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A review of treatment modalities for periodic migrainous neuralgia
345
Pain, 31 (1987) 345-352 Elsevier PAI 01143
A review of treatment modalities for periodic migrainous neuralgia Mark Wake and Edward Hitchcock of3~rrningh~~
University
L)epartment of Neurosurgery,
Midland Centre for Neurosurgery Ho&y Lane, Bjrming~am (U.K..)
und Neuroiog?t,
(Received 4 November 1986, revised received 7 July 1987, accepted I2 August 1987)
Ten cases of periodic migrainous neuralgia are reported. Clinical details of the cases Summary including presentation and clinical course are typical of this condition. Treatment of these 10 cases encompasses medical, minor surgical and major surgical procedures. The results demonstrate that if medical tr~tm~t fails or there are severe drug side effects, trigeminal rhizotomy may be effective in producing iong-term pain relief. If pain recurs or trigeminal rhizotomy is ineffective, section of the greater and lesser superficial petrosal nerves should be considered. Key words: Periodic migrainous neuralgia; Trigeminal rhizotomy; Petrosal nerves
Introduction Definition
Periodic migrainous neuralgia is a condition consisting of ‘unilateral pain principally in the ocular, frontal and temporal areas recurring in separate bouts with daily attacks for several months usually with rhinorrhoea or lacrimation’ [7]. A syndrome of facial pain accompa~ed by facial autonomic disturbance is known by a variety of names (red migraine, sphenopalatine neuralgia, erythroprosopalgia, sympathetic hemicrania, migrainous (cilia@ neuralgia, atypical neuralgia with sympathetic phenomena, carotodynia, vidian nerve neuralgia, autonomic facie-cephalgia, histamine cephalgia, petrosal neuralgia, painful vascular disorder of the head, facial migrainous neuralgia, trigeminal migraine, cluster headache and periodic migrainous neuralgia). The condition deserves distinction
Correspondence to: Prof. E. Hitchcock, Midland Centre for Neurosurgery and Neurology, Holly Lane, Smethwick, WarIey, West Midlands B67 7JX, U.K.
0304-3959/87/$03.50 0 1987 Elsevier Science Publishers B.V. (Biomedical Division)
3&i
from other atypical neuralgias because of the prominent autonomic disorders accompanying the pain and because for this group a relatively simple operation may procure complete relief, The disease is characterised by a remittent course recurring at intervals of months or years and lasting for days, weeks or months. The attacks of pain occur less frequently than in trigeminal neuralgia and there are no ‘trigger spots.’ It is often remarkable for the extraordinary regularity of its onset which is often in the early hours of the morning, some 3 or 4 h after the patient has retired to bed. The pain is throbbing, burning, aching or lancinating and is usually experienced in the upper half of the face. commonly affecting the eye. It is often associated with pain in the temporal region. cheek or neck but remains strictly unilateral. The associated autonomic disturbances are often striking. Ptosis or hyperhidrosis on the affected side have also been noticed but many of the autonomic manifestations may be attributed to parasympathetic overactivity. The complete autonomic disturbance comprises conjunctival injection, lacrimation and mucous discharge or blockage of the nose, again all strictly unilateral. Flushing of the face and sweating are uncommon manifestations but the affected part of the face may be described as ‘puffy or swollen.’ Patients quickly recognise exacerbating factors. White and Sweet [ 111 mention the reclining position as being ‘intolerable’ and suggest that this is due to further engorgement of head vessels. Thus it is that during attacks patients tend to rise from their beds and restlessly pace their bedrooms. Alcohol and heat may induce an attack during the period of vulnerability and tend to exacerbate the pain once produced.
Clinical material Ten patients with a diagnosis of periodic ~gr~nous neuralgia have been treated by medical, minor surgical and major surgical methods. The results are presented with long-term follow-up. The male/female ratio was l/l with an age range of 20-65 years at time of presentation; all social classes were represented. Details of presentation and treatment are given chronologically.
This 35-year-old man developed severe right facial pain which iasted for 3 weeks. The pain was throbbing in character and associated with marked conjunctival injection and lacrimation. The symptoms started at night and continued until the following day. Then followed an 11 month pain-free period after which the pain recurred. This episode lasted for 2 weeks. Eleven months later a further episode of pain associated with autonomic features occurred and at this stage the patient was commenced on ergotamine. The patient remained pain-free for 18 months whilst on ergotamine but subsequently both pain and autonomic features returned despite therapy (Table I).
