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Headache
Series Editor: Paolo Martelletti
DeryaUludüz
MarceloValença
AynurÖzge
MarioFernandoPrietoPeresEditors
Rare Causes
ofHeadache
Disorders
Case Based Diagnostic Approach
Headache
Series Editor
PaoloMartelletti, Roma,Italy
The purpose of this Series, endorsed by the European Headache Federation (EHF),
is to describe in detail all aspects of headache disorders that are of importance in
primary care and the hospital setting, including pathophysiology, diagnosis,
management, comorbidities, and issues in particular patient groups. A key feature of
the Series is its multidisciplinary approach, and it will have wide appeal to internists,
rheumatologists, neurologists, pain doctors, general practitioners, primary care
givers, and pediatricians. Readers will nd that the Series assists not only in
understanding, recognizing, and treating the primary headache disorders, but also in
identifying the potentially dangerous underlying causes of secondary headache
disorders and avoiding mismanagement and overuse of medications for acute
headache, which are major risk factors for disease aggravation. Each volume is
designed to meet the needs of both more experienced professionals and medical
students, residents, and trainees.
Derya Uludüz • Marcelo Valença
Aynur Özge • Mario Fernando Prieto Peres
Editors
Rare Causes of Headache
Disorders
Case Based Diagnostic Approach
Editors
Derya Uludüz
School of Medicine, Neurology Department
Istanbul University Cerrahpasa
Samatya, Türkiye
Marcelo Valença
Neurology and Neuroscience
Federal University of Pernambuco
Recife, Brazil
Aynur Özge
Mersin State Hospital
Mersin, Turkey
Mario Fernando Prieto Peres
Universidade de São Paulo
São Paulo, SP, Brazil
ISSN 2197-652X ISSN 2197-6538 (electronic)
Headache
ISBN 978-3-032-10241-6 ISBN 978-3-032-10242-3 (eBook)
https://doi.org/10.1007/978-3-032-10242-3
© The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature
Switzerland AG 2026
This work is subject to copyright. All rights are solely and exclusively licensed by the Publisher, whether
the whole or part of the material is concerned, specically the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on microlms or in any other physical way, and
transmission or information storage and retrieval, electronic adaptation, computer software, or by similar
or dissimilar methodology now known or hereafter developed.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication
does not imply, even in the absence of a specic statement, that such names are exempt from the relevant
protective laws and regulations and therefore free for general use.
The publisher, the authors and the editors are safe to assume that the advice and information in this book
are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the
editors give a warranty, expressed or implied, with respect to the material contained herein or for any
errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional
claims in published maps and institutional afliations.
This Springer imprint is published by the registered company Springer Nature Switzerland AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
If disposing of this product, please recycle the paper.
Foreword
When we think about headaches, our minds naturally go to the common culprits: the
dull ache of a tension headache, the throbbing misery of a migraine, the searing pain
of a cluster headache, or the stuffy pressure of a sinus issue. These are the daily
realities for more than a billion people. But beyond this familiar landscape exists a
rarer and more complex territory—the realm of uncommon headache disorders.
These are not merely bad headaches but often distinct medical conditions or symptoms of a serious underlying problem. Understanding them is crucial because they
challenge both patients and doctors to look deeper. They remind us that the sensation of head pain can be a messenger for a wide array of intricate physiological
malfunctions.
Consider, for instance, headaches that are intimately tied to the very pressure that
surrounds our brain. There are conditions where the body produces too much cerebrospinal uid or fails to absorb it properly, leading to a build-up of pressure inside
the skull. This can cause a persistent, daily headache often worse in the morning,
and crucially, it can threaten vision by swelling the optic nerves. Conversely, the
opposite problem can occur—a leak of this same uid, perhaps from a tiny tear in
the spine’s lining. This results in a profound low-pressure headache, one with a telltale signature: it is excruciating when you sit or stand but vanishes almost completely when you lie at. Then there are the vascular crises, where the blood vessels
in the brain itself suddenly constrict in a wave of spasms, leading to a sudden,
explosive thunderclap headache that can peak in seconds, often triggered by some-
thing as simple as an orgasm or a moment of exertion.
