Achal Kumar

AS Cohen

C Ertsey

D Bouhassira

G Bussone

Headache Classification Subcommittee of the International Headache Society

JA Pareja

JW Berg ter

MS Matharu

Prabhat Singh

R Benoliel

R Guerreiro

Rakesh Kumar Gupta

Sushil Kumar Rahi

T Blatter

Vimal Kumar Paliwal

Journal of Headache and Pain

English

PubMed

Springer - Publisher Connector

BRIEF REPORT
Short-lasting unilateral neuralgiform headache with conjunctival
injection and tearing (SUNCT) with preserved refractory period:
report of three cases
Vimal Kumar Paliwal • Prabhat Singh •
Achal Kumar • Sushil Kumar Rahi •
Rakesh Kumar Gupta
Received: 16 November 2011 / Accepted: 12 December 2011 / Published online: 7 January 2012
 The Author(s) 2012. This article is published with open access at Springerlink.com
Abstract
Background Short-lasting unilateral neuralgiform head-
ache with conjunctival injection and tearing (SUNCT) and
short-lasting unilateral neuralgiform attacks with cranial
autonomic features (SUNA) are rare primary headache
syndromes characterized by spontaneous or triggered
attacks of unilateral, brief, multiple, orbitofrontal pain
associated with ipsilateral autonomic features. SUNCT is
considered as a subset of SUNA. In SUNA, there may be
cranial autonomic symptoms other than conjunctival
injection and lacrimation, or either of two is present.
SUNCT/SUNA can be triggered immediately after or at the
decrescendo phase of the ongoing attack without any
intervening refractory period. Refractory period is usually
present in trigeminal neuralgia. Absent refractory period is
thought to reliably differentiate SUNCT/SUNA from tri-
geminal neuralgia and has been proposed for inclusion into
the International Classification of Headache Disorders
(ICHD) diagnostic criteria for SUNCT.
Case reports We report three patients of SUNCT syn-
drome with preserved intervening refractory period of
variable duration observed at different times.
Discussion Trigeminal neuralgias with autonomic fea-
tures, SUNA and SUNCT share a common pathophysio-
logical mechanism and actually represent a continuum. It is
well known that patient with trigeminal neuralgia may
transform into SUNCT/SUNA. Similarly, being a contin-
uum, the presence or the absence of refractory period and
its duration may change in a patient with SUNCT/SUNA at
different time points.
Conclusion The presence of refractory period should not
exclude the diagnosis of SUNCT in a patient with other
clinical features suggestive of SUNCT.
Keywords Short-lasting unilateral neuralgiform
headache with conjunctival injection and tearing (SUNCT) 
Refractory period  Trigeminal neuralgia with autonomic
features  Short-lasting unilateral neuralgiform attacks with
cranial autonomic features (SUNA)
Introduction
Short-lasting unilateral neuralgiform headache with con-
junctival injection and tearing (SUNCT) is a rare primary
headache characterized by short-lasting unilateral orbito-
temporal neuralgiform attacks with conjunctival injection,
tearing and rhinorrhoea. Attacks are usually severe, brief
and vary in duration from 5 to 240 s and in frequency from
1/day to 30/hour [1]. Unlike trigeminal neuralgia, SUNCT
patients can trigger headache without an intervening
refractory period [2]. Absent refractory period has been
proposed as mandatory criteria for diagnosing SUNCT. We
report three cases of SUNCT syndrome with preserved
refractory period. All three cases had superior cerebellar
artery loop around the root entry zone of ipsilateral tri-
geminal nerve.
Electronic supplementary material The online version of this
article (doi:10.1007/s10194-011-0412-7) contains supplementary
material, which is available to authorized users.
V. K. Paliwal (&)  P. Singh  A. Kumar  S. K. Rahi
Department of Neurology, Sanjay Gandhi Postgraduate Institute
of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
e-mail: dr_vimalkpaliwal@rediffmail.com
R. K. Gupta
Department of Radiology, Sanjay Gandhi Postgraduate Institute
of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
123
J Headache Pain (2012) 13:167–169
DOI 10.1007/s10194-011-0412-7
Case 1
A 62-year gentleman complained of recurrent brief
(20–30 s) episodes of right periorbital pain for last 2 years.
Starting with 1–5 episodes/day, his pain attacks increased
to 20–50 attacks/day for last 2 months. In between attacks,
he remained asymptomatic. Pain was severe, non-throb-
bing, stabbing and most episodes were accompanied by
lacrimation and redness of right eye. Pain produced visible
distress but without motor restlessness (supplementary
video). There was no photophobia, phonophobia, vomiting
or exacerbation of pain with head movement. Pain could be
triggered by touching right periorbital area and mouth
opening. Patient reported absent refractory period. There
was no family history of migraine. NSAIDS did not relieve
his pain. Examination of the patient was unremarkable. A
triggered attack with conjunctival congestion and lacri-
mation was witnessed (supplementary video). Refractory
period of variable duration (1–10 min) was observed at
different occasions. MRI of the head revealed vascular loop
around right trigeminal nerve (Fig. 1a). A trial of indo-
methacin and oxygen inhalation failed to produce any
response. Oxcarbazepine (900 mg/day) reduced the fre-
quency of headache attacks. Oxcarbazepine induced seda-
tion and ataxia precluded further increase in its dose.
Lamotrigine was added (200 mg/day). It produced remis-
sion for last 6 months.
Case 2
A 56-year gentleman complained of intermittent, brief
