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Trigeminal neuralgia and multiple sclerosis
Trigeminal neuralgia and multiple sclerosis A c o m p l e x diagnosis David Gale, BDS, FDSRCS (Eng), a Stephen Prime, PhD, FDSRCPS, MRCPath, b, and M.J. Campbell, MBBS, FRCP, c Bristol, U.K. BRISTOL DENTAL HOSPITAL, UNIVERSITY OF BRISTOL, AND FRENCHAY HOSPITAL
Trigeminal neuralgia is a well-recognized complication in patients with multiple sclerosis. A case report is presented that describes multiple sclerosis in a middle-aged man with otherwise classical unilateral trigeminal neuralgia who demonstrated a variable response to pharmacologic and surgical intervention. The case highlights the difficulties of diagnosis when trigeminal neuralgia occurs concurrently with multiple sclerosis. (ORAl,SURGORAl,Mm ORAl,PATHOI,ORAl,RADIOI,ENDOD1995;79:398-401)
Trigeminal neuralgia is a debilitating disorder that typically occurs in middle to old age with a slight female predominance. The disease is commonly idiopathic although it is thought that the most likely pathogenesis involves demyelinization of the retrogasserian ganglionic fibers just before entry into the pons, with or without associated localized compression.l, 2 Classically, the pain is experienced within and is limited to the anatomic distribution of one or more divisions of the trigeminal nerve. The pain is paroxysmal, very severe, and has been described as a sharp shooting electric shock with pain-free remissions between attacks. Eating, washing, and talking can act on trigger zones to precipitate the pain. Only 3% to 10% of cases are bilateral. The condition is usually treated with carbamazepine or other anticonvulsants and drugs,3, 4 but surgical management including cryotherapy, alcohol injections, thermocoagulation, neurectomy, radiofrequency, and intracranial microvascular decompression have also been used. 5 Some 2% to 7% of patients with trigeminal neuralgia also have multiple sclerosis. 6-s Multiple sclerosis is characterized by an idiopathic demyelination in the central nervous system (CNS) and both environmental and genetic ( H L A DR2) factors are thought to predispose to the condition. Demyelination within the trigeminal nerve pathways is thought to be responsible for the neuralgia, which can be indistinguishable from classical idiopathic trigeminal neuralgia, 9 although local sensory impairment may be present in some cases. Multiple sclerosis is more common in females (male to female ratio is 2:3) and commonly apaSenior House Officer,Bristol Dental Hospital. bprofessor,Divisionof Oral Medicine,Pathology,and Microbiology, Universityof Bristol. cConsultant Neurologist, Department of Neurology, Frenchay Hospital. Copyright | 1995 by Mosby-Year Book, Inc. 1079-2104/95/$3.00 + 0 7/17/60623
398
pears in the third and fourth decade. Diagnosis of multiple sclerosis is made predominantly on clinical grounds, and commonly there is an extended history of multiple neurologic symptoms such as visual disturbances (optic neuritis, field defects, and diplopia) and brain stem or spinal cord dysfunction (paraesthesia, sensory loss, ataxia, motor weakness). The clinical diagnosis may be supported by neurophysiologic conduction tests, magnetic resonance imaging (MRI) and computed tomography imaging, 1,2 and biochemical assays of the cerebrospinal fluid (for example, globulin levels).9 Although there is no curative treatment for multiple sclerosis, systemic steroids are commonly used to control acute relapses, and other drugs are available to control certain symptoms, such as, spasticity or neurogenic bladder dysfunction. A case is reported of classical trigeminal neuralgia in which multiple sclerosis appeared to be a late development and that raises questions regarding the diagnosis of both multiple sclerosis and trigeminal neuralgia.
