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Meige syndrome: An unusual cause of involuntary facial movements
Meige syndrome: An unusual cause of involuntary facial movements Mark SCHOOL
R. Stevens, D.D.S., * and Mark OF MEDICINE.
MIAMI
E. Wang, B.D.S.,*
Miami,
Fla.
UNIVERSITY
Dental and allied health professionals are on occasion confronted with patients who exhibit abnormal facial movements. These patients may be seeking a diagnosis or may relate a specific problem resulting from the uncontrolled and involuntary orofacial movements. A complete description of the various conditions associated with abnormal facial movements is beyond the scope of this article. Instead. these authors present a case with dental symptoms that were masking a more serious underlying progressive neurologic disorder. Appropriate referral to the neurology service is essential so that treatment of the underlying cause may precede, rather than follow, empiric management of these patients’ symptoms. (ORAL SURC ORAL MED ORAL PATHOL 1988;66:427-9)
A bnormal, disordered, involuntary movements are classified as dyskinesias.l The relatively slow and rhythmic nature of these movements distinguishes them from the twisting and writhing pattern seen in chorea. When the affected muscles exhibit abnormal tone, the term dystonia is used. A syndrome comprising dystonias of the oral, facial, lingual, and cervical musculature in association with blepharospasm was first described by a French neurologist, Henri Meige, in 1910; he called this syndrome spasme facial median.2,3 An alternative eponym for this condition, proposed by Marsden, 4 is Brulghel’s syndrome. The uncommon nature of this affliction, combined with better documentation of similar dyskinesias and diseases of extrapyramidal origin, has lead to confusion and misdiagnosis. Jankovic and Ford,= in 1982, prospectively evaluated 100 patients with blepharospasm and orofacial-cervical dystonias, the largest series studied to date. They report that the syndrome was most frequently confused with functional disorders, including nervous habits, neurosis, myasthenia gravis, Parkinson’s disease, tardive dyskinesia, temporomandibular joint dysfunction syndrome, Huntington’s chorea, and facial tics. A contributing reason for the confusion that enshrouds this disease may be the incomplete presentation of symptoms at varying stages of the disease process. We hope that this article will offer a comprehensive account of the syndrome: its clinical features, factors that distin*Division
of Oral
and Maxillofacial
Surgery.
guish it from other facial dyskinesias, and treatment modalities currently being employed to aid those suffering from this condition. CASE
REPORT
A 49-year-old Oriental woman sought treatment for cheek biting and fracturing of anterior teeth. She was examined by several dentists and oral-maxillofacial surgeons for her complaints. The patient underwent a tkmporomandibular joint work-up that consisted of a comprehensive examination, tomograms, and splint and physical therapy with occlusal equilibrations; all of these procedures provided no explanation of or relief from her recurrent symptoms. Her complaints persisted, and she was placed in short-term intermaxillary fixation with guiding elastics in an attempt to control her paroxysmal parafunctional habit. Eventually the posterior second molars were removed because of the failure of multiple conservative treatments. Clinical examination revealed a pleasant, middle-aged Oriental woman in moderate psychological distress., She exhibited a small, involuntary, continual positional movement of her lower jaw in a protrusive direction. Masse&r muscles were hypertrophied bilaterally and moderately tender to palpation. Her dentition showed marked attrition, and incisors were shortened and sharp. The remainder of her clinical examination and the review of systems did not help explain her current condition. Her past medical history was significant only for the present illness of approximately 1 year’s duration and for intermittent treatment of exogenous depression caused by loss of her spouse 18 months earlier. The patient was subsequently referred to the Department of Neurology, where a diagnosis of Meige syndrome 427
428
Stevens and Wong
Oral Sutg October 1988
The pathophysiology of Meige syndrome is poorly understood. Certain clinical findings point to the possible organic nature of the disease. Computed tomographic scans of the head were abnormal in 34% of cases studied by Jankovic and Ford.2 The pathologic findings included evidence of cerebral atrophy and various lucencies. The relationship of Meige syndrome to other known dysfunctions of the basal ganglia and rostra1 brainstem (for instance, generalized dystonias, Parkinson’s disease, essential tremors, and Wilson’s disease) provides further circumstantial evidence of the possible organic origins. The effects of various cholinergic and dopaminergic agents on patients suffering from Meige syndrome offer yet another clue to the pathologic process involved. It has been found that, in general, the use of agents that deplete dopamine levels (eg, tetrabenazine) or decrease dopaminergic or cholinergic receptor activity (eg, lisuride, trihexylphenidyl) resulted in various degrees of relief from the symptoms. This finding would appear to implicate relative cholinergic hyperactivity or a central dopamine preponderance as responsible causes.2’5-7 However, failure to obtain consistent results with the pharmacologic agents used suggests that the total mechanism of the disease has yet to be elucidated.
