Short-lasting
unilateral neuralgiform headache with
conjunctival injection and tearing syndrome
treated with microvascular decompression of the
trigeminal nerve: case report
Alfonso Lagares, Pedro A. Gomez, Angel
Pérez-Nuñez,
Ramiro D. Lobato, Ana Ramos
Department of
Neurosurgery, Hospital 12 de Octubre, Madrid,
Spain
Short-lasting unilateral neuralgiform
headache with conjunctival injection and tearing
(SUNCT) syndrome is a very rare disorder
characterized by short-lasting neuralgiform
unilateral pain affecting the
orbital-periorbital area, lasting for 5 to 250
seconds, sometimes precipitated by stimuli in
the trigeminal area, and associated with
autonomic phenomena consisting mainly of
conjunctival injection, tearing, and rhinorrhea
(19). Although many different pharmacological
treatments have been tried, no single treatment
has achieved a sure and definite response in
these patients. There also have been reports of
patients treated with surgery, with variable
results. To our knowledge, microvascular
decompression has been reported as successful in
only two previous cases. However, the case of a
patient who did not respond to two different
attempts of trigeminal root decompression also
has been reported. We present the case of a
patient affected by this syndrome who responded
to microvascular decompression.
CASE REPORT
A 56-year-old woman
was referred to our clinic because she had
experienced pain in the distribution of the
first left trigeminal branch during the previous
2 years. The pain was described as electric or
lancinating, spreading over the left orbit and
forehead as well as the left frontal and
temporal areas of the hair. She experienced
paroxysms lasting from a few seconds to 1 to 2
minutes superimposed over a dull sensation of
pain involving the same territory. The paroxysms
were triggered by touching the eye or the left
side of the face, chewing, yawning,
washing her hair, and even light. Although the
paroxysms were triggered by a light touch or
chewing, she was able to talk or touch herself
while having the paroxysm. Sometimes, she also
had pain in her throat or left ear. During
attacks of pain, she experienced tearing and
ipsilateral conjunctival injection, eyelid edema
and rhinorrhea, as well as intense photophobia.
The attacks had no refractory period and
normally disappeared while the patient was
sleeping.
She had been
administered different treatments, with no
response, before coming to our clinic.
Carbamazepine, gabapentin, topiramate, and
phenytoin had been tried with no response at
all. We attempted indomethacin, oxygen, and
another trial of carbamazepine and baclofen also
without response. The patient was hypertense and
could not tolerate verapamil because it
increased or even provoked pain paroxysms.
Neurological
examination results were normal, with no
trigeminal hypesthesia and a normal corneal
reflex. A magnetic resonance imaging (MRI)
examination revealed a vascular structure
compressing and distorting the left trigeminal
root. Because no medical treatment had been
useful and the patient was seriously
incapacitated by photophobia and pain, we
decided to perform a microvascular decompression
of the trigeminal root.
At surgery, there was clear compression of
the trigeminal root by a superior cerebellar
artery loop that was resolved by interposing a
Teflon patch. The patient awoke from the
operation without pain, and all the accompanying
signs and symptoms, such as photophobia, had
disappeared. The postoperative course was
uneventful, and a control MRI scan showed
resolution of the trigeminal root compression.
Two years after treatment, the patient remains
asymptomatic.
DISCUSSION
Autonomic features,
rare in neuropathic pain, are more common in
vascular pain syndromes. However, the
association between a neuritic or neuralgiform
pain and autonomic features such as tearing or
conjunctival injection are present in several
head pain syndromes, such as cluster headache,
paroxysmal hemicrania, hemicrania continua, and
SUNCT syndrome, which are grouped as the
so-called trigeminal autonomic cephalalgias.
These short-lasting headaches are characterized
by unilateral trigeminal pain occurring in
association with prominent ipsilateral cranial
autonomic features.