CASE REPORT A 43-year-old white man came to the Department of Oral Medicine with a 2-week history of intense pain of short durations in the region of the left maxilla and mandible corresponding to the distribution of the maxillary and mandibular divisions of the trigeminal nerve. The pain was described as an electric shock and was associated with a burning sensation in this region. There was no organic orofacial pathologic reason for these symptoms, and a provisional diagnosis of trigeminal neuralgia was made. Baseline hematology was essentially normal although there was a macrocytosis (mean corpuscular volume = 100 femtolitres) and raised plasma liver enzymes (gamma-glutamyl transferase = 122 units/l), consistent with an increased alcohol intake. The patient was given 300 mg carbamazepine daily. Subsequent breakthrough pain forced a carbamazepine increase to 600 mg daily, although this was not tolerated well and resulted in marked drowsiness. The carbamazepine, however, was maintained at 600 mg daily and was supple-
ORAL SURGERY ORAL MEDICINEORAL PATHOLOGY
Gale, Prime, and Campbell
399
Volume 79, Number 3
Fig. 1. Axial section through brain stem (T2W image) shows high intensity lesion (arrow) in left sensory root entry zone consistent with demyelination.
Fig. 2. Lateral view of brain in midline (T2W image) shows pericollosal high intensity lesion (arrow) typical of demyelination, that is, multiple sclerosis.
mented with 200 mg phenytoin. Three days later the patient returned in considerable distress and extreme pain. A local anesthetic block relieved the left mental neuralgia and, therefore, cryotherapy was performed at the mental foramen. This resulted in complete pain relief for some 20 months without medication. The patient returned with pain specifically associated with the lower left second molar tooth. Eating, exposure to
cold fluids, or touching the mucosa overlying the ascending ramus resulted in episodic explosions of pain in this region. The situation was somewhat complicated by the fact that the lower left second molar was hyperalgesic to ethyl chloride and that there was evidence of both lateral periodontal and periapical bone loss. Total analgesia was experienced from an inferior alveolar nerve block before the extraction of this tooth. After the local anesthetic effect dissipated,
400
Gale, Prime, and Campbell
however, a more classical, excruciating neuralgic-type pain was evident. The carbamazepine was restarted (400 mg daily), 'and cryosurgery was performed again to the left mental nerve. Limited success was achieved with the cryosurgery and a neurosurgical opinion was obtained in view of the recurrence pattern. Microvascular decompression of the left trigeminal nerve was carried out by vaporizing a left mesencephalic vein with a left mastoid exposure. Although there was a transient loss in the sense of balance and paraesthesia in the left mental region and the left auricular and preauricular regions, the patient remained painfree for almost 2 years. The patient returned after 2 years with classical trigeminal neuralgia relating to the left mental region. Further cryosurgery relieved these symptoms immediately, and the patient remained painfree for another 11 months without medication. After 11 months, the patient returned again complaining of pain associated with the left maxillary division of the trigeminal nerve. A bupivacaine block immediately controlled the pain, a carious upper left premolar tooth was extracted, and 400 mg carbamazepine daily was prescribed. A neurologic opinion was obtained and resulted in a diagnosis of multiple sclerosis. This diagnosis was made on the basis of a left optic neuritis (diagnosed privately some 10 years previously and not reported by the patient until this late stage) and the present situation of bilateral optic disk pallor, ataxic nystagmus, sensory loss of the maxillary and mandibular divisions of the trigeminal nerve, a depressed left corneal reflex, and some imbalance on turning. An MRI scan revealed periventricular demyelination classical of multiple sclerosis (Fig. 1) and a left pons lesion that corresponded exactly to the trigeminal nerve nuclei and sensory root entry zone (Fig. 2). The patient is currently being maintained on 400 mg carbamazepine daily and is painfree. There remains some dizziness on turning and residual facial paresthesia. DISCUSSION Trigeminal neuralgia is a well-recognized manifestation in patients with multiple sclerosis. 6-8 In those cases where multiple sclerosis is associated with trigeminal neuralgia, there appears to be an earlier age of onset (50 years as opposed to 63 years of age in classical trigeminal neuralgia), 8 the symptoms are commonly bilateral (31% as opposed to 3% to 10% of cases of classical trigeminal neuralgia), 6, 8 and the condition is frequently refractory to carbamazepine. 5 The patient reported in this case was 43 years of age, had unilateral trigeminal neuralgia, and had a variable response to carbamazepine. Furthermore, although microvascular decompression is normally contraindicated in cases of multiple sclerosis in association with trigeminal neuralgia, in the present case this surgical procedure resulted in pain relief for some 2 years. Interestingly, a recent case report also de-
