The Journal of Emergency
Medicine,
Vol. 8, pp. 571-574.
1990
BLEPHAROSPASM-OROMANDIBULAR CEREBELL~P~NTINE
Printed in the USA. Copyright 0 1990 Pergamon Press plc
DYSTONIA ASSOCIATED WITH A LEFT ANGLE MENINGIOMA
John A. Persing, MD,* Amy Muir, BA,* Daniel G. Becker, BA,* Joseph J. Jankovic, MD,t Richard L. Anderson, MD,* Richard F. Edlich, MD, PhD* *Department of Plastic Surgery, University of Virginia School of Medicine, Charlottesville, Virginia, the TDepartment of Neurology, Baylor College of Medicine, Houston, Texas, and the *Department of Ophthalmology, University of Utah Health Sciences Center, Salt Lake City, Utah Reprint address: Richard F. Edlich, MD, Php., University of Virginia School of Medicine, Box 332, Charlottesville, VA 22908
?? Abstract - Blepharospasm-oromandibuhtr dystonia is
times, spasms of the tongue, mandibular muscles, and muscles of the floor of the mouth also occur. This syndrome occurs more often in females than in males (2: 1) and most often between the ages of 30 and 70 years (3,4). It is our purpose to describe the first case report of #‘a patient with a cerebellopontine angle meningioma that was associated with the development of this syndrome.
characterized by the presence of spasms of the orbicularis oculi (blepharospasm) and of the lower facial or oromandibular muscles. A patient with this syndrome is presented in which a left cerebellopontine angle meningioma appeared to act as a triggering mechanism for the develop ment of this disorder. On the basis of this report, we recommend that physicians search for this tumor in patients with this disorder.
CASE REPORT 0 Keywords - blepharospasm; oromandibular; dystonia; meningioma
A 58-year-old white woman complained of frequent involuntary eye lid closure, and photophobia for six months. She experienced a sudden onset of photophobia (“uncomfortable glare when looking at the sun”) while sunbathing at the beach, which persisted until admission and was associated with dry eyes. Her frequent involuntary blinking progressed to episodes of forced eyelid closure that was worsened by reading, writing, exercise, and emotional stress, disappearing during sleep. The patient was also troubled by tenseness of her neck muscles. The persistence of these symptoms caused considerable anxiety, and eventually deep depression. Pertinent past history included a successful removal of a benign mixed tumor of the left parotid gland 26 years before this admission. She had never received antiemetic, antipsychotic, or neuroleptic drug treatment and had no family history of dystonia. Mental examination was normal. Pupillary and extraocular muscle functions were normal. No visual field defect was detected. There was no evidence of trichiasis, blepharitis, entropion, or a superficial foreign body. Cornea1 reflexes were intact; all other cranial nerves were normal. Her bilateral blepharospasm, exacerbated
INTRODUCTION In 1910, Meige (1) described a syndrome of blepharo-
spasm and oromandibular dystonia that was characterized by spasms of the orbicularis oculi (blepharospasm) and of the lower facial or oromandibular muscles. Marsden (2) coined the term “Brueghel’s syndrome” as an eponym for blepharospasm or oromandibular dystonia of adult onset. The clinical features of this syndrome are fairly uniform. In its classical form, it is characterized by the presence of spasms of the orbicularis oculi that produce bilateral blepharospasm and that are accompanied by mild to moderate spasms of the frontalis and involvement of the middle and lower facial muscles. At
This study was supported by generous gifts from Mr. John McCrane, New York, New York, and H.E. Ghassan I. Shaker, London, England. RECEIVED: 21 November 1989; FINAL SUBMISSIONRECEIVED:20 ACCEPTED: 17 April 1990 571
March 1990;
0736-4679/90 $3.00 + .OO
572
J. A. Persing, A. Muir, D. G. Becker, J. J. Jankovic, R. L. Anderson, R. F. Edlich
by light directed at her eyes, was evident. The patient’s gait and motor system were normal. There were no sensory deficits, and plantar responses were flexor. She was admitted to the hospital for further evaluation. A computed tomographic scan revealed a 2.5 cm diameter spherical calcified mass in the left cerebellopontine angle (Figure 1). The tumor seemed to be invaginated into adjacent cerebellum with little displacement of the brain stem. Through a left occipital craniectomy, the tumor was found arising from the posterior aspect of the petrous bone, extending up to the tentorium, and projecting into adjacent cerebellum. A 1 cm margin of cerebellum was resected with the tumor. Biopsy confirmed that the tumor was a meningioma. This surgical procedure was followed by the disappearance of bilateral blepharospasm for approximately 6 months. The recurrence of blepharospasm was associated with involuntary contractions of her upper neck muscles anteriorly and bilaterally. During the next four years, further progression of her neurologic symptoms was evident. Her involuntary eyelid closure caused functional blindness and became associated with significant oromandibular dystonia. She displayed frequent
