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Oral Facial Dystonia Triggered by Speech
Case Reports
4. Brown DD, Juhl RP, Warner SL: Decreased bioavailability of digoxin due to hypercholesterolemic interventions. Circulation 58: 164-172, 1978 5. Northcutt RC, Steil IN, Hollifield JW, et at: The
influence of cholestyramine on thyroxine absorption. JAMA 208:1857-1861, 1969 6. Carruthers SG, Dujovne CA: Cholestyramine and spironolactone and their combination in digitoxin elimination. Clin Pharmacol Ther 27:184-187, 1980
7. Robinson DS, Benjamin DM, McCormack JJ: Interaction of warfarin and nonsystemic gastrointestinal drugs. Clin Pharmacol Ther 12:491-495, 1971
8. Johansson C, Adamsson U, Stierner U, et al: Interaction by cholestyramine on the uptake of hydrocortisone in the gastrointestinal tract. Acta Med Scand 204:509-512, 1978 9. Ti TY, Giles HG, Sellers EM: Probable interaction of loperamide and cholestyramine. Can Med Assoc J 23:607-608, 1978
10. Friedel RO, Roskind MA: Relationship of blood
levels of Sinequan to clinical effects in the treatment of depression in aged patients, in Mendels J
(ed): Sinequan (Doxepin HCI): A Monograph of Recent Clinical Studies. Amsterdam, Excerpta Medica, 1975, pp 52-54 II. Hollister LE: Plasma concentrations of tricyclic antidepressants in clinical practice. J Clin Psychiatry 43:66-69, 1982 12. Boyer WF, Friedel RO: Antidepressant and antipsychotic plasma levels. Psychiatr Clin N Am 7:601-610, 1984 13. Greenblatt DJ, Sellars EM, Koch-Weser J: Impor-
tance of protein binding for the interpretation of serum or plasma drug concentrations. J Clin Pharmacol 22:259-263, 1982 14. APA Task Force Report: Tricyclic antidepressants, blood level measurements and clinical outcome. Am J Psychiatry 142:155-162, 1985 15. Amsterdam J, Brunswick D, Mendels J: The clinical application of tricyclic antidepressant pharmacokinetics and plasma levels. Am J Psychiatry 137:653-662, 1980 16. Risch SC, Kalin NH, Janowsky DS, et al: Indica-
tions and guidelines for plasma tricyclic antidepressant concentration monitoring. J Clin Psychopharmacol 1:59-63, 1981
Oral Facial Dystonia Triggered by Speech GEORGE
W.
PAULSON,
JANICE BARNES,
umerous variants of focal dystonia can be considered psychogenic, although there is an increasing tendency to recognize most dystonias as organic in origin. We have seen four patients with severe dystonic movement triggered by speech. The cause and pathophysiology are uncertain, and the condition is severe and may be recognized by any physician or
N
Received October 14, 1986; revised January 21, 1987; accepted February 9, 1987. From the Department of Neurology, The Ohio State University; and the Riverside Methodist Hospital, Columbus, Ohio. Address reprint requests to Dr. Paulson, Department of Neurology, The Ohio State University, 452 Means Hall, 1655 Upham Drive, Columbus, OH 43210. Copyright 0 1988 The Academy of Psychosomatic Medicine.
M.D.
PH.D.
psychologist. There is no evidence that speech therapy, pharmacotherapy, or surgery will totally eliminate the phenomena. Case Report This 48-year~ld man had a three-year history of speech problems consisting of sudden spasms in his neck and throat, often triggered by even a brief (3Q..second) period of normal speech. Because of his complaint about speech he had had surgery for a benign thyroid nodule two years earlier. When he spoke there was a feeling of neck strain; if he continued talking, his speech seemed forced. This could be partially overcome by arching his head backward, and he could produce a whisper by rais-
PSYCHOSOMATICS
Case Reports
ing the pitch of his voice. There was little or no secondary relationship to stress, and the process was present when he was alone or with others. He had no writer's cramp, no history to suggest MS or other neurologic disorders, and no progressive rigidity or stiffness. There was no blepharospasm and he had never been depressed. He had never received neuroleptics. Examination, including careful neurologic examination and direct inspection of the vocal cords, was entirely normal. CT examination, EMG, and CSF were all normal. A trial of baclofen, alprazolam, trihexyphenidyl, and various antidepressants or anticonvulsants was without major benefit. One year later he again received trihexyphenidyl, the most useful of the drugs, and at a dose of 15 mg per day he was significantly improved.