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY March 1995
scribes multiple sclerosis occurring some 2 years after diagnosis of trigeminal neuralgia. 1~ The question remains as to whether multiple sclerosis was possibly a subsequent development after the trigeminal neuralgia diagnosis or, alternatively, whether trigeminal neuralgia symptoms occurred in an undiagnosed multiple sclerosis patient, u Figs. 1 and 2 show the magnetic resonance images of demyelination plaques from the present case, consistent with multiple sclerosis located in the nucleus and sensory root entry zone of the trigeminal nerve. The patient in this report revealed at a late stage that he had sought an ophthalmologic opinion for impaired vision in the left eye approximately 10 years before he first presented with trigeminal neuralgia and, at that time, he was diagnosed as having optic neuritis. Optic neuritis is commonly a precursor of multiple sclerosis, but a diagnosis of multiple sclerosis requires other clinical signs or abnormal investigations. 9 In the present situation, the clinical diagnosis of multiple sclerosis was predominantly made on the basis of ophthalmologic disturbances bilaterally and was supported by an abnormal M R I brain scan; this procedure was not available in 1977 when the optic neuritis was first diagnosed. Furthermore, the distinction between multiple sclerosis and idiopathic trigeminal neuralgia can be further complicated by the fact that carbamazepine can by itself cause nystagmus and diplopia. That multiple sclerosis developed after the initial trigeminal neuralgia diagnosis in the present case is supported by the fact that microvascular decompression was successful for some 2 years. This case study clearly indicates the advizability of a neurologic consultation 2, 5 in cases of uncontrolled, bilateral, and atypical trigeminal neuralgia. It is important to be aware of other symptoms that are indicative of multiple sclerosis such as retrobulbar neuritis, diplopia, ataxia, sensory disturbance, or motor weakness 9 and, hopefully, to exclude this debilitating disorder at an early stage. We thank Dr. J. W. Eveson for critical reading of the manuscript. REFERENCES
1. RosenkopfKL. Current conceptsconcerningthe aetiologyand treatment of trigeminal neuralgia. J Craniomandibular Pract 1989;7:312-8. 2. Darlow LA, Brooks ML, Quinn PD. Magnetic resonance imaging in the diagnosis of trigeminal neuralgia. J Oral Maxillofac Surg 1992;50:621-6. 3. Srisintorn S. Diagnosis of trigeminal neuralgia. Dent Update 1991;18:304. 4. Zakrzewska JM. Medical management of trigeminal neuralgia. Br Dent J 1990;168:399-401.
Gale, Prime, and Campbell
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY
401
Volume 79, Number 3 5. Zakrzewska JM. Surgical management of trigeminal neuralgia. Br Dent J 1991;170:61-2. 6: Friedman CE. Trigeminal neuralgia in a patient with multiple sclerosis. J Endodont 1989;15:379-80. 7. Linderoth B, Hakanson S. Paroxysmal facial pain in disseminated sclerosis treated by retrogasserian glycerol injection. Acta Neurol Scand 1989;80:341-6. 8. Brisman R. Trigeminal neuralgia and multiple sclerosis. Arch Neurol 1987;44:379-81. 9. Wilkinson IMS. Multiple sclerosis: essential neurology. Oxford: Blackwell Scientific, 1993:135-45. 10. Neilson K, Field EA. Trigeminal neuralgia: a cautionary tale. Br Dent J 1994;176:68-70.
11. Jensen TS, Rasmussen P, Reske-Nielson E. Association of trigeminal neuralgia with multiple sclerosis: clinical and pathological features. Acta Neurol Scand 1982;65:182-9.
Reprint requests: Professor S.S. Prime Division of Oral Medicine, Pathology, and Microbiology Bristol Dental Hospital Lower Maudlin Street Bristol B21 2LY
AVAILABILITY OF JOURNAL BACK ISSUES As a service to our subscribers, copies of back issues of ORAL SURGERY, ORAL MEDICINE, ORAL PATHOLOGY, ORAL RADIOLOGY,AND ENDODONTICS for the preceding 5 years are maintained and are available for purchase from the publisher, Mosby-Year Book, Inc., at a cost of $9.00 per issue. The following quantity discounts are available: 25% off on quantities of 12 to 23, and one third off on quantities of 24 or more. Please write to Mosby-Year Book, Inc., Subscription Services, 11830 Westline Industrial Drive, St. Louis, MO 63146-3318, or call (800)453-4351 or (314)453-4351 for information on availability of particular issues. If unavailable from the publisher, photocopies of complete issues are available from University Microfilms International, 300 N. Zeeb Rd., Ann Arbor, MI 48106 (313)761-4700.
