Movement Disorders Vol. 5, No. 2, 1990, pp. 127-133 0 1990 Movement Disorder Society
Psychogenic Dyskinesias in Patients with Organic Movement Disorders Ranjit Ranawaya, David Riley, and Anthony Lang Movement Disorders CliniclToronto Western Hospital, 25 Leonard Avenue, Suite 101, Toronto, Ontario, Canada
Summary: The association of psychogenic neurological features with organic neurological disease is commonly acknowledged. However, the occurrence of psychogenic dyskinesias in patients with underlying organic movement disorders is not well recognized. Six cases of psychogenic dyskinesias complicating preexisting organic movement disorders are described. This possibility must be carefully considered and excluded before an unusual movement disorder can be considered entirely psychogenic. Key Words: Dyskinesias, psychogenicDystonia-Tremor.
It is well recognized that patients with organic neurological disease may demonstrate additional “functional” neurological signs and symptoms. These features complicate their investigation, diagnosis, and management. Possibly the best-known example of this association is the occurrence of “pseudoseizures” accompanying a true seizure disorder (1). However, it is not generally appreciated that psychologically based movement disorders may become superimposed on preexisting true organic movement disorders. Experience with six such patients reported here indicates that when faced with the presence of a bizarre and obviously “functional” dyskinesia, the possibility must be considered that a true “organic” movement disorder has been embellished or enhanced by the presence of underlying psychopathology.
tremor of the hands and a 3-year history of involuntary lip-smacking movements. She had a history of migraine for which she had received propranolol and pizotyline, and she also took conjugated estrogen for postmenopausal symptomatology . There had been no history of neuroleptic drug use. On examination there was an intermittent side-toside head tremor with less frequent side-to-side rocking head movements of larger amplitude. At times the head was held in a retrocollic posture, with the chin deviated to either shoulder, more commonly to the right. The head tremor and rocking increased with concentration on various tasks. There was frequent lip-smacking and she tended to voluntarily clench the teeth and talk through a strained mouth to overcome the lip and jaw movements. There was a low-amplitude postural and action tremor of the hands. Otherwise, results of the neurological examination were normal. Treatment with lorazepam, reserpine, and withdrawal of the estrogen compound failed to lessen the facial and head movements. She continued to have periodic “spells” of generalized body shaking unwitnessed in the office, and lorazepam was found to shorten these episodes. While in the hospital for an unrelated problem she was finally witnessed to have one of the shaking spells, during which there was pronounced side-to-side, one to two per second, head
CASE 1 A 72-year-old woman was first seen in 1984 complaining of occasional spells of generalized shaking that lasted 2 4 h. These were superimposed on a 4-year history of head tremor and mild action
Address correspondence and reprint requests to Dr. A. Lang, Movement Disorders Clinic/Toronto Western Hospital, 25 Leonard Avenue, Suite 101, Toronto, Ontario, Canada MST 2R2.
127
128
R . RANAWAYA ET AL.
rocking, with additional forward and backward shoulder movements and arm jerking. The lipsmacking movements were also increased at this time. She was awake and alert throughout the “spell.” Concentration on various tasks caused the new head, shoulder, and arm movements to dampen and she could also hold them in check for a short period when asked to do so. Injection of normal saline resulted in an immediate resolution of these more severe movements but had no effect on her underlying head titubation and facial dyskinesia. Continuous oral placebo therapy lessened the frequency of her episodic shaking, and her family physician successfully used p a r e n t e d placebo (normal saline) when these paroxysms recurred. CASE 2 A 42-year-old woman was well until the age of 35, when she developed pain in the back of the neck, followed several months later by involuntary pulling of the head to the right. She was diagnosed as having spasmodic torticollis in 1980. An accessory nerve block provided only partial relief and surgery was not undertaken. She remained unchanged until early 1982, when she abruptly developed sideto-side head shaking that remained for several weeks and then subsided spontaneously, leaving her with the previous torticollis. Again, late in 1982, she had a similar exacerbation of head shaking. This subsided within 3 days of treatment with haloperido1 0.25 mg b.i.d. and cyclobenzaprine, without return on discontinuation of these drugs. In March 1983, she again had a spontaneous development of head shaking and within hours of taking a single dose of haloperidol, these movements again subsided, only to return several weeks later with a further resolution minutes after taking a dose of haloperidol. Between then and 1987 she experienced similar episodes, some lasting several days, others only minutes to hours. Each of these would completely resolve, leaving the underlying torticollis. More recent episodes failed to improve with low doses of haloperidol. She was otherwise well aside from multiple unrelated somatic complaints. A younger brother was said to have excessive eye blinking as a child. During the course of the 4 years that she was followed, the most consistent abnormality on examination was typical intermittent dystonic pulling of the chin up and to the right, which she was able to control with minimal contact of the hand to the right
Movement Disorders, Vol. 5 , No. 2 , 1990
side of the chin or left side of the face. There were superimposed additional intermittent rapid jerking movements of the head to the left. During an Amytal (amobarbital) interview no change in the movements or neck posture was apparent. She showed evidence of a state of chronic tension related to financial stresses but no other psychopathology. During an exacerbation of more severe “shaking” there was rapid side-to-side movement of the head, which varied in amplitude and frequency. Often the movement spanned the full range of lateral rotation, with side-to-side rapid movements >60° back and forth alternatively to the right and left of the midline. Occasionally the head was held to one side or the other for a few seconds. While concentrating on various tasks, the head shaking often decreased or completely subsided. The head shaking stopped during the act of writing a word and returned between words when the pen was lifted off the page. While performing finger-to-nose testing the head would shake rapidly and wildly from side to side throughout the activity except on touching the nose, at which time the movement would stop completely. Voluntarily holding the head to one extreme or the other caused the shaking to abate. The patient denied the feeling of an urge to perform the movement or other premonitory sensation, sense of relief after the movement, or any degree of voluntary control. The remainder of her neurological examination was normal. Her underlying spasmodic torticollis failed to respond to a variety of medications including anticholinergics and benzodiazepine drugs, carbamazepine, and haloperidol.
