- Abstract : Two patients suffering
from SUNCT syndrome are presented. Some features
are remarkable. The first patient was a
69-year-old man whose first crisis was located
in the right supraorbital region. After a
4-month spontaneous remission, the pain returned
to the upper part of the cheek, radiating to the
supraciliary region on the same side, with
lacrimation and conjunctival injection.
Rhinorrhea was absent. The painful attacks were
triggered by head movements. Clinical
improvement occurred with carbamazepine
treatment. The second patient was a 48-year-old
woman whose painful attacks lasted from 30 to 45
seconds followed by a burning sensation lasting
2 hours. Autonomic signs such as conjunctival
injection, lacrimation, and edema and
ipsilateral ptosis of the upper lid were rather
marked. There was never any rhinorrhea. Her
attacks were triggered by head and eye
movements. She responded to the administration
of corticosteroids and carbamazepine. According
to these features, the two patients had SUNCT
syndrome, and the positive carbamazepine
response suggests a relationship with trigeminal
neuralgia.
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Since Sjaastad et al described it in 1978, this
syndrome acquired greater attention when a
detailed description and the term SUNCT appeared
in 1989. The clinical manifestations consist of
shortlasting crises of a neuralgic-type pain,
felt unilaterally, and centered in the
orbital/periorbital area; of moderate or severe
intensity and accompanied by conjunctival
injection, tearing, and rhinorrhea. Twenty-one
cases have been reported to date. Since the
beginning, reference has been made to the
similarities of SUNCT and cluster headache, as
both show autonomic signs, and also to
first-division trigeminal neuralgia because of
the neuralgic characteristics of this pain.
However, Sjaastad and Kruszewski have made a
particular emphasis on highlighting the
differences so as to grant SUNCT its own
identity. Worldwide experience of this syndrome
is still limited, so new case descriptions are
required to obtain better knowledge and
understanding of it. For that reason, two
patients diagnosed in Argentina are presented
here.
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- CASE HISTORIES
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- Case 1.-A 69-year-old man who, in
1994, had neuralgic pain crises of 10 to 20
seconds duration and moderate intensity. Located
in the medial right supraciliary area, such
attacks were triggered by a gentle touch of the
area and in some cases by lateral head
movements. There was no pairs between paroxysms,
and he never had a nighttime crisis. Analgesics
did not provide significant relief. Towards the
second half of that year, there was a
spontaneous remission for 4 months. By February
1995, daily painful crises returned. They were
episodes of neuralgiform pain similar to a 15-
to 20-second electric current, and with the
peculiarity of beginning in the upper part of
the cheek and radiating to the supraciliary
region on the same side. As it went by behind
the eye, it caused conjunctival injection and
profuse tearing. There was no rhinorrhea.
Attacks recurred between three and five times a
day, and were especially triggered by touching
the right supraciliary and upper palpebral
areas, and by mastication, laughter,
yawning, and
lateral head movements to the right.
Neurological examination was always normal, as
were a brain CT scan and magnetic resonance
imaging (MRl). There were no remarkable
antecedents. After unsuccessful treatment with
several analgesics, in June of the same year,
treatment with daily carbamazepine 600 mg was
initiated. In a 2-month period, the interval
between crises begun to increase and after 3
months of treatment, medication was gradually
discontinued with only one or two attacks per
week.
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- Case 2.-A 48-year-old woman consulted
a physician in April of 1997 due to a painful
condition in the right orbitofrontal area. She
experienced between six and seven episodes
daily. Each consisted of an abrupt and intense
peak of neuralgic pain like a spark or electric
shock of 30 to 45 seconds duration and followed
by a painful burning feeling which persisted
from 35 minutes to 2 hours.
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- The neuralgic pain began in the medial
canthus of the right eye and then involved a
triangular orbitofrontal area and, with less
intensity, the upper half of her right cheek.
During the height of the pain, there was ocular
congestion, tearing, edema, and ipsilateral
palpebral ptosia. Crises were spontaneous, and
also triggered by touching the periorbital area,
brushing her teeth,
yawning,
masticating, washing her hair, exposure to
strong wind, lateral head movements to the
right, and rapid eye movements. Most of the
attacks occurred between 6 AM and 8 PM, but she
also had some nocturnal attacks, apparently
triggered by movements. After the painful
episodes, she suffered from hyperesthesia in the
affected area and the skin in the frontal region
ipsilaterally looked rough and irregular like
sandpaper.