The treatment modalities currently being employed comprise two categories: pharmacologic agents that are known to possess either dopaminergic or cholinergic activity and pharmacologic or surgical procedures designed to relieve the major symptoms of the disease. Individual patient’s responses to the various pharmacologic agents have been extremely variable, with different efficacies and varying periods of relief obtained. Jankovic and Ford report that 22% of their patients had a marked and lasting improvement (more than 3 months) of symptoms from at least one of the drugs employed. Sixty-nine percent of their patients had lesser responses. Agents that have been tried include tetrabenazine (a dopamine-depleting agent), lithium (believed to act by blocking dopamine receptor sensitivity),2 trihexylphenidyl (a choline agonist that may provide competitive inhibition of choline transport),6 and clonazepam (a serotonergic agent). Failure to control the disease process by one of these regimens has lead to direct attention to the most incapacitating manifestations. Blepharospasm, which in its extreme form may lead to functional blindness, has been managed by selective sectioning of the zygomatic and palpebral branches of the seventh nerve in combination with a brow lift.2 The direct injection of botulin into the musculature of the eyelids has also been reported useful in gaining temporary relief.4 The management of the dental aspects of the disease should conserve as much tissue as possible. Recognition of the disease state and its clinical course will aid the practitioner in arriving at a suitable timetable for treatment.
TREATMENT
DISCUSSION
was made. The patient was placed on a trial treatment of tetrabenazine that satisfactorily controlled her orofacial spasms. The patient will require further oral surgical and dental follow-up in the event of future refractory responses to medications and for treatment of local dental problems. PATHOPHYSIOLOGY
MODALITIES
Because the pathophysiologic issues of Meige syndrome have not yet been determined, treatment of the disease has so far been directed primarily against the symptoms. As described earlier, prompt diagnosis of this relatively rare condition is difficult. In Jankovic and Ford’s review the mean duration between onset of symptoms and correct diagnosis was 6.6 years. Often, the patients were subjected to multiple trials of pharmacologic agents, psychotherapy, and surgical attempts at relieving symptoms. Patients underwent temporomandibular joint surgery, extractions of teeth, and occlusal equilibration in an effort to correct the dental manifestations of this disease. Although these treatment modalities should not be derided, it is important to stress that they should follow, not precede, attempts at gaining control of the disease through more conservative means.
Meige syndrome, in its complete form, is manifested by the spontaneous onset of repetitive, dystonic, nonrhythmic contractions of the circumoral, labial, lingual, and masticatory musculature in combination with blepharospasm.2zs*6 The muscular spasms are bilateral and last from a few seconds to as much as half a mintue. Some authors place importance on the duration of the tonic spasms; the spasms in tardive dyskinesia characteristically last less than 5 seconds, whereas in Meige syndrome they continue longer.8 The disease usually affects adults and usually appears spontaneously in the fifth to sixth decades of life.’ However, the condition has also been diagnosed in teenaged patients,5 and in Jankovic and Ford’s series 17% of the patients experienced an onset of the disease before age 40 years. Women are more commonly affected than men in a ratio that approximates 2:l.
