. 2017;77(3–4):175–9. https://doi.org/10.1159/000456004.
11. Lee D, Batista PM, McMackin KK, Ha A, Trani J, Carpenter JP, Lombardi JV.Intraoperative
blood pressure lability carries a higher risk of headache after carotid endarterectomy. J Vasc
Surg. 2022;75(2):592–598.e1. https://doi.org/10.1016/j.jvs.2021.08.070.
12. Dolan JG, Mushlin AI.Hypertension, vascular headaches, and seizures after carotid endarterectomy. Case report and therapeutic considerations. Arch Intern Med. 1984;144(7):1489–91.
13. Pearce J.Headache after carotid endarterectomy. Br Med J. 1976;2:85–6. PMID: 1276821.
PMCID: PMC1687795. https://doi.org/10.1136/bmj.2.6027.85.
14. Leviton A, Caplan L, Salzman E.Severe headache after carotid endarterectomy. Headache.
1975;15(3):207–10.
15. Leira EC, Cruz-Flores S, Leacock RO, Abdulrauf SI.Sumatriptan can alleviate headaches due
to carotid artery dissection. Headache. 2001;41(6):590–1.
0.2001.041006590.x.
https://doi.org/10.1136/neurintsurg- 2019- 015621.
https://doi.org/10.1111/j.1526- 4610.1975.hed1503207.x.
https://doi.org/10.1111/j.1526- 4610.1994.
https://doi.org/10.1046/j.1526- 461
P. S. FaroSantos

Chapter 24
Headache Attributed toCerebral Venous
Thrombosis (CVT)
DeryaUluduz andAynurÖzge
24.1 Introduction
Cerebral venous thrombosis (CVT) is a complex and potentially life-threatening
condition characterized by the formation of a blood clot in the dural venous sinuses
or cerebral veins, leading to increased intracranial pressure (ICP) and potential
venous infarction [1–3]. This results in signicant morbidity and mortality if not
promptly diagnosed and treated. CVT can affect both the supercial and deep
venous systems of the brain, leading to a variety of clinical manifestations, including venous infarction and cerebral edema. CVT is relatively rare compared to arterial strokes, with an estimated annual incidence of about 3–4 per million people.
However, it can be more common in specic populations such as young women and
those with prothrombotic conditions.
Headache is a primary symptom of CVT, occurring in up to 90% of cases, and
can sometimes present as the sole symptom, complicating its diagnosis [1–4]. The
headache associated with CVT can be diverse, ranging from subacute onset to thunderclap headache patterns, often with diffuse or bilateral characteristics [5, 6]. This
variability, combined with the absence of specic diagnostic markers, makes early
detection crucial yet challenging. The lack of a typical headache pattern and the
potential for isolated headache presentation without other neurological signs further
complicate the diagnosis, leading to delays in treatment initiation [4, 5]. The epidemiology of CVT-related headaches underscores their diagnostic challenge, with
headache being the sole presentation in approximately 15% of patients [4, 7].
D. Uluduz (*)
Medical Faculty, Neurology Department, Istanbul University Cerrahpasa, Samatya, Türkiye
A. Özge
Mersin State Hospital, Mersin, Turkey
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_24
227© The Author(s), under exclusive license to Springer Nature