CASE 3 A 71-year-old woman complained of tremor and a gait disturbance. Her husband had noted a mild tremor in her hands and head for 1-2 years although the patient had not been aware of it. Her grandmother had severe hand and head tremor without features of Parkinson’s disease. The patient was otherwise well until she was involved in a motor vehicle accident in January 1986, in which she sustained a whiplash injury but no significant head trauma. This was followed by neck pain that later involved the shoulders and back. Two months after the accident she suddenly developed stuttering speech, a rubbery, limp feeling in the legs, and paroxysmal generalized whole body “tremors.” Because of increasing tremor she was admitted to the
PSYCHOGENIC D YSKINESIAS IN ORGANIC MOVEMENT DISORDERS hospital. A psychosomatic disorder was considered and trifluoperazine therapy resulted in restlessness and parkinsonism. Subsequently, carbidopal levodopa (Sinemet), benztropine, and bromocriptine were tried unsuccessfully. Her severe generalized body “tremors” gradually improved, leaving her with constant less severe body “shaking” and inability to walk. The shaking involved the entire body, particularly the head and abdominal muscles. It would improve on lying down, She was aware of milder shaking of her hands, which was not disabling. Self-care tasks were only impaired by the more generalized “shaking.” A gait disorder developed while she was in the hospital and progressed to the point that she could barely support herself. By mid June 1986 she required the use of a wheelchair. She had a multitude of other unrelated somatic complaints. Litigation for the motor vehicle accident was still pending. Examination in October 1986 showed a rhythmical tremor of the head in both the side-to-side and anteroposterior directions as well as a bobbing tremor of the trunk and abdominal muscles, which at times reached marked proportions. Her voice shook with the proximal trunk tremor. There was a proximal postural tremor of the arms that increased distally with action. This increase was very variable, at times being most prominent with the finger toward the nose and at other times reaching the examiner’s finger. On bringing a cup to the mouth, the trunk tremor was most noticeable, but there was only minimal arm tremor. Very little tremor was seen in her writing. On performance of various tasks, the abdominal tremor behaved unpredictably. At times, it would abate completely and at other times would shake between the movements that she was concentrating on. On lying down, the tremor subsided completely for variable periods of time. At this time, she briefly developed a lowamplitude parkinsonian tremor of the right thumb and index finger. However, this dampened considerably when she performed tasks with the left hand. She had difficulty rising from a chair due to a pronounced increase in the trunk shaking. She tended to stand with her knees and hips flexed, occasionally rocking side to side with the right foot inverted. Shaking increased with attempted walking and the legs were held stiffly and dragged across the floor. The flexed posture of the knees and hips was maintained throughout walking but despite this, as well as histrionic swaying from side to side and back-
129
ward stumbling, she never fell. Results of the remainder of the examination were normal aside from giving way on testing of hip flexor strength. CASE 4 A 53-year-old woman had a long-standing history of psychiatric problems including depression and preoccupation with an alleged sexual assault at the age of 24 that occurred under “involuntary” hypnosis. There was also a history of repeated parental physical abuse as a child. She first experienced abnormal movements during the second trimester of her first pregnancy. These comprised spasmodic contractions of her upper and lower limbs. These symptoms abated with delivery. From her description, the movements were similar to those that occurred subsequently. During her second pregnancy 2 years later, she had a miscarriage and claimed that recurrent involuntary movements were a contributing factor. Again, these were paroxysmal twitching and jerking movements of the arms and legs that occurred for several moments at a time. She had undergone previous psychiatric admissions for depression and continuing preoccupation with the events encountered at the age of 24. Three years before our assessment she underwent a further course of hypnotherapy during which time she developed involuntary pulling of the head to the right (torticollis). There was a questionable history of less marked pulling and abnormal posturing that had existed for some 10 years before this. Some time after the development of the more pronounced neck posturing, she developed a recurrence of rapid jerking movements of her limbs, especially her arms. These occurred primarily on lying in bed, and prevented sleep. Although the torticollis markedly lessened in the supine position, lying down also resulted in side-to-side head jerking in addition to the abnormal arm movements. Both forms of movement disorder (torticollis and paroxysmal head and arm movements in the supine position) were bothersome and disabling, the movements in bed possibly most of all, because of their interference with sleep. She was in the process of a litigation procedure against the hypnotherapist who was treating her when the recent exacerbation of abnormal movements began. Psychiatric examination revealed some evidence of depression as well as constant preoccupation with the assault at the age of 24 and the insistent belief that her present symptomatology had been
Movement Disorders, Vol. 5 . N o . 2, 1990
130
R . RANAWAYA ET AL.
caused by hypnotherapy. She demonstrated intermittent deviation of the chin toward the right shoulder, which could voluntarily be returned to the midline for short periods. There was hypertrophy of the left sternocleidomastoid muscle. On assuming the supine position, the more sustained contractions of the left sternocleidomastoid muscle lessened and she could maintain the head in the midposition for prolonged periods. However, at this time she developed a series of bizarre abnormal movements. Initially she showed a slow repetitive shoulder elevation accompanied by right arm hyperpronation with subsequent return to the relaxed supine position as the shoulder fell. These movements were followed 30-60 s later by rapid, simultaneous, bilateral shoulder shrugging ( 1 4 Hz) and arm pronation, then by a rotatory flinging of the head from one side to the other (1-2 Hz) accompanied by alternating shoulder shrugging. After a further 30-60 s, the pattern of movements changed again to maintenance of the head to the extreme right or left side accompanied by ipsilateral upward shoulder jerks (1-4 Hz) to the chin, followed by a slow rotation (0.3 Hz) of the head from one side to the other, often with a rachetlike or cogwheel component. She maintained her eyes closed throughout but was fully alert and was able to speak; however, the movements would abate if she was participating in any conversation. All of these movements subsided immediately on sitting up and the more tonic deviation of the head to the right returned again. CASE 5 A 23-year-old woman was first seen in 1985 complaining of involuntary movements of her neck. Nine years previously she had a hyperextension injury to her neck, resulting in severe pain in the neck and shoulders, treated with simple analgesic medications and a collar. During the next month she developed involuntary “flopping” of her head forward and side to side. She described her head pulling in the opposite direction to that which she wished to direct it. She found it impossible to hold her head up and frequently would use her hands over the forehead and chin to keep it steady. The neck movements had progressed to their maximum severity during a 1-month period and had remained unchanged since then. Various therapies had been tried including physiotherapy, traction, ultrasound, heat packs, and chiropractic, without benefit. She had a long history of psychiatric illness with multiple admissions for depression. She had under-
