Objective assessment of autonomic signs during triggered

first division trigeminal neuralgia

JA Pareja1, M Baron1, P Gili2, J Yanguela2, AB Caminero3, JL Dobato1,
FJ Barriga1, L Vela1 & M Sanchez-del-Ro1
1
3

Department of Neurology, Fundacion Hospital Alcorcon, Madrid, 2Department of Ophthalmology, Fundacion Hospital Alcorcon, Madrid, and
Department of Neurology, Hospital NaSa de Sonsoles, Avila, Spain

Pareja JA, Baron M, Gili P, Yanguela J, Caminero AB, Dobato JL, Barriga FJ,
Vela L & Sanchez-del-Ro M. Objective assessment of autonomic signs during
triggered first division trigeminal neuralgia. Cephalalgia 2002; 22:251255. London.
ISSN 0333-1024
A total of 26 episodes of V-1 trigeminal neuralgia attacks have been recorded in two
female patients. Autonomic phenomena were assessed according to a semiquantitative
scale. Attacks lasted 175 s. Mild lacrimation without conjunctival hyperaemia,
rhinorrhea or ptosis was observed, even in relatively long lasting episodes. This is
in clear contradiction with SUNCT (shortlasting, unilateral, neuralgiform headache with
conjunctival injection, tearing and rhinorrhea) attacks that are always dramatically
accompanied by both lacrimation and conjunctival injection of the symptomatic side
from the very onset of symptoms. Carbamazepine provided complete and sustained
relief of symptoms in both patients. Herein we will show differential autonomic features
of V-1 trigeminal neuralgia vs. SUNCT that will both aid the clinician to distinguish both
syndromes and stress that both entities are nosologicaly different. u Head and face,
neuralgia, SUNCT, trigeminal neuralgia, unilateral headaches
Juan A. Pareja, Department of Neurology, Fundacion Hospital Alcorcon, C/Budapest 1,
Alcorcon-Madrid, Spain. Tel. 34916219513, fax 34916219714,
e-mail japareja@fhalcorcon.es Received 19 February 2001, accepted 27 November 2001

Introduction
The controversy regarding the nosological independence
of SUNCT (shortlasting, unilateral, neuralgiform headache with conjunctival injection, tearing and rhinorrhea) from V-1 trigeminal neuralgia is still a subject
of debate. SUNCT syndrome resembles V-1 trigeminal
neuralgia in several features (unilaterality of pain,
triggering mechanisms, brevity and frequency of
attacks) (1, 2). This similitude may be further supported
by cases of SUNCT secondary to structural lesions in the
posterior phosa (3, 4) where compression of the trigeminal nerve has been taken as pathogenically crucial.
Even conventional treatment of V-1 trigeminal neuralgia
successfully used in SUNCT, such as Gassers ganglion
compression (5), has been claimed as evidence of the
similitude between both disorders. Indeed, reports
claiming that SUNCT may be a transformed trigeminal
neuralgia (6), or that SUNCT could be a trigeminal
# Blackwell Science Ltd Cephalalgia, 2002, 22, 251255

neuralgia with lacrimation (7), have also been the subject

of discussion.
On the other hand, there are numerous clinical
differences between SUNCT and trigeminal neuralgia
(sex distribution, temporal profile of attacks, refractory
periods, autonomic signs and response to treatment) that
make us consider them distinct nosological entities
(8). V-1 trigeminal neuralgia patients may report mild
autonomic signs (usually lacrimation) that tend to
appear during severe attacks and after suffering for
years from headache (8), whereas SUNCT attacks are
characterized by both conjunctival injection and lacrimation appearing in a dramatic and rather explosive
way from the onset of symptoms.
The principal aim of the present study was to
document the autonomic signs in V-1 trigeminal
neuralgia in order to obtain objective information to
further dissect and clarify the differences with other
pain syndromes.
251

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JA Pareja et al.