Volume Number
Meige syndrome
66 4
The most common symptom for which patients seek treatment is blepharospasm, preceded by or associated with eye irritation, tearing, photophobia, and increased blinking.2 Sixty-one percent of the patients reviewed by Jankovic and Ford proceeded to develop the complete syndrome. The severity of the symptoms is variable, as is the period between initial onset of the disease and maximal severity; this period ranges from 2 months to 18 years.5 This wide range has added to the difficulty of correctly diagnosing Meige syndrome. Patients who did not initially complain of blepharospasm reported a variety of symptoms. Of interest to the dental profession are those patients whose chief complaint was facial stiffness, dysarthria, spasmodic dysphonia, involuntary jaw and tongue movements that may dislodge dentures, cheek biting, nocturnal bruxism, temporomandibular joint pain, and facial pain associated with muscle spasms2 Several “trigger” factors have been reported. Emotional stress is considered to be the most important exacerbating factor.2 Sedation has been found to relieve the symptoms.5 The muscular spasms also disappear with sleep. Other trigger factors included glare, looking up, reading, watching television, fatigue, walking, and talking.2,5 A history of depression and psychiatric disorders is commonly associated with Meige syndrome. Marsden4 reports an incidence of 36%, and 41% of TolosaV patients suffered concomitantly from depression. Another feature of the disease that strongly suggests its origins is the strong family history of movement disorders or other neurologic disorders. Meige syndrome has been reported in sisters9 and in
429
patients with a family history of essential tremor, Parkinson’s disease, and abnormal facial tics. A genetic mode of inheritance supports Jankovic and Ford’s contention that presentations of isolated blepharospasm or isolated orofacial-cervical dyskinesia (that is, patients who have one symptom but not the other) may represent formes frustes of the disease. REFERENCES 1. Gould Medical Dictionarv. Revised ed. New York: McGrawHill, 1972. 2. Jankovic J, Ford J. Blepharospasm and orofacial cervical dystonia: clinical and pharmacological findings in 100 patients. Ann Neurol 1983;13:4021 I. 3. Tolosa ES, Klawans HL. Meige’s disease: a clinical form of facial convulsion, bilateral and medial. Neurology 1979; 361635-l. 4. Marsden CD. Blepharospasm-oromandibular syndrome (Brulghel’s syndrome): a variant of adult-onset torsion dystonia? Neural Neurosurg Psychiatry 1976;39:1204-9. 5. Tolosa ES. Clinical features of meige’s disease: idiopathic orofacial dystonia. Arch Neural 198 1;36: 147-5 1. 6. Coats ME. Meige’s disease and a positive treatment response with deanol. Milit Med 1985;150:152-3. 7. Nutt JG, Hammerstad JP. Blepharospasm and oromandibular dvstonia (Meiae’s svndrome) in sisters. Ann Nemo1 1980;$:189-91: . 8. Glazer WM, Moore DC, Hanse TC, Brenner LM. Meige’s svndrome and tardive dvskinesia. Am J Psychiatry 1983; <40:798-9. 9. Mauriello JA. Blepharospasm, Meige syndrome and hemifacial spasm: treatment with botulinurn toxin. Neurology 1985;35: 1499-500. Reprint requesls to: Dr. Mark R. Stevens Division of Oral and Maxillofacial School of Medicine Miami University Jackson Memorial Hospital 1611 N.W. 12th Ave. Miami, FL 33 136
Surgery
R. Stevens, D.D.S., * and Mark OF MEDICINE.
MIAMI
E. Wang, B.D.S.,*
Miami,
Fla.
UNIVERSITY
Dental and allied health professionals are on occasion confronted with patients who exhibit abnormal facial movements. These patients may be seeking a diagnosis or may relate a specific problem resulting from the uncontrolled and involuntary orofacial movements. A complete description of the various conditions associated with abnormal facial movements is beyond the scope of this article. Instead. these authors present a case with dental symptoms that were masking a more serious underlying progressive neurologic disorder. Appropriate referral to the neurology service is essential so that treatment of the underlying cause may precede, rather than follow, empiric management of these patients’ symptoms. (ORAL SURC ORAL MED ORAL PATHOL 1988;66:427-9)
A bnormal, disordered, involuntary movements are classified as dyskinesias.l The relatively slow and rhythmic nature of these movements distinguishes them from the twisting and writhing pattern seen in chorea. When the affected muscles exhibit abnormal tone, the term dystonia is used. A syndrome comprising dystonias of the oral, facial, lingual, and cervical musculature in association with blepharospasm was first described by a French neurologist, Henri Meige, in 1910; he called this syndrome spasme facial median.2,3 An alternative eponym for this condition, proposed by Marsden, 4 is Brulghel’s syndrome. The uncommon nature of this affliction, combined with better documentation of similar dyskinesias and diseases of extrapyramidal origin, has lead to confusion and misdiagnosis. Jankovic and Ford,= in 1982, prospectively evaluated 100 patients with blepharospasm and orofacial-cervical dystonias, the largest series studied to date. They report that the syndrome was most frequently confused with functional disorders, including nervous habits, neurosis, myasthenia gravis, Parkinson’s disease, tardive dyskinesia, temporomandibular joint dysfunction syndrome, Huntington’s chorea, and facial tics. A contributing reason for the confusion that enshrouds this disease may be the incomplete presentation of symptoms at varying stages of the disease process. We hope that this article will offer a comprehensive account of the syndrome: its clinical features, factors that distin*Division
of Oral
and Maxillofacial
Surgery.
