OR WAIT null SECS
Five words that are guaranteed to annoy your patientwith a diagnosis of psychogenic movementdisorder (PMD) are It's all in your head.It's the worst thing you can say, said Katie Kompoliti,MD, associate professor of neurological sciencesat Rush University Medical Center in Chicago.
Five words that are guaranteed to annoy your patient with a diagnosis of psychogenic movement disorder (PMD) are "It's all in your head."
"It's the worst thing you can say," said Katie Kompoliti, MD, associate professor of neurological sciences at Rush University Medical Center in Chicago. Patients who hear this are likely to reject the diagnosis and seek further opinions; some will visit half a dozen specialists or more. They can run up thousands of dollars in health care costs and be subjected to unnecessary drug regimens and risky procedures.
"Patients can end up worse than they were at the onset of the disorder," said Joseph Jankovic, MD, professor of neurology and director of the Movement Disorders Clinic at Baylor College of Medicine in Houston. Finally, their chances of recovery appear to decrease the longer their condition goes untreated. This is why it is essential to make an accurate diagnosis in a reasonable amount of time and to deliver the news with sensitivity. The good news? Patients who accept their diagnosis and follow through with treatment have a good chance of recovery.
"It's a very difficult diagnosis," said Vanessa K. Hinson, MD, director of the Movement Disorders Program at the Medical University of South Carolina in Charleston. "But it's so important to make it, because if you do, these patients can get better."
DEFINING THE DISORDER
A patient with a PMD may exhibit any of the movements seen in organic movement disorders, including tremor, dystonia, chorea, bradykinesia, myoclonus, tics, athetosis, ballism, and lack of coordination. The person may also have disrupted speech and gait.1
The most common cause of PMD is a conversion disorder, in which psychological stressors cause the person to experience physical symptoms. The second most common cause is somatization disorder, in which the person seeks medical attention for recurrent and changing somatic complaints. Neurologists need to remember that the symptoms of conversion and somatization disorders are not under the person's conscious control.2
A less common cause is factitious disorder, in which the person achieves a psychological gain from his or her disease. As with conversion disorder and somatization disorder, persons with factitious disorder generally do not produce their symptoms intentionally, although some patients are malingerers, meaning that they produce their symptoms intentionally to achieve financial or other gain. Malingering is the least common cause of the disorder.1
Patients with PMD account for an estimated 2% to 3% of those in movement disorder clinics,3 and some centers report even higher rates. For example, about 15% of patients evaluated in the Baylor College of Medicine Movement Disorders Clinic have a psychogenic cause for their symptoms.4
"I think the reason [for the high rates at our center] is that community neurologists recognize the common movement disorders and refer the atypical ones to movement disorder centers," said Jankovic. "Many, if not most, of these cases turn out to be psychogenic."
The average age at onset ranges from 37 to 50 years, and women account for more than half of cases-estimates range from 61% to 87%.3,5,6 Although they usually occur alone, 10% to 15% are associated with organic neurological disorders.7 Many patients have a coexisting axis I diagnosis, such as major depression or an anxiety disorder, or an axis II diagnosis, such as personality disorder.
Known risk factors for PMD include a history of sexual abuse or rape, surgery or other physical trauma, and highly emotionally stressful life events.5,8,9
NOT A Dx OF EXCLUSION
Jankovic said that he is seeing more cases of PMD than ever before, probably because the disease is being recognized more frequently. "In the past, cases would often be misdiagnosed," he said.
Neurologists have plenty of ways to make the diagnosis, however. Hinson emphasized that there are specific clinical characteristics that the neurologist should be looking for and that the diagnosis should not be viewed as a diagnosis of exclusion.
For example, PMDs tend to come on abruptly. "It will often be very specific; the patient will tell me, 'I was sitting in the fourth row of church, it was 10:30 in the morning, I felt a cold sweat on my face, my arm started shaking,'" said Kompoliti. The symptoms will also progress quickly, which is unusual in an organic movement disorder.