Beyond pressure and blood vessels, the structure of the nervous system itself can
be a source. A congenital malformation where brain tissue extends into the spinal
canal can cause headaches triggered by coughing or straining. While the fear of a
brain tumor is common, it is a rare cause of headache; when it does occur, the pain
is usually part of a larger picture, including personality changes, seizures, or weakness. We must also include the searing, electric shock-like pains of cranial neuralgias, which are less a constant ache and more a series of lightning strikes to the face,
triggered by the slightest touch or gust of wind.
v
vi
Foreword
The overarching theme with these rare headache disorders is that they are frequently red ags. They demand medical attention not because the pain itself is
always worse but because what they represent is a system in distress. A headache
that is new and progressive in an older person, one that comes with fever and confusion, or one that changes dramatically with posture, is the brain’s way of signaling
that something specic and potentially dangerous is wrong. In essence, exploring
these rare causes expands our understanding of headache from a simple condition to
a complex language of symptoms, urging us to listen carefully to what the pain is
trying to say.
This book superbly addresses and catalogs this wealth of clinical information
and represents a veritable goldmine for the clinician. Tackling diagnostic uncertainties in this area of headache medicine is the real challenge. The more well-known
primary headaches belong to patients who navigate life with established diagnoses,
whereas the rare forms, being sometimes life-threatening, require impeccable clinical knowledge. It is a reference volume that is essential for the physical or virtual
library of every clinician who, from whatever discipline, approaches rare headaches
and for whom a missed diagnosis is not an option.
Unitelma Sapienza Università di Roma PaoloMartelletti
Roma, Italy
Contents
Part I Migraine
1 Hemiplegic Migraine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
Elcio Juliato Piovesan and Marco Antonio Takashi Utiumi
2 Migrainous Status . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
Mauro Eduardo Jurno and Ligia Barros de Oliveira
3 Migrainous Infarction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23
Alessandro Borrelli, Fabiana Ursitti, and Massimiliano Valeriani
4 Migraine Aura-Triggered Seizure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
Ilaria Notaristefano, Valentina Baglioni, and Vincenzo Guidetti
5 Episodic Syndromes That May Be Associated with Migraine:
Recurrent Gastrointestinal Disturbance . . . . . . . . . . . . . . . . . . . . . . . . 41
Toshiyuki Hikita
6 Episodic Syndromes That May Be Associated with Migraine:
Cyclical Vomiting Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49
Ishaq Abu-Arafeh
7 Episodic Syndromes That May Be Associated with Migraine:
Abdominal Migraine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61
Arife Cimen Atalar, Ebru Nur Vanli-Yavuz, and Pinar Topaloğlu
8 Episodic Syndromes That May Be Associated with Migraine:
Benign Paroxysmal Vertigo . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75
Tiago Gomes De Paula and Thais Rodriges Villa
Part II Trigeminal Autonomic Cephalalgias (TACs)
9 Short-Lasting Unilateral Neuralgiform Headache Attacks
with Conjunctival Injection and Tearing (SUNCT) . . . . . . . . . . . . . . . 83
Utku Topbaş, Nevra Öksüz, and Aynur Özge
vii
viii
Contents
10 Short-Lasting Unilateral Neuralgiform Headache Attacks
with Cranial Autonomic Symptoms (SUNA) . . . . . . . . . . . . . . . . . . . . 93
Gabriele Sebastianelli, Pubudu Amarasena, and Francesca Puledda