contractions of her platysma, digastric, stemocleidomastoid, muscles of facial expression, and jaw muscles causing grimacing, lip pursing, and jaw clenching. Her speech became dysarthric as she appeared to speak through clenched teeth. Magnetic resonance imaging was noncontributory with no evidence of tumor recurrence, cerebral atrophy, plaques, or lesions. Visual and auditory evoked potentials were normal. Enzyme immunoassays (5) for HTLV-I antibodies and enzyme-linked immunosorbent assay (6) for HTLV-III antibodies were negative. A rapid plasma reagent test for syphilis was negative. An electromyogram demonstrated no evidence of synkinetic activation, with normal size of motor units and without fibrillation. The bursts of muscle discharges were bilaterally synchronous. These findings suggested a central process responsible for muscle spasms. Her blepharospasm and orofacial dystonia have been managed by medical and surgical therapies. Botulinum toxin injections into orbicularis oculi, orbicularis oris, paranasal, masseter, and digastric muscles have provided temporary diminution in the magnitude of her involuntary contractions (7). The mechanism of action of botulinurn toxin is to interfere with the release of acetylcholine from the nerve terminals, without affecting the response of nerves and muscles to direct electrical stimulation or application of acetylcholine. In this manner, it leads to paralysis, and with long-term exposure, to atrophy of muscles. Because her bilateral blepharospasm became relatively refractory to botulinurn toxin injections, upper and lower lid orbicularis oculi resection was undertaken for relief of blepharospasm (8,9). She has recently entered a clinical trial of tetrabenazine for management of dystonia (10).
DISCUSSION
Figure 1. Computed tomographic 6can: left cerebellopontlne angle rounded extm-axlal mass (arrow) enhances brightly and homogen8ously.
Involuntary orofacial4ervical movements are frequently rapid, continuous, and stereotypic. When they are caused by chronic treatment with either neuroleptic, antipsychotic, or antiemetic medications, they are called tardive dyskinesia (11). The symptoms of tardive dyskinesia consist usually of involuntary, repetitive movements of the lips and tongue (tongue thrusting and lip smacking). Prompt diagnosis is important because withdrawal of the offending drug may lead to recovery. Orofacial-cervical dyskinesia, either choreic or dystonic, may occur spontaneously in patients with no prior exposure to drugs. The most common cause of spontaneous orofacial dyskinesia is Meige syndrome. Numerous etiologic factors have been implicated in this syndrome. A genetic predisposition may be important, as suggested by the occasional familial occurrence of some of these cases (10,12,13). Immunologic disorders such as rheu-
Blepharospasm-oromandibular
Dystonia
573
matoid arthritis, myasthenia gravis, Sjiigren syndrome, and thyroid abnormalities may predispose susceptible patients to develop this syndrome (10). Neurologic disorders included in the differential diagnosis of Meige syndrome, such as apraxia of eyelid opening, blepharospasm due to ocular disease, benign tic, functional blepharospasm, and hemifacial spasm, can usually be distinguished without difficulty by the emergency physician on the basis of several clinical features (14). Apraxia of eyelid opening is due to a supranuclear inhibition of the levator palpebrae superioris with normal function of the orbicularis oculi muscle. Because ocular disease such as blepharitis, keratoconjunctival sicca, trichiasis, entropion, or a superficial foreign body can cause secondary reflex eyelid blinking, a thorough ophthalmologic examination is needed before the diagnosis of Meige syndrome can be made. Benign tics may be due to intermittent contractions of the orbicularis oculi muscles, and resolution often occurs spontaneously within days to weeks. Functional or hysterical blepharospasm usually begins abruptly and is often precipitated by emotional stress; affected patients are usually much younger than those with Meige syndrome. Hemifacial spasm is caused by sudden contractions of the muscles innervated by the facial nerve. In many cases, the facial nerve is compressed by a vascular lesion near its exit from the brain stem. The emergency medical physician should also perform a complete neurologic examination. Patients with multiple sclerosis, Parkinson disease, tardive dyskinesia, and various other movement disorders have an increased risk for the development of blepharospasm ( 11,15,16). A patient suspected of having Meige syndrome should be referred to a neurologist for further evaluation, which should include computed tomographic scan of the brain.