The onset is often related to a precipitating event, such as surgery, the death of a loved one, divorce, rape, or financial stress. "The person basically starts having a multitude of abnormal movements right after this precipitating event," said Hinson.
There are also typical clinical signs. For example, the movements tend to be incongruent with organic disease, and there may be multiple abnormal movements. "So I might see a patient who has tremor and myoclonus and chorea and on top of that, a speech and a gait disorder," said Hinson.
The movements tend to exhibit distractibility and variability and increase with attention to the affected body part. "The movements may vary throughout the examination," said Hinson. "When the examiner leaves the room, the movement improves, and when we pay special attention to the limb in question, the movement suddenly is exacerbated."
Other characteristics are deliberate slowness of movement; entrainment; coactivation; association with false weakness, sensory loss, and pain; lack of responsiveness to drugs for organic movement disorders; and response to placebo drugs and suggestion.
A simple way to check for entrainment is to have the patient mimic a movement at a specific frequency. "If the patient has a tremor in the left hand at a certain frequency, we have them do rapid alternating finger tapping movements with their right hand at a given frequency, and often times we will see that the psychogenic tremor will assume the frequency of the voluntary movement," said Hinson. A good test for distractibility, she said, is to have the patient count backward from 100 in sevens and see if this affects his movements.
Jankovic reported that he will sometimes use the power of suggestion to check for a PMD. For example, he might tell a patient with intermittent tremor that vibration can trigger the tremor in some patients and then apply a tuning fork to the affected part of the body. "In most cases, the patient suddenly develops tremor, even though it's not due to any organic cause, but just to my suggestion." He said that a converse suggestion also could work: suggesting that the tuning fork will reduce the tremor.
"I have hundreds and hundreds of videotapes that show complete resolution of the tremor with the power of suggestion," he said. This strategy helps define the phenomenology and provides further support for the diagnosis.
Another test that can be used to aid in diagnosis is the administration of a placebo. Hinson recommended giving placebos in a double-blind fashion-and only with the patient's consent, to avoid feelings of deception or mistrust. She also cautioned that organic movement disorders may temporarily improve in response to placebo administration, so placebo response should not be used as the sole criterion for a diagnosis of a PMD.
Kompoliti agreed that neurologists need to be cautious with these diagnoses. For example, she pointed out that it is possible for more than 1 movement disorder-such as Parkinson and chorea-to coexistent in the same patient. Furthermore, certain movement disorders, such as dystonia, can be bizarre in their presentation and their triggers.
Neurologists may need to perform tests such as MRI or spinal fluid analysis to exclude organic causes of the symptoms. However, said Jankovic, these tests usually already have been performed by the time he sees a patient at his center. "I rely on examination," he said. "There's no blood test that can tell someone they have PMD."
Neurologists also can use tests such as EMG-based tremor analysis and EMG analysis of myoclonus to quantify movement disorders. "With an EMG, you can document this entrainment phenomenon much more specifically than you can just visually because you can show the frequency of the tremor, you can show exactly the changes of frequency of the tremor with distraction," said Hinson, who noted that she rarely uses this test.
European physicians sometimes use functional brain scans such as [123I]-CIT single-photon-emission CT and fluorodopa positron emission tomography scans to check for dopaminergic cell loss in the brain, but these are used primarily in research settings in the United States.
DIAGNOSIS DOMAIN Of NEURO MOVEMENT SPECIALIST
It is difficult for a neurologist who doesn't see a lot of patients with PMD to make a diagnosis, conceded Jankovic. "I would say that the vast majority of these patients-80% or 90%-had another diagnosis before they saw us," he said.
There is also a risk that someone with an organic movement disorder will be misdiagnosed with a PMD. "Before we disclose the diagnosis to the patient, we want to be 100% sure that this is a correct diagnosis and that we have excluded all organic causes," said Jankovic. This is why it is a good idea to refer patients with a suspected PMD to a movement disorder center.