Different pathophysiological
mechanisms have been proposed to explain the
connection between pain in the trigeminal
territory and autonomic features. Goadsby and
Lipton (6) hypothesized that these headaches are
characterized by an activation between
trigeminal afferents, which would give rise to
pain, and cranial parasympathetic efferents,
which would be responsible for autonomic
features, via the trigeminal-autonomic
activation reflex. The pathway of this reflex
could consist of a brainstem connection between
the trigeminal and the parasympathetic outflow
accompanying the VIIth cranial nerve. There is
experimental evidence supporting the connection
between trigeminal afferents and changes in
parasympathetic outflow, such as occurrence of
increased cerebral and extracerebral blood flow
via vasodilation after trigeminal ganglion
stimulation in animals and humans. Moreover,
these cranial autonomic symptoms could be
prominent in trigeminal autonomic cephalalgias
resulting from a possible central disinhibition
of the trigeminal-autonomic reflex. Positron
emission tomography and functional MRI studies
performed during pain bouts have evidenced
abnormal ipsilateral hypothalamic activation in
patients with cluster headache and SUNCT
syndrome (14, 15). These findings all seem to
suggest that there are hypothalamic-trigeminal
connections and that the hypothalamus has a
modulatory role on the nociceptive and autonomic
pathways. Thus, a dysfunction involving these
structures could be responsible for the
association of facial pain and autonomic
features characteristic of these headaches.
SUNCT syndrome, first described by
Sjaastad et al. (19) in 1989, is characterized
by unilateral facial pain, maximal in the
ophthalmic distribution of the trigeminal nerve,
especially in the orbital or periorbital
regions, forehead, and temple (6, 10, 13, 17,
19). The individual attacks are brief, lasting
from 5 to 250 seconds, although attacks lasting
hours have been described. Patients can
precipitate attacks by touching trigger zones
within the trigeminal territory and also by
eating, chewing, talking, or coughing. The
intensity of the pain ranges from moderate to
severe, although patients characteristically can
touch themselves or talk while the pain is
present. Pain is described as stabbing, burning,
or electric shock-like. Cranial autonomic
symptoms are very prominent in SUNCT syndrome,
and characteristically both ipsilateral
conjunctival injection and lacrimation accompany
every attack. Rhinorrhea, eyelid edema, ptosis,
miosis, and facial redness also can be
present.
SUNCT syndrome
should be differentiated from essential
trigeminal neuralgia (6, 13). Trigeminal
neuralgia that involves only the territory of
the first branch is very infrequent, occurring
in only 1 to 4% of cases (18). In contrast to
SUNCT syndrome, patients cannot talk or touch
their face during attacks, and pain normally
spreads over time to other trigeminal
territories. Bouts of pain are shorter in
trigeminal neuralgia than in SUNCT syndrome, and
the typical refractory period after the pain
attack is absent in patients with SUNCT
syndrome. In addition, autonomic symptoms are
uncommon in trigeminal neuralgia. Benoliel and
Sharav (1) reviewed the occurrence of autonomic
symptoms in a series of 22 patients diagnosed
with trigeminal neuralgia. Six patients had
lacrimation with the attacks, but none of them
had first division involvement and all responded
at some time to carbamazepine. Sjaastad et al.
(18) also reviewed a group of patients with
first-division trigeminal neuralgia and
concluded that although autonomic symptoms, such
as lacrimation, can be present in patients with
trigeminal neuralgia, the combination of more
than two autonomic symptoms is very rare.
Autonomic symptoms are much more pronounced in
SUNCT syndrome, and there is response at some
time to carbamazepine in trigeminal neuralgia.
However, some connection between these two pain
syndromes may exist, because there are patients
with trigeminal neuralgia in whom SUNCT syndrome
eventually develops (3, 9). At presentation, the
symptoms of our patient could have suggested the
diagnosis of trigeminal neuralgia with
first-division involvement. However, the
presence of several prominent autonomic
phenomena accompanying pain attacks, the
duration of the attacks, and the fact that the
patient could talk or touch herself during the
pain bouts, together with the lack of response
to therapeutic doses of carbamazepine and many
other drugs, made the diagnosis of SUNCT
syndrome almost certain.