Methods
Patients
Two patients suffering from pure V-1 trigeminal
neuralgia (i.e. not extended to V-2 or V-3 territories)
were studied during numerous acute episodes. Neurological and ophthalmologic examinations did not reveal
underlying abnormalities. Routine blood analysis
including ESR and brain/orbital MRI were also within
normal limits. Treatment with standard oral doses of
carbamazepine completely controlled symptoms. Diagnosis of idiopathic trigeminal neuralgia fulfilled IHS
diagnostic criteria (9). Patients gave written consent for
withdrawal of treatment, the induction of the attacks
and the recording.

Patient 1
A 79-year-old right-handed female with a 8-year history
of right primary V-1 trigeminal neuralgia was successfully treated with carbamazepine (600 mg daily). The
patient reported having suffered from brief episodes of
several seconds of lancinating pain, some of which were
accompanied by ipsilateral lacrimation. She denied
reddening of the eye, nasal stuffiness, rhinorrhea,
changes in pupil size or palpebral width. Attacks
could be induced by light tapping of the temple and
by blowing the nose.
The patient agreed to stop medication in order to
record typical provoked attacks. Three days after
discontinuing medication a severe symptomatic period
developed. On the day of the recording the patient
triggered a total of seven attacks, of which three
could be studied in detail. After one day of study
carbamazepine was resumed providing complete relief
of symptoms.

Experimental setting
Patients were placed in front of a video camera and
asked to provoke attacks based on their previous
experience, such as by touching the forehead, temporal
area or blowing the nose. Both patients were videotaped
and in patient 1 photographs were also taken (Fig. 1).
All images were obtained under the same conditions
of light, magnification and exposure parameters using
a high-resolution camera (ZEISS FF 450 IR Fundus
camera) and digital image archiving system (Visupac/
System 450 Digital). In order to get a better image of
the bulbar and tarsal conjunctiva the lower and upper
eyelids were lifted and patients were told to look up.
Images were obtained both during and in between
attacks. The contralateral eye during the attack and both
eyes during the interictal period were used as control.
The following autonomic parameters were evaluated:
lacrimation, presence of hyperaemia and oedema in
bulbar and tarsal conjunctiva, rhinorrhea, ptosis, eyelid
swelling and changes in pupil diameter. If a tear was
observed during an attack, it was removed with the aid
of a tissue before induction of the next episode. Relevant
autonomic phenomena were quantified according to a
previously described scale (Table 1 (10)).
Duration of attacks was measured by a stopwatch.
Start-time was defined as the time when the patient
felt the onset of pain, and stop-time as the time the
patient considered pain completely abated. Estimation
of the degree of autonomic phenomena observed in
real time was independently assessed by two of the
authors (JAP, MB). In order to rule out any subjective
bias two ophthalmologists ( JY and PG) also evaluated
the autonomic phenomena by watching the videotapes
and photographs. Only close-up images were analysed.
Inter-rater reliability was of 100%.

Patient 2

Results

A 58-year-old right-handed female diagnosed de novo

(5 months after the onset of symptoms) with right V-1
trigeminal neuralgia, agreed to allow us to witness
several attacks before starting treatment with carbamazepine. Attacks were provoked by light touch of the
forehead and temporal area. On the day of the recording
and after several hours of observation the patient did
not present spontaneous attacks, therefore she proceeded to trigger 23 attacks within a period of 2 h,
after which carbamazepine was started and complete
response was achieved and sustained for the past
5 years.

Duration of attacks
A total of 26 attacks were recorded (three in patient 1
and 23 in patient 2). Duration of attacks was
175 s (patient 1, 124 s; patient 2, 175 s). According
to the patients own estimation previous attacks
usually lasted less than 1015 s. However, in
patient 2, 61% of the 23 attacks recorded lasted
more than 15 s (Table 2). This same patient considered
that the triggered attacks were more severe and
lasted longer than the apparently spontaneous generated
ones.
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Autonomic signs during trigeminal neuralgia