The diagnosis should be made by a neurologist who specializes in movement disorders rather than by a psychiatrist or psychologist, emphasized Jankovic. He said that it is extremely frustrating to diagnose a PMD, send the patient to a psychologist or a psychiatrist for treatment, and have that specialist tell them they are psychologically "fine" and that their disease is organic-a surprisingly common occurrence.
Hinson agreed that a neurologist who specializes in movement disorders should be tasked with making the diagnosis of a PMD, but she pointed out that psychiatrists can help refine the diagnosis and identify whether the PMD is related to a conversion disorder, somatoform disorder, factitious disorder, or to malingering.
Hinson reported that neurologists often refer cases to her when they are uncertain about the diagnosis. Other times, the referring neurologist has made the diagnosis but the patient has refused to accept it. Finally, some neurologists shy away from making the diagnosis themselves because they fear that the patient will not be receptive to it.
REACHING THE PATIENT
How can neurologists get patients to accept a diagnosis of PMD?
"You need to deliver the diagnosis with a lot of sensitivity so that the patient trusts the doctor, does not feel rejected, and buys into the treatment process," said Hinson. The first step is to acknowledge that the patient has a movement disorder. "I say, for example, 'You have a tremor disorder. There's no doubt about that.'" Hinson then goes on to explain that there are different causes of tremor. "I say that there's parkinsonian tremor, there's essential tremor, there's drug-induced tremor, and there's psychogenic tremor."
She then goes through each of these causes and explains why the patient's disease either does or does not fit the criteria. "I say, your tremor does not fit into Parkinson's type tremor because of this and that, and patients can understand this . . . They think, 'my tremor is different.'"
Finally, she delivers her conclusion: "It's not Parkinson's, it's not essential tremor, it's not drug- induced; your tremor falls in the category of psychogenic tremor. So what does that mean?" She then explains to the patient that the emotion center in the brain is tightly linked with the motion center in the brain, and if there's an emotional disturbance, the body can react with motor symptoms.
"I often make the analogy that on a small scale, if someone who is supposed to speak in public is nervous, their hands might start shaking, or if they're embarrassed, their face might get red, so emotional feelings can translate into physical symptoms." She said that most patients are receptive to the analogy and understand what she is saying.
"Then I underline that the patient has a movement disorder but that the origin of the movement disorder is not cell death or neurodegenerative disease like in Parkinson's. I stress that the disorder is an abnormal function of the brain that is triggered by emotions."
Hinson also emphasizes that the disease is curable and that the movements can go away because brain damage is not involved. She said that patients like hearing that brain damage is not an issue and that they can "get better."
Finally, Hinson tells them that the only way for them to improve is to follow through with treatment from a psychiatrist. "Because I tell them where I'm coming from and how I make the diagnosis, they're usually 'with' me [about the diagnosis and treatment recommendation] and will follow up with my psychiatric colleague."
Jankovic said that about 99% of his patients accept the diagnosis, but admitted that this wasn't always the case. "I'll be honest with you, when I first started seeing these patients, my success rate was much lower than that. I didn't know exactly how to approach them."
He agreed with Hinson that a sensitive delivery is essential. Too often, patients have the impression that the physician does not believe them and thinks the problem is "all in their head." "My feeling is that I have to convey to them that I do believe them, I do believe that what they're telling me bothers them, and that these symptoms are not entirely under their control."
Jankovic said that he emphasizes stress as the cause of the disorder and attempts to identify what type of stress might be at play. "I would say that in 80% or 90% of cases we can clearly identify what the stress factor might be at the time of the initial visit." He added that some patients will initially deny having stress as a precipitating factor but that further interviews with the patient, family members, and friends will generally reveal a specific stress trigger.
Kompoliti agreed that the diagnosis is difficult for patients to accept. She likes to emphasize the positive. "I tell them, 'this is the best news I could have given you today. If you had Parkinson disease, it would have been bad news: it's progressive, you have it for the rest of your life, and it could put you in a wheelchair, but you don't have that.'"