The role of the meningioma in our patient’s clinical course is unclear. It may have served as an external triggering factor in an otherwise asymptomatic patient (17). Alternatively, the tumor may have compressed the central brainstem, an area previously implicated in the pathogenesis of secondary (and possibly primary) cranial-cervical dystonia (18-2 1). The reason for the spontaneous remission of the syndrome for 6 months after tumor removal is also uncertain. Spontaneous remissions of more than a few days are exceptional, but can occur during the first year of illness and can last several months (17). Frequently, with time, the dystonic spasms become more frequent and intense and in the complete form of the disorder extend to the middle and lower facial muscles, and even to the oromandibular ones. Medical and surgical treatment can attenuate the intensity of the dystonic spasms in Meige syndrome. Without better understanding of the causes and biochemical mechanisms of Meige syndrome, drug therapy for this disorder will remain symptomatic and empirical at best (10). Tetrabenazine, a dopamine-depleting agent, results in marked and lasting improvement in many patients. Lithium and anticholinergic agents are also helpful in selected patients. Haloperidol, previously reported to be effective in this syndrome is used only minimally because of concern for the possible risk of tardive dyskinesia. However, it is not known whether drug therapy can alter the natural course of the disease. Because meningioma has now been found as a possible triggering mechanism for blepharospasm-oromandibular dystonia, we recommend that physicians be aware of the possible relationship of this tumor and this neurologic disorder. The successful removal of this tumor, however, may not alter the natural progression of this neurologic disorder.
REFERENCES 1. Meige H. Les convulsions de la face, une forme clinique de convulsion faciale, bilat&ale et mkdiane. Revue neurologique (Paris). 1910;20:437-43. dystonia syndrome 2. Marsden CD. Blepharospasm-oromandibular (Brueghel’s syndrome). J Neural Neurosurg Psychiatry. 1976;39: 1204-9. 3. Tolosa ES, Klawans HL. Meige’s disease. A clinical form of facial convulsions, bilateral and medial. Arch Neurol. 1979;36: 635-7. 4. Casey DE. Pharmacology of blepharospasm-oromandibular dystonia syndrome. Neurology (NY). 1980;30:690-5. 5. Centers for Disease Control. Licensure of screening tests for antibody to human t-lymphotropic virus type I. MMWR. 1988;37: 736-40. 6. Weiss SH, Goedert JJ, Samgadharan MG, Bodner AJ. The AIDS Seroepidemiology Collaborative Working Group, Gal10 RC, Blattner WA. Screening test for HTLV-III (AIDS Agent) antibodies specificity, sensitivity, and applications. JAMA. 1985;253: 221-5.
