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9.2 Case Presentation
A 67-year-old male Hasan was admitted to our tertiary headache clinic for severe
headaches. His headache was localized to the right temporal and periorbital regions,
lasting around one minute. The pain was excruciatingly severe; he described it as if
something had screwed into his right eye for one minute before stopping suddenly.
When pain occurred, he felt a tingling sensation in his periorbital area, and his right
eye was reddish. He complained of tears in his right eye. His headache was triggered by certain activities, such as eating, talking, and washing his face. When the
pain starts, it occurs ve to ten times an hour, most of the day, with attacks occurring
daily or every other day for three years. When he came to the headache clinic, he
seemed to accept his headache episodes. He said, “I believe that this pain came to
my head because God wanted to punish me for the sins I committed.” He had coronary artery disease and hypertension, and he was using acetylsalicylic acid, metoprolol, and ramipril for chronic diseases. There was no family history to explain this
pain. Neurologic examinations were remarkable except for right eye ptosis. Both
indomethacin and oxygen tests were negative. Diagnosis of short-lasting unilateral
neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)
was made. Imaging with contrast-enhanced magnetic resonance imaging (MRI) and
MR Angiography showed no vascular or structural abnormalities. We admitted the
patient to the neurology unit and started treatment with intravenous (IV) lidocaine
(infusion 1.5mg/kg/h). The headache was completely resolved by the third day of
treatment. We continued the treatment for an additional 24hours to ensure the headaches did not return. After that, we sent the patient home and gradually started
lamotrigine treatment, tapering it to 300mg per day. During the rst follow-up visit
after six weeks, the patient still reported headaches, but they were relieved by about
50%. To improve the situation, we added topiramate to the treatment and gradually
increased the dosage to 200mg per day. After four weeks, the patient reported a
95% reduction in headache symptoms.
9.3 Headache Characteristics inSUNCT
Pain in SUNCT is almost always unilateral. The pain is of short duration (1–600s)
and is always accompanied by autonomic symptoms. If conjunctival injection and
tears are present, it is called SUNCT.Pain is usually described by patients as shooting, stabbing electrical-shock like [3, 7]. Rarely it can be described as throbbing [3].
Pain intensity is severe or very severe. In most patients, pain is localized to the V1
branch of the trigeminal nerve, while pain tends to radiate to the V2 and V3 branches
and even to the cervical branches. In some, the pain may also be in the neck, where
cervical branches carry the pain [3, 8] (Fig.9.1). While attacks occur mostly spontaneously, they are triggered by stimuli such as talking, eating, touching the face,
and shaving, as in trigeminal neuralgia [7, 8]. While there is a refractory period
9 Short-Lasting Unilateral Neuralgiform Headache Attacks with Conjunctival…
Fig. 9.1 Distribution of pain in SUNCT
85
between attacks in trigeminal neuralgia, this refractory period is absent in SUNCT
[7]. Even immediately after the pain has ended, pain may be stimulated with triggers. Four different pain patterns are dened for SUNCT (Fig.9.2).
1. Single or repetitive stabs: Each episode lasts on average 60s (1–600s). Attacks
recur periodically throughout the day (average 20–30). There is no long refractory period between episodes [3].
2. Plateau like pattern: The pain peaks very quickly (max. 2–3s) and continues at
a constant intensity for 5–600s and then abruptly stops [3].
3. Saw tooth pattern: It consists of stabbing pain superimposed on severe pain at
the base. The stabbing comes in a regular pattern and is superimposed on the
pain at the base. The duration of pain can last up to 20minutes [7].
4. Plateau-like with exacerbations: A form of pain in which random, very short
(1–2s) spikes are added to the constant pain (5–300s) [3].
Since the duration of pain can last up to 600s, it can be misdiagnosed as paroxysmal hemicrania. In SUNCT, attacks are unresponsive to indomethacin, which is
always responsive in paroxysmal hemicrania. During pain, patients usually have
psychomotor agitation as in cluster headache. However, some patients with shortterm pain and somatic triggers tend to wait until the pain subsides. They prefer to
remain immobile to avoid triggers [3]. Attacks recur frequently during the day
(1–200 times). The ICHD-3 diagnostic criteria for SUNCT are as follows
(Table9.1) [1].