Movement Disorders, Vol. 5 , No. 2 , 1990
gone two surgical procedures for abdominal pain and was subsequently diagnosed as having irritable bowel syndrome. She had noted a mild postural tremor for many years. A maternal aunt was said to have torticollis. Various medications had been prescribed for short periods for her depression and neck problems. These included lithium, doxepin, trazodone, haloperidol, and chlorpromazine. Pimozide was used for 5 years without benefit. In November 1984, transcutaneous electrical nerve stimulation (TENS) resulted in immediate and marked reduction in the abnormal movements. On examination with the TENS activated, she showed occasional anterior flexion movements of the neck with her head slightly tilted and turned toward the right shoulder. There was a mild postural tremor of the outstretched hands. The rernainder of the neurological examination was normal. When she turned the TENS off there was an immediate change in her status. The neck began to rock from side to side, particularly with anterocollis, as well as tortional movements with the chin to either shoulder, the movements being either slow or more rapid and jerky. These movements interfered with walking and writing. She made frequent grimacing movements in attempting to control the neck. When she switched the TENS on, again there was an immediate change. The neck stopped rocking and within 2-5 s she was able to lift her head straight and hold it without the rocking movements, although there were occasional anterocollic movements as before. As the neck rocking subsided she would regularly breathe a loud sigh of relief. Examination 2 years later again revealed intermittent anterocollis with additional turn and tilt to the right. She regularly held her hand to her face or behind her neck (geste antagoniste) to control these movements. The more variable and bizarre head rocking and jerking was less severe than when first seen and she no longer required the use of TENS. However, she now demonstrated intermittent inversion of the right foot and claw-like posturing of the hands with the thumb held into the palm. CASE 6 In 1983, a 28-year-old man had a sudden onset of tremor in the right hand, initially at rest and later with action. In mid-1985, he had a severe depression requiring hospitalization and treatment with a variety of drugs including neuroleptic and tricyclic antidepressant drugs. He believed that the tremor worsened with these agents. The tremor caused
PSYCHOGENIC D YSKINESIAS IN ORGANIC MOVEMENT DISORDERS spillage and sloppy handwriting. On direct questioning, he also admitted to involuntary excessive tightening of his hand on the pen while writing, which antedated the use of any neuroleptic agents. At the time of the interview, he was taking imipramine 50 mg t.i.d. and butalbital (Fiorinal) for migraine. He took benztropine intermittently, which he had found to have a mild dampening effect on the tremor. Family history and the remainder of the functional inquiry were negative. On examination, there was a subjective reduction in all sensory modalities on the right side of the body, including vibration over the forehead and mediastinum. There was an intermittent, variable amplitude tremor in the right arm, which was present particularly when his attention was drawn to that limb. With any form of mental or physical distraction, the tremor subsided completely. The tremor was present at rest. With the arms outstretched, there was a similar low-amplitude pronation/ supination tremor. The postural tremor subsided completely with distraction as well as the performance of finger-to-nose testing with that hand. The tremor was evident on holding a full cup of water outstretched but again, subsided with any distraction. It began immediately when the distracting maneuvre (e.g., finger tapping with the left hand) was stopped. There was no increase in tremor at the endpoints of holding a cup out or bringing it to the mouth. There was no tremor on writing. However, he held the pen tightly, the script became more cramped as he continued, and he complained of excessive tightness in the hand. Investigation results, including a slit-lamp examination, were entirely normal. He was reassured and during the next 6 months he had a marked spontaneous improvement in the tremor. He continued to experience writing difficulties and examination once again revealed typical features of writer’s cramp. DISCUSSION In the same fashion that “functional” neurological signs and symptoms may be superimposed on organic neurological disease, these six patients had “psychogenic dyskinesias” complicating a preexisting organic movement disorder. Three patients (cases 2, 4,5 ) had typical dystonic spasmodic torticollis, one (case 6) had writer’s cramp, three (cases 1,3,5) had essential tremor (familial in one), and one of these (case 1) had orofacial dyskinesia, probably a form of cranial dystonia (Meige’s syn-
131
drome) combined with mild retrocollis. One patient (case 4) had probably had similar psychogenic dyskinesias earlier in life before the development of the torticollis, but the most recent exacerbation occurred after the onset of torticollis. In one patient (case 9, the psychogenic dyskinesia seems to have begun at approximately the same time as her underlying movement disorder. In the remaining patients the organic dyskinesias had been present for variable periods (usually several years) before the onset of the psychogenic movement disorder. In five patients (cases l , 2, 4, 5 , 6), the new movement disorder was phenomenologically different from the original dyskinesia, whereas in only one (case 3) was it similar (i.e., tremor). The psychogenic dyskinesias usually affected the same part of the body as the underlying organic movement disorder did as well as contiguous regions not originally involved. In these patients it was the new psychogenic dyskinesia that caused the greatest concern and disability whereas the original movement disorder was usually tolerable. In two patients (cases 3 and 6), the underlying movement disorder had been completely overlooked by the patients and the referring physicians. The new movement disorders were considered psychogenic because of associated historical, clinical, and behavioral features and the response to suggestion and/or placebo therapy. When there was any doubt about the “functional” nature of these movements, patients were followed long enough to document further supportive evidence of this diagnosis. This evidence was usually the behavior of the movement disorder that was inconsistent or incongruous with other known (organic) dyskinesias. In reviewing the concept of psychogenic dystonia, Fahn and Williams (2) suggested criteria for subdividing patients into categories based on certainty of diagnosis. These included (a) documented psychogenic dystonia, (b) clinically established psychogenic dystonia, (c) probable psychogenic dystonia, and (d) possible psychogenic dystonia. We would favor expanding the application of these authors’ criteria (2) to the diagnosis of psychogenic movement disorders in general, with one major qualification, supported by the experience reported here. In the “probable” and “possible” categories, Fahn and Williams include patients in whom the movements are consistent and congruent with the classic movement disorder but who have other neurological signs that are definitely psychogenic (e.g., false weakness, false sensory findings) or who have