341
TABLE
I
RESULTS
OF TREATMENT
IN 10 CASES OF PERIODIC
Key: -, not attempted; 0, no pain relief; + + + , long-term complete pain relief. Case no.
Ergotamine
Peripheral nerve block
1
++
_
2 3 4
Side effects 0 0
_ _
5 6 7 8 9 10
Side effects ++ +++ ++ _ ++
0 _ _ _ -
+, partial
MIGRAINOUS
pain relief;
NEURALGIA
+ +. temporary
complete
pain relief:
Trigeminal ganglion block
Percutaneous rhizotomy
Trigeminal rhizotomy
Petrosal nerve section
_
_
_
_
+++ _
_
_ _
_
0 -
_
_
_
+++
_
_
+
+
_
_
+ -
_
+++ _
0
0
_
+++
0 0 -
+++
+
Case 2 A 20-year-old man developed severe left frontal pain associated with left-sided nasal obstruction, conjunctival injection and lacrimation. The pain continued at intervals of 3-4 months and lasted for 3-4 weeks. Recurrent attacks of pain occurred each night. The patient was commenced on ergotamine but the early development of established side effects prevented continuation of treatment. Both pain and autonomic features were still present after a 12 year follow-up (Table I). Case 3 A 29-year-old woman developed pain in the right side of her face. The pain was throbbing in character in the right cheek and forehead and was associated with right-sided lacrimation and nasal obstruction. The pain occurred in the early morning and lasted for l-2 h. Each episode lasted 6-7 weeks separated by pain-free periods of approximately 3 months. This pattern continued over 20 years. Initially the patient was treated by ergotamine but the patient’s symptoms remained unaffected. Subsequently, a percutaneous right trigeminal ganglion block was performed with alcohol. This produced hypoaesthesia in all 3 divisions of the trigeminal nerve but there was no pain relief. This demonstrates the phenomenon that the pain of period migrainous neuralgia may continue in areas of the face rendered hypoaesthetic or anaesthetic. Six months later a temporal craniotomy with right trigeminal rhizotomy and cervical sympathectomy was performed. Twenty-five years after surgery the patient has required no further treatment (Table I). Case 4 A 38-year-old acter, associated
man developed with right-sided
severe right-sided facial pain, throbbing in charlacrimation and rhinorrhoea and flushing of the
348
right cheek. The pain was in the right fronto-temporal region occurring in the early morning and lasting for a few hours. This occurred for 2-3 weeks alternating with pain-free periods of similar length. Initially the patient was treated with ergotamine but this failed to relieve the patient’s symptoms. Later, percutaneous injection of the right trigeminal ganglion with alcohol produced anaesthesia in the third division of the trigeminal nerve to both light touch and pin prick and a reduction in the sensation in the first and second divisions of the trigeminal nerve. There was, however, no pain relief. A middle fossa craniectomy and division of the right trigeminal nerve were therefore performed later during the same admission. Post-operative testing of trigeminal sensation. however, demonstrated that there had been no significant change. Additionally, the patient’s pain was not relieved. Eighteen months later a re-exploration of the trigeminal nerve was performed via a posterior fossa approach. residual trigeminal sensory root fibres were identified and divided at the cavum trigeminae. This procedure was followed by total anaesthesia in all 3 divisions of the trigeminal nerve but unfortunately no pain relief. The patient was then lost to follow-up (Table I).
A 34-year-old man developed herpes zoster in the first division of the trigeminal nerve. This was followed by right-sided facial pain and sweating. Two years later, after a blow to the right frontal region, the pain became much more severe. The pain was in the right frontal region and associated with constriction of the right pupil and a partial ptosis. It was also accompanied by flushing and sweating in the right frontal region and exacerbated by ingestion of alcohol. The patient was commenced on ergotamine treatment but the development of early side effects prevented continuation of treatment. Six months later a right supraorbital and supratrochlear nerve block with alcohol produced anaesthesia but no pain relief. A right temporal fossa exploration with section of the greater and lesser superficial petrosal nerves was performed followed by a 20 year period of pain relief with no autonomic features (Table I). clue 6 A 63-year-old woman developed sudden onset of severe right periorbital pain associated with lacrimation and right-sided rhinorrhoea. The pain occurred early morning, lasting for approximately 1 h and the attacks occurred at 2 weekly intervals. Long-term ergotamine treatment was instituted. This produced a 6 year However, when the pain recurred a period when the patient remained pain-free. temporal fossa exploration and section of the greater and lesser superficial petrosal nerves was performed. One year later, when last reviewed, she remained pain-free and subsequently was lost to follow-up (Table 1).
