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British Journal of Neurosurgery 2000; 14(1): 33-39

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ORIGINAL ARTICLE

Bilateral trigeminal neuralgia: a therapeutic dilemma

L.TACCONI & J. B. MILES

Department of Neurosurgery,Walton Centre for Neurology and Neurosurgery, Liverpool, UK

Abstract
In order to illustrate the inherent problems of managing bilateral trigeminal neuralgia a retrospective study of the 16 cases
of bilateral trigeminal neuralgia, out of just over 300 cases of trigeminal neuralgia, treated over a 14-year period, has been
performed. All the patients, presented with a typical history of trigeminal neuralgia and underwent surgical exploration. Pain
relief was initially achieved in all cases; however, only four remained cured, three have become pain free after additional
rhizotomy, a further one after peripheral cryotherapy and four with medical treatment. Four patients have had bilateral
operations for trigeminal neuralgia, but in two cases the pain was relieved on one side only. Bilateral trigeminal neuralgia
presents special problems of management with respect to underlying neuropathology (e.g. multiple sclerosis), the need for
the limitation of the use of ablative techniques in order to minimise the disability of bilateral sensory and motor dysfunction,
and the relatively poor response to microvascular decompression. These factors emphasize the multifactorial nature of the
cause of trigeminal neuralgia. Magnetic resonance tomographic angiography is now available and is important in determining
the range of therapeutic options for this group of patients.

Key words: bilateral, evoked potential, facial pain, microvascular decompression, trigeminal neuralgia.

Introduction age 54.9) and the duration of symptoms from 1 to 28


years (mean 6.9). There was no difference in the sex
Idiopathic trigeminal neuralgia (TGN) is usually a
unilateral disease and bilateral cases are quite rare.^ distribution. In one patient, the initial presentation
Bilateral TGN (BTGN) has been reported to have an was with bilateral pain. In six cases (37.5%) the pain
incidence which varies between 0.3 and 6%.^"' had started on the lefr side, while in the remaining
While the treatment of idiopathic TGN has become cases it began on the right side. The second and third
relatively standarized, there remains a major branches were the most commonly involved. Involve-
therapeutic dilemma in how to treat bilateral cases. ment of a single branch was present in six patients.
Ablative techniques carry the obligatory risk of Generally, the pain was more severe on the side of
sensory and/or motor trigeminal deficits which, if the initial presentation.
bilateral, may cause the patient to have serious Two patients (12%) were first degree relatives
problems with speaking and eating, and therefore the (father and son) suffering from Charcot-Marie-
use of non-ablative methods must be considered. Tooth disease; two had multiple sclerosis (MS) and
To illustrate the magnitude of the problem, we another one had probable inclusion body myositis.
have retrospectively reviewed 16 cases of BTGN from Magnetic resonance tomographic angiography
a total of 302 patients (5.2%) operated on for TGN (MRTA)^ was performed in all cases and was useful
in our centre over the last 14 years. in showing a vascular compression in 81 % (13 cases).
In nine cases it was bilaterally positive including the
two patients with MS.
Patients and methods
The median interval between the appearance of
A total of 16 patients with BTGN presented to the the first symptom on one side and the onset on the
Walton Centre for Neurology and Neurosurgery in opposite side was 5.1 years (range between 7 days
Liverpool (UK) between 1983 and 1997, and have and 19 years). Six patients had previously received
been analysed. All presented as clinically typical of other forms of treatment such as percutaneous radi-
TGN according to Rasmussen's classification.^ ofrequency coagulation or peripheral nerve cryo-
The ages ranged between 24 and 78 years (mean therapy. All of them presented with established mild

Correspondence to. Professor J. Miles, Department of Neurological Sciences, Walton Centre for Neurology and Neurosurgery, Lower Lane,
Fazakerley, Liverpool L97 LJ, UK. Fax: 0151 529 5509. E-mail: MILES-J@WCNN.CO.UK
Received for publication 9 March 1999. Accepted 5 August 1999.
ISSN 0268-8697 print/ISSN 1360-046X online/00/010033-07 The Neurosurgical Foundation
34 L. Tacconi &J. B. Miles