Movement Disorders, Vol. 5 , No. 2 , 1990
132
R . RANAWAYA ET AL. TABLE 1. Patient characteristics
Case
Age
Sex
Underlying dyskinesia
Psychogenic dyskinesia
Confirming features
Additional “functional” neurology
Psychiatric features
Level of certaintyn
Head, shoulders, arm rocking
Placebo
Anxiety, multiple somatic complaints
Documented
Paroxysmal side-to-side head shaking
Distractability, incongruity, immediate (minutes) response to haloperidol
Chronic anxiety
Essential tremor
Generalized whole-body tremors (initially paroxysmal)
Incongruity
Clinically established (“Documented” if immediate response to haloperidol is accepted as placebo) Clinically established
Essential tremor, facial dyskinesia, retrocollis Spasmodic torticollis
Psychogenic gait disturbance
4
53
F
Spasmodic torticollis
Paroxysmal head, trunk, arm rocking
Incongruity, present only when lying down
5
23
F
Spasmodic torticolli s, essential tremor
Head rocking
Incongruity, abrupt and striking improvement with TENS
6
28
M
Writer’s cramp
Tremor
Incongruity, distractability, spontaneous improvement
Right hemisensory loss
Post MVA, litigation pending, multiple somatic complaints Long-standing depression, personality disorder, litigation pending Long-standing depression
Long-standing depression
Clinically established
Clinically established (“Documented” if immediate response to TENS is accepted as placebo) Clinically established
Applying criteria of Fahn and Williams (2). TENS, transcutaneous electrical nerve stimulation; MVA, motor vehicle accident. a
multiple somatizations. Thus the criteria, as they stand, do not allow for the possible coexistence of organic and psychogenic conditions. Applying these criteria rigidly, we would have diagnosed our patients as having only psychogenic dyskinesias. Our experience emphasizes that organic and psychogenic movement disorders are not mutually exclusive. Indeed, 1 of the 21 cases of Fahn and Williams (case 5) had underlying idiopathic torsion dystonia before presenting with new symptoms which were an embellishment of his dystonia associated with pain. These latter symptoms were documented to be psychogenic. Taking into account this disagreement in diagnostic criteria, all of the patients reported here would otherwise (i.e., distinct from their underlying organic movement disorder) be classified as having “documented” or “clinically established” psychogenic dyskinesias. Criteria for ‘’documented psychogenic dyskinesias” would require that the abnormal movements be persistently relieved by psychotherapy, by the
Movement Disorders, Vol. 5, No. 2 , 1990
clinician using psychological suggestion including physiotherapy, or by the administration of placebos. “Clinically established psychogenic dyskinesias” are inconsistent over time or are incongruent with “organic” movement disorders. In addition, patients demonstrate one or more of the following: other neurological signs that are definitely psychogenic, multiple somatizations, and an obvious psychiatric disturbance. The bizarre, inconsistent, and rapidly changing nature of many of the movements documented in our patients were quite incongruous with “organic” dyskinesias. Resolution of the movement disorder with distraction or concentration on a task (e.g., sequential finger tapping, mental arithmetic) is a useful clinical feature, although we have not taken this behavior in isolation to indicate a diagnosis of psychogenic dyskinesia. In our experience, tics and akathitic movements frequently (if not always) behave in the same fashion whereas other dyskinesias typically worsen or remain unchanged. Patient 2 gave a family history
PSYCHOGENIC D YSKtNESIAS IN ORGANIC MOVEMENT DISORDERS
compatible with a tic disorder; however, her abnormal movements were clinically atypical and lacked additional subjective features of tics. As indicated in Table 1, using the criteria outlined above, case 1 could be classified as “documented,” cases 3, 4, and 6 could be classified as “clinically established,” and cases 2 and 5 could be classified as either, depending on whether or not one accepts their respective responses to haloperidol (resolution in minutes) and TENS as comparable to placeboinduced benefit. We have seen several other patients who represent examples of “probable” and “possible” psychogenic dyskinesias combined with organic movement disorders but have chosen to exclude them from this report because of diagnostic uncertainty. In four patients, the psychogenic dyskinesia was originally paroxysmal. In one, the paroxysms resolved, leaving a persistent, less severe psychogenic dyskinesia. The paroxysmal nature of abnormal movements should serve as a possible clue to a psychogenic origin. Bressman et al. (3) have reported that 11 of their 25 patients with paroxysmal nonkinesigenic dystonia were psychogenic, and 7 of Fahn and Williams’ 21 patients with psychogenic dystonia had paroxysmal movements (2). Obviously other “organic” causes of paroxysmal dyskinesias (4) must be excluded before a primary psychogenic source can be accepted. The underlying psychological disturbances in our patients are listed in Table 1. There were no uniform or consistent psychiatric abnormalities in this group. Litigation was pending in two patients (cases 3 and 4) and additional long-standing psychopathology was evident in one of these. Two patients had multiple somatic complaints, one with associated depression. Of note is the lack of behavioral characteristics typical of the “histrionic” or “hysterical” personality. This concurs with the general ex-
133
perience in psychogenic neurological disorders, in which -80% lack the “hysterical” personality (5). Psychogenic movement disorders are not common in terms of percentage of patients seen with organic movement disorders. In Fahn and Williams’ experience with a total of 793 patients with idiopathic dystonia, only 21 patients had documented and clinically established psychogenic dystonia, representing only 2.6% of the dystonia population. If 18 patients with probable and possible psychogenic dystonia are added to the above, the final figure is 4.6% (2). In Marsden’s experience with 400 patients with various forms of dystonia, only 5 (1.25%) cases had a confident diagnosis of hysteria (5). We estimate that approximately 1&15% of all the patients we diagnosed as having a psychogenic dyskinesia have had evidence of an additional underlying organic movement disorder. This experience is comparable to a 10-37% incidence of true epilepsy in patients with pseudoseizures (1,6). Hence, we would caution physicians not to overdiagnose psychogenic movement disorders and emphasize the need to carefully consider the possibility of an additional underlying “organic” movement disorder when faced with a bizarre psychogenic dyskinesia. REFERENCES 1 . Lesser RP, Lueders H, Dinner DS. Evidence for epilepsy is rare in patients with psychogenic seizures. Neurology 1983;33:502-504. 2. Fahn S, Williams DT. Psychogenic dystonia. Adv Neurol 1988;50:431 4 5 . 3 . Bressman SB, Fahn S , Burke RE. Paroxysmal nonkinesigenic dystonia. Adv Neurol 1988;50:403-413. 4. Weiner WJ, Lang AE. Movement disorders: a comprehensive survey. New York: Fntura Press, 1989. 5 . Marsden CD. Hysteria-a neurologist’s view. Psycho/ Med 1986;16277-288. 6. Krumholz A, Niedermeyer E. Psychogenic seizures: a clinical study with follow-up data. Neurology 1983;33:498-502.