86
a
de
bc
Fig. 9.2 Headache subtypes seen on SUNCT
Table 9.1 Diagnostic criteria for SUNCT
Diagnostic criteria for short-lasting unilateral neuralgiform headache
A.At least 20 attacks fullling criteria B through D
B.Moderate or severe unilateral head pain, with orbital, supra-orbital, temporal and/or other
trigeminal distribution, lasting for 1–600seconds and occurring as single stabs, a series of
stabs, or in a sawtooth pattern
C.At least one of the following ve cranial autonomic symptoms or signs, ipsilateral to the
pain:
1. Conjunctival injection and/or lacrimation
2. Nasal congestion and/or rhinorrhea
3. Eyelid edema
4. Forehead and facial sweating
5. Miosis and/or ptosis
D.Attacks have a frequency of at least one a day
E.Not better accounted for by another ICHD-3 diagnosis
Diagnostic Criteria for SUNCT
A.Attacks fullling criteria for short-lasting unilateral neuralgiform headache attacks
B.Both conjunctival injection and lacrimation (tearing) ipsilateral to the pain
Episodic SUNCT: Attacks occurring in periods lasting from seven days to one year, separated by
pain-free periods lasting at least three months
Chronic SUNCT: Attacks occurring without a remission period, or with remissions lasting less
than three months, for at least one year.
U. Topbaş et al.
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87
9.4 Diagnosis Algorithm
When patient complains for one side locked severe headache with cranial autonomic symptoms:
Step 1: Ask for duration and number of attacks in one day. Notably, all trigeminal
autonomic cephalgia headaches except cluster headache occur at least one per
day. If more than ten attacks, then cluster headache is unlikely. If the patient
complains severe headache more continuously than half hour, diagnosis of
SUNCT/SUNA is unlikely.
Step 2: Ask for the prominence of cranial autonomic symptoms and the presence of
refractory periods. Trigeminal neuralgia has a refractory period. If the pain
doesn’t have a refractory period, SUNCT/SUNA is likely.
Step 3: Make the indometacin test to rule out paroxysmal hemicrania. Give 100mg
of indomethacin IM.If the pain resolves, paroxysmal hemicrania is likely the
diagnosis.
Step 4: When pain arises, give the patient 8–12L/min oxygen with a mask. If the
pain resolves or reduces in intensity, a diagnosis of cluster headache is likely.
Step 5: If the indomethacin and oxygen tests were negative, SUNCT or SUNA is
most likely the diagnosis. If the pain is frequent and disabling, give the patient IV
lidocaine. If the pain resolves or reduces, SUNCT/SUNA is most likely the
diagnosis.
Step 6: Search for secondary causes, prefer imaging with MRI and MR Angiography
for structural and vascular abnormalities.
9.5 Secondary SUNCT
SUNCT is included in primary headaches, but its association with structural anomalies in the brain has been reported. Meningitis, trauma, posterior fossa lesions, and
pituitary lesions can be counted among the secondary causes [8, 9]. Some of the
secondary causes are listed in Table9.2
9.6 Management
SUNCT pain can be very disabling for patients, especially when occurs frequently.
It is a challenging situation for patients and some patients may consider suicide
because of this pain. Therefore, once diagnosed, treatment should start as soon as
possible. If the patient’s attacks are persistent and we can see cranial autonomic
ndings in the patient examination, we should start acute pain treatment as soon as
possible.
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U. Topbaş et al.
Table 9.2
causes of SUNCT
: Secondary
Cerebellopontine arteriovenous
malformation
Cavernous hemangioma
Brainstem infarction
Pilocytic astrocytoma expanding to the
trigeminal root
Vascular loops compressing the
trigeminal nerve
Parietal and parieto-occipital space
occupying lesions
Pathologic white matter lesions in
multiple sclerosis
Vertebral artery dissection
Neuromyelitis optica spectrum disorder
Cerebellopontine angle meningioma
Macroadenoma and microadenoma in
pituitary
In acute pain, intravenous 2% lidocaine solution should be given as a continuous
infusion at a dose of 1.5–3 mg/kg/h with monitoring [10]. Electrocardiogram
(ECG), liver, and kidney function tests should be performed, and hypomagnesemia
and hypokalemia should be corrected before starting the drug. It should never be
used in the presence of atrioventricular (AV) block on ECG.Heart rate and blood
pressure should be monitored every 5minutes for the rst 30minutes, heart rate and
blood pressure should be monitored every 15minutes for the next 4hours and every
2–4 hours thereafter, and lidocaine should be stopped in case of abnormality.