to severe sensory impairment on the treated side. Unfortunately, she remained totally free from pain
One of them had undergone microvascular for only a matter of weeks. MRTA confirmed satisfac-
decompression (MVD) elsewhere. All patients tory bilateral decompression. At 24 months follow-up,
underwent surgical exploration. MVD was performed she has recurrent BTGN that is satisfactorily relieved
in 14 patients, in four (25%) this was done bilaterally by 1 g of carbamazepine daily in conjunction with
and two underwent posterior fossa rhizotomy. clonazepam 1 mg nocte.
Vascular compression of the trigeminal nerve by
an artery at the root entry zone on the symptomatic
Case 13
side was confirmed in 12 cases (seven left side); a
vein was found in two cases and in two patients there Idiopathic BTGN. This 71-year-old woman
was a combination of artery and vein. In those who presented after 15 years of bilateral facial pain
underwent bilateral exploration neurovascular involving the second and third division on each side.
compression (artery and/or vein) was identified in all Carbamazepine gave some relief, but it had to be
cases confirming the preoperative MRTA findings. discontinued because of severe side effects. Pheny-
Immediate surgical outcome was successful, in toin and baclofen gave no relief. Radiofrequency
terms of pain control, in all patients. At a median coagulation on the right side (the worse side) gave
follow-up of 36 months (range between 1 and 120 only a temporary improvement with sensory impair-
months) only four patients had been cured by MVD. ment. MRTA showed typical bilateral vascular
The others (75%) had recurrence of their pain (two compression. Bilateral MVD was performed at the
bilaterally). Four of these (one bilateral) have achieved same procedure. A vein was found on the right side,
pain control with medication (three with while an artery was the source of compression on the
carbamazepine, one with lamotrigine), three after left hand side. The postoperative recovery was
additional posterior fossa rhizotomy and one by unremarkable and the pain disappeared. At 5 years
further cryotherapy (short follow-up). In total, seven follow-up she is still pain free.
patients (44%) have been cured by surgery alone and
four (25%) controlled by surgery and medication.
Case 3
Two patients with unilateral pain recurrence are
considering posterior fossa rhizotomy. BTGN and MS. A 71-year-old man had suffered 8
Complications were: one patient had a cerebro- years' typical left-sided TGN involving the second
spinal fiuid leak cured by lumbar puncture; one a division. In the last 2 years he also experienced pain
temporary paresis of the IV cranial nerve; and one, in the same distribution on the opposite side. He had
an elderly quadriplegic patient with MS, died of suffered with MS for the past 30 years, resulting in
pneumonia 3 weeks after the operation. Table I such a severe quadriparesis that he was chair bound
summarizes the clinical and surgical features. and could not feed himself. The pain was either so
severe as to preclude eating or with even moderate
doses of carbamazepine and apparent pain suppres-
Illustrative cases sion, he was rendered too sedated to be fed. He had
been treated with repeated radiofrequency coagula-
Case 2 tion, eight times on one side and 10 times on the
Idiopathic BTGN. A 33-year-old woman, presented other, with recent periods of relief limited to only
with typical BTGN. The pain had started 18 months weeks. Neurological examination showed unilateral
previously on the left side involving the second and masseter weakness and bilateral patchy trigeminal
third divisions of the trigeminal nerve. The third sensory loss in addition to the profound quadri-
branch on the right side had been involved for 12 paresis.
months. Initially, the pain was controlled with a daily MRTA was positive bilaterally. As the right-sided
gram of carbamazepine, however, owing to the onset pain was so much more severe then the left and the
of side effects, the drug had to be reduced and eventu- latter having had the more effective recent radiofre-
ally discontinued. quency coagulation, it was decided to proceed with a
The patient's neurological examination was right-sided MVD. At surgery, an artery was found
unremarkable with no clinical or radiographic and easily dissected away from the nerve. The first
evidence of MS. few postoperative days were unremarkable with disap-
MRTA clearly showed trigeminal vascular compres- pearance of the pain but unfortunately he died from
sion at the root entry zone bilaterally and the patient pneumonia 3 weeks after the operation.
opted for a simultaneous surgical decompression of
both trigeminal nerves. The surgicalfindingscoincided
with the MRTA appearance. The superior cerebellar Case 6
artery was dissected and pushed away on the left BTGN and MS. A 47-year-old woman had suffered
side, while on the right, an artery and two veins were with typical left-sided TGN involving the second
separated from the nerve and the veins divided. The branch, for 1 year. In the last month she also
surgical recovery was quick and uncomplicated. experienced right-sided pain involving the same
Bilateral trigeminal neuralgia 35