Movement Disorders, Vol. 5 , N o . 2 , 1990
Psychogenic Dyskinesias in Patients with Organic Movement Disorders Ranjit Ranawaya, David Riley, and Anthony Lang Movement Disorders CliniclToronto Western Hospital, 25 Leonard Avenue, Suite 101, Toronto, Ontario, Canada
Summary: The association of psychogenic neurological features with organic neurological disease is commonly acknowledged. However, the occurrence of psychogenic dyskinesias in patients with underlying organic movement disorders is not well recognized. Six cases of psychogenic dyskinesias complicating preexisting organic movement disorders are described. This possibility must be carefully considered and excluded before an unusual movement disorder can be considered entirely psychogenic. Key Words: Dyskinesias, psychogenicDystonia-Tremor.
It is well recognized that patients with organic neurological disease may demonstrate additional “functional” neurological signs and symptoms. These features complicate their investigation, diagnosis, and management. Possibly the best-known example of this association is the occurrence of “pseudoseizures” accompanying a true seizure disorder (1). However, it is not generally appreciated that psychologically based movement disorders may become superimposed on preexisting true organic movement disorders. Experience with six such patients reported here indicates that when faced with the presence of a bizarre and obviously “functional” dyskinesia, the possibility must be considered that a true “organic” movement disorder has been embellished or enhanced by the presence of underlying psychopathology.
tremor of the hands and a 3-year history of involuntary lip-smacking movements. She had a history of migraine for which she had received propranolol and pizotyline, and she also took conjugated estrogen for postmenopausal symptomatology . There had been no history of neuroleptic drug use. On examination there was an intermittent side-toside head tremor with less frequent side-to-side rocking head movements of larger amplitude. At times the head was held in a retrocollic posture, with the chin deviated to either shoulder, more commonly to the right. The head tremor and rocking increased with concentration on various tasks. There was frequent lip-smacking and she tended to voluntarily clench the teeth and talk through a strained mouth to overcome the lip and jaw movements. There was a low-amplitude postural and action tremor of the hands. Otherwise, results of the neurological examination were normal. Treatment with lorazepam, reserpine, and withdrawal of the estrogen compound failed to lessen the facial and head movements. She continued to have periodic “spells” of generalized body shaking unwitnessed in the office, and lorazepam was found to shorten these episodes. While in the hospital for an unrelated problem she was finally witnessed to have one of the shaking spells, during which there was pronounced side-to-side, one to two per second, head
CASE 1 A 72-year-old woman was first seen in 1984 complaining of occasional spells of generalized shaking that lasted 2 4 h. These were superimposed on a 4-year history of head tremor and mild action
Address correspondence and reprint requests to Dr. A. Lang, Movement Disorders Clinic/Toronto Western Hospital, 25 Leonard Avenue, Suite 101, Toronto, Ontario, Canada MST 2R2.
127
128
R . RANAWAYA ET AL.
rocking, with additional forward and backward shoulder movements and arm jerking. The lipsmacking movements were also increased at this time. She was awake and alert throughout the “spell.” Concentration on various tasks caused the new head, shoulder, and arm movements to dampen and she could also hold them in check for a short period when asked to do so. Injection of normal saline resulted in an immediate resolution of these more severe movements but had no effect on her underlying head titubation and facial dyskinesia. Continuous oral placebo therapy lessened the frequency of her episodic shaking, and her family physician successfully used p a r e n t e d placebo (normal saline) when these paroxysms recurred. CASE 2 A 42-year-old woman was well until the age of 35, when she developed pain in the back of the neck, followed several months later by involuntary pulling of the head to the right. She was diagnosed as having spasmodic torticollis in 1980. An accessory nerve block provided only partial relief and surgery was not undertaken. She remained unchanged until early 1982, when she abruptly developed sideto-side head shaking that remained for several weeks and then subsided spontaneously, leaving her with the previous torticollis. Again, late in 1982, she had a similar exacerbation of head shaking. This subsided within 3 days of treatment with haloperido1 0.25 mg b.i.d. and cyclobenzaprine, without return on discontinuation of these drugs. In March 1983, she again had a spontaneous development of head shaking and within hours of taking a single dose of haloperidol, these movements again subsided, only to return several weeks later with a further resolution minutes after taking a dose of haloperidol. Between then and 1987 she experienced similar episodes, some lasting several days, others only minutes to hours. Each of these would completely resolve, leaving the underlying torticollis. More recent episodes failed to improve with low doses of haloperidol. She was otherwise well aside from multiple unrelated somatic complaints. A younger brother was said to have excessive eye blinking as a child. During the course of the 4 years that she was followed, the most consistent abnormality on examination was typical intermittent dystonic pulling of the chin up and to the right, which she was able to control with minimal contact of the hand to the right
Movement Disorders, Vol. 5 , No. 2 , 1990
side of the chin or left side of the face. There were superimposed additional intermittent rapid jerking movements of the head to the left. During an Amytal (amobarbital) interview no change in the movements or neck posture was apparent. She showed evidence of a state of chronic tension related to financial stresses but no other psychopathology. During an exacerbation of more severe “shaking” there was rapid side-to-side movement of the head, which varied in amplitude and frequency. Often the movement spanned the full range of lateral rotation, with side-to-side rapid movements >60° back and forth alternatively to the right and left of the midline. Occasionally the head was held to one side or the other for a few seconds. While concentrating on various tasks, the head shaking often decreased or completely subsided. The head shaking stopped during the act of writing a word and returned between words when the pen was lifted off the page. While performing finger-to-nose testing the head would shake rapidly and wildly from side to side throughout the activity except on touching the nose, at which time the movement would stop completely. Voluntarily holding the head to one extreme or the other caused the shaking to abate. The patient denied the feeling of an urge to perform the movement or other premonitory sensation, sense of relief after the movement, or any degree of voluntary control. The remainder of her neurological examination was normal. Her underlying spasmodic torticollis failed to respond to a variety of medications including anticholinergics and benzodiazepine drugs, carbamazepine, and haloperidol.