Trigeminal neuralgia is a well-recognized complication in patients with multiple sclerosis. A case report is presented that describes multiple sclerosis in a middle-aged man with otherwise classical unilateral trigeminal neuralgia who demonstrated a variable response to pharmacologic and surgical intervention. The case highlights the difficulties of diagnosis when trigeminal neuralgia occurs concurrently with multiple sclerosis. (ORAl,SURGORAl,Mm ORAl,PATHOI,ORAl,RADIOI,ENDOD1995;79:398-401)
Trigeminal neuralgia is a debilitating disorder that typically occurs in middle to old age with a slight female predominance. The disease is commonly idiopathic although it is thought that the most likely pathogenesis involves demyelinization of the retrogasserian ganglionic fibers just before entry into the pons, with or without associated localized compression.l, 2 Classically, the pain is experienced within and is limited to the anatomic distribution of one or more divisions of the trigeminal nerve. The pain is paroxysmal, very severe, and has been described as a sharp shooting electric shock with pain-free remissions between attacks. Eating, washing, and talking can act on trigger zones to precipitate the pain. Only 3% to 10% of cases are bilateral. The condition is usually treated with carbamazepine or other anticonvulsants and drugs,3, 4 but surgical management including cryotherapy, alcohol injections, thermocoagulation, neurectomy, radiofrequency, and intracranial microvascular decompression have also been used. 5 Some 2% to 7% of patients with trigeminal neuralgia also have multiple sclerosis. 6-s Multiple sclerosis is characterized by an idiopathic demyelination in the central nervous system (CNS) and both environmental and genetic ( H L A DR2) factors are thought to predispose to the condition. Demyelination within the trigeminal nerve pathways is thought to be responsible for the neuralgia, which can be indistinguishable from classical idiopathic trigeminal neuralgia, 9 although local sensory impairment may be present in some cases. Multiple sclerosis is more common in females (male to female ratio is 2:3) and commonly apaSenior House Officer,Bristol Dental Hospital. bprofessor,Divisionof Oral Medicine,Pathology,and Microbiology, Universityof Bristol. cConsultant Neurologist, Department of Neurology, Frenchay Hospital. Copyright | 1995 by Mosby-Year Book, Inc. 1079-2104/95/$3.00 + 0 7/17/60623
398
pears in the third and fourth decade. Diagnosis of multiple sclerosis is made predominantly on clinical grounds, and commonly there is an extended history of multiple neurologic symptoms such as visual disturbances (optic neuritis, field defects, and diplopia) and brain stem or spinal cord dysfunction (paraesthesia, sensory loss, ataxia, motor weakness). The clinical diagnosis may be supported by neurophysiologic conduction tests, magnetic resonance imaging (MRI) and computed tomography imaging, 1,2 and biochemical assays of the cerebrospinal fluid (for example, globulin levels).9 Although there is no curative treatment for multiple sclerosis, systemic steroids are commonly used to control acute relapses, and other drugs are available to control certain symptoms, such as, spasticity or neurogenic bladder dysfunction. A case is reported of classical trigeminal neuralgia in which multiple sclerosis appeared to be a late development and that raises questions regarding the diagnosis of both multiple sclerosis and trigeminal neuralgia.
CASE REPORT A 43-year-old white man came to the Department of Oral Medicine with a 2-week history of intense pain of short durations in the region of the left maxilla and mandible corresponding to the distribution of the maxillary and mandibular divisions of the trigeminal nerve. The pain was described as an electric shock and was associated with a burning sensation in this region. There was no organic orofacial pathologic reason for these symptoms, and a provisional diagnosis of trigeminal neuralgia was made. Baseline hematology was essentially normal although there was a macrocytosis (mean corpuscular volume = 100 femtolitres) and raised plasma liver enzymes (gamma-glutamyl transferase = 122 units/l), consistent with an increased alcohol intake. The patient was given 300 mg carbamazepine daily. Subsequent breakthrough pain forced a carbamazepine increase to 600 mg daily, although this was not tolerated well and resulted in marked drowsiness. The carbamazepine, however, was maintained at 600 mg daily and was supple-
ORAL SURGERY ORAL MEDICINEORAL PATHOLOGY
Gale, Prime, and Campbell
399
Volume 79, Number 3
Fig. 1. Axial section through brain stem (T2W image) shows high intensity lesion (arrow) in left sensory root entry zone consistent with demyelination.