Other conditions such as
cluster headache and migraine should be included
in the differential diagnosis of this syndrome.
Both have longer attack durations (minutes to
hours), and cluster headache has low attack
frequencies. A key diagnostic point is the
presence in our patient of a clear-cut
trigeminal trigger, which is characteristic of
SUNCT syndrome. The lack of response of our
patient to indomethacin and the aggravation of
her symptoms by calcium channel antagonists,
which normally are useful in migraine and
migraine-like syndrome, also support the
diagnosis (6).
Many different
pharmacological treatments have been tried on
patients with SUNCT syndrome. This condition is
refractory to treatments that are useful in
other headache syndromes, such as carbamazepine,
indomethacin, paracetamol, 5-hydroxytryptamine
agonists, calcium channel antagonists,
-blockers, prednisolone, phenytoin, and
baclofen. Calcium channel antagonists are said
even to worsen the pain symptoms (6). There are
some reports of the efficacy of new
antiepileptic drugs such as gabapentin or
lamotrigine (4, 7, 12), but these are still
scarce. Surgical treatment also has been tried.
Some patients have responded to ablative
procedures directed to the trigeminal ganglion
such as balloon compression or glycerol
rhizolysis (8, 16). Two patients have been
treated successfully with microvascular
decompression (5, 11), but another underwent the
same procedure twice without success (2). In
this last patient, an offending vascular
structure was not found during surgery, but in
one of the successfully treated patients, MRI
did show a vascular compression before surgery,
as occurred in our patient. Although surgical
treatment should be reserved as the last
therapeutic choice, when a vascular compression
of the trigeminal root is identified, as
occurred in our case, microvascular
decompression can be effective in treating this
syndrome. However, more experience is needed to
determine the value of this technique in the
treatment of this complex syndrome.
COMMENTS
Short-lasting
unilateral neuralgiform headache with
conjunctival injection and tearing syndrome is a
rare condition hardly ever encountered by those
dealing with pain and may be confused with other
pain types with autonomic origin. Therefore, its
differential diagnosis and treatment are
challenging and disputable. As was stated by the
authors, making an interpretation about the
treatment on the basis of only one case may be
misleading. In planning the treatment modality,
minimally invasive methods could be more
appropriate as an initial step. This article is
valuable because it shows that microvascular
decompression may be applied in patients
resistant to all treatment choices
Yücel
Kanpolat Ankara, Turkey
The authors have
described an unusual syndrome that was
successfully treated with microvascular
decompression. This is a very rare phenomenon
and is characterized by very short-lasting
tic-like pain that affects the periorbital area
and is associated with conjunctival injection,
excessive tearing, rhinorrhea, and complaints of
blurred vision. The latter symptoms clearly
differentiate this syndrome from classical
trigeminal neuralgia. This short-lasting
neuralgiform pain is most commonly confused with
cluster headache, although the prolonged
character of cluster is a key diagnostic issue.
There often is a trigger similar to that in
trigeminal neuralgia; sometimes, the phenomenon
is precipitated by exposure to bright light.
Patients often have throat or ear pain, so
confusion with glossopharyngeal neuralgia
occurs.
Medications commonly
used for trigeminal neuralgia or other
neuropathic pain sometimes help. Migraine
therapies have been disappointing. The etiology
is generally unknown; this is one of the few
cases in which trigeminal root compression has
been demonstrated. I have approached these
patients as if they had trigeminal neuralgia;
unfortunately, however, the medical therapies
are not very effective. Failure to respond to
carbamazepine is one of the diagnostic points of
this syndrome, separating it from typical
trigeminal neuralgia. Most direct surgical
procedures on the ganglion have not been very
successful, and only a few patients have been
treated by microvascular decompression. The
success of this surgery makes it likely that
patients with from this rare syndrome can be
identified by its clinical features and through
failure of the usually successful medical
treatments. Microvascular decompression may
offer more successful therapy for patients who
have failed virtually all other treatments.
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