Her next step is to explain how important it is to act quickly to begin treatment because patients with recent-onset PMD have the best chance of getting better.
REFERRAL: PSYCHIATRIST OR PSYCHOLOGIST?
Treatment may involve sessions with a psychiatrist, a psychologist, or both. Hinson prefers to refer patients to a psychiatrist who has a special interest in somatoform disorders, but said any reputable psychiatrist would be suitable. If patients are recruiting their own mental health professional, Hinson requests that the patient have the psychiatrist call her. "The psychiatrist often needs reassurance from the neurologist that the condition is indeed psychogenic," she said.
The psychiatrist will devise a treatment plan that might include psychotherapy (either psychodynamic therapy or cognitive behavioral therapy); stress management and relaxation techniques such as biofeedback, yoga, and meditation; and medication for associated depression and anxiety. Many psychiatrists will refer the patient to a psychologist for the psychotherapy or stress management components. In addition, some patients may benefit from treatment by a physical or occupational therapist to reestablish normal motor function.
About half of Hinson's patients in whom a PMD diagnosis is made are requested to check back with Hinson after 3 months of once-weekly psychotherapy. "Some patients do well with the reassurance that someone is overseeing the treatment process," she said.
Jankovic's preference is to send patients directly to psychologists, especially those who specialize in stress management techniques such as biofeedback. He and his colleagues at Baylor have recently embarked on an independent review board-approved study of transcutaneous nerve stimulation as a means of "relaxing" the abnormally contracting muscles.
Few studies have looked at the long-term outcome of PMD. However, there is evidence that persons who are least likely to recover are those who are symptomatic for more than 6 months; those with insidious onset of PMD; and those with a primary psychiatric diagnosis of hypochondriasis, factitious disorder, or malingering.
In the largest longitudinal study to date (228 patients seen at the Baylor Movement Disorders Clinic), nearly 57% of patients improved, 22% worsened, and 21% remained the same after an average follow-up of 3.4 years. Patients who were most likely to recover were those in good physical health, with positive perceptions of their social life, who perceived that they were receiving effective treatment, who eliminated stressors in their life, and who were treated with a specific medication.10
"We found that patients who initially accept their diagnosis do well," said Jankovic. Hinson agreed. "I think we can prevent a lot of harm by delivering the diagnosis quickly and precisely and not shying away from it," she said. "We can see very good success with the appropriate treatment."
REFERENCES1. Hinson VK, Haren WB. PMDs. Lancet Neurol. 2006;5:695-700.
2. Lees AJ. Odd and unusual movement disorders. J Neurol Neurosurg Psychiatry. 2002;72(suppl 1):I17-I21.
3. Factor SA, Podskalny GD, Molho ES. PMDs: frequency, clinical profile, and characteristics. J Neurol Neurosurg Psychiatry. 1995;59:406-412.
4. Jankovic J, Vuong KD, Thomas M. Psychogenic tremor: long-term outcome. CNS Spectr. 2006;11:501-508.
5. Williams DT, Ford B, Fahn S. Phenomenology and psychopathology related to PMDs. Adv Neurol. 1995;65:231-257.
6. Hinson VK, Cubo E, Comella CL, et al. Rating scale for PMDs: scale development and clinimetric testing. Mov Disord. 2005;20:1592-1597.
7. Ranawaya R, Riley D, Lang A. Psychogenic dyskinesias in patients with organic movement disorders. Mov Disord. 1990;5:127-133.
8. Marsden CD. Hysteria-a neurologist's view. Psychol Med. 1986;16:277-288.
9. Feinstein A, Stergiopoulos V, Fine J, Lang AE. Psychiatric outcome in patients with a PMD: a prospective study. Neuropsychiatry Neuropsychol Behav Neurol. 2001;14:169-176.
10. Thomas M, Vuong KD, Jankovic J. Long-term prognosis of patients with PMDs. Parkinsonism Relat Disord. 2006;12:382-387.