A 65-year-old man developed left frontal pain, associated with left-sided rhinorrhoea, facial sweating
orbital and temporal pain and flushing. The pain was
349
described as being similar to ‘toothache’ and predo~nantly occurred during the night, The pain tended to occur for 2-7 consecutive nights each episode lasting for approximately 2 h. Between episodes there were generally prolonged pain-free episodes lasting up to 3 months. Ergotamine treatment was commenced and this produced pain relief. However, it was necessary for the patient to take the ergotamine during each night and this produced troublesome sleep disturbance. One year later a temporal fossa exploration and section of both greater and lesser superficial petrosal nerves was attempted. This was unsuccessful and no nerve tissue was demonstrated in the resected specimen. Re-exploration was therefore performed a few weeks later, the petrosal nerves were now identified and sectioned. However, due to equivocal responses of the patient to stimulation of the cut nerves the ipsilateral trigeminal nerve was also sectioned. This produced anaesthesia in the second and third divisions of the trigeminal nerve and a 1 month period of complete pain relief. After this period the pain recurred but was now well controlled by occasional medical treatment for a 15 year follow-up period (Table I). A 5%year-old woman developed recurrent severe pain in the left upper gum radiating across the face towards the left eye and ear lasting approximately 1 h and associated with facial flushing. This was treated successfully by ergotamine. The patient remained pain-free for 4 years after which side effects of the therapy occurred. In addition, further autonomic features developed including ipsilateral nasal obstruction and lacrimation. A temporal fossa exploration with division of the left greater superficial petrosal nerve was performed. This abolished the autonomic features but the pain continued, although reported as less severe. Re-exploration was therefore performed 2 weeks later than the left trigeminal nerve was sectioned together with cervical sympathectomy. This produced appropriate anaesthesia and at last follow-up, some 12 years later, she had complete pain relief and no autonomic features (Table I). Case 9
A 33-year-old woman developed right orbital and periorbital pain together with right frontal pain which had a burning character and was associated with conjunctival injection and lacrimation. A percutaneous right trigeminai ganglion injection with alcohol was performed. This produced partial anaesthesia in the first and second divisions of the trigeminal nerve to light touch and pin prick and was associated with a reduction in intensity of the facial pain. Her pain was of sufficient intensity that a trial of ergotamine was undertaken but this produced no further pain relief. However, due to persistent symptoms (although reduced in intensity) 10 years later a temporal fossa exploration with division of the greater and lesser superficial petrosal nerves was performed. This failed to produce any further p&n relief (Table I). Case 10
This 65-year-old lady developed severe left periorbital pain radiating down the left side of the nose. The pain lasted 3-4 h occurring every night and often waking
her from sleep. It was associated with left-sided nasal obstruction, rhinorrhoea and lacrimation. She described a sensation of the face feeling ‘puffy’ during episodes of pain. The pain was made worse by ingestion of alcohol. Initially she was treated with a combination of methylsergide and lithium with partial pain relief for 1 year. Subsequently she received inter~ttent courses of methylser~de with some pain relief but with unaltered autonomic symptoms. However, despite medical treatment she complained of increasingly severe pain for the next 18 months. She was therefore treated by a percutaneous radiofrequency rhizotomy to the left trigeminal nerve. This caused partial anaesthesia in the maxillary division of the trigeminal nerve but produced no pain relief. Her autonomic features also persisted. One month later a left temporal craniectomy and division of greater and lesser superficial petrosal nerves were performed. This produced complete pain relief with cessation of her autonomic features. After 1 year follow-up she remains pain-free with no autonomic features.