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36 L. Tacconi &J. B. Miles

distribution. She had been diagnosed with MS 10 From an epidemiological point of view, BTGN is
years previously, when she had presented with optic somewhat different from the unilateral condition.
neuritis. MRTA was bilaterally positive. As she could Pollack et al., in a review of 30 cases of BTGN out of
not tolerate carbamazepine, she underwent MVD on a total of 664 cases found in the first group a higher
the left side which was the more affected side. At percentage of females, a higher rate of familial cases
operation, an ectatic superior cerebellar artery was and an increased occurrence of additional cranial
identified and displaced with a non-absorbable nerve dysfunction. ^'^ The familial incidence found in
sponge. At 2 years follow-up she had no pain on the this study reached a very high statistical significance
left side and has approximately monthly fiashes of (p<0.001) when compared with the control group.^
pain on the right side. In our series the ratio female/male was equal. One
patient, only, presented with additional VII cranial
Cases 5 and 7 nerve dysfunction (case 12) and only two had a history
BTGN and Charcot-Marie-Tooth disease (familial of familial incidence (cases 5 and 7).
cases). Case 7 was a 48-year-old man who presented We noticed, as did others,' a lapse of many years
with a 24-year history of right-sided TGN involving between the onset of pain on one side and the develop-
the second and third division, and a 10-year history ment of the pain on the other. In our series, however,
of left-sided pain involving mainly the second branch. the pain began simultaneously bilaterally in only one
He, like his son, suffered from Charcot-Marie-Tooth of our 16 patients.
Disease. At another hospital he underwent alcohol
injection and radioftequency coagulation on the right Incidence
side, which gave him only short-term relief from the
pain, but left him with sensory impairment on this A large review of 14,692 cases of TGN in 1969 by
side of the face. A gram of carbamazepine per day White and Sweet, found a 3.3% incidence of
ceased being effective so in 1987 he underwent a right- BTGN,'^ while 32 out of 269 consecutive patients in
sided MVD. The finding at operation was that of a Brisman's series (11.9%), were affected.'^The differ-
typical arterial compression. This procedure stopped ence in incidence can, probably, be explained on the
the pain for only 4 months. A MRTA, performed in our basis of the prevalence of MS in the series''' or
unit, showed vascular compression only on the left side because of the different length of the follow-up. The
and the patient opted for a MVD on this side. At longer the history, the more likely it is that there will
surgery, two veins were found, coagulated and divided be an appearance of BTGN. Peet and Schneider
at the root entry zone. The procedure has stopped his reported that the incidence of bilaterality in their
pain and at 5 years follow-up he only suffers occasional patients increased firom 2.7% when they were first
right-sided twitches of pain, well controlled by a small seen to 4.9% at their latest follow-up." However, the
dose of medication. incidence of pure idiopathic bilateral TGN seems to
His 24-year-old son (case 5) presented with a 4-year be 1-5%.''5
history of right-sided 2nd and 3rd division TGN and
1 year history of left sided 3rd division TGN. MRTA Concomitant illness
showed bilateral neurovascular compression of the
trigeminal nerves at the root entry zone. He Approximately 1% of patients with MS develop TGN
underwent bilateral simultaneous MVD with the find- and 2-4% of patients with TGN have evidence of
ings of a single vein compression on the right side MS."*-'^ In 10-20% of cases TGN can be the
and an arterial compression on the left side. presenting symptoms in otherwise asymptomatic
Unfortunately, he remained totally free from pain for patients with MS.'^ MS should be considered in any
only 2 weeks then the pain recurred bilaterally, initially young patient who develops TGN, particularly if it is
mild in form. Repeat MRTA showed satisfactory bilateral." In Jensen's et al. series seven out of 22
decompression on the left side, but a suspect vessel patients (32%) with MS had bilateral TGN and this
high incidence was significant (p<0.01).''' It seems
was present on the opposite side. He opted for repeat
that in patients with MS and TGN, BTGN can
exploration on the right side. A small vein of suspect
develop twice as often.'^ In a series of seven patients
dimensions was the only finding and so a partial
with trigeminal neuralgia and MS a vascular compres-
rhizotomy was undertaken. Two years postopera-
sion of the nerve was seen in 5 of the cases^ versus
tively, he is pain free on the right side, but he suffers an incidence of 85-95% of cases in the so called
mild TGN on the opposite side, controlled by idiopathic trigeminal neuralgia group.^
intermittent moderate doses of carbamazepine.
Trigeminal neuralgia has been reported to be
Discussion associated, although very rarely, with various connec-
tive tissue disorders, especially systemic sclerosis.^'"^^
Epidemiology
The reason for this tendency is not clear, but it does
The first few cases of BTGN^'^ were reported more not seem to be due to vasculitis.
than 200 years after the first description of TGN by In our series, we have two patients with established
Locke in 1677.''" MS (cases 3 and 6), one with atypical myositis disease
Bilateral trigeminal neuralgia 37