Fig. 2. Lateral view of brain in midline (T2W image) shows pericollosal high intensity lesion (arrow) typical of demyelination, that is, multiple sclerosis.
mented with 200 mg phenytoin. Three days later the patient returned in considerable distress and extreme pain. A local anesthetic block relieved the left mental neuralgia and, therefore, cryotherapy was performed at the mental foramen. This resulted in complete pain relief for some 20 months without medication. The patient returned with pain specifically associated with the lower left second molar tooth. Eating, exposure to
cold fluids, or touching the mucosa overlying the ascending ramus resulted in episodic explosions of pain in this region. The situation was somewhat complicated by the fact that the lower left second molar was hyperalgesic to ethyl chloride and that there was evidence of both lateral periodontal and periapical bone loss. Total analgesia was experienced from an inferior alveolar nerve block before the extraction of this tooth. After the local anesthetic effect dissipated,
400
Gale, Prime, and Campbell
however, a more classical, excruciating neuralgic-type pain was evident. The carbamazepine was restarted (400 mg daily), 'and cryosurgery was performed again to the left mental nerve. Limited success was achieved with the cryosurgery and a neurosurgical opinion was obtained in view of the recurrence pattern. Microvascular decompression of the left trigeminal nerve was carried out by vaporizing a left mesencephalic vein with a left mastoid exposure. Although there was a transient loss in the sense of balance and paraesthesia in the left mental region and the left auricular and preauricular regions, the patient remained painfree for almost 2 years. The patient returned after 2 years with classical trigeminal neuralgia relating to the left mental region. Further cryosurgery relieved these symptoms immediately, and the patient remained painfree for another 11 months without medication. After 11 months, the patient returned again complaining of pain associated with the left maxillary division of the trigeminal nerve. A bupivacaine block immediately controlled the pain, a carious upper left premolar tooth was extracted, and 400 mg carbamazepine daily was prescribed. A neurologic opinion was obtained and resulted in a diagnosis of multiple sclerosis. This diagnosis was made on the basis of a left optic neuritis (diagnosed privately some 10 years previously and not reported by the patient until this late stage) and the present situation of bilateral optic disk pallor, ataxic nystagmus, sensory loss of the maxillary and mandibular divisions of the trigeminal nerve, a depressed left corneal reflex, and some imbalance on turning. An MRI scan revealed periventricular demyelination classical of multiple sclerosis (Fig. 1) and a left pons lesion that corresponded exactly to the trigeminal nerve nuclei and sensory root entry zone (Fig. 2). The patient is currently being maintained on 400 mg carbamazepine daily and is painfree. There remains some dizziness on turning and residual facial paresthesia. DISCUSSION Trigeminal neuralgia is a well-recognized manifestation in patients with multiple sclerosis. 6-8 In those cases where multiple sclerosis is associated with trigeminal neuralgia, there appears to be an earlier age of onset (50 years as opposed to 63 years of age in classical trigeminal neuralgia), 8 the symptoms are commonly bilateral (31% as opposed to 3% to 10% of cases of classical trigeminal neuralgia), 6, 8 and the condition is frequently refractory to carbamazepine. 5 The patient reported in this case was 43 years of age, had unilateral trigeminal neuralgia, and had a variable response to carbamazepine. Furthermore, although microvascular decompression is normally contraindicated in cases of multiple sclerosis in association with trigeminal neuralgia, in the present case this surgical procedure resulted in pain relief for some 2 years. Interestingly, a recent case report also de-
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY March 1995