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- Neurological examination, except for the
above description, was always normal, as were a
brain CT scan and MRI. lndomethacin,
amitriptyline, ergotamine, and verapamil
treatments were not helpful. The later use of
prednisolone (60 mg daily for 5 days and then 20
mg daily for 10 days) and carbamazepine (800 mg
per day for 11 weeks) brought benefit by
reducing the painful crises to one or two a day
and then providing complete relief. This cluster
of painful attacks was preceded 2 years before
by another episode which had a prolonged
sawtoothlike temporal pattern. The previous
clinical history was not otherwise
remarkable.
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- Comments
- The patients presented here show some
clinical characteristics reminiscent of SUNCT
syndrome.
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- Location of pain : In both
cases, the most painful location was in the
orbital area, even when in the first patient,
the painful discharge started in the upper part
of the cheek and radiated rapidly to the eye. In
the second patient. there was a painful feeling
in the upper half of the ipsilateral cheek. In
our interpretation, the main localization of
pain was still in the orbital periorbital
region.
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- Intensity and duration of pain
: In both cases, the intensity of the
crises was between moderate and intense, hang an
electric current characteristic. In patient 1,
every episode lasted between 15 and 20 seconds
and in patient 2, every paroxysm was initiated
by an intense peak of pain of about 30 seconds,
followed by a "plateau" of lesser intensity
which could last between 35 minutes and 2 hours.
Attacks with atypical prolonged features, as in
this case, were described by Pareja and
coworkers in 1996.
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- Frequency : In one case, there
were between three and five exclusively daytime
crises and in the other case there were also
nocturnal crises, which were the first to
disappear when the number of attacks
decreased.
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- Triggering factors : Both
patients had similar triggers located in either
the first or in the second division of the
trigeminal nerve. Additionally, painful attacks
were triggered by head rotation, and in the
second patient by rapid lateral eye movements.
These features have been reported in SUNCT by
Sjaastad et al.
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- Accompanying signs : The first
patient had conjunctival injection and profuse
tearing when the painful discharge was felt
behind the eye. In the second patient, there was
edema and palpebraI ptosis and the skin in the
ipsilateral frontal region looked rough and
irregular like "sandpaper." Neither patient had
miosis or rhinorrhea. The prominent autonomic
phenomena are consistent with SUNCT syndrome, in
contradistinction to trigeminai neuralgia, in
which such features are rarely seen.
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- Medication : Patient 1 had a
partial remission with carbamazepine after 3
months of treatment. In patient 2, the crises
were reduced to one or two a day after 16 days
of combined treatment with carbamazepine and
corticosteroids and 9 weeks of carbamazepine
alone.
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- In both patients, the differential diagnoses
included cluster headache, trigeminal neuralgia,
chronic paroxysmal hemicrania, and Raeder
syndrome. Organic pathologies were excluded by
imaging studies. The neuralgic characteristics
of the pain, location, duration, accompanying
autonomic symptoms, and extratrlgeminal
triggers, all make the condition described by
the two patients similar to SUNCT syndrome. A
remarkable characteristic is the fact that in
both patients, the location of pain was not
confined to the first trigeminal division, but,
in part, also involved the second division. Also
notable was the prolonged duration of each
crisis in patient 2, as well as the skin changes
in the frontal area.
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- We have to mention, especially, the response
to treatment. No analgesic was beneficial. The
only favorable response occurred with
carbamazepine, alone in one case and given with
corticosteroids in the other. We believe
medication was actually responsible for
improvement, since both patients had a long
history of pain and with these drugs their
crises disappeared or were greatly reduced over
a 2- to 3-month period. We do not believe this
was just a spontaneous remission. Even though it
is mentioned that one of the characteristics of
SUNCT is its partial or lack of response to
carbamazepine, we, nevertheless, believe that
our cases fitted properly into this syndrome and
due to all the features described above, we
believe it is closer to trigeminal neuralgia
than cluster headache and the like.
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