253

Figure 1 Differential conjunctival signs in V-1 trigeminal neuralgia vs. SUNCT syndrome. Panel A shows the eye ipsilateral to the
symptomatic side during a V-1 trigeminal neuralgia attack (patient 1). Note the absence of lacrimation and conjunctival injection.
Panel B represents the same eye as in panel A in between two attacks. Panel C is the contralateral eye during the same attack to
demonstrate absence of lacrimation or conjunctival injection. Note: the eye shown in panels AC is from the same patient. Panel D
shows the symptomatic eye of a different patient during a SUNCT attack, to demonstrate a clear conjunctival hyperaemia and
lacrimation (outer corner of the eye) as opposed to V-1 trigeminal neuralgia. Pictures were taken with a ZEISS FF 450 IR Fundus
camera, Panasonic 3 ccd camera (7683576 pixels).

Table 1 Grading of ocular signs of lacrimation and

conjunctival injection (10)
Lacrimation
Mild (+): increased conjunctival brightness to increased
conjunctival meniscus
Moderate (++): tears clearly seen but hardly overflowing
the palpebral edge
Severe (+++): tears running down the face
Conjunctival injection
Mild (+): increase in the number of vessels seen but not
clearly dilated
Moderate (++): clearly dilated vessels
Severe (+++): engorged, tortuous and intensely
red/violaceous coloured vessels.

Conjunctival hyperaemia
In none of the observed attacks in patient 2 was there
any appreciable conjunctival injection. However, in
patient 1 we observed a selective mild injection of the
tarsal conjunctiva on the symptomatic side (Fig. 1A)
without obvious hyperaemia of the bulbar conjunctiva.
In this case this selective hyperaemia could either be
explained by retraction of the symptomatic inferior
eyelid in order to take the photographs or secondary to
an antialgic contraction of the eyelids.

Other autonomic signs

There was no ptosis, engorgement of the vessels of
the eyelids, swelling of eyelids, changes in pupil size,
rhinorrhea or reported nasal stuffiness in any of the
attacks recorded.

Lacrimation
Assessment of lacrimation revealed production of
moderate amounts of ipsilateral lachrymal fluid,
enough to be detected by the naked eye of the
investigators but insufficient to overflow the lower
eyelid and form a tear. During six attacks (23%) a
single well-formed tear hardly leaving the eyelid was
documented.
# Blackwell Science Ltd Cephalalgia, 2002, 22, 251255

Contralateral eye and interictal period

The contralateral eye did not show changes in any of
the parameters evaluated during pain, and did not
differ from its appearance during the interictal period
(Fig. 1C). The affected eye during the interictal period
revealed no asymmetries with the contralateral eye
(Fig. 1B).

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JA Pareja et al.

Table 2 Semiquantitative estimation of some ocular signs

during V-1 trigeminal neuralgia attacks

Attack no.*
1.1
1.2
1.3
2.1
2.2
2.3
2.4
2.5
2.6
2.7
2.8
2.9
2.10
2.11
2.12
2.13
2.14
2.15
2.16
2.17
2.18
2.19
2.20
2.21
2.22
2.23

Duration (s)
12
16
8
5
15
12
18
14
13
20
21
22
13
25
23
22
18
21
15
9
16
13
17
28
22
20

Lacrimation
++
++
+
0
0
+
++
+
++
+
++
++
+
+
+
+
+
+
+
+
0
0
+
+
0
+

Table 3 Summary of differential autonomic features between

V-1 trigeminal neuralgia and SUNCT estimated by the same
semiquatitative method (10)

Conjunctival
injection
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0

*The first digit corresponds to the patient number and the

second digit to the number of the attack.

Discussion
We had the opportunity of witnessing and recording
multiple stereotypical V-1 trigeminal neuralgia attacks,
providing an objective assessment of the attacks and
avoiding the patients recall bias. In a total of 26 episodes
of V-1 trigeminal neuralgia attacks, recorded paucity
of autonomic signs has objectively been demonstrated,
even in relatively long lasting episodes. Although
caution is required as only two patients were studied,
it seems that in V-1 trigeminal neuralgia, lacrimation,
when present, is meagre and hardly ever accompanied
by conjunctival injection (8). This is in clear contradiction
with SUNCT attacks that are always dramatically
accompanied by both lacrimation and conjunctival
congestion of the symptomatic side from the very
onset of symptoms, Table 3 (1, 2).
Generally, SUNCT attacks last three times as long
as V-1 trigeminal neuralgia attacks (11). We had the
exceptional opportunity of witnessing 23 V-1 trigeminal
neuralgia attacks lasting more than 10 s (range 1228).