7. Scott AB, Kennedy RA, Stubbs HA. Botulinum: a toxin injection as a treatment for blepharospasm. Arch Gpthalmol. 1985;103: 347-50. 8. Gilhun WN, Anderson RL. Blepharospasm surgery: an anatomical approach. Arch Opthalmol. 1981;99:1056-62. 9. McCord CD Jr, Shore J, Putnam JR. Treatment of essential blepharospasm; IIz a modification of exposure for the muscle stripping technique. Arch Gpthalmol. 1984;102:269-73. 10. Jankovic J, Ford J. Blepharospasm and orofaciakervical dystonia: clinical and pharmacological findings in 100 patients. Ann Neurol. 1983;13:402-11. 11. Ananth J, Edelmuth E, Dargan B. Meige’s syndrome associated with neuroleptic treatment. Am J Psychiatry. 1988;145:513-5. 12. Tolosa ES. Clinical features of Meige’s disease (idopathic orofacial dystonia): a report of 17 cases. Arch Neurol. 1981;38:147-51. 13. Nutt JG, Hammerstad JP. Blepharospasm and oromandibular dystonia (Meige’s syndrome) in sisters. Ann Neurol. 1981;9: 189-91. 14. Kennedy RI-I, Bartley GB, Flanagan JC, Waller RR. Treatment of
574
J. A. Persing, A. Muir, D. G. Becker, J. J. Jankovic, R. L. Anderson, R. F. Edlich
blepharospasm with botulinum toxin. Mayo Clin Proc. 1989;64: 1085-90. 15. Henderson JW. Essential blepharospasm. Tram Am Ophthalmol Sot. 1956;54:453-520. 16. Jankovic J, Havins WE, Wilkins RB. Blinking and blepharospasm: mechanism, diagnosis, and management. JAMA. 1982;248: 3160-4. 17. Tolosa ES, Marti MJ. Blepharospasm-oromandibular dystonia syndrome (Meige’s syndrome): clinical aspects. In: Jankovic J, Tolosa ES, eds. Advances in Neurology, vol 49. New York: Raven Press: 1988:73-84.
18. Jankovic J, Pate1 SC. Blepharospasm associated with brainstem lesions. Neurology. 1983;33:12374. 19. Jankovic J. Blepharospasm with basal ganglia lesions. Arch Neurol. 1986;43:866-8. 20. Jankovic J, Patten BM. Blepharospasm and autoimmune disease. Movement Disord. 1987;2:159-63. 21. Jankovic J, Nutt JG. Blepharospasm and cranial-cervical dystonia (Meige’s syndrome): familial occurrence. In: Jankovic J, Tolosa ES, eds. Advances in neurology, vol. 49, New York: Raven Press; 1988:117-23.
Medicine,
Vol. 8, pp. 571-574.
1990
BLEPHAROSPASM-OROMANDIBULAR CEREBELL~P~NTINE
Printed in the USA. Copyright 0 1990 Pergamon Press plc
DYSTONIA ASSOCIATED WITH A LEFT ANGLE MENINGIOMA
John A. Persing, MD,* Amy Muir, BA,* Daniel G. Becker, BA,* Joseph J. Jankovic, MD,t Richard L. Anderson, MD,* Richard F. Edlich, MD, PhD* *Department of Plastic Surgery, University of Virginia School of Medicine, Charlottesville, Virginia, the TDepartment of Neurology, Baylor College of Medicine, Houston, Texas, and the *Department of Ophthalmology, University of Utah Health Sciences Center, Salt Lake City, Utah Reprint address: Richard F. Edlich, MD, Php., University of Virginia School of Medicine, Box 332, Charlottesville, VA 22908
?? Abstract - Blepharospasm-oromandibuhtr dystonia is
times, spasms of the tongue, mandibular muscles, and muscles of the floor of the mouth also occur. This syndrome occurs more often in females than in males (2: 1) and most often between the ages of 30 and 70 years (3,4). It is our purpose to describe the first case report of #‘a patient with a cerebellopontine angle meningioma that was associated with the development of this syndrome.
characterized by the presence of spasms of the orbicularis oculi (blepharospasm) and of the lower facial or oromandibular muscles. A patient with this syndrome is presented in which a left cerebellopontine angle meningioma appeared to act as a triggering mechanism for the develop ment of this disorder. On the basis of this report, we recommend that physicians search for this tumor in patients with this disorder.