(case 12) and two with Charcot-Marie-Tooth disease dolorosa,^"'*' but recent publications do not support
(cases 5 and 7). The remainder did not have any this belief.*^ Its success rate is also lower when
clinical or radiographic evidence of MS. However, it compared with the other techniques.^^
is possible that they may later develop this disease, Percutaneous microcompression (PM) can be suit-
although their mean age of 52.7 years, seems to be able also for patients with first division pain and seems
well above the age when MS normally presents itself. to carry less sensory impairment;*^ however, the
predictable duration of pain relief has yet to be
established.
Aetiology
The morbidity associated with all these procedures
Vascular compression at the nerve root entry zone is (RFC, PGI, PM), when used bilaterally, must be
the main causative factor in TGN^'^'^^ with about bourne in mind. The patient may end up being unable
10% of cases due to cerebellopontine masses such as to eat, either because of total loss of sensation or
tumour^^ and aneurysm.^^ Demyelinating plaques, masticatory paralysis.'^
syringobulbia and brain stem infarct have an incidence Microvascular decompression (MVD) for
of 1%.25'3 trigeminal neuralgia seems to be the most physi-
The aetiology of BTGN is undoubtedly too ological way to deal with this problem.^^ The success
complex to be explained only on the bases of a rate with unilateral cases is very high 80-90% at 1
mechanical compression at the root entry zone. Most year follow-up, with a very low rate in term of
likely, a multifactorial mechanism plays an important permanent neurological sequelae^^'*"* and a mortality
role with, possible, central and peripheral influences. rate less than 0.5%.^"^ All patients with TGN should
Likewise the position of the MS plaque in the have MRTA. We have demonstrated, in a previous
central part of the root entry zone and the efficacy of publication, the utility, the extreme sensitivity and
central acting anticonvulsant therapy again, seems to specificity of this technique in demonstrating neurov-
validate this hypothesis.This might explain the surgical ascular compression in patients with TGN.*^ As a
failure of pain control, even, in patients with positive result, open surgical procedures can be recom-
intraoperative findings of neurovascular compres- mended with confidence in any case where MRTA is
sion. positive. A long-term analysis substantiates that MVD
carries the highest rate of long-term cure, the lowest
Treatment recurrence rate, and the least trigeminal sensory and
motor loss,*''"*^ which is vitally important when
The aim of treatment for TGN is suppression of applied to bilateral disease. Careful quantitative
pain, but with due consideration to the quality of life sensory neurology has shown that in fact, MVD
on prolonged treatment. Optimally, MRI of the brain restores to normal the subtle sensory deficits found
should always be performed to exclude the presence in TGN.'*^''*^ The electrophysiological restitution of
of a structural pathology, before starting medical treat- normal conduction can also be demonstrated at the
ment with carbamazepine.^''^^ Approximately 50% time of surgery."*^
of patients do not achieve long-term relief with
medical therapy either because of drug tolerance or MVD offers the option of cure and is, in our
toxic side effects.^^ When medical treatment fails, opinion, the treatment of choice, particularly in
then different surgical options must be considered. patients with involvement of the ophthalmic division.
There is no doubt that interrupting the pathway at It can be recommended for all patients considered fit
any location along the trigeminal nerve can relieve enough for surgery, regardless of their age. While the
the pain;^*"^^ however, the duration of pain relief success rate of MVD alone in BTGN in our series is
and the specific side efifects will be different depending only four out of 16 cases, the quality of pain relief
upon which technique is used and particularly where with normal neurological function, the extremely low
it is applied anatomically. mortality, 0.3% and morbidity rate (if we consider all
Percutaneous ablations (peripheral neurectomy, the cases of TGN operated on by MVD in our Unit)
glycerol injection, radiofrequency or balloon micro- makes it an essential consideration for treatment, in
compression) are easily performed, but the relief is presence of a positive MRTA.
temporary and results in varying degrees of deficits When neurovascular compression is not
in terms of sensory impairment and motor dysfunc- demonstrated by MRTA, decompressive surgery may
tion. Percutaneous radiofrequency coagulation (RFC) be excluded but partial root section of the trigeminal
can suppress the pain but it is associated with obliga- nerve in the root entry zone can still be considered.
tory sensory loss,^^ with up to 19% risk of postopera- The advantages of partial root section by open opera-
tive dysaesthetic complication^ and 4% incidence of tion at the root entry zone (REZ) are that it assures
corneal anaesthesia.^^ This procedure should not be protection of motor function, it provides longer or
used in patients with pain involving the first distribu- permanent pain relief, while, in a manner as yet
tion of the trigeminal nerve. unexplained, virtually always leaves some light touch
Percutaneous glycerol injection (PGI) was said to sensation (authors' observation). This makes it safer
be, rarely, associated with dysaesthesias or anaesthesia when used to treat TGN in the first division; it also
38 L. Tacconi & J. B. Miles

has a much lower risk of provoking anaesthesia dolo- help in establishing the efficacy of MVD, which is,
when possible, be the best physiological option.
Simultaneously bilateral MVD is a feasible and
reasonable option' if MRTA is positive bilaterally
and the pains of equal significance, such that removal Acknowledgements
of one would not allow reduction in medication. This We thank Professor H. B. Coakham, Frenchay
would not only save the patients from two operations Hospital, Bristol (UK), for allowing us to include a
with their attendant risks and complication, but also case of his whom we investigated.
save them from bearing the pain for an unnecessary
length of time.
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