CASE 3 A 71-year-old woman complained of tremor and a gait disturbance. Her husband had noted a mild tremor in her hands and head for 1-2 years although the patient had not been aware of it. Her grandmother had severe hand and head tremor without features of Parkinson’s disease. The patient was otherwise well until she was involved in a motor vehicle accident in January 1986, in which she sustained a whiplash injury but no significant head trauma. This was followed by neck pain that later involved the shoulders and back. Two months after the accident she suddenly developed stuttering speech, a rubbery, limp feeling in the legs, and paroxysmal generalized whole body “tremors.” Because of increasing tremor she was admitted to the
PSYCHOGENIC D YSKINESIAS IN ORGANIC MOVEMENT DISORDERS hospital. A psychosomatic disorder was considered and trifluoperazine therapy resulted in restlessness and parkinsonism. Subsequently, carbidopal levodopa (Sinemet), benztropine, and bromocriptine were tried unsuccessfully. Her severe generalized body “tremors” gradually improved, leaving her with constant less severe body “shaking” and inability to walk. The shaking involved the entire body, particularly the head and abdominal muscles. It would improve on lying down, She was aware of milder shaking of her hands, which was not disabling. Self-care tasks were only impaired by the more generalized “shaking.” A gait disorder developed while she was in the hospital and progressed to the point that she could barely support herself. By mid June 1986 she required the use of a wheelchair. She had a multitude of other unrelated somatic complaints. Litigation for the motor vehicle accident was still pending. Examination in October 1986 showed a rhythmical tremor of the head in both the side-to-side and anteroposterior directions as well as a bobbing tremor of the trunk and abdominal muscles, which at times reached marked proportions. Her voice shook with the proximal trunk tremor. There was a proximal postural tremor of the arms that increased distally with action. This increase was very variable, at times being most prominent with the finger toward the nose and at other times reaching the examiner’s finger. On bringing a cup to the mouth, the trunk tremor was most noticeable, but there was only minimal arm tremor. Very little tremor was seen in her writing. On performance of various tasks, the abdominal tremor behaved unpredictably. At times, it would abate completely and at other times would shake between the movements that she was concentrating on. On lying down, the tremor subsided completely for variable periods of time. At this time, she briefly developed a lowamplitude parkinsonian tremor of the right thumb and index finger. However, this dampened considerably when she performed tasks with the left hand. She had difficulty rising from a chair due to a pronounced increase in the trunk shaking. She tended to stand with her knees and hips flexed, occasionally rocking side to side with the right foot inverted. Shaking increased with attempted walking and the legs were held stiffly and dragged across the floor. The flexed posture of the knees and hips was maintained throughout walking but despite this, as well as histrionic swaying from side to side and back-
129
ward stumbling, she never fell. Results of the remainder of the examination were normal aside from giving way on testing of hip flexor strength. CASE 4 A 53-year-old woman had a long-standing history of psychiatric problems including depression and preoccupation with an alleged sexual assault at the age of 24 that occurred under “involuntary” hypnosis. There was also a history of repeated parental physical abuse as a child. She first experienced abnormal movements during the second trimester of her first pregnancy. These comprised spasmodic contractions of her upper and lower limbs. These symptoms abated with delivery. From her description, the movements were similar to those that occurred subsequently. During her second pregnancy 2 years later, she had a miscarriage and claimed that recurrent involuntary movements were a contributing factor. Again, these were paroxysmal twitching and jerking movements of the arms and legs that occurred for several moments at a time. She had undergone previous psychiatric admissions for depression and continuing preoccupation with the events encountered at the age of 24. Three years before our assessment she underwent a further course of hypnotherapy during which time she developed involuntary pulling of the head to the right (torticollis). There was a questionable history of less marked pulling and abnormal posturing that had existed for some 10 years before this. Some time after the development of the more pronounced neck posturing, she developed a recurrence of rapid jerking movements of her limbs, especially her arms. These occurred primarily on lying in bed, and prevented sleep. Although the torticollis markedly lessened in the supine position, lying down also resulted in side-to-side head jerking in addition to the abnormal arm movements. Both forms of movement disorder (torticollis and paroxysmal head and arm movements in the supine position) were bothersome and disabling, the movements in bed possibly most of all, because of their interference with sleep. She was in the process of a litigation procedure against the hypnotherapist who was treating her when the recent exacerbation of abnormal movements began. Psychiatric examination revealed some evidence of depression as well as constant preoccupation with the assault at the age of 24 and the insistent belief that her present symptomatology had been
Movement Disorders, Vol. 5 . N o . 2, 1990
130
R . RANAWAYA ET AL.
caused by hypnotherapy. She demonstrated intermittent deviation of the chin toward the right shoulder, which could voluntarily be returned to the midline for short periods. There was hypertrophy of the left sternocleidomastoid muscle. On assuming the supine position, the more sustained contractions of the left sternocleidomastoid muscle lessened and she could maintain the head in the midposition for prolonged periods. However, at this time she developed a series of bizarre abnormal movements. Initially she showed a slow repetitive shoulder elevation accompanied by right arm hyperpronation with subsequent return to the relaxed supine position as the shoulder fell. These movements were followed 30-60 s later by rapid, simultaneous, bilateral shoulder shrugging ( 1 4 Hz) and arm pronation, then by a rotatory flinging of the head from one side to the other (1-2 Hz) accompanied by alternating shoulder shrugging. After a further 30-60 s, the pattern of movements changed again to maintenance of the head to the extreme right or left side accompanied by ipsilateral upward shoulder jerks (1-4 Hz) to the chin, followed by a slow rotation (0.3 Hz) of the head from one side to the other, often with a rachetlike or cogwheel component. She maintained her eyes closed throughout but was fully alert and was able to speak; however, the movements would abate if she was participating in any conversation. All of these movements subsided immediately on sitting up and the more tonic deviation of the head to the right returned again. CASE 5 A 23-year-old woman was first seen in 1985 complaining of involuntary movements of her neck. Nine years previously she had a hyperextension injury to her neck, resulting in severe pain in the neck and shoulders, treated with simple analgesic medications and a collar. During the next month she developed involuntary “flopping” of her head forward and side to side. She described her head pulling in the opposite direction to that which she wished to direct it. She found it impossible to hold her head up and frequently would use her hands over the forehead and chin to keep it steady. The neck movements had progressed to their maximum severity during a 1-month period and had remained unchanged since then. Various therapies had been tried including physiotherapy, traction, ultrasound, heat packs, and chiropractic, without benefit. She had a long history of psychiatric illness with multiple admissions for depression. She had under-