Treatment can be extended for up to seven days until the patient’s pain is relieved [10].
In patients without frequent and disabling attacks, preventive treatment should
be initiated. We recommend lamotrigine at a dose of 100–400mg/day in the rst
step [11]. The treatment goal should be reached by increasing the dose by 25mg/
day in the rst two weeks, 50mg/day in the third and fourth weeks, and 50mg once
a week after initiation. Generally, 200mg/day is the maintenance dose for patients,
but it may be necessary to increase the dose up to 400mg/day to be considered
lamotrigine-refractory. Since the success of alternative therapies may be low in
lamotrigine-resistant patients, preventive treatment with lamotrigine should be
insisted [11].
In patients who cannot tolerate lamotrigine or have refractory pain, gabapentin,
oxcarbazepine, topiramate, carbamazepine, and duloxetine can be started [11].
They may also be considered as an adjunct to lamotrigine. Additional oral preventive therapies are summarized in Table9.3.
If oral treatment fails or is not tolerated, occipital nerve blocks and botulinum
toxin applications can be performed. Occipital nerve blockade is performed with
2% lidocaine and 8mg dexamethasone in a 1:1 mixture and approximately 1/4 of
9 Short-Lasting Unilateral Neuralgiform Headache Attacks with Conjunctival…
89
Table 9.3
Oral medical treatments used for the treatment of SUNCT [12]
Drug
Lamotrigine 100–400mg/
Oxcarbazepine 300–2400mg/
Gabapentin 600–3600mg/
Topiramate 50–400mg/
Carbamazepine 200–1600mg/
Maintenance
dose Adverse effect Comment
day
day
day
day
day
Tremor, Stevens-Johnson
syndrome, inuenza-like
illness, headache
Hyponatremia, nausea,
dizziness, anxiety, ataxia,
depressed mood
Somnolence, tiredness,
dizziness, fatigue, central
nervous system (CNS)
depressed mood, ataxia
Drowsiness, depressed mood,
nephrolithiasis, anxiety,
cognitive dysfunction, angle
closure glaucoma
Cognitive dysfunction,
dizziness, ataxia, anxiety,
depressed mood, nausea,
hyponatremia
Primary therapeutic agent,
gradually adjust dosage
Could demonstrate superior
efcacy compared to
carbamazepine
Alternative treatment option
for SUNCT
Consider supplementary
treatment options improved
therapeutic outcomes in
SUNCT
Titrate slowly
Demonstrates reduced
therapeutic efcacy relative
to alternative medications
the cases respond [4, 11]. Botulinum toxin can be applied to the painful area at
1.5 cm intervals on the epidermis, 2.5–5 IU. This treatment, which is useful in
patients with trigeminal neuralgia, may also be effective in patients with SUNCT [4,
12]. Calcitonin gene-related peptide (CGRP) antagonist therapies are benecial in
some patient groups [12].
9.7 Refractory SUNCT andSurgical Treatments
Patients who do not respond to oral therapies and other transitional therapies are
considered refractory SUNCT.We use surgical treatment options for patients with
refractory SUNCT.Microvascular decompression (MVD), occipital nerve stimulation (ONS), and deep brain stimulation (DBS) are among these surgical options.
MVD is one of the options that can be used as a treatment, especially in the presence
of an aberrant trigeminal vascular loop [13]. ONS aims at pain desensitization by
continuous occipital nerve stimulation, resulting in increase in the trafc in the trigeminal pain pathway [14]. DBS treatment applied to the posterior hypothalamus
and ventral tegmentum of the mesencephalon showed near-complete improvement
in refractory SUNCT patients [15]. Although it is a very effective treatment, due to
serious adverse effects (hemorrhage in the brain stem, vertigo, permanent diplopia),
this treatment should be kept as a reserve for refractory patients.
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9.8 Conclusion
SUNCT is a severe and disabling headache associated with unilateral cranial autonomic syndromes. It is crucial to accurately diagnose SUNCT and differentiate it
from other types of autonomic headaches. Patients with severe and frequent headache attacks should be treated acutely to stop the pain. In the presence of SUNCT,
structural and vascular anomalies should be ruled out, at least by neuroimaging. In
the absence of response to prophylactic treatments, occipital nerve blockade and
botulinum toxin applications can be tried. In refractory cases, appropriate surgical
procedures may be performed.