Discussion The characteristic feature of each of the four cases we have seen was severe paroxysmal facial dystonia in a middle-aged male who manifested no other extrapyramidal features. The dystonia was triggered by speech and resulted in pulling of the mouth and chin down or to one side. Speech became high pitched at the time of the spasm, and prior to the dystonic spasms, the patient noticed a tight feeling in his mouth. There was major involvement of the lower half of the face and of the anterior cervical muscles and oral musculature, but the upper face was not affected. Sixty seconds of speech were sufficient to trigger the spasms; they immediately ceased when the patient stopped talking. The higher voice ranges were affected differently than lower ranges, and a whistle or a whisper did not always trigger the reaction. None of the patients had overt psychiatric disease. There was no evidence that anticholinergics, minor anxiolytics, butyrophenones, or sedation provided significant benefit, but massive doses of anticholinergics were mildly helpful. In contrast to essential tremor which is ameliorated by alcohol,' alcohol had no major effect, good or bad. Dystonia includes several varieties of sustained postural abnormality and can be associated with tremor or with choreoathetosis. The classical form of dystonia, dystonia muscuVOLUME 29 • NUMBER 2. SPRING 1988
lorum deformans, is not usually accentuated by an effort to speak and does not involve the facial musculature more than the limbs. Some patients with dystonic movement and patients with athetosis may have an overflow or spread of movements to the face when there is major effort or with "action dystonia." Genetically, dystonia musculorum deformans may be either recessive or dominant, but many sporadic cases are seen. 2 In our cases with oral facial dystonia, we observed no obvious inherited tendency. In addition to the heritable dystonias, numerous fragmentary or focal dystonias have been described by Marsden,3 who might categorize the condition we are describing as a laryngo-pharyngeal dystonia. The fragmentary dystonia do fall in the borderland between psychiatry and neurology, as is true with writer's cramp, and some are associated with an incessant mouth movement reminiscent of tardive dyskinesia. Most fragmentary dystonias are probably organic and portions of a larger spectrum; all respond poorly to therapy.· Other forms of the fragmentary dystonias may come to the attention of physicians and speech pathologists. Meige's syndrome includes mouth movements and blepharospasm, and begins in the middle years of life. 5•6 This well-described entity responds poorly to therapy, may slowly worsen, and is not simply psychogenic blepharospasm. In patients with Meige's syndrome, the chin often thrusts forward or moves from side to side, the eyes close forcefully at the same time, and the individual may be severely incapacitated because of transient inability to see. Rarely these patients will also complain of interference with speech. The movements and the eye blinks of Meige's syndrome can be blocked transiently by whistling or by speech, as if by distraction or increased neuronal inhibition. Such cases of blepharospasm clearly differ from tardive dyskinesia in appearance. Tardive dyskinesia/· K which usually follows prolonged use of phenothiazines, does include a tendency to exhibit a writhing tongue or puckering mouth movements, but it rarely affects speech. Spastic dysphonia, another cause for a "tight voice," is distinct from 137
Case Reports
oral facial dystonia, and in most patients there is no apparent tightness of the facial musculature. The cause of spastic dysphonia is uncertain, and some cases do present extrapyramidal features in other portions of the body. There are conditions that can be interpreted as "cross-talk," an uncontrollable or inappropriate spread of impulses within the nervous system. This does not refer to ephaptic spread, but to common phenomena such as a peculiar tingling sensation in the flank when an individual has actually scratched a different place in the body. Such cross-talk may be thought of as akin to faintly overhearing (because of spread in the wires) a different conversation while on the telephone. It is certainly true, with or without the cross-talk phenomena, that inappropriate spread can occur within the nervous system. For example, there are familial or idiopathic mirror movements in which one hand moves in a mirror image of the movements of the other hand. The mechanism of such motor or sensory spread remains unclear. At times, distinct clinical entities do result from local irritation and instability in select areas, e.g., hemifacial spasm or trigeminal neuralgia. 9 As is true in many of the phenomena described above, the mechanism of the oral facial dystonia seen in our patients remains obscure. Numerous drugs, including butyrophenones, anticholinergics, and sedatives, have been tried for these patients. None have been dramatically helpful. Some of the patients do learn to
partially control the movements, usually by frequent stops during conversations, but they usually cannot control the dystonia completely. Because the basic voice is usually normal in our patients and because spastic dysphonia is not usually associated with the bizarre muscle movement of the neck and chin, oral facial dystonia can be distinguished from spastic dysphonia. At this time we not only lack an explanation, we also lack a proper name for this condition. "Oral facial dystonia triggered by speech" is clumsy, and oral mandibular dystonia or laryngo-pharyngeal dystonia are also unsatisfactory. Currently, we feel that this difficult condition is best considered as a "focal" or "fragmentary" dystonia. The most effective approach for managing these patients is to offer a trial of anticholinergics, administered with a slow increase. Butyrophenones may also help, but levodopa is not likely to be effective. Speech therapy may help to control overflow phenomena, and this can facilitate communication. Some patients will need therapy for depression. A more complete pathologic explanation and better therapy are needed. There is no reason to believe the phenomenon is primarily psychogenic. Generally speaking, information about the pathology of each of the dystonias is limited. There are, in contrast to the movement disorder of Parkinson's disease, no completely accepted biochemical or pathologic concomitants of dystonia. More research is needed to address these issues.