SUNCT*
V1-neuralgia{

Lacrimation

Conjunctival
injection

Duration (s)

+++
+/++

+++
0

80140
528

*Twenty-two attacks in three patients (10). {Twenty-six attacks

in two patients, present series.

These unusual relatively long-lasting attacks have given

us the chance to compare the pain and accompanying
features of V-1 trigeminal neuralgia attacks vs. equivalent shorter episodes of SUNCT. This is important
because attacks of V-1 trigeminal neuralgia of long
duration may overlap the shortest SUNCT attacks
(1020 s) (11). By doing so we ruled out any uncertainty
regarding the paucity of local autonomic signs in V-1
trigeminal neuralgia due to the short duration of the
attacks (less than 10 s) (8). It should be noted that it is
widely acknowledged that SUNCT attacks lasting
1020 s already present full-blown autonomic accompaniments 1 to 2 s after onset of pain (1). Our data show
the almost complete absence of autonomic signs in even
relatively long attacks of V-1 trigeminal neuralgia,
clarifying that duration of the episode is independent
of the accompaniments, and nosologically separating
V-1 trigeminal neuralgia attacks from other similar
cephalgias.
It has been proposed that in some patients SUNCT
might have evolved from trigeminal neuralgia (6, 7).
The question is whether it is possible that both disorders consecutively appear in some patients, as has
been reported in clustertic (12, 13) and CPHtic (14, 15),
or rather a unique polymorphous condition may
exhibit two clinical variants (V-1 trigeminal neuralgia
and SUNCT). In V-1 trigeminal neuralgia attacks, as
opposed to SUNCT attacks, autonomic signs appear
either occasionally or are present to a minor extent (8).
Although in theory incomplete attacks of SUNCT with
less overt autonomic accompaniments might well occur
with what we know so far, V-1 trigeminal neuralgia
and SUNCT attacks seem to represent two different
disorders (8).
The clinical differences described above between
SUNCT and V-1 trigeminal neuralgia could well
follow a difference in the threshold for activation of
only the trigeminal system, as in V-1 trigeminal
neuralgia or the additional recruitment of other systems
such as the parasympathetic, that would lead to
the appearance of more or less profuse conjunctival
injection, lacrimation and rhinorrhea as observed in
# Blackwell Science Ltd Cephalalgia, 2002, 22, 251255

Autonomic signs during trigeminal neuralgia

SUNCT. The cessation of the attack and following
refractory period in V-1 trigeminal neuralgia suggests
the presence of a strong central inhibitory system that
is lacking or malfunctioning in SUNCT. Recently, the
reported activation in the hypothalamus during a
SUNCT attack has been claimed as pathogenically
relevant as the hypothalamus has connections to the
superior salivary nucleus, responsible for the autonomic
signs observed (16). Nevertheless, we still face the problem of a difficult diagnosis in these patients unless future
biological markers are available.
In conclusion, there seem to be quantitative and qualitative differences regarding local autonomic accompaniments between SUNCT and V-1 trigeminal
neuralgia. According to our data, absence of conjunctival
injection and mild lacrimation characterize V-1 trigeminal neuralgia, while marked lacrimation and
conjunctival injection appear in tandem during SUNCT
attacks, and both signs should, in the future, be
incorporated together as one solid diagnostic criterion
of SUNCT as it strengthens the differences with V-1
trigeminal neuralgia. However, further research is
necessary as inter- and intraindividual differences in
autonomic signs accompanying V-1 trigeminal neuralgia
may occur.

Acknowledgements
The work described here was supported in part by the PPA
Humphrey-IHS-GlaxoSmithkline 2000 Research Fellowship
(MSDR).

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