Movement Disorders, Vol. 5 , No. 2 , 1990
gone two surgical procedures for abdominal pain and was subsequently diagnosed as having irritable bowel syndrome. She had noted a mild postural tremor for many years. A maternal aunt was said to have torticollis. Various medications had been prescribed for short periods for her depression and neck problems. These included lithium, doxepin, trazodone, haloperidol, and chlorpromazine. Pimozide was used for 5 years without benefit. In November 1984, transcutaneous electrical nerve stimulation (TENS) resulted in immediate and marked reduction in the abnormal movements. On examination with the TENS activated, she showed occasional anterior flexion movements of the neck with her head slightly tilted and turned toward the right shoulder. There was a mild postural tremor of the outstretched hands. The rernainder of the neurological examination was normal. When she turned the TENS off there was an immediate change in her status. The neck began to rock from side to side, particularly with anterocollis, as well as tortional movements with the chin to either shoulder, the movements being either slow or more rapid and jerky. These movements interfered with walking and writing. She made frequent grimacing movements in attempting to control the neck. When she switched the TENS on, again there was an immediate change. The neck stopped rocking and within 2-5 s she was able to lift her head straight and hold it without the rocking movements, although there were occasional anterocollic movements as before. As the neck rocking subsided she would regularly breathe a loud sigh of relief. Examination 2 years later again revealed intermittent anterocollis with additional turn and tilt to the right. She regularly held her hand to her face or behind her neck (geste antagoniste) to control these movements. The more variable and bizarre head rocking and jerking was less severe than when first seen and she no longer required the use of TENS. However, she now demonstrated intermittent inversion of the right foot and claw-like posturing of the hands with the thumb held into the palm. CASE 6 In 1983, a 28-year-old man had a sudden onset of tremor in the right hand, initially at rest and later with action. In mid-1985, he had a severe depression requiring hospitalization and treatment with a variety of drugs including neuroleptic and tricyclic antidepressant drugs. He believed that the tremor worsened with these agents. The tremor caused
PSYCHOGENIC D YSKINESIAS IN ORGANIC MOVEMENT DISORDERS spillage and sloppy handwriting. On direct questioning, he also admitted to involuntary excessive tightening of his hand on the pen while writing, which antedated the use of any neuroleptic agents. At the time of the interview, he was taking imipramine 50 mg t.i.d. and butalbital (Fiorinal) for migraine. He took benztropine intermittently, which he had found to have a mild dampening effect on the tremor. Family history and the remainder of the functional inquiry were negative. On examination, there was a subjective reduction in all sensory modalities on the right side of the body, including vibration over the forehead and mediastinum. There was an intermittent, variable amplitude tremor in the right arm, which was present particularly when his attention was drawn to that limb. With any form of mental or physical distraction, the tremor subsided completely. The tremor was present at rest. With the arms outstretched, there was a similar low-amplitude pronation/ supination tremor. The postural tremor subsided completely with distraction as well as the performance of finger-to-nose testing with that hand. The tremor was evident on holding a full cup of water outstretched but again, subsided with any distraction. It began immediately when the distracting maneuvre (e.g., finger tapping with the left hand) was stopped. There was no increase in tremor at the endpoints of holding a cup out or bringing it to the mouth. There was no tremor on writing. However, he held the pen tightly, the script became more cramped as he continued, and he complained of excessive tightness in the hand. Investigation results, including a slit-lamp examination, were entirely normal. He was reassured and during the next 6 months he had a marked spontaneous improvement in the tremor. He continued to experience writing difficulties and examination once again revealed typical features of writer’s cramp. DISCUSSION In the same fashion that “functional” neurological signs and symptoms may be superimposed on organic neurological disease, these six patients had “psychogenic dyskinesias” complicating a preexisting organic movement disorder. Three patients (cases 2, 4,5 ) had typical dystonic spasmodic torticollis, one (case 6) had writer’s cramp, three (cases 1,3,5) had essential tremor (familial in one), and one of these (case 1) had orofacial dyskinesia, probably a form of cranial dystonia (Meige’s syn-
131
drome) combined with mild retrocollis. One patient (case 4) had probably had similar psychogenic dyskinesias earlier in life before the development of the torticollis, but the most recent exacerbation occurred after the onset of torticollis. In one patient (case 9, the psychogenic dyskinesia seems to have begun at approximately the same time as her underlying movement disorder. In the remaining patients the organic dyskinesias had been present for variable periods (usually several years) before the onset of the psychogenic movement disorder. In five patients (cases l , 2, 4, 5 , 6), the new movement disorder was phenomenologically different from the original dyskinesia, whereas in only one (case 3) was it similar (i.e., tremor). The psychogenic dyskinesias usually affected the same part of the body as the underlying organic movement disorder did as well as contiguous regions not originally involved. In these patients it was the new psychogenic dyskinesia that caused the greatest concern and disability whereas the original movement disorder was usually tolerable. In two patients (cases 3 and 6), the underlying movement disorder had been completely overlooked by the patients and the referring physicians. The new movement disorders were considered psychogenic because of associated historical, clinical, and behavioral features and the response to suggestion and/or placebo therapy. When there was any doubt about the “functional” nature of these movements, patients were followed long enough to document further supportive evidence of this diagnosis. This evidence was usually the behavior of the movement disorder that was inconsistent or incongruous with other known (organic) dyskinesias. In reviewing the concept of psychogenic dystonia, Fahn and Williams (2) suggested criteria for subdividing patients into categories based on certainty of diagnosis. These included (a) documented psychogenic dystonia, (b) clinically established psychogenic dystonia, (c) probable psychogenic dystonia, and (d) possible psychogenic dystonia. We would favor expanding the application of these authors’ criteria (2) to the diagnosis of psychogenic movement disorders in general, with one major qualification, supported by the experience reported here. In the “probable” and “possible” categories, Fahn and Williams include patients in whom the movements are consistent and congruent with the classic movement disorder but who have other neurological signs that are definitely psychogenic (e.g., false weakness, false sensory findings) or who have