Part III Other Primary Headache Disorders
11 Primary Cough Headache . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109
PingKun Chen, Hung Yi Wu, and Shuu-Jiun Wang
12 Primary Exercise Headache . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 119
Arão Belitardo de Oliveira, Yohannes Woubishet Woldeamanuel,
and Julio Pascual
13 Primary Headache Associated with Sexual Activity . . . . . . . . . . . . . . 127
Füsun Mayda Domaç
14 Cold-Stimulus Headache . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135
Daniella Araújo de Oliveira and Marcelo Valença
15 External-Pressure Headache . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145
Erlene Roberta Ribeiro dos Santos, Juliana Ramos de Andrade,
and Welber Sousa Oliveira
16 Epicrania Fugax . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155
Gonçalo Diniz Pinho and Raquel Gil Gouveia
17 Nummular Headache . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165
Tülay Yılmaz and Betül Baykan
Part IV Headache Attributed to Cranial
or Cervical Vascular Disorder
18 Headache Attributed to Arteriovenous Malformation . . . . . . . . . . . . 177
Cláudio Manoel Brito, Elder Machado Sarmento,
and Leonardo Sarmento
19 Dural Arteriovenous Fistula (DAVF) Attributed Headache . . . . . . . . 185
Ece Yanık and Doğa Vurallı
20 Headache Attributed to Cavernous Angioma . . . . . . . . . . . . . . . . . . . . 193
Marcelo Valença and Luiz Severo Bem Junior
21 Headache Attributed to Angiitis of the Central Nervous System:
Giant Cell Arteritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 201
Luiz Paulo Queiroz and Andressa Miozzo Soares
Contents
22 Headache or Facial or Neck Pain Attributed to Cervical
Carotid or Vertebral Artery Dissection . . . . . . . . . . . . . . . . . . . . . . . . . 209
Ş. Ozan Dörtkol and Esme Ekizoğlu
23 Post-endarterectomy Headache . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 221
Paulo Sergio Faro Santos
24 Headache Attributed to Cerebral Venous Thrombosis (CVT) . . . . . . 227
Derya Uluduz and Aynur Özge
25 Headache Attributed to Reversible Cerebral
Vasoconstriction Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 241
Marcelo Valença and Luciana Patrízia Alves de Andrade-Valença
26 Cerebral Autosomal Dominant Arteriopathy and Subcortical
Infarcts Leukoencephalopathy (CADASIL)
Attributed Headache. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 247
Utku Topbaş, Nevra Öksüz, and Aynur Özge
27 Headache Attributed to Mitochondrial Encephalopathy,
Lactic Acidosis, and Stroke-Like Episodes (MELAS) . . . . . . . . . . . . . 255
Nana Nino Tatishvili, Teona Shatirishvili, and Soa Tatishvili
28 Headache Attributed to Moyamoya Angiopathy . . . . . . . . . . . . . . . . . 263
Esra Kochan Kizilkilic and Derya Uluduz
ix
29 Migraine-Like Aura Attributed to Cerebral Amyloid
Angiopathy (CAA) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 273
Maria Eduarda Nobre
30 Headache Attributed to Pituitary Apoplexy . . . . . . . . . . . . . . . . . . . . . 279
Carolina Martins, Jesuíno Albino, Juliana Andrade,
and Marcelo Valença
Part V Headache Attributed to Non-vascular Intracranial Disorder
31 Headache Attributed to Low Cerebrospinal
Fluid (CSF) Pressure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 293
Marcio Nattan Portes Souza and Diogo Guilherme Leão Edelmuth
32 Headache Attributed to Neurosarcoidosis . . . . . . . . . . . . . . . . . . . . . . 305
Mohamad Ali Hussein, Marco Antonio Nihi,
and Camila Carneiro Ferreira
33 Headache with Neurological Deficits and CSF Lymphocytosis
(HaNDL Syndrome) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 313
Amr Hassan, Rehab Magdy, and Mona Hussein
34 Headache Attributed to Chiari Malformation Type I (CM1) . . . . . . . 321
Claudia Baptista Tavares