228
D. Uluduz and A. Özge
Understanding the relationship between CVT and headache is essential for improving diagnostic accuracy and patient outcomes.
24.2 Case Presentation
A 24-year-old primigravida female presented to the emergency department with a
generalized tonic–clonic seizure 2weeks postpartum following an uneventful vaginal delivery. The seizure lasted approximately 3min, and she remained unconscious
for 20min afterwards. In the preceding 3days, she had been experiencing an excruciating headache that began insidiously but progressively worsened. The pain was
predominantly occipital, radiating down to her neck and extending towards the vertex. She described it as a relentless, throbbing sensation, akin to an intense pressure
building inside her skull. The headache was resistant to simple analgesics and was
exacerbated by movement, particularly when she changed positions or attempted to
stand. She also reported a sense of heaviness in her head, making it difcult for her
to concentrate or engage in routine activities. Throughout this period, she developed
associated symptoms of nausea and vomiting, which became more pronounced as
the headache intensied. Bright lights and loud noises aggravated her discomfort,
forcing her to retreat into a dark and quiet room. Despite her worsening symptoms,
she attributed them to postpartum fatigue and dehydration, delaying medical attention until the convulsive episode occurred.
Upon arrival, the patient appeared drowsy, with uctuating consciousness. Her
vital signs were stable, with a temperature of 36.6°C, a heart rate of 70bpm, blood
pressure of 110/60mmHg, a respiratory rate of 22 breaths per minute, and an oxygen saturation of 99% on room air. A general physical examination revealed no
abnormalities. Neurological evaluation and fundoscopic examination were unremarkable. Her medical and family history, including thromboembolic conditions,
were unremarkable, and she had no history of drug use or hormonal therapy. Given
her young age and lack of signicant past medical history, neuroimaging was performed to rule out vascular pathologies.
A non-contrast computed tomography (CT) scan revealed hyperdensity in the
superior sagittal sinus, suggestive of a dense clot sign, with no evidence of parenchymal hemorrhage (Picture 24.1). Magnetic resonance imaging (MRI) with
T2-FLAIR (uid-attenuated inversion recovery) sequences demonstrated hyperintense signals in the cortical and deep venous structures, indicative of venous congestion, without signs of infarction or hemorrhagic transformation (Picture 24.2).
MR venography (MRV) conrmed the absence of ow voids in the superior sagittal
sinus and transverse sinus, indicative of venous occlusion, without any arteriovenous malformation or aneurysmal dilation (Picture 24.3). Laboratory investigations,
including a complete blood count, biochemical screening, coagulation prole,
inammatory markers, and a thrombophilia panel, yielded normal results. Specic
tests for autoimmune and inherited thrombophilic conditions, such as

24 Headache Attributed toCerebral Venous Thrombosis (CVT)
Picture 24.1 Cranial CT demonstrates hyperdensity within the superior sagittal sinus, consistent
with the dense sinus sign
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Picture 24.2 Axial T2-FLAIR images; hyperintensity along the internal cerebral veins and vein
of Galen, consistent with impaired venous drainage
antiphospholipid antibodies, Factor V Leiden mutation, protein C/S deciency, and
lupus anticoagulant, were negative.
The diagnosis of postpartum cerebral venous sinus thrombosis (CVT) was established, and anticoagulation therapy with heparin was initiated, later transitioning to
therapeutic oral warfarin. On the fourth day of hospitalization, she experienced

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Picture 24.3 MR venography; reduced or absent ow in the right transverse and sigmoid sinuses,
and interrupted ow in the superior sagittal sinus
D. Uluduz and A. Özge
another generalized tonic–clonic seizure lasting 2min, prompting the initiation of
levetiracetam at a dose of 1000mg per day. She was discharged with full neurological recovery and was advised to continue oral anticoagulation therapy.
24.3 Case Discussion
Cerebral venous sinus thrombosis is a rare but serious postpartum complication,
often presenting with seizures, headaches, and focal neurological decits. Pregnancy
and the puerperium increase the risk due to hypercoagulability and endothelial
changes. The diagnosis relies on a high degree of clinical suspicion and conrmatory imaging, with MR venography being the gold standard for detecting venous
occlusions. In this case, the patient presented with postpartum headache, seizure,
and focal neurological decits, necessitating urgent neuroimaging. The CT scan
revealed a dense clot sign, while MRI and MR venography conrmed sinus thrombosis. The absence of identiable thrombophilic risk factors suggested that puerperium was the primary contributing factor.
Early anticoagulation is the cornerstone of treatment, preventing thrombus propagation and promoting recanalization. Seizures are common in CVT patients,
requiring antiepileptic therapy in cases of recurrent episodes. The patient’s favorable outcome highlights the importance of early recognition and timely intervention. This case underscores the need for heightened awareness of CVT in postpartum
patients presenting with new-onset headaches, seizures, or neurological decits.