References
I. Larsen TA, Caine DB: Essential tremor. Clin Neuropharmacol 6: 185-206, 1983 2. Zeman W: Dystonia: an overview. Adv Neurol 14:91-103, 1976 3. Marsden CD: Dystonia: the spectrum of the disease, in, Yahr, MD, The Basal Ganglia. New York, Raven Press, 1976, pp 351-367 4. Paulson GW: Torsion dystonia, in Johnson RT, Mosby CV (eds): Current Therapy in Neurologic Disease. St. Louis, CV Mosby Co, 1985, pp 264266 5. Duvoisin RC: Meige syndrome: relief on high-dose anticholinergic therapy. Clin Neuropharmacol
6:63-66, 1983 6. Tolosa ES, Lai CW: Meige disease: striatal dopaminergic preponderance. Neurology 29: 11261130,1979 7. Goetz CG, Klawans HL: Tardive dyskinesia. Neurol Clin 2:605-614, 1984 8. Paulson GW: Tardive dyskinesia. Annu Rev Med 26:75-81,1975 9. Cook BR, Janetta PJ: Tic convulsive: results in II cases treated with microvascular decompression of the fifth and seventh cranial nerves. J Neurosurg 61:949-955, 1984
PSYCHOSOMATICS
4. Brown DD, Juhl RP, Warner SL: Decreased bioavailability of digoxin due to hypercholesterolemic interventions. Circulation 58: 164-172, 1978 5. Northcutt RC, Steil IN, Hollifield JW, et at: The
influence of cholestyramine on thyroxine absorption. JAMA 208:1857-1861, 1969 6. Carruthers SG, Dujovne CA: Cholestyramine and spironolactone and their combination in digitoxin elimination. Clin Pharmacol Ther 27:184-187, 1980
7. Robinson DS, Benjamin DM, McCormack JJ: Interaction of warfarin and nonsystemic gastrointestinal drugs. Clin Pharmacol Ther 12:491-495, 1971
8. Johansson C, Adamsson U, Stierner U, et al: Interaction by cholestyramine on the uptake of hydrocortisone in the gastrointestinal tract. Acta Med Scand 204:509-512, 1978 9. Ti TY, Giles HG, Sellers EM: Probable interaction of loperamide and cholestyramine. Can Med Assoc J 23:607-608, 1978
10. Friedel RO, Roskind MA: Relationship of blood
levels of Sinequan to clinical effects in the treatment of depression in aged patients, in Mendels J
(ed): Sinequan (Doxepin HCI): A Monograph of Recent Clinical Studies. Amsterdam, Excerpta Medica, 1975, pp 52-54 II. Hollister LE: Plasma concentrations of tricyclic antidepressants in clinical practice. J Clin Psychiatry 43:66-69, 1982 12. Boyer WF, Friedel RO: Antidepressant and antipsychotic plasma levels. Psychiatr Clin N Am 7:601-610, 1984 13. Greenblatt DJ, Sellars EM, Koch-Weser J: Impor-
tance of protein binding for the interpretation of serum or plasma drug concentrations. J Clin Pharmacol 22:259-263, 1982 14. APA Task Force Report: Tricyclic antidepressants, blood level measurements and clinical outcome. Am J Psychiatry 142:155-162, 1985 15. Amsterdam J, Brunswick D, Mendels J: The clinical application of tricyclic antidepressant pharmacokinetics and plasma levels. Am J Psychiatry 137:653-662, 1980 16. Risch SC, Kalin NH, Janowsky DS, et al: Indica-
tions and guidelines for plasma tricyclic antidepressant concentration monitoring. J Clin Psychopharmacol 1:59-63, 1981
Oral Facial Dystonia Triggered by Speech GEORGE
W.