Movement Disorders, Vol. 5 , No. 2 , 1990
132
R . RANAWAYA ET AL. TABLE 1. Patient characteristics
Case
Age
Sex
Underlying dyskinesia
Psychogenic dyskinesia
Confirming features
Additional “functional” neurology
Psychiatric features
Level of certaintyn
Head, shoulders, arm rocking
Placebo
Anxiety, multiple somatic complaints
Documented
Paroxysmal side-to-side head shaking
Distractability, incongruity, immediate (minutes) response to haloperidol
Chronic anxiety
Essential tremor
Generalized whole-body tremors (initially paroxysmal)
Incongruity
Clinically established (“Documented” if immediate response to haloperidol is accepted as placebo) Clinically established
Essential tremor, facial dyskinesia, retrocollis Spasmodic torticollis
Psychogenic gait disturbance
4
53
F
Spasmodic torticollis
Paroxysmal head, trunk, arm rocking
Incongruity, present only when lying down
5
23
F
Spasmodic torticolli s, essential tremor
Head rocking
Incongruity, abrupt and striking improvement with TENS
6
28
M
Writer’s cramp
Tremor
Incongruity, distractability, spontaneous improvement
Right hemisensory loss
Post MVA, litigation pending, multiple somatic complaints Long-standing depression, personality disorder, litigation pending Long-standing depression
Long-standing depression
Clinically established
Clinically established (“Documented” if immediate response to TENS is accepted as placebo) Clinically established
Applying criteria of Fahn and Williams (2). TENS, transcutaneous electrical nerve stimulation; MVA, motor vehicle accident. a
multiple somatizations. Thus the criteria, as they stand, do not allow for the possible coexistence of organic and psychogenic conditions. Applying these criteria rigidly, we would have diagnosed our patients as having only psychogenic dyskinesias. Our experience emphasizes that organic and psychogenic movement disorders are not mutually exclusive. Indeed, 1 of the 21 cases of Fahn and Williams (case 5) had underlying idiopathic torsion dystonia before presenting with new symptoms which were an embellishment of his dystonia associated with pain. These latter symptoms were documented to be psychogenic. Taking into account this disagreement in diagnostic criteria, all of the patients reported here would otherwise (i.e., distinct from their underlying organic movement disorder) be classified as having “documented” or “clinically established” psychogenic dyskinesias. Criteria for ‘’documented psychogenic dyskinesias” would require that the abnormal movements be persistently relieved by psychotherapy, by the
Movement Disorders, Vol. 5, No. 2 , 1990
clinician using psychological suggestion including physiotherapy, or by the administration of placebos. “Clinically established psychogenic dyskinesias” are inconsistent over time or are incongruent with “organic” movement disorders. In addition, patients demonstrate one or more of the following: other neurological signs that are definitely psychogenic, multiple somatizations, and an obvious psychiatric disturbance. The bizarre, inconsistent, and rapidly changing nature of many of the movements documented in our patients were quite incongruous with “organic” dyskinesias. Resolution of the movement disorder with distraction or concentration on a task (e.g., sequential finger tapping, mental arithmetic) is a useful clinical feature, although we have not taken this behavior in isolation to indicate a diagnosis of psychogenic dyskinesia. In our experience, tics and akathitic movements frequently (if not always) behave in the same fashion whereas other dyskinesias typically worsen or remain unchanged. Patient 2 gave a family history
PSYCHOGENIC D YSKtNESIAS IN ORGANIC MOVEMENT DISORDERS
compatible with a tic disorder; however, her abnormal movements were clinically atypical and lacked additional subjective features of tics. As indicated in Table 1, using the criteria outlined above, case 1 could be classified as “documented,” cases 3, 4, and 6 could be classified as “clinically established,” and cases 2 and 5 could be classified as either, depending on whether or not one accepts their respective responses to haloperidol (resolution in minutes) and TENS as comparable to placeboinduced benefit. We have seen several other patients who represent examples of “probable” and “possible” psychogenic dyskinesias combined with organic movement disorders but have chosen to exclude them from this report because of diagnostic uncertainty. In four patients, the psychogenic dyskinesia was originally paroxysmal. In one, the paroxysms resolved, leaving a persistent, less severe psychogenic dyskinesia. The paroxysmal nature of abnormal movements should serve as a possible clue to a psychogenic origin. Bressman et al. (3) have reported that 11 of their 25 patients with paroxysmal nonkinesigenic dystonia were psychogenic, and 7 of Fahn and Williams’ 21 patients with psychogenic dystonia had paroxysmal movements (2). Obviously other “organic” causes of paroxysmal dyskinesias (4) must be excluded before a primary psychogenic source can be accepted. The underlying psychological disturbances in our patients are listed in Table 1. There were no uniform or consistent psychiatric abnormalities in this group. Litigation was pending in two patients (cases 3 and 4) and additional long-standing psychopathology was evident in one of these. Two patients had multiple somatic complaints, one with associated depression. Of note is the lack of behavioral characteristics typical of the “histrionic” or “hysterical” personality. This concurs with the general ex-
133
perience in psychogenic neurological disorders, in which -80% lack the “hysterical” personality (5). Psychogenic movement disorders are not common in terms of percentage of patients seen with organic movement disorders. In Fahn and Williams’ experience with a total of 793 patients with idiopathic dystonia, only 21 patients had documented and clinically established psychogenic dystonia, representing only 2.6% of the dystonia population. If 18 patients with probable and possible psychogenic dystonia are added to the above, the final figure is 4.6% (2). In Marsden’s experience with 400 patients with various forms of dystonia, only 5 (1.25%) cases had a confident diagnosis of hysteria (5). We estimate that approximately 1&15% of all the patients we diagnosed as having a psychogenic dyskinesia have had evidence of an additional underlying organic movement disorder. This experience is comparable to a 10-37% incidence of true epilepsy in patients with pseudoseizures (1,6). Hence, we would caution physicians not to overdiagnose psychogenic movement disorders and emphasize the need to carefully consider the possibility of an additional underlying “organic” movement disorder when faced with a bizarre psychogenic dyskinesia. REFERENCES 1 . Lesser RP, Lueders H, Dinner DS. Evidence for epilepsy is rare in patients with psychogenic seizures. Neurology 1983;33:502-504. 2. Fahn S, Williams DT. Psychogenic dystonia. Adv Neurol 1988;50:431 4 5 . 3 . Bressman SB, Fahn S , Burke RE. Paroxysmal nonkinesigenic dystonia. Adv Neurol 1988;50:403-413. 4. Weiner WJ, Lang AE. Movement disorders: a comprehensive survey. New York: Fntura Press, 1989. 5 . Marsden CD. Hysteria-a neurologist’s view. Psycho/ Med 1986;16277-288. 6. Krumholz A, Niedermeyer E. Psychogenic seizures: a clinical study with follow-up data. Neurology 1983;33:498-502.
Movement Disorders, Vol. 5 , N o . 2 , 1990
