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I. Notaristefano et al.
Chapter 5
Episodic Syndromes That May
BeAssociated withMigraine: Recurrent
Gastrointestinal Disturbance
ToshiyukiHikita
5.1 Introduction
The third edition of the International Classication of Headache Disorders (ICHD-3)
lists recurrent gastrointestinal disturbance as a subsection of 1.6 Episodic syndromes that may be associated with migraine [1]. It states that such syndromes often
precede or coincide with the onset of migraine [2]. Subsection 1.6.1 Recurrent gastrointestinal disturbance comprises 1.6.1.1 Cyclical vomiting syndrome and 1.6.1.2
Abdominal migraine. These conditions are discussed in Chaps. 6 and 7 of this publication, “Migraine: Episodic Syndromes That May Be Associated with Migraine:
Cyclical Vomiting Syndrome,” and “Migraine: Episodic Syndromes That May Be
Associated with Migraine: Abdominal Migraine.”
In the second edition of the International Classication of Headache Disorders
(ICHD-2), cyclical vomiting, abdominal migraine, and benign paroxysmal vertigo
of childhood were listed as subcategories of childhood periodic syndrome that are
commonly precursors of migraine [3]. Recurrent gastrointestinal disturbance was
added as a subcategory in the ICHD-3, which also provided the diagnostic criteria.
These are shown in Table5.1. Previously used terms for this condition have included
chronic abdominal pain, functional abdominal pain, functional dyspepsia, irritable
bowel syndrome, and functional abdominal pain syndrome. The ICHD-3 description of recurrent gastrointestinal disturbance is: “Recurrent episodic attacks of
abdominal pain and/or discomfort, nausea and/or vomiting, occurring infrequently,
chronically or at predictable intervals, that may be associated with migraine.”
T. Hikita (*)
Department of Pediatrics, Teikyo University School of Medicine, Itabashi-Ku, Tokyo, Japan
Hikita Pediatric Clinic, Kiryu City, Gunma, Japan
e-mail: t-hikita@ra2.so-net.ne.jp
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_5
41© The Author(s), under exclusive license to Springer Nature
42
T. H ikit a
Table 5.1
gastrointestinal disturbance
A. At least ve attacks with distinct episodes of abdominal pain and/or discomfort and/or
B.Normal gastrointestinal examination and evaluation
C.Not attributed to another disorder
International Classication of Headache Disorders-3 diagnostic criteria for recurrent
nausea and/or vomiting
5.2 Pathophysiology
Although the pathophysiology of recurrent gastrointestinal disturbance is unclear, it
is thought to be related to migraine. Symptoms may be intensied by stress, and it
is believed to have a neurological etiology. Cases have been reported in which triptan preparations, which are used to treat migraine, are effective. These studies suggest that serotonin metabolism may be involved [2, 4–8]. Primary headache
disorders, including migraine, are among the most common neurological diseases.
Episodic syndromes that may be related to migraine, including recurrent gastrointestinal disturbances such as abdominal migraine and cyclical vomiting, often precede the onset of migraine or co-occur. They also may resemble the pathology of
migraine [9].
5.3 Case Presentation
A 3-year and 5-month-old girl presented with recurrent upper abdominal pain
occurring one to three times per week, with each episode lasting between 10min
and 1h. These episodes were sometimes accompanied by diarrhea or vomiting.
Before the onset of pain, she often stopped eating and would lie down. She occasionally reported headaches and showed signs of photophobia and reduced appetite.
During episodes, she preferred quiet environments and sometimes had difculty
sleeping due to the discomfort. Her medical history included atopic dermatitis and
car sickness. There was a positive family history of typical migraine with aura in her
mother. The patient was diagnosed with recurrent abdominal pain by the pediatrician and was monitored clinically, though no specic diagnostic investigations were
performed. Until she was 6years and 10months old, the patient experienced episodes of abdominal pain around her navel. This was accompanied by nausea, but no
vomiting or diarrhea. She would sometimes complain of headaches when she had
abdominal pain. There were no warning signs preceding the episodes. During the
periods of pain, the patient was sensitive to sound and light, and her face was pale.
From around the age of 8years and 10months, the patient complained of severe
pulsating pain in the left temporal region that occurred approximately twice a month
5 Episodic Syndromes That May Be Associated with Migraine: Recurrent…
43
for 2–4h. The pain worsened with routine physical activity and was accompanied
by photophobia, phonophobia, and nausea. The patient was diagnosed with migraine
without aura. Acetaminophen was effective against the headaches.
The patient did not experience abdominal pain after the age of 9 years and
6months. However, since the age of 9years and 6months, the patient has continued
to experience migraines, with headaches occurring approximately twice a month at
15years and 2months. Acetaminophen remains effective, and the patient is under
ongoing outpatient observation.