PAULSON,
JANICE BARNES,
umerous variants of focal dystonia can be considered psychogenic, although there is an increasing tendency to recognize most dystonias as organic in origin. We have seen four patients with severe dystonic movement triggered by speech. The cause and pathophysiology are uncertain, and the condition is severe and may be recognized by any physician or
N
Received October 14, 1986; revised January 21, 1987; accepted February 9, 1987. From the Department of Neurology, The Ohio State University; and the Riverside Methodist Hospital, Columbus, Ohio. Address reprint requests to Dr. Paulson, Department of Neurology, The Ohio State University, 452 Means Hall, 1655 Upham Drive, Columbus, OH 43210. Copyright 0 1988 The Academy of Psychosomatic Medicine.
M.D.
PH.D.
psychologist. There is no evidence that speech therapy, pharmacotherapy, or surgery will totally eliminate the phenomena. Case Report This 48-year~ld man had a three-year history of speech problems consisting of sudden spasms in his neck and throat, often triggered by even a brief (3Q..second) period of normal speech. Because of his complaint about speech he had had surgery for a benign thyroid nodule two years earlier. When he spoke there was a feeling of neck strain; if he continued talking, his speech seemed forced. This could be partially overcome by arching his head backward, and he could produce a whisper by rais-
PSYCHOSOMATICS
Case Reports
ing the pitch of his voice. There was little or no secondary relationship to stress, and the process was present when he was alone or with others. He had no writer's cramp, no history to suggest MS or other neurologic disorders, and no progressive rigidity or stiffness. There was no blepharospasm and he had never been depressed. He had never received neuroleptics. Examination, including careful neurologic examination and direct inspection of the vocal cords, was entirely normal. CT examination, EMG, and CSF were all normal. A trial of baclofen, alprazolam, trihexyphenidyl, and various antidepressants or anticonvulsants was without major benefit. One year later he again received trihexyphenidyl, the most useful of the drugs, and at a dose of 15 mg per day he was significantly improved.
Discussion The characteristic feature of each of the four cases we have seen was severe paroxysmal facial dystonia in a middle-aged male who manifested no other extrapyramidal features. The dystonia was triggered by speech and resulted in pulling of the mouth and chin down or to one side. Speech became high pitched at the time of the spasm, and prior to the dystonic spasms, the patient noticed a tight feeling in his mouth. There was major involvement of the lower half of the face and of the anterior cervical muscles and oral musculature, but the upper face was not affected. Sixty seconds of speech were sufficient to trigger the spasms; they immediately ceased when the patient stopped talking. The higher voice ranges were affected differently than lower ranges, and a whistle or a whisper did not always trigger the reaction. None of the patients had overt psychiatric disease. There was no evidence that anticholinergics, minor anxiolytics, butyrophenones, or sedation provided significant benefit, but massive doses of anticholinergics were mildly helpful. In contrast to essential tremor which is ameliorated by alcohol,' alcohol had no major effect, good or bad. Dystonia includes several varieties of sustained postural abnormality and can be associated with tremor or with choreoathetosis. The classical form of dystonia, dystonia muscuVOLUME 29 • NUMBER 2. SPRING 1988
lorum deformans, is not usually accentuated by an effort to speak and does not involve the facial musculature more than the limbs. Some patients with dystonic movement and patients with athetosis may have an overflow or spread of movements to the face when there is major effort or with "action dystonia." Genetically, dystonia musculorum deformans may be either recessive or dominant, but many sporadic cases are seen. 2 In our cases with oral facial dystonia, we observed no obvious inherited tendency. In addition to the heritable dystonias, numerous fragmentary or focal dystonias have been described by Marsden,3 who might categorize the condition we are describing as a laryngo-pharyngeal dystonia. The fragmentary dystonia do fall in the borderland between psychiatry and neurology, as is true with writer's cramp, and some are associated with an incessant mouth movement reminiscent of tardive dyskinesia. Most fragmentary dystonias are probably organic and portions of a larger spectrum; all respond poorly to therapy.