Division
of the petrosal nerves -
surgical technique
Greater and lesser superficial petrosal nerve section may be performed by temporal fossa exploration under general anaesthesia. In a properly prepared and positioned patient a temporal craniectomy is performed and the temporal lobe gently retracted extradurally. The greater and lesser superficial petrosal nerves may be identified with the aid of the operating microscope. These nerves emerge from the anterior aspect of the petrous bone and pass across the floor of the middle cranial fossa towards foramina lacerum and ovale respectively. The patient may then be woken from anaesthesia and the nerves stimulated in turn using a direct current at 50 c/set, 2 msec duration and 5 V square wave pulses applied via bipolar electrodes. In cases where it can be established that stimulation of these nerves accurately reproduces the patient’s pain symptoms (with or without autonomic features) the nerves are divided after the patient has been re-anaesthetized. The wound is then closed in a conventional way. The results have demonstrated this to be a safe and often effective procedure for periodic migrainous neuralgia.
Discussion The vaso-dilatory phenomena accompanying this syndrome are often marked. An attack can be provoked with a subcutaneous injection of histamine [4] and many patients obtain relief from ergotamine [8]. Cobb and Finesinger [l] showed that the greater superficial petrosal nerves carried the afferent vase-~lato~ fibres to the brain’s pia mater and also secreto-motor and vaso-dilator fibres to the lacrimal gland, nasal mucosa, pharynx, palate and upper lip. Reviewing this evidence Gardner et al. [3] thought that periodic parasym-
351
pathetic discharges along one greater superficial petrosal nerve might account for the pain. Their results in dividing this nerve in 13 patients were excellent in 25%, fair to good in 50% and failed in 25%. In some cases the cut fibres were assumed to regenerate since lacrimation and pain recurred and both pain relief and a dry eye were obtained by cutting the nerve again. In the operative failures they suggested that other vaso-dilator pathways peripheral to the point of section or in a separate pathway resulted in continuing parasympathetic discharge and continuing pain. Although the operation described appeared reasonably effective, there was no certainty that the afferent pathway lay in the greater superficial petrosal nerve alone for both the middle meningeal artery and the lesser superficial petrosal nerve were also divided. A recurrence rate after section of petrosal nerves of up to 53.6% has been reported in other series [lo] but our experience differs with these results. White and Sweet [ll] undertook other studies by electrical stimulation of the suspected pathways under local anaesthesia, reporting their results in 6 patients. In 4 out of 5 patients stimulation of the greater superficial petrosal nerve produced pain locahsed to ear, eye or adjoining parts of the head and face. Because this pain could only be produced by stimulation of the central end of the cut nerve they concluded that the pain was related to afferents in the nerve rather than a response of other pathways to vaso-dilation induced by the afferent fibres. In 2 patients, however, stimulation of the middle meningeal artery also reproduced the pain. The evidence from this small series of patients suggests that more than one pain pathway may be involved although the autonomic phenomena are clearly related to parasympathetic discharges along the petrosal nerves. The pain experienced in this syndrome depends partly upon the release into tissues of bradykinin and serotonin-like substance around the dilated arteries and it is tempting to suggest that as a result of parasympathetic impulses and vaso-dilatation a similar substance is produced in the affected areas, pain then passing along either petrosal or trigeminal afferent pathways 191. Treatment modalities for periodic migranous neuralgia include medical (ergotamine, methylsergide, lithium) [6] semi-invasive surgical techniques or major surgery. The clinical material reported here illustrates that in cases of failed medical treatment or when side effects prohibit the use of these drugs, surgery is indicated. A first-stage trigeminal nerve rhizotomy with or without sphenopalative ganglion block using alcohol [S] or cryosurgical techniques [2] may be effective. If this fails temporal fossa craniectomy/craniotomy and greater and lesser superficial petrosal nerve section may prove to be worthwhile.
We are grateful to Veronica Turner for typing the manuscript and to past colleagues at the Department of Surgical Neurology, University of Edinburgh, for allowing us to record some of their cases.