guish it from other facial dyskinesias, and treatment modalities currently being employed to aid those suffering from this condition. CASE
REPORT
A 49-year-old Oriental woman sought treatment for cheek biting and fracturing of anterior teeth. She was examined by several dentists and oral-maxillofacial surgeons for her complaints. The patient underwent a tkmporomandibular joint work-up that consisted of a comprehensive examination, tomograms, and splint and physical therapy with occlusal equilibrations; all of these procedures provided no explanation of or relief from her recurrent symptoms. Her complaints persisted, and she was placed in short-term intermaxillary fixation with guiding elastics in an attempt to control her paroxysmal parafunctional habit. Eventually the posterior second molars were removed because of the failure of multiple conservative treatments. Clinical examination revealed a pleasant, middle-aged Oriental woman in moderate psychological distress., She exhibited a small, involuntary, continual positional movement of her lower jaw in a protrusive direction. Masse&r muscles were hypertrophied bilaterally and moderately tender to palpation. Her dentition showed marked attrition, and incisors were shortened and sharp. The remainder of her clinical examination and the review of systems did not help explain her current condition. Her past medical history was significant only for the present illness of approximately 1 year’s duration and for intermittent treatment of exogenous depression caused by loss of her spouse 18 months earlier. The patient was subsequently referred to the Department of Neurology, where a diagnosis of Meige syndrome 427
428
Stevens and Wong
Oral Sutg October 1988
The pathophysiology of Meige syndrome is poorly understood. Certain clinical findings point to the possible organic nature of the disease. Computed tomographic scans of the head were abnormal in 34% of cases studied by Jankovic and Ford.2 The pathologic findings included evidence of cerebral atrophy and various lucencies. The relationship of Meige syndrome to other known dysfunctions of the basal ganglia and rostra1 brainstem (for instance, generalized dystonias, Parkinson’s disease, essential tremors, and Wilson’s disease) provides further circumstantial evidence of the possible organic origins. The effects of various cholinergic and dopaminergic agents on patients suffering from Meige syndrome offer yet another clue to the pathologic process involved. It has been found that, in general, the use of agents that deplete dopamine levels (eg, tetrabenazine) or decrease dopaminergic or cholinergic receptor activity (eg, lisuride, trihexylphenidyl) resulted in various degrees of relief from the symptoms. This finding would appear to implicate relative cholinergic hyperactivity or a central dopamine preponderance as responsible causes.2’5-7 However, failure to obtain consistent results with the pharmacologic agents used suggests that the total mechanism of the disease has yet to be elucidated.
The treatment modalities currently being employed comprise two categories: pharmacologic agents that are known to possess either dopaminergic or cholinergic activity and pharmacologic or surgical procedures designed to relieve the major symptoms of the disease. Individual patient’s responses to the various pharmacologic agents have been extremely variable, with different efficacies and varying periods of relief obtained. Jankovic and Ford report that 22% of their patients had a marked and lasting improvement (more than 3 months) of symptoms from at least one of the drugs employed. Sixty-nine percent of their patients had lesser responses. Agents that have been tried include tetrabenazine (a dopamine-depleting agent), lithium (believed to act by blocking dopamine receptor sensitivity),2 trihexylphenidyl (a choline agonist that may provide competitive inhibition of choline transport),6 and clonazepam (a serotonergic agent). Failure to control the disease process by one of these regimens has lead to direct attention to the most incapacitating manifestations. Blepharospasm, which in its extreme form may lead to functional blindness, has been managed by selective sectioning of the zygomatic and palpebral branches of the seventh nerve in combination with a brow lift.2 The direct injection of botulin into the musculature of the eyelids has also been reported useful in gaining temporary relief.4 The management of the dental aspects of the disease should conserve as much tissue as possible. Recognition of the disease state and its clinical course will aid the practitioner in arriving at a suitable timetable for treatment.
TREATMENT
DISCUSSION
was made. The patient was placed on a trial treatment of tetrabenazine that satisfactorily controlled her orofacial spasms. The patient will require further oral surgical and dental follow-up in the event of future refractory responses to medications and for treatment of local dental problems. PATHOPHYSIOLOGY
MODALITIES
Because the pathophysiologic issues of Meige syndrome have not yet been determined, treatment of the disease has so far been directed primarily against the symptoms. As described earlier, prompt diagnosis of this relatively rare condition is difficult. In Jankovic and Ford’s review the mean duration between onset of symptoms and correct diagnosis was 6.6 years. Often, the patients were subjected to multiple trials of pharmacologic agents, psychotherapy, and surgical attempts at relieving symptoms. Patients underwent temporomandibular joint surgery, extractions of teeth, and occlusal equilibration in an effort to correct the dental manifestations of this disease. Although these treatment modalities should not be derided, it is important to stress that they should follow, not precede, attempts at gaining control of the disease through more conservative means.