CASE REPORT 0 Keywords - blepharospasm; oromandibular; dystonia; meningioma
A 58-year-old white woman complained of frequent involuntary eye lid closure, and photophobia for six months. She experienced a sudden onset of photophobia (“uncomfortable glare when looking at the sun”) while sunbathing at the beach, which persisted until admission and was associated with dry eyes. Her frequent involuntary blinking progressed to episodes of forced eyelid closure that was worsened by reading, writing, exercise, and emotional stress, disappearing during sleep. The patient was also troubled by tenseness of her neck muscles. The persistence of these symptoms caused considerable anxiety, and eventually deep depression. Pertinent past history included a successful removal of a benign mixed tumor of the left parotid gland 26 years before this admission. She had never received antiemetic, antipsychotic, or neuroleptic drug treatment and had no family history of dystonia. Mental examination was normal. Pupillary and extraocular muscle functions were normal. No visual field defect was detected. There was no evidence of trichiasis, blepharitis, entropion, or a superficial foreign body. Cornea1 reflexes were intact; all other cranial nerves were normal. Her bilateral blepharospasm, exacerbated
INTRODUCTION In 1910, Meige (1) described a syndrome of blepharo-
spasm and oromandibular dystonia that was characterized by spasms of the orbicularis oculi (blepharospasm) and of the lower facial or oromandibular muscles. Marsden (2) coined the term “Brueghel’s syndrome” as an eponym for blepharospasm or oromandibular dystonia of adult onset. The clinical features of this syndrome are fairly uniform. In its classical form, it is characterized by the presence of spasms of the orbicularis oculi that produce bilateral blepharospasm and that are accompanied by mild to moderate spasms of the frontalis and involvement of the middle and lower facial muscles. At
This study was supported by generous gifts from Mr. John McCrane, New York, New York, and H.E. Ghassan I. Shaker, London, England. RECEIVED: 21 November 1989; FINAL SUBMISSIONRECEIVED:20 ACCEPTED: 17 April 1990 571
March 1990;
0736-4679/90 $3.00 + .OO
572
J. A. Persing, A. Muir, D. G. Becker, J. J. Jankovic, R. L. Anderson, R. F. Edlich
by light directed at her eyes, was evident. The patient’s gait and motor system were normal. There were no sensory deficits, and plantar responses were flexor. She was admitted to the hospital for further evaluation. A computed tomographic scan revealed a 2.5 cm diameter spherical calcified mass in the left cerebellopontine angle (Figure 1). The tumor seemed to be invaginated into adjacent cerebellum with little displacement of the brain stem. Through a left occipital craniectomy, the tumor was found arising from the posterior aspect of the petrous bone, extending up to the tentorium, and projecting into adjacent cerebellum. A 1 cm margin of cerebellum was resected with the tumor. Biopsy confirmed that the tumor was a meningioma. This surgical procedure was followed by the disappearance of bilateral blepharospasm for approximately 6 months. The recurrence of blepharospasm was associated with involuntary contractions of her upper neck muscles anteriorly and bilaterally. During the next four years, further progression of her neurologic symptoms was evident. Her involuntary eyelid closure caused functional blindness and became associated with significant oromandibular dystonia. She displayed frequent
contractions of her platysma, digastric, stemocleidomastoid, muscles of facial expression, and jaw muscles causing grimacing, lip pursing, and jaw clenching. Her speech became dysarthric as she appeared to speak through clenched teeth. Magnetic resonance imaging was noncontributory with no evidence of tumor recurrence, cerebral atrophy, plaques, or lesions. Visual and auditory evoked potentials were normal. Enzyme immunoassays (5) for HTLV-I antibodies and enzyme-linked immunosorbent assay (6) for HTLV-III antibodies were negative. A rapid plasma reagent test for syphilis was negative. An electromyogram demonstrated no evidence of synkinetic activation, with normal size of motor units and without fibrillation. The bursts of muscle discharges were bilaterally synchronous. These findings suggested a central process responsible for muscle spasms. Her blepharospasm and orofacial dystonia have been managed by medical and surgical therapies. Botulinum toxin injections into orbicularis oculi, orbicularis oris, paranasal, masseter, and digastric muscles have provided temporary diminution in the magnitude of her involuntary contractions (7). The mechanism of action of botulinurn toxin is to interfere with the release of acetylcholine from the nerve terminals, without affecting the response of nerves and muscles to direct electrical stimulation or application of acetylcholine. In this manner, it leads to paralysis, and with long-term exposure, to atrophy of muscles. Because her bilateral blepharospasm became relatively refractory to botulinurn toxin injections, upper and lower lid orbicularis oculi resection was undertaken for relief of blepharospasm (8,9). She has recently entered a clinical trial of tetrabenazine for management of dystonia (10).