352
References i Cobb, S. and Finesinger, J.E., Cerebral circulation, Arch. Neural. Psych&. (Chic.). 2X (1932) 1243-1256. 2 Cook, N., Cryosurgery of migraine, Headache, 12 (1973) 143- 150. 3 Gardner, W.J.. Stowell. A. and Dutlinger R.. Resection of the greater superficial petrosal nerve in the treatment of unilateral headache. .I. Neurosurg., 4 (1947) 105-I 14. 4 Horton, B.T., Histamine cephalgia, Lancet, i (1952) 92. 5 Kittrelle, J.P.. Grouze, D.S. and Seybold. M.E., Cluster headache: local anaesthetic abortive agents, Arch. Neural. (Chic.). 42 (1985) 496498. 6 Kudrow, L.. Cluster Headache, Oxford University Press. London, 19X0, pp. 125-140. 7 Merskey, H. (Ed.), Classification of chronic pain. Pain. Suppi. 3 (1986) S74. 8 Saxena. P.R.. The effect of antimigraine drugs on the vascular responses of 5hydroxytryptamine and related hiogenic substances on the external carotid bed of dogs: possible pharmacological implications to their antimigraine action, Headache, 12 (1972) 44. 9 Sicuteti. F., Geppetti. P., Marabini, S. and Lembeck. F.. Pain relief by somatostatin in attacks of cluster headache, Pain. 18 (1984) 3599365. 10 Stowell, A.. Physiologic mechanisms and treatment of histamine in petrosal neuralgia, Headache. 9 (1970) 187-194. 11 White J.C. and Sweet. W.H.. Pain and the Neurosurgeon. Thomas. Springfield, IL. 1969. pp. 345-372.
Pain, 31 (1987) 345-352 Elsevier PAI 01143
A review of treatment modalities for periodic migrainous neuralgia Mark Wake and Edward Hitchcock of3~rrningh~~
University
L)epartment of Neurosurgery,
Midland Centre for Neurosurgery Ho&y Lane, Bjrming~am (U.K..)
und Neuroiog?t,
(Received 4 November 1986, revised received 7 July 1987, accepted I2 August 1987)
Ten cases of periodic migrainous neuralgia are reported. Clinical details of the cases Summary including presentation and clinical course are typical of this condition. Treatment of these 10 cases encompasses medical, minor surgical and major surgical procedures. The results demonstrate that if medical tr~tm~t fails or there are severe drug side effects, trigeminal rhizotomy may be effective in producing iong-term pain relief. If pain recurs or trigeminal rhizotomy is ineffective, section of the greater and lesser superficial petrosal nerves should be considered. Key words: Periodic migrainous neuralgia; Trigeminal rhizotomy; Petrosal nerves
Introduction Definition
Periodic migrainous neuralgia is a condition consisting of ‘unilateral pain principally in the ocular, frontal and temporal areas recurring in separate bouts with daily attacks for several months usually with rhinorrhoea or lacrimation’ [7]. A syndrome of facial pain accompa~ed by facial autonomic disturbance is known by a variety of names (red migraine, sphenopalatine neuralgia, erythroprosopalgia, sympathetic hemicrania, migrainous (cilia@ neuralgia, atypical neuralgia with sympathetic phenomena, carotodynia, vidian nerve neuralgia, autonomic facie-cephalgia, histamine cephalgia, petrosal neuralgia, painful vascular disorder of the head, facial migrainous neuralgia, trigeminal migraine, cluster headache and periodic migrainous neuralgia). The condition deserves distinction
Correspondence to: Prof. E. Hitchcock, Midland Centre for Neurosurgery and Neurology, Holly Lane, Smethwick, WarIey, West Midlands B67 7JX, U.K.
0304-3959/87/$03.50 0 1987 Elsevier Science Publishers B.V. (Biomedical Division)
3&i
from other atypical neuralgias because of the prominent autonomic disorders accompanying the pain and because for this group a relatively simple operation may procure complete relief, The disease is characterised by a remittent course recurring at intervals of months or years and lasting for days, weeks or months. The attacks of pain occur less frequently than in trigeminal neuralgia and there are no ‘trigger spots.’ It is often remarkable for the extraordinary regularity of its onset which is often in the early hours of the morning, some 3 or 4 h after the patient has retired to bed. The pain is throbbing, burning, aching or lancinating and is usually experienced in the upper half of the face. commonly affecting the eye. It is often associated with pain in the temporal region. cheek or neck but remains strictly unilateral. The associated autonomic disturbances are often striking. Ptosis or hyperhidrosis on the affected side have also been noticed but many of the autonomic manifestations may be attributed to parasympathetic overactivity. The complete autonomic disturbance comprises conjunctival injection, lacrimation and mucous discharge or blockage of the nose, again all strictly unilateral. Flushing of the face and sweating are uncommon manifestations but the affected part of the face may be described as ‘puffy or swollen.’ Patients quickly recognise exacerbating factors. White and Sweet [ 111 mention the reclining position as being ‘intolerable’ and suggest that this is due to further engorgement of head vessels. Thus it is that during attacks patients tend to rise from their beds and restlessly pace their bedrooms. Alcohol and heat may induce an attack during the period of vulnerability and tend to exacerbate the pain once produced.