5.4 Case Discussion
Based on the intensity and duration of abdominal pain and the frequency of vomiting, this case did not meet the diagnostic criteria for abdominal migraine or cyclical
vomiting syndrome. Although the patient in this case also complained of headaches,
in some cases of recurrent gastrointestinal disturbance, headaches do not accompany the abdominal pain. This case met the diagnostic criteria for recurrent gastrointestinal disturbance (Table5.1) [1]. Provided organic disease is ruled out, patients
with recurrent abdominal pain are observed, but no further examinations are
required. If the diagnostic criteria for abdominal migraine are met during the course
of the disease, the patient is treated for abdominal migraine. In the ICHD-2, the
diagnostic criterion for abdominal migraine was abdominal pain for 1h or more, but
this was changed to 2h or more in the ICHD-3 [1, 3]. A previous study of recurrent
abdominal pain and abdominal migraines found that recurrent abdominal pain,
which is relatively mild, is more common than abdominal migraines [10]. When the
duration of abdominal migraine was extended from 1 to 2h in the diagnostic criteria, the number of cases decreased. Relatively mild cases that do not meet the diagnostic criteria for abdominal migraine or cyclical vomiting syndrome should be
diagnosed as recurrent gastrointestinal disturbance and observed for any progression. A previous study of cyclical vomiting syndrome conducted by the authors
followed 25 cases for >5years. Among these, 4 (16%) patients developed abdominal migraine and 11 (44%) developed migraine (Fig.5.1) [11]. This suggests a
strong relationship among cyclical vomiting syndrome, a subtype of recurrent gastrointestinal disturbance, abdominal migraine, and migraine. In addition, cases have
been reported in which sumatriptan, a migraine medication, was effective against
cyclical vomiting syndrome and abdominal migraine [4–8]. Other drugs that are
effective in the prevention of migraine headaches have also been found effective in
the treatment of cyclical vomiting syndrome. This suggests that these conditions
share a common pathophysiology.
44
Fig. 5.1 Outcomes of 25 patients with cyclical vomiting syndrome. In 2008, all 25 patients had
cyclical vomiting syndrome and did not have migraines at that time. Over the next 5years, 11
patients (44%) developed migraines, and 4 patients (16%) developed abdominal migraines. By
2013, more than 5years after 2008, 1 patient (4%) still had cyclical vomiting syndrome, 7 patients
(28%) had migraines, and 17 patients (68%) had no symptoms
T. H ikit a
5.5 Diagnostic Algorithm
Step 1: Clinical suspicion.
• Identify key symptoms:
– Recurrent distinct episodes of abdominal pain and/or discomfort and/or
nausea and/or vomiting.
– Family history of migraine.
Step 2: Initial diagnostic workup.
• Inspection, auscultation, palpation:
– No organic cause of the symptom.
– No trauma.
Step 3: Apply ICHD-3 diagnostic criteria (Table5.1) [1]
Step 4: Classication re-evaluation.
• Reassess headache classication for episodic syndrome that may be associ-
ated with migraine.
– See Chaps. 6 and 7.
5 Episodic Syndromes That May Be Associated with Migraine: Recurrent…
45
Table 5.2
ACTH adrenocorticotropic hormone, ADH antidiuretic hormone, ALT alanine aminotransferase,
CT computed tomography, GI gastrointestinal, γ-GTP gamma-glutamyl transpeptidase, HVA
homovanillic acid, MRI magnetic resonance imaging, VMA vanillylmandelic acid
Differential diagnosis and useful examinations for recurrent gastrointestinal disturbance
Digestive disease: Chronic appendicitis, abnormality (intestinal malrotation), mucosal disorder
(ulcer, esophagitis, Crohn’s disease), pancreatitis, hepatobiliary disease (cholelithiasis,
choledochal cyst)
Blood examination: Blood count, ALT, γ-GTP, amylase, lipase.
Urinalysis: VMA, HVA,.
Fecal examination: Fecal culture.
Diagnostic imaging: Abdominal ultrasonography, abdominal ultrasonography,
esophagogastroduodenoscopy, upper GI series.
Neurological disorder: Brain tumor, intracranial hypertension, abdominal migraine, abdominal
epilepsy
Diagnostic imaging: Brain MRI and/or CT, electroencephalogram.
Otolaryngologic disorder: Chronic sinusitis, vestibular disorder
Diagnostic imaging: Paranasal sinus CT.
Urological diseases: Kidney stones, hydronephrosis, etc.
Diagnostic imaging: Abdominal ultrasonography.
Urinalysis: Uric acid, ca/Cr ratio.