DISCUSSION
Figure 1. Computed tomographic 6can: left cerebellopontlne angle rounded extm-axlal mass (arrow) enhances brightly and homogen8ously.
Involuntary orofacial4ervical movements are frequently rapid, continuous, and stereotypic. When they are caused by chronic treatment with either neuroleptic, antipsychotic, or antiemetic medications, they are called tardive dyskinesia (11). The symptoms of tardive dyskinesia consist usually of involuntary, repetitive movements of the lips and tongue (tongue thrusting and lip smacking). Prompt diagnosis is important because withdrawal of the offending drug may lead to recovery. Orofacial-cervical dyskinesia, either choreic or dystonic, may occur spontaneously in patients with no prior exposure to drugs. The most common cause of spontaneous orofacial dyskinesia is Meige syndrome. Numerous etiologic factors have been implicated in this syndrome. A genetic predisposition may be important, as suggested by the occasional familial occurrence of some of these cases (10,12,13). Immunologic disorders such as rheu-
Blepharospasm-oromandibular
Dystonia
573
matoid arthritis, myasthenia gravis, Sjiigren syndrome, and thyroid abnormalities may predispose susceptible patients to develop this syndrome (10). Neurologic disorders included in the differential diagnosis of Meige syndrome, such as apraxia of eyelid opening, blepharospasm due to ocular disease, benign tic, functional blepharospasm, and hemifacial spasm, can usually be distinguished without difficulty by the emergency physician on the basis of several clinical features (14). Apraxia of eyelid opening is due to a supranuclear inhibition of the levator palpebrae superioris with normal function of the orbicularis oculi muscle. Because ocular disease such as blepharitis, keratoconjunctival sicca, trichiasis, entropion, or a superficial foreign body can cause secondary reflex eyelid blinking, a thorough ophthalmologic examination is needed before the diagnosis of Meige syndrome can be made. Benign tics may be due to intermittent contractions of the orbicularis oculi muscles, and resolution often occurs spontaneously within days to weeks. Functional or hysterical blepharospasm usually begins abruptly and is often precipitated by emotional stress; affected patients are usually much younger than those with Meige syndrome. Hemifacial spasm is caused by sudden contractions of the muscles innervated by the facial nerve. In many cases, the facial nerve is compressed by a vascular lesion near its exit from the brain stem. The emergency medical physician should also perform a complete neurologic examination. Patients with multiple sclerosis, Parkinson disease, tardive dyskinesia, and various other movement disorders have an increased risk for the development of blepharospasm ( 11,15,16). A patient suspected of having Meige syndrome should be referred to a neurologist for further evaluation, which should include computed tomographic scan of the brain.
The role of the meningioma in our patient’s clinical course is unclear. It may have served as an external triggering factor in an otherwise asymptomatic patient (17). Alternatively, the tumor may have compressed the central brainstem, an area previously implicated in the pathogenesis of secondary (and possibly primary) cranial-cervical dystonia (18-2 1). The reason for the spontaneous remission of the syndrome for 6 months after tumor removal is also uncertain. Spontaneous remissions of more than a few days are exceptional, but can occur during the first year of illness and can last several months (17). Frequently, with time, the dystonic spasms become more frequent and intense and in the complete form of the disorder extend to the middle and lower facial muscles, and even to the oromandibular ones. Medical and surgical treatment can attenuate the intensity of the dystonic spasms in Meige syndrome. Without better understanding of the causes and biochemical mechanisms of Meige syndrome, drug therapy for this disorder will remain symptomatic and empirical at best (10). Tetrabenazine, a dopamine-depleting agent, results in marked and lasting improvement in many patients. Lithium and anticholinergic agents are also helpful in selected patients. Haloperidol, previously reported to be effective in this syndrome is used only minimally because of concern for the possible risk of tardive dyskinesia. However, it is not known whether drug therapy can alter the natural course of the disease. Because meningioma has now been found as a possible triggering mechanism for blepharospasm-oromandibular dystonia, we recommend that physicians be aware of the possible relationship of this tumor and this neurologic disorder. The successful removal of this tumor, however, may not alter the natural progression of this neurologic disorder.