Clinical material Ten patients with a diagnosis of periodic ~gr~nous neuralgia have been treated by medical, minor surgical and major surgical methods. The results are presented with long-term follow-up. The male/female ratio was l/l with an age range of 20-65 years at time of presentation; all social classes were represented. Details of presentation and treatment are given chronologically.
This 35-year-old man developed severe right facial pain which iasted for 3 weeks. The pain was throbbing in character and associated with marked conjunctival injection and lacrimation. The symptoms started at night and continued until the following day. Then followed an 11 month pain-free period after which the pain recurred. This episode lasted for 2 weeks. Eleven months later a further episode of pain associated with autonomic features occurred and at this stage the patient was commenced on ergotamine. The patient remained pain-free for 18 months whilst on ergotamine but subsequently both pain and autonomic features returned despite therapy (Table I).
341
TABLE
I
RESULTS
OF TREATMENT
IN 10 CASES OF PERIODIC
Key: -, not attempted; 0, no pain relief; + + + , long-term complete pain relief. Case no.
Ergotamine
Peripheral nerve block
1
++
_
2 3 4
Side effects 0 0
_ _
5 6 7 8 9 10
Side effects ++ +++ ++ _ ++
0 _ _ _ -
+, partial
MIGRAINOUS
pain relief;
NEURALGIA
+ +. temporary
complete
pain relief:
Trigeminal ganglion block
Percutaneous rhizotomy
Trigeminal rhizotomy
Petrosal nerve section
_
_
_
_
+++ _
_
_ _
_
0 -
_
_
_
+++
_
_
+
+
_
_
+ -
_
+++ _
0
0
_
+++
0 0 -
+++
+
Case 2 A 20-year-old man developed severe left frontal pain associated with left-sided nasal obstruction, conjunctival injection and lacrimation. The pain continued at intervals of 3-4 months and lasted for 3-4 weeks. Recurrent attacks of pain occurred each night. The patient was commenced on ergotamine but the early development of established side effects prevented continuation of treatment. Both pain and autonomic features were still present after a 12 year follow-up (Table I). Case 3 A 29-year-old woman developed pain in the right side of her face. The pain was throbbing in character in the right cheek and forehead and was associated with right-sided lacrimation and nasal obstruction. The pain occurred in the early morning and lasted for l-2 h. Each episode lasted 6-7 weeks separated by pain-free periods of approximately 3 months. This pattern continued over 20 years. Initially the patient was treated by ergotamine but the patient’s symptoms remained unaffected. Subsequently, a percutaneous right trigeminal ganglion block was performed with alcohol. This produced hypoaesthesia in all 3 divisions of the trigeminal nerve but there was no pain relief. This demonstrates the phenomenon that the pain of period migrainous neuralgia may continue in areas of the face rendered hypoaesthetic or anaesthetic. Six months later a temporal craniotomy with right trigeminal rhizotomy and cervical sympathectomy was performed. Twenty-five years after surgery the patient has required no further treatment (Table I). Case 4 A 38-year-old acter, associated
man developed with right-sided
severe right-sided facial pain, throbbing in charlacrimation and rhinorrhoea and flushing of the
348
right cheek. The pain was in the right fronto-temporal region occurring in the early morning and lasting for a few hours. This occurred for 2-3 weeks alternating with pain-free periods of similar length. Initially the patient was treated with ergotamine but this failed to relieve the patient’s symptoms. Later, percutaneous injection of the right trigeminal ganglion with alcohol produced anaesthesia in the third division of the trigeminal nerve to both light touch and pin prick and a reduction in the sensation in the first and second divisions of the trigeminal nerve. There was, however, no pain relief. A middle fossa craniectomy and division of the right trigeminal nerve were therefore performed later during the same admission. Post-operative testing of trigeminal sensation. however, demonstrated that there had been no significant change. Additionally, the patient’s pain was not relieved. Eighteen months later a re-exploration of the trigeminal nerve was performed via a posterior fossa approach. residual trigeminal sensory root fibres were identified and divided at the cavum trigeminae. This procedure was followed by total anaesthesia in all 3 divisions of the trigeminal nerve but unfortunately no pain relief. The patient was then lost to follow-up (Table I).