References
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Chapter 10
Short-Lasting Unilateral Neuralgiform
Headache Attacks withCranial Autonomic
Symptoms (SUNA)
GabrieleSebastianelli , PubuduAmarasena , andFrancescaPuledda
10.1 Introduction
Short-lasting unilateral neuralgiform headache attacks are a group of primary headache disorders classied under trigeminal autonomic cephalalgias (TACs). They
share some clinical characteristics with TACs, including strictly unilateral head pain
in the trigeminal territory and cranial autonomic symptoms (CAS). Their prevalence and frequency are uncertain. A recent meta-analysis revealed that they are
present in approximately 0.32% of patients evaluated for headache or facial pain
[1]. Additionally, a population-based nationwide registry in Norway reported a
1-year prevalence of 1.2 per 100,000 [2], representing the rarest headache disorders
among the TACs.
The third edition of the International Classication of Headache Disorders
(ICHD-3) [3] recognizes two different subtypes depending on the presence of the
associated CAS: short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform
headache attacks with cranial autonomic symptoms (SUNA). However, it is unclear
whether these represent two separate entities or different manifestations of the same
disease [1].
G. Sebastianelli
Sapienza University of Rome, Department of Medico-Surgical Sciences and Biotechnologies,
Polo Pontino, Latina, Italy
P. Amarasena · F. Puledda (
Headache Group, Wolfson Sensory, Pain and Regeneration Centre, King’s College London,
London, UK
e-mail: francesca.puledda@kcl.ac.uk
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_10
*)
93© The Author(s), under exclusive license to Springer Nature
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G. Sebastianelli et al.
This chapter provides a comprehensive overview of the clinical characteristics
and pathophysiological mechanisms of SUNA, aiming to improve the identication
and distinction of this condition from other TACs and offer strategies for its
management.
10.2 Pathophysiology
The exact pathophysiology of SUNA and its relationship to SUNCT remains
unknown. Their pathophysiology involves a complex interaction between central
and peripheral nervous structures, with the trigeminal autonomic reex (TAR)
playing a pivotal role in developing the prominent CAS that accompany the pain.
TAR is composed of an afferent and an efferent limb, the trigeminal and the facial
nerve, respectively. Various nociceptive triggers can activate the afferent limb,
which transmits the stimulus to the second-order neurons into the trigeminocervical complex (TCC). The TCC has a reex connection into the pons with the superior salivatory nucleus (SSN). The efferent pathway arises from the preganglionic
parasympathetic bers of the SSN, which through the greater supercial petrosal
nerve are conveyed to the sphenopalatine ganglion (SPG), where postganglionic
parasympathetic bers arise and innervate nasal and lacrimal glands
(Fig.10.1) [4–6].
A peripheral neurovascular conict with morphological changes is highly
prevalent on the symptomatic side in patients with SUNA and SUNCT, and this
could be responsible for the peripheral ignition of pain [7]. This suggests a possible shared pathophysiology with trigeminal neuralgia (TN), indicating a potential overlap between these conditions [7]. However, central permissive mechanisms,
such as the disinhibition of the TAR, are needed for the development of prominent
CAS and the reoccurrence of attacks without the refractory period [8, 9], which
distinguishes SUNA from TN.The posterior hypothalamus was shown to activate
during spontaneous SUNCT attacks [10, 11] and was hypothesized to be responsible for the central disinhibition of TAR [8, 9], playing a pivotal role in the pathophysiology of short-lasting unilateral neuralgiform headache attacks [10]. Indeed,
the posterior hypothalamus is connected with the TCC by the trigeminohypothalamic and reticulohypothalamic tracts, by which it receives nociceptive signals
from areas innervated by the trigeminal system (such as cranial skin, meninges,
and intracranial blood vessels) [12, 13]. Through the secretion of orexin neuropeptides, the posterior hypothalamus can regulate autonomic functions and modulate the nociception activity of the TCC, including nociceptive processing of
meningeal input [14]. Furthermore, several studies have demonstrated that hypothalamic deep brain stimulation reduces pain in patients with drug-resistant SUNA
and SUNCT, conrming its central role in the pathophysiology of these conditions [15–17].