REFERENCES 1. Meige H. Les convulsions de la face, une forme clinique de convulsion faciale, bilat&ale et mkdiane. Revue neurologique (Paris). 1910;20:437-43. dystonia syndrome 2. Marsden CD. Blepharospasm-oromandibular (Brueghel’s syndrome). J Neural Neurosurg Psychiatry. 1976;39: 1204-9. 3. Tolosa ES, Klawans HL. Meige’s disease. A clinical form of facial convulsions, bilateral and medial. Arch Neurol. 1979;36: 635-7. 4. Casey DE. Pharmacology of blepharospasm-oromandibular dystonia syndrome. Neurology (NY). 1980;30:690-5. 5. Centers for Disease Control. Licensure of screening tests for antibody to human t-lymphotropic virus type I. MMWR. 1988;37: 736-40. 6. Weiss SH, Goedert JJ, Samgadharan MG, Bodner AJ. The AIDS Seroepidemiology Collaborative Working Group, Gal10 RC, Blattner WA. Screening test for HTLV-III (AIDS Agent) antibodies specificity, sensitivity, and applications. JAMA. 1985;253: 221-5.
7. Scott AB, Kennedy RA, Stubbs HA. Botulinum: a toxin injection as a treatment for blepharospasm. Arch Gpthalmol. 1985;103: 347-50. 8. Gilhun WN, Anderson RL. Blepharospasm surgery: an anatomical approach. Arch Opthalmol. 1981;99:1056-62. 9. McCord CD Jr, Shore J, Putnam JR. Treatment of essential blepharospasm; IIz a modification of exposure for the muscle stripping technique. Arch Gpthalmol. 1984;102:269-73. 10. Jankovic J, Ford J. Blepharospasm and orofaciakervical dystonia: clinical and pharmacological findings in 100 patients. Ann Neurol. 1983;13:402-11. 11. Ananth J, Edelmuth E, Dargan B. Meige’s syndrome associated with neuroleptic treatment. Am J Psychiatry. 1988;145:513-5. 12. Tolosa ES. Clinical features of Meige’s disease (idopathic orofacial dystonia): a report of 17 cases. Arch Neurol. 1981;38:147-51. 13. Nutt JG, Hammerstad JP. Blepharospasm and oromandibular dystonia (Meige’s syndrome) in sisters. Ann Neurol. 1981;9: 189-91. 14. Kennedy RI-I, Bartley GB, Flanagan JC, Waller RR. Treatment of
574
J. A. Persing, A. Muir, D. G. Becker, J. J. Jankovic, R. L. Anderson, R. F. Edlich
blepharospasm with botulinum toxin. Mayo Clin Proc. 1989;64: 1085-90. 15. Henderson JW. Essential blepharospasm. Tram Am Ophthalmol Sot. 1956;54:453-520. 16. Jankovic J, Havins WE, Wilkins RB. Blinking and blepharospasm: mechanism, diagnosis, and management. JAMA. 1982;248: 3160-4. 17. Tolosa ES, Marti MJ. Blepharospasm-oromandibular dystonia syndrome (Meige’s syndrome): clinical aspects. In: Jankovic J, Tolosa ES, eds. Advances in Neurology, vol 49. New York: Raven Press: 1988:73-84.
18. Jankovic J, Pate1 SC. Blepharospasm associated with brainstem lesions. Neurology. 1983;33:12374. 19. Jankovic J. Blepharospasm with basal ganglia lesions. Arch Neurol. 1986;43:866-8. 20. Jankovic J, Patten BM. Blepharospasm and autoimmune disease. Movement Disord. 1987;2:159-63. 21. Jankovic J, Nutt JG. Blepharospasm and cranial-cervical dystonia (Meige’s syndrome): familial occurrence. In: Jankovic J, Tolosa ES, eds. Advances in neurology, vol. 49, New York: Raven Press; 1988:117-23.