24 Headache Attributed toCerebral Venous Thrombosis (CVT)
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Prompt diagnosis and appropriate management are crucial in preventing complications and ensuring a good prognosis.
24.4 Pathophysiology
The primary mechanisms underlying cerebral venous thrombosis (CVT)-related
headaches involve venous outow obstruction, increased intracranial pressure
(ICP), cortical vein infarction, cerebral edema, and venous hemorrhage. Each of
these mechanisms contributes to the complex headache prole associated with
CVT, making diagnosis and management challenging (Fig.24.1).
Venous Outow Obstruction and Increased Intracranial Pressure (ICP) When a
venous sinus or cerebral vein becomes occluded, normal venous drainage is disrupted, leading to venous congestion. This congestion results in a secondary increase
in ICP due to impaired cerebrospinal uid (CSF) absorption at the arachnoid granulations. The pressure buildup within the cranial vault can exert direct mechanical
stress on pain-sensitive structures, including the dura mater and meningeal vessels,
which are richly innervated by nociceptive bers originating from the trigeminovascular system. The elevated ICP manifests clinically as a headache that is often
Fig. 24.1 Venous obstruction triggers inammation, blood–brain barrier disruption, increased
intracranial pressure, and nociceptive activation, ultimately leading to cortical infarction, edema,
and headache

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D. Uluduz and A. Özge
described as severe, progressive, and diffuse. It is frequently exacerbated by Valsalva
maneuvers, such as coughing, straining, or positional changes, due to transient
increases in venous pressure that further compromise cerebral venous drainage
[8–10]. Additionally, elevated ICP can lead to papilledema, which may contribute to
visual disturbances and worsen the headache through secondary optic nerve involvement [11].
Cortical Vein Infarction and Neuroinammation In addition to venous congestion, CVT can lead to cortical vein infarction, a consequence of sustained venous
hypertension and hypoxia [4, 8]. The resultant ischemic injury triggers an inammatory cascade involving microglial activation, cytokine release (e.g., interleukin-1β
(IL-1β), tumor necrosis factor alpha [TNF-α]), and increased permeability of the
blood–brain barrier (BBB) [12].
This inammatory response plays a pivotal role in sensitizing pain pathways.
The activation of nociceptive bers within the dura and cortex leads to increased
release of pain-producing mediators such as substance P, calcitonin gene-related
peptide (CGRP), and bradykinin [13]. These molecules amplify pain transmission
by lowering the activation threshold of trigeminovascular afferents, making the
headache more persistent and refractory to conventional analgesics [14].
Cerebral Edema and Its Contribution to Headache Pathophysiology Cerebral
edema is another signicant contributor to CVT-related headaches and can be classied into cytotoxic and vasogenic edema. Cytotoxic edema results from cellular
energy failure due to ischemia, leading to intracellular water accumulation. In contrast, vasogenic edema arises from BBB disruption, allowing plasma proteins and
uids to enter the extracellular space [8, 9, 15]. Both forms of edema exacerbate
ICP elevation and contribute to worsening headache symptoms. The expansion of
brain tissue within the xed cranial vault intensies meningeal and vascular stretch,
further activating pain-sensitive structures. This can lead to diffuse, non-localized
headaches that mimic primary headache disorders such as migraines or tension-type
headaches, complicating the differential diagnosis.
Venous Hemorrhage and Meningeal Irritation
Venous hemorrhage is a potential
consequence of severe venous hypertension leading to vessel rupture. The extravasation of blood into cerebral tissue can irritate the meninges, further exacerbating
headache severity. Hemorrhagic transformation of venous infarcts adds another
layer of complexity by increasing localized mass effect and potentiating secondary
inammatory responses [15]. The presence of hemosiderin–laden macrophages and
blood degradation products can induce oxidative stress, leading to prolonged activation of nociceptive pathways. This results in a persistent, throbbing headache that
may resemble a thunderclap headache or other secondary headache disorders such
as subarachnoid hemorrhage.
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