(10–90 s) attacks of severe right supra-orbital lancinating
pain attacks for 1 year. Episodes of attacks increased to
25–30/day from 1 week from initial 1–6/day. Inter-attack
period was asymptomatic. Lacrimation, mild redness of
right eye and rhinorrhoea (with longer attacks) accompa-
nied most attacks. Pain could be triggered by touching right
forehead, spitting and chewing. There was no photophobia,
phonophobia, vomiting, associated motor restlessness or
pain exacerbation with head movement. There was no his-
tory of refractory period. There was no family history of
migraine. Carbamazepine, topiramate, amitriptyline, gaba-
pentin and pregabalin received in last 1 year did not work.
Neurological examination was normal. A triggered attack
conforming to history was witnessed. An intervening
refractory period of 10 min or more was observed in most
witnessed episodes. MRI of the head showed superior cer-
ebellar artery loop around right trigeminal nerve (Fig. 1b).
A trial of indomethacin and oxygen inhalation did not
relieve his pain. With lamotrigine (200 mg/day) he
achieved remission for last 1 year.
Case 3
A 64-year gentleman presented with recurrent, brief
(10–30 s) attacks of severe, stabbing pain involving right
periorbital area for 6 months. For 1 month, his attacks
increased to 5–30/day from initial 1–2/day. Most episodes
were accompanied by lacrimation, redness and partial
drooping of right eye lid (with longer attacks). There was no
photophobia, phonophobia, vomiting or restlessness.
Touching right eyelid and teeth brushing could trigger
attack. Patient was not sure about the refractory period.
There was no family history of migraine. At the time of
consultation, he was taking carbamazepine (600 mg/day)
for a week with resultant 2–3 attacks/day. His clinical
examination was normal. One spontaneous attack (25 s
long) with conjunctival congestion and lacrimation was
witnessed. Repeated attempts to trigger the headache failed;
however, few spontaneous attacks were witnessed upon
drug discontinuation. His MRI of the head showed superior
cerebellar artery loop around both trigeminal nerves
(Fig. 1c). He was restarted on carbamazepine (400 mg/day)
which promptly abolished his spontaneous attacks.
Fig. 1 Axial MRI fast imaging employing steady-state acquisition (FIESTA) sequence showing superior cerebellar artery loop (white arrow
heads) at root entry zone of trigeminal nerve (white arrows) on right side (a, b) and on both sides (c)
168 J Headache Pain (2012) 13:167–169
123
Discussion
Clinical features of our cases conform to diagnostic criteria
of SUNCT (ICHD 2nd ed) and does not comply with
diagnosis of migraine, paroxysmal hemicrania and cluster
headache [3]. However, these attacks are similar to ‘‘tri-
geminal neuralgia with autonomic symptoms’’ which is
indistinguishable from short-lasting unilateral neuralgiform
headache with cranial autonomic features (SUNA) [4–6].
Unlike SUNCT/SUNA, refractory period is usually present
in trigeminal neuralgia. Absent refractory period is thought
to reliably differentiate SUNCT/SUNA from trigeminal
neuralgia and has been proposed as mandatory criteria for
diagnosis of SUNCT [2]. Our patients 1 and 2 could not
demonstrate absent refractory period despite giving history
of same. Owing to overwhelming pain with every trigger,
they possibly presumed that a trigger just at termination of
ongoing attack will also induce pain. Hence, history of
refractory period (as derived from telephonic interview)
needs objective verification.
Trigeminal autonomic reflex with central disinhibition of
variable extent is thought to produce a continuum of tri-
geminal neuralgia, SUNA and SUNCT [1]. Most patients
with these headaches show variation in pain intensity,
attack frequency and severity of autonomic symptoms. The
variations may be good enough to switch a patient from
trigeminal neuralgia to SUNA/SUNCT [7–9]. Variations in
severity of autonomic features and duration of refractory
period were observed in all our patients. We propose that at
the extreme of the continuum represented by SUNCT, little
variation in the number, length and severity of pain attacks
and length of refractory period (or its absence) may be seen.
All our patients had aberrant vascular loops around
ipsilateral trigeminal nerve. Secondary SUNCT may have
atypical features, but refractory period in these cases was
not studied [7–9]. With paucity of literature, it is difficult to
speculate that refractory period could be a more frequent
feature of secondary SUNCT.
SUNCT caused by ipsilateral superior cerebellar artery
vascular loop near the root entry zone of trigeminal nerve is
rare [10, 11]. These aberrant vessels can be delineated by
special MRI techniques like fast imaging employing
steady-state acquisition (FIESTA). Once identified, vas-
cular decompression over trigeminal nerve may be offered
in those with failed medical treatment [11].
We conclude that a brief refractory period may exist in
SUNCT patients. Refractory period may be prospectively
studied in a reasonable cohort before its application as
criteria for diagnosing SUNCT.
Conflict of interest None.
Open Access This article is distributed under the terms of the
Creative Commons Attribution License which permits any use, dis-
tribution and reproduction in any medium, provided the original
author(s) and source are credited.
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Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) with preserved refractory period: report of three cases

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