scribes multiple sclerosis occurring some 2 years after diagnosis of trigeminal neuralgia. 1~ The question remains as to whether multiple sclerosis was possibly a subsequent development after the trigeminal neuralgia diagnosis or, alternatively, whether trigeminal neuralgia symptoms occurred in an undiagnosed multiple sclerosis patient, u Figs. 1 and 2 show the magnetic resonance images of demyelination plaques from the present case, consistent with multiple sclerosis located in the nucleus and sensory root entry zone of the trigeminal nerve. The patient in this report revealed at a late stage that he had sought an ophthalmologic opinion for impaired vision in the left eye approximately 10 years before he first presented with trigeminal neuralgia and, at that time, he was diagnosed as having optic neuritis. Optic neuritis is commonly a precursor of multiple sclerosis, but a diagnosis of multiple sclerosis requires other clinical signs or abnormal investigations. 9 In the present situation, the clinical diagnosis of multiple sclerosis was predominantly made on the basis of ophthalmologic disturbances bilaterally and was supported by an abnormal M R I brain scan; this procedure was not available in 1977 when the optic neuritis was first diagnosed. Furthermore, the distinction between multiple sclerosis and idiopathic trigeminal neuralgia can be further complicated by the fact that carbamazepine can by itself cause nystagmus and diplopia. That multiple sclerosis developed after the initial trigeminal neuralgia diagnosis in the present case is supported by the fact that microvascular decompression was successful for some 2 years. This case study clearly indicates the advizability of a neurologic consultation 2, 5 in cases of uncontrolled, bilateral, and atypical trigeminal neuralgia. It is important to be aware of other symptoms that are indicative of multiple sclerosis such as retrobulbar neuritis, diplopia, ataxia, sensory disturbance, or motor weakness 9 and, hopefully, to exclude this debilitating disorder at an early stage. We thank Dr. J. W. Eveson for critical reading of the manuscript. REFERENCES
1. RosenkopfKL. Current conceptsconcerningthe aetiologyand treatment of trigeminal neuralgia. J Craniomandibular Pract 1989;7:312-8. 2. Darlow LA, Brooks ML, Quinn PD. Magnetic resonance imaging in the diagnosis of trigeminal neuralgia. J Oral Maxillofac Surg 1992;50:621-6. 3. Srisintorn S. Diagnosis of trigeminal neuralgia. Dent Update 1991;18:304. 4. Zakrzewska JM. Medical management of trigeminal neuralgia. Br Dent J 1990;168:399-401.
Gale, Prime, and Campbell
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY
401
Volume 79, Number 3 5. Zakrzewska JM. Surgical management of trigeminal neuralgia. Br Dent J 1991;170:61-2. 6: Friedman CE. Trigeminal neuralgia in a patient with multiple sclerosis. J Endodont 1989;15:379-80. 7. Linderoth B, Hakanson S. Paroxysmal facial pain in disseminated sclerosis treated by retrogasserian glycerol injection. Acta Neurol Scand 1989;80:341-6. 8. Brisman R. Trigeminal neuralgia and multiple sclerosis. Arch Neurol 1987;44:379-81. 9. Wilkinson IMS. Multiple sclerosis: essential neurology. Oxford: Blackwell Scientific, 1993:135-45. 10. Neilson K, Field EA. Trigeminal neuralgia: a cautionary tale. Br Dent J 1994;176:68-70.
11. Jensen TS, Rasmussen P, Reske-Nielson E. Association of trigeminal neuralgia with multiple sclerosis: clinical and pathological features. Acta Neurol Scand 1982;65:182-9.
Reprint requests: Professor S.S. Prime Division of Oral Medicine, Pathology, and Microbiology Bristol Dental Hospital Lower Maudlin Street Bristol B21 2LY
AVAILABILITY OF JOURNAL BACK ISSUES As a service to our subscribers, copies of back issues of ORAL SURGERY, ORAL MEDICINE, ORAL PATHOLOGY, ORAL RADIOLOGY,AND ENDODONTICS for the preceding 5 years are maintained and are available for purchase from the publisher, Mosby-Year Book, Inc., at a cost of $9.00 per issue. The following quantity discounts are available: 25% off on quantities of 12 to 23, and one third off on quantities of 24 or more. Please write to Mosby-Year Book, Inc., Subscription Services, 11830 Westline Industrial Drive, St. Louis, MO 63146-3318, or call (800)453-4351 or (314)453-4351 for information on availability of particular issues. If unavailable from the publisher, photocopies of complete issues are available from University Microfilms International, 300 N. Zeeb Rd., Ann Arbor, MI 48106 (313)761-4700.