Metabolic or endocrine disease: Mitochondrial disease, urea cycle disorder, fatty acid
metabolism disease, acute intermittent porphyria, diabetes mellitus, Addison disease,
pheochromocytoma, hereditary angioedema
Blood examination: Electrolyte, blood glucose, catecholamine, blood gas, lactic acid, amino
acid, ammonia, ACTH, ADH.
Urinalysis: Ketone body, organic acid, carnitine, porphobilinogen.
Other: Munchausen syndrome, anxiety, depression, pregnancy
Blood examination: hCG.
Urinalysis: Drug test.
Other: Psychological consult.
Diagnostic criteria for recurrent gastrointestinal disturbance have been described
in the ICHD-3. An essential step in diagnosing recurrent gastrointestinal disturbance is to rule out organic diseases. For example, the criteria presented in ICHD-3
are in Table5.1 [1]. It is always necessary to perform appropriate examinations
before making a diagnosis of recurrent gastrointestinal disturbance. Differential
diagnosis and practical examinations are described in Table5.2. There is no specic
examination for recurrent gastrointestinal disturbance; a combination of examinations, as in Table5.2, must be conducted.
5.6 Management
In cases of recurrent gastrointestinal disturbance where the abdominal pain is severe
or lasts for extended durations, blood tests and imaging are required. In such cases,
the same examinations as those for abdominal migraine and cyclical vomiting
46
T. H ikit a
syndrome are performed to rule out other conditions (Chaps. 6 and 7). One of the
diagnostic criteria for recurrent gastrointestinal disturbance is normal gastrointestinal examination and evaluation results. In this case, the mother had migraines, and
the child suffered from car sickness. Although the child’s condition in this case
persisted for a long time, it did not worsen. No medication was prescribed for the
abdominal pain, and the patient was monitored without blood or imaging examination. Eventually, the pain improved. In mild cases of recurrent gastrointestinal disturbance, routine examination is not necessary.
5.7 Conclusion
Recurrent gastrointestinal disturbances include cyclical vomiting syndrome and
abdominal migraine. However, it also includes cases of recurrent abdominal pain
and vomiting that do not meet the diagnostic criteria for these conditions. Patients
may have a family history of migraine. Some cases progress to migraine during
follow-up. If no neurological ndings are found on examination, symptoms often
improve with careful follow-up without unnecessary examination. However, it is
advisable to explain to the family that, if the condition persists, the patient may
develop migraine in the future.
References
1. Headache Classication Committee of the International Headache Society (IHS). 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.
2. Irwin S, Barmherzig R, Gelfand A. Recurrent gastrointestinal disturbance: abdominal
migraine and cyclic vomiting syndrome. Curr Neurol Neurosci Rep. 2017;17:21. https://doi.
org/10.1007/s11910- 017- 0731- 4.
3. Headache Classication Subcommittee of the International Headache Society. The international classication of headache disorders: 2nd edition. Cephalalgia. 2004;24(Suppl):1–160.
4. Benson JM, Zorn SL, Book LS. Sumatriptan in the treatment of cyclic vomiting. Ann
Pharmacother. 1995;29:997–9. https://doi.org/10.1177/106002809502901008.
5. Kowalczyk M, Parkman H, Ward L. Adult cyclic vomiting syndrome successfully treated
with intranasal sumatriptan. J Gen Intern Med. 2010;25:88–91. https://doi.org/10.1007/
s11606- 009- 1162- y.
6. Kakisaka Y, Wakusawa K, Haginoya K, Saito A, Uematsu M, Yokoyama H, Sato T, Tsuchiya
S. Efcacy of sumatriptan in two pediatric cases with abdominal pain-related functional
gastrointestinal disorders: does the mechanism overlap that of migraine? J Child Neurol.
2010;25:234–7. https://doi.org/10.1177/0883073809336875.
7. Hikita T, Kodama H, Kaneko S, Amakata K, Ogita K, Mochizuki D, Kaga F, Nakamoto N,
Fujii Y, Kikuchi A.Sumatriptan as a treatment for cyclic vomiting syndrome: a clinical trial.
Cephalalgia. 2011;31:504–7. https://doi.org/10.1177/0333102410390398.
5 Episodic Syndromes That May Be Associated with Migraine: Recurrent…
8. Calhoun AH, Pruitt AP.Injectable sumatriptan for cyclic vomiting syndrome in adults: a case
series. Headache. 2014;54:1526–30. https://doi.org/10.1111/head.12444.
9. Abu-Arafeh I, Gelfand AA. The childhood migraine syndrome. Nat Rev Neurol.
2021;17:449–58.
10. Hikita T.Prevalence of abdominal migraine and recurrent abdominal pain in a Japanese clinic.
Pediatr Int. 2016;58:669–71. https://doi.org/10.1111/ped.13005.