· Other forms of the fragmentary dystonias may come to the attention of physicians and speech pathologists. Meige's syndrome includes mouth movements and blepharospasm, and begins in the middle years of life. 5•6 This well-described entity responds poorly to therapy, may slowly worsen, and is not simply psychogenic blepharospasm. In patients with Meige's syndrome, the chin often thrusts forward or moves from side to side, the eyes close forcefully at the same time, and the individual may be severely incapacitated because of transient inability to see. Rarely these patients will also complain of interference with speech. The movements and the eye blinks of Meige's syndrome can be blocked transiently by whistling or by speech, as if by distraction or increased neuronal inhibition. Such cases of blepharospasm clearly differ from tardive dyskinesia in appearance. Tardive dyskinesia/· K which usually follows prolonged use of phenothiazines, does include a tendency to exhibit a writhing tongue or puckering mouth movements, but it rarely affects speech. Spastic dysphonia, another cause for a "tight voice," is distinct from 137
Case Reports
oral facial dystonia, and in most patients there is no apparent tightness of the facial musculature. The cause of spastic dysphonia is uncertain, and some cases do present extrapyramidal features in other portions of the body. There are conditions that can be interpreted as "cross-talk," an uncontrollable or inappropriate spread of impulses within the nervous system. This does not refer to ephaptic spread, but to common phenomena such as a peculiar tingling sensation in the flank when an individual has actually scratched a different place in the body. Such cross-talk may be thought of as akin to faintly overhearing (because of spread in the wires) a different conversation while on the telephone. It is certainly true, with or without the cross-talk phenomena, that inappropriate spread can occur within the nervous system. For example, there are familial or idiopathic mirror movements in which one hand moves in a mirror image of the movements of the other hand. The mechanism of such motor or sensory spread remains unclear. At times, distinct clinical entities do result from local irritation and instability in select areas, e.g., hemifacial spasm or trigeminal neuralgia. 9 As is true in many of the phenomena described above, the mechanism of the oral facial dystonia seen in our patients remains obscure. Numerous drugs, including butyrophenones, anticholinergics, and sedatives, have been tried for these patients. None have been dramatically helpful. Some of the patients do learn to
partially control the movements, usually by frequent stops during conversations, but they usually cannot control the dystonia completely. Because the basic voice is usually normal in our patients and because spastic dysphonia is not usually associated with the bizarre muscle movement of the neck and chin, oral facial dystonia can be distinguished from spastic dysphonia. At this time we not only lack an explanation, we also lack a proper name for this condition. "Oral facial dystonia triggered by speech" is clumsy, and oral mandibular dystonia or laryngo-pharyngeal dystonia are also unsatisfactory. Currently, we feel that this difficult condition is best considered as a "focal" or "fragmentary" dystonia. The most effective approach for managing these patients is to offer a trial of anticholinergics, administered with a slow increase. Butyrophenones may also help, but levodopa is not likely to be effective. Speech therapy may help to control overflow phenomena, and this can facilitate communication. Some patients will need therapy for depression. A more complete pathologic explanation and better therapy are needed. There is no reason to believe the phenomenon is primarily psychogenic. Generally speaking, information about the pathology of each of the dystonias is limited. There are, in contrast to the movement disorder of Parkinson's disease, no completely accepted biochemical or pathologic concomitants of dystonia. More research is needed to address these issues.
References
I. Larsen TA, Caine DB: Essential tremor. Clin Neuropharmacol 6: 185-206, 1983 2. Zeman W: Dystonia: an overview. Adv Neurol 14:91-103, 1976 3. Marsden CD: Dystonia: the spectrum of the disease, in, Yahr, MD, The Basal Ganglia. New York, Raven Press, 1976, pp 351-367 4. Paulson GW: Torsion dystonia, in Johnson RT, Mosby CV (eds): Current Therapy in Neurologic Disease. St. Louis, CV Mosby Co, 1985, pp 264266 5. Duvoisin RC: Meige syndrome: relief on high-dose anticholinergic therapy. Clin Neuropharmacol
6:63-66, 1983 6. Tolosa ES, Lai CW: Meige disease: striatal dopaminergic preponderance. Neurology 29: 11261130,1979 7. Goetz CG, Klawans HL: Tardive dyskinesia. Neurol Clin 2:605-614, 1984 8. Paulson GW: Tardive dyskinesia. Annu Rev Med 26:75-81,1975 9. Cook BR, Janetta PJ: Tic convulsive: results in II cases treated with microvascular decompression of the fifth and seventh cranial nerves. J Neurosurg 61:949-955, 1984
PSYCHOSOMATICS