A 34-year-old man developed herpes zoster in the first division of the trigeminal nerve. This was followed by right-sided facial pain and sweating. Two years later, after a blow to the right frontal region, the pain became much more severe. The pain was in the right frontal region and associated with constriction of the right pupil and a partial ptosis. It was also accompanied by flushing and sweating in the right frontal region and exacerbated by ingestion of alcohol. The patient was commenced on ergotamine treatment but the development of early side effects prevented continuation of treatment. Six months later a right supraorbital and supratrochlear nerve block with alcohol produced anaesthesia but no pain relief. A right temporal fossa exploration with section of the greater and lesser superficial petrosal nerves was performed followed by a 20 year period of pain relief with no autonomic features (Table I). clue 6 A 63-year-old woman developed sudden onset of severe right periorbital pain associated with lacrimation and right-sided rhinorrhoea. The pain occurred early morning, lasting for approximately 1 h and the attacks occurred at 2 weekly intervals. Long-term ergotamine treatment was instituted. This produced a 6 year However, when the pain recurred a period when the patient remained pain-free. temporal fossa exploration and section of the greater and lesser superficial petrosal nerves was performed. One year later, when last reviewed, she remained pain-free and subsequently was lost to follow-up (Table 1).
A 65-year-old man developed left frontal pain, associated with left-sided rhinorrhoea, facial sweating
orbital and temporal pain and flushing. The pain was
349
described as being similar to ‘toothache’ and predo~nantly occurred during the night, The pain tended to occur for 2-7 consecutive nights each episode lasting for approximately 2 h. Between episodes there were generally prolonged pain-free episodes lasting up to 3 months. Ergotamine treatment was commenced and this produced pain relief. However, it was necessary for the patient to take the ergotamine during each night and this produced troublesome sleep disturbance. One year later a temporal fossa exploration and section of both greater and lesser superficial petrosal nerves was attempted. This was unsuccessful and no nerve tissue was demonstrated in the resected specimen. Re-exploration was therefore performed a few weeks later, the petrosal nerves were now identified and sectioned. However, due to equivocal responses of the patient to stimulation of the cut nerves the ipsilateral trigeminal nerve was also sectioned. This produced anaesthesia in the second and third divisions of the trigeminal nerve and a 1 month period of complete pain relief. After this period the pain recurred but was now well controlled by occasional medical treatment for a 15 year follow-up period (Table I). A 5%year-old woman developed recurrent severe pain in the left upper gum radiating across the face towards the left eye and ear lasting approximately 1 h and associated with facial flushing. This was treated successfully by ergotamine. The patient remained pain-free for 4 years after which side effects of the therapy occurred. In addition, further autonomic features developed including ipsilateral nasal obstruction and lacrimation. A temporal fossa exploration with division of the left greater superficial petrosal nerve was performed. This abolished the autonomic features but the pain continued, although reported as less severe. Re-exploration was therefore performed 2 weeks later than the left trigeminal nerve was sectioned together with cervical sympathectomy. This produced appropriate anaesthesia and at last follow-up, some 12 years later, she had complete pain relief and no autonomic features (Table I). Case 9
A 33-year-old woman developed right orbital and periorbital pain together with right frontal pain which had a burning character and was associated with conjunctival injection and lacrimation. A percutaneous right trigeminai ganglion injection with alcohol was performed. This produced partial anaesthesia in the first and second divisions of the trigeminal nerve to light touch and pin prick and was associated with a reduction in intensity of the facial pain. Her pain was of sufficient intensity that a trial of ergotamine was undertaken but this produced no further pain relief. However, due to persistent symptoms (although reduced in intensity) 10 years later a temporal fossa exploration with division of the greater and lesser superficial petrosal nerves was performed. This failed to produce any further p&n relief (Table I). Case 10
This 65-year-old lady developed severe left periorbital pain radiating down the left side of the nose. The pain lasted 3-4 h occurring every night and often waking
her from sleep. It was associated with left-sided nasal obstruction, rhinorrhoea and lacrimation. She described a sensation of the face feeling ‘puffy’ during episodes of pain. The pain was made worse by ingestion of alcohol. Initially she was treated with a combination of methylsergide and lithium with partial pain relief for 1 year. Subsequently she received inter~ttent courses of methylser~de with some pain relief but with unaltered autonomic symptoms. However, despite medical treatment she complained of increasingly severe pain for the next 18 months. She was therefore treated by a percutaneous radiofrequency rhizotomy to the left trigeminal nerve. This caused partial anaesthesia in the maxillary division of the trigeminal nerve but produced no pain relief. Her autonomic features also persisted. One month later a left temporal craniectomy and division of greater and lesser superficial petrosal nerves were performed. This produced complete pain relief with cessation of her autonomic features. After 1 year follow-up she remains pain-free with no autonomic features.