11. Hikita T, Kodama H, Ogita K, Kaneko S, Nakamoto N, Mimaki M.Cyclic vomiting syndrome
in infants and children: a clinical follow-up study. Pediatr Neurol. 2016;57:29–33. https://doi.
org/10.1016/j.pediatrneurol.2016.01.001.
https://doi.org/10.1038/s41582- 021- 00497- 6.
47
Chapter 6
Episodic Syndromes That May
BeAssociated withMigraine: Cyclical
Vomiting Syndrome
IshaqAbu-Arafeh
6.1 Introduction
Cyclical vomiting syndrome (CVS) is a childhood syndrome related to migraine.
CVS presents with specic clinical features and follows a dened clinical pattern in
its prognosis and response to treatment, similar to those of childhood migraine.
CVS is characterised by recurrent stereotypical episodes of intense nausea and
vomiting lasting between 2 and 72h, interspaced by periods of full return to normal
health. Children continue to have normal general health, growth and development.
Although interest in CVS and research into its clinical characteristics have increased
over the past 20–30years, the initial description of the condition dates back to the
late nineteenth century [1]. CVS has attracted the attention of general paediatricians, paediatric gastroenterologists, neurologists and headache specialists. The
denition and criteria for the diagnosis, therefore, were developed as a migraine
disorder within the International Classication of Headache Disorders (ICHD) and
as a Functional Gastrointestinal Disorder by Gastroenterologists [2, 3]. CVS has
also been reported in adult patients of all ages and is increasingly recognised as an
common cause for patients referred for gastroenterology clinics [4].
This review will cover the epidemiology, pathogenesis, clinical features, progno-
sis, treatment and the impact of CVS on patients’ quality of life.
The onset of CVS can occur at any age, but is most common during early childhood. Several studies reported the peak age of onset of CVS to be around 5–6years
[5]. Both male and female children are affected almost equally. CVS have been
reported from all over the world with a comparable prevalence regardless of ethnic
or socio-economic background. The prevalence of CVS is estimated at around 2%
in children between the ages of 5–15years in Scotland, Australia and Turkey [5–7],
I. Abu-Arafeh (*)
Paediatric Neurosciences Unit, Royal Hospital for Children, Glasgow, UK
e-mail: ishaq.abu-arafeh2@nhs.scot
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_6
49© The Author(s), under exclusive license to Springer Nature
50
with an estimated incidence of 3.15/100,000 children per year [8]. The prevalence
of CVS in adult patients is not known, but at least one in ten new patients referred
to a gastroenterology clinic may have CVS [9]. There is a very close and consistent
relationship between CVS and migraine. Children with a personal or family history
of migraine have an increased risk of presenting with CVS, and children with CVS
have an increased risk of developing migraine late in childhood or in early adult life.
I. Abu-Arafeh
6.2 Pathogenesis
Despite the close relationship between migraine and CVS, the pathogenesis of CVS
continues to be a subject of speculation and controversy. Underlying genetic predisposition for CVS has been shown in clinical and epidemiological studies to be
closely associated with migraine. The molecular genetics of both migraine and CVS
are very likely to be complex and polygenic, and continue to be a target for research.
However, single-gene mutations have been reported in patients with familial hemiplegic migraine involving the voltage-gated calcium channel (CACNA1A), sodiumpotassium ATPase channels (ATP1A2), and the voltage-gated sodium channels
(SCN1A) and PRRT2 [10]. In some patients with CVS, a single-gene mutation on
the Ryanodine Receptor 2 (RYR2) has been detected. It may have a signicant association with the disease [11].
Mitochondrial gene mutations and polymorphism have also been investigated to
delineate the suspected maternal role in the disease inheritance [12–14].
Mechanisms that initiate and maintain an episode of CVS may involve one or
more pathways including autonomic and neuroendocrine dysfunction, hypothalamicpituitary- adrenal axis activation and the dysregulation of the central nervous system
emetic centre and chemoreceptor trigger zone (CTZ) [12, 13, 15]. Dysregulation of
the cannabinoid type 1 receptor (CB1) in the brain may also play a role in the pathogenesis of CVS, as has been shown in chronic use of cannabis and a possible disorder of endogenous cannabinoid metabolism presenting with a syndrome of recurrent
vomiting [16]. Functional MRI (magnetic resonance imaging) studies have shown
altered intrinsic connectivity of the sensorimotor network, specically salience to
the mid-posterior insula, in patients with migraine and CVS compared to healthy
control individuals [17].
6.3 Case Presentation
Adam presented at the age of 6years with a chief complaint of recurrent episodes
of intense nausea and vomiting with no apparent reason. His parents reported that
he had been seen and assessed by physicians many times since he was 3years old.
Adam is well and healthy between episodes, and he has achieved excellent growth
and development.