Meige syndrome, in its complete form, is manifested by the spontaneous onset of repetitive, dystonic, nonrhythmic contractions of the circumoral, labial, lingual, and masticatory musculature in combination with blepharospasm.2zs*6 The muscular spasms are bilateral and last from a few seconds to as much as half a mintue. Some authors place importance on the duration of the tonic spasms; the spasms in tardive dyskinesia characteristically last less than 5 seconds, whereas in Meige syndrome they continue longer.8 The disease usually affects adults and usually appears spontaneously in the fifth to sixth decades of life.’ However, the condition has also been diagnosed in teenaged patients,5 and in Jankovic and Ford’s series 17% of the patients experienced an onset of the disease before age 40 years. Women are more commonly affected than men in a ratio that approximates 2:l.
Volume Number
Meige syndrome
66 4
The most common symptom for which patients seek treatment is blepharospasm, preceded by or associated with eye irritation, tearing, photophobia, and increased blinking.2 Sixty-one percent of the patients reviewed by Jankovic and Ford proceeded to develop the complete syndrome. The severity of the symptoms is variable, as is the period between initial onset of the disease and maximal severity; this period ranges from 2 months to 18 years.5 This wide range has added to the difficulty of correctly diagnosing Meige syndrome. Patients who did not initially complain of blepharospasm reported a variety of symptoms. Of interest to the dental profession are those patients whose chief complaint was facial stiffness, dysarthria, spasmodic dysphonia, involuntary jaw and tongue movements that may dislodge dentures, cheek biting, nocturnal bruxism, temporomandibular joint pain, and facial pain associated with muscle spasms2 Several “trigger” factors have been reported. Emotional stress is considered to be the most important exacerbating factor.2 Sedation has been found to relieve the symptoms.5 The muscular spasms also disappear with sleep. Other trigger factors included glare, looking up, reading, watching television, fatigue, walking, and talking.2,5 A history of depression and psychiatric disorders is commonly associated with Meige syndrome. Marsden4 reports an incidence of 36%, and 41% of TolosaV patients suffered concomitantly from depression. Another feature of the disease that strongly suggests its origins is the strong family history of movement disorders or other neurologic disorders. Meige syndrome has been reported in sisters9 and in
429
patients with a family history of essential tremor, Parkinson’s disease, and abnormal facial tics. A genetic mode of inheritance supports Jankovic and Ford’s contention that presentations of isolated blepharospasm or isolated orofacial-cervical dyskinesia (that is, patients who have one symptom but not the other) may represent formes frustes of the disease. REFERENCES 1. Gould Medical Dictionarv. Revised ed. New York: McGrawHill, 1972. 2. Jankovic J, Ford J. Blepharospasm and orofacial cervical dystonia: clinical and pharmacological findings in 100 patients. Ann Neurol 1983;13:4021 I. 3. Tolosa ES, Klawans HL. Meige’s disease: a clinical form of facial convulsion, bilateral and medial. Neurology 1979; 361635-l. 4. Marsden CD. Blepharospasm-oromandibular syndrome (Brulghel’s syndrome): a variant of adult-onset torsion dystonia? Neural Neurosurg Psychiatry 1976;39:1204-9. 5. Tolosa ES. Clinical features of meige’s disease: idiopathic orofacial dystonia. Arch Neural 198 1;36: 147-5 1. 6. Coats ME. Meige’s disease and a positive treatment response with deanol. Milit Med 1985;150:152-3. 7. Nutt JG, Hammerstad JP. Blepharospasm and oromandibular dvstonia (Meiae’s svndrome) in sisters. Ann Nemo1 1980;$:189-91: . 8. Glazer WM, Moore DC, Hanse TC, Brenner LM. Meige’s svndrome and tardive dvskinesia. Am J Psychiatry 1983; <40:798-9. 9. Mauriello JA. Blepharospasm, Meige syndrome and hemifacial spasm: treatment with botulinurn toxin. Neurology 1985;35: 1499-500. Reprint requesls to: Dr. Mark R. Stevens Division of Oral and Maxillofacial School of Medicine Miami University Jackson Memorial Hospital 1611 N.W. 12th Ave. Miami, FL 33 136
Surgery