Division
of the petrosal nerves -
surgical technique
Greater and lesser superficial petrosal nerve section may be performed by temporal fossa exploration under general anaesthesia. In a properly prepared and positioned patient a temporal craniectomy is performed and the temporal lobe gently retracted extradurally. The greater and lesser superficial petrosal nerves may be identified with the aid of the operating microscope. These nerves emerge from the anterior aspect of the petrous bone and pass across the floor of the middle cranial fossa towards foramina lacerum and ovale respectively. The patient may then be woken from anaesthesia and the nerves stimulated in turn using a direct current at 50 c/set, 2 msec duration and 5 V square wave pulses applied via bipolar electrodes. In cases where it can be established that stimulation of these nerves accurately reproduces the patient’s pain symptoms (with or without autonomic features) the nerves are divided after the patient has been re-anaesthetized. The wound is then closed in a conventional way. The results have demonstrated this to be a safe and often effective procedure for periodic migrainous neuralgia.
Discussion The vaso-dilatory phenomena accompanying this syndrome are often marked. An attack can be provoked with a subcutaneous injection of histamine [4] and many patients obtain relief from ergotamine [8]. Cobb and Finesinger [l] showed that the greater superficial petrosal nerves carried the afferent vase-~lato~ fibres to the brain’s pia mater and also secreto-motor and vaso-dilator fibres to the lacrimal gland, nasal mucosa, pharynx, palate and upper lip. Reviewing this evidence Gardner et al. [3] thought that periodic parasym-
351
pathetic discharges along one greater superficial petrosal nerve might account for the pain. Their results in dividing this nerve in 13 patients were excellent in 25%, fair to good in 50% and failed in 25%. In some cases the cut fibres were assumed to regenerate since lacrimation and pain recurred and both pain relief and a dry eye were obtained by cutting the nerve again. In the operative failures they suggested that other vaso-dilator pathways peripheral to the point of section or in a separate pathway resulted in continuing parasympathetic discharge and continuing pain. Although the operation described appeared reasonably effective, there was no certainty that the afferent pathway lay in the greater superficial petrosal nerve alone for both the middle meningeal artery and the lesser superficial petrosal nerve were also divided. A recurrence rate after section of petrosal nerves of up to 53.6% has been reported in other series [lo] but our experience differs with these results. White and Sweet [ll] undertook other studies by electrical stimulation of the suspected pathways under local anaesthesia, reporting their results in 6 patients. In 4 out of 5 patients stimulation of the greater superficial petrosal nerve produced pain locahsed to ear, eye or adjoining parts of the head and face. Because this pain could only be produced by stimulation of the central end of the cut nerve they concluded that the pain was related to afferents in the nerve rather than a response of other pathways to vaso-dilation induced by the afferent fibres. In 2 patients, however, stimulation of the middle meningeal artery also reproduced the pain. The evidence from this small series of patients suggests that more than one pain pathway may be involved although the autonomic phenomena are clearly related to parasympathetic discharges along the petrosal nerves. The pain experienced in this syndrome depends partly upon the release into tissues of bradykinin and serotonin-like substance around the dilated arteries and it is tempting to suggest that as a result of parasympathetic impulses and vaso-dilatation a similar substance is produced in the affected areas, pain then passing along either petrosal or trigeminal afferent pathways 191. Treatment modalities for periodic migranous neuralgia include medical (ergotamine, methylsergide, lithium) [6] semi-invasive surgical techniques or major surgery. The clinical material reported here illustrates that in cases of failed medical treatment or when side effects prohibit the use of these drugs, surgery is indicated. A first-stage trigeminal nerve rhizotomy with or without sphenopalative ganglion block using alcohol [S] or cryosurgical techniques [2] may be effective. If this fails temporal fossa craniectomy/craniotomy and greater and lesser superficial petrosal nerve section may prove to be worthwhile.
We are grateful to Veronica Turner for typing the manuscript and to past colleagues at the Department of Surgical Neurology, University of Edinburgh, for allowing us to record some of their cases.
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