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Twisted in Pain: The Multidisciplinary Treatment Approach to Functional Dystonia

2019; Lippincott Williams & Wilkins; Volume: 27; Issue: 6 Linguagem: Inglês

10.1097/hrp.0000000000000237

1465-7309

Yogesh Khachane, Kasia Kozlowska, Blanche Savage, Georgia McClure, Gretel Butler, Nicola Gray, Andrea Worth, Samantha Mihailovich, David L. Perez, Helene Helgeland, George P. Chrousos,

Pain Management and Placebo Effect

CASE HISTORY Jai was a 14-year-old boy referred to our consultation-liaison team by a pediatric rheumatologist for treatment of functional dystonia in the neck, a subtype of functional neurological symptom disorder (FND).1* Jai lived in a suburban area of a small city with his parents, Mr. and Mrs. P, and his 16-year-old brother, Kumar. Both his parents held academic positions at the university. Mrs. P had a history of generalized anxiety disorder. Kumar was a perfectionist who was academically oriented and achieved well at school, whereas Jai was more engaged in music; he liked to compose songs and to play them on the keyboard. The medical consultations leading up to the diagnosis of FND and referral to our team had been complicated, involving numerous specialists and teams, each with their own frameworks for investigation and assessment. Presentation Six weeks prior to presentation to our hospital, Jai suddenly developed left-sided neck pain while getting up from the lawn. On standing, he found that his neck had flexed and twisted to the left side. After two weeks of treatment by the family doctor—one week of analgesics and a second week of analgesics and physiotherapy—Jai's pain and posture had not improved. He was admitted to his local hospital under the orthopedic team. In the hospital Jai had a computerized tomography (CT) scan of his cervical spine. The results of the scan were difficult to interpret because Jai had not been able to straighten his neck. A subluxation (displacement of bones) of the spine could not be ruled out. Treatment included an opioid analgesic (oxycodone) and neck traction (see Figure 1, Panel A) which gave Jai some relief from the pain. During the weeklong admission, Jai developed weakness in the legs.Figure 1: Panel A. Jai treated with traction under an orthopedic team. Panel B. Jai sitting in the wheelchair wearing the Miami J neck collar with his body and neck twisted over the wheelchair hand rests. Panel C. Functional physiotherapy: learning to do stairs toward the latter part of the treatment process.At discharge, Jai and his parents were told that all test results had been normal and that the neck would eventually correct itself. No explanation was given for the weakness in the legs. Mrs. P reported that she had found the interaction with the orthopedic team uncomfortable and distressing. She felt that they were communicating to her that Jai had been wasting their precious time. The discharge plan was for Jai to continue analgesia, diazepam, and the neck brace (Miami J collar) for six weeks. Although Jai could not walk and was in a wheelchair when he was discharged, no hospital assistance was provided to help Mrs. P with the task of moving Jai to and into the car. Jai ended up falling just outside the car while attempting to transfer from the wheelchair to the car. In the car he experienced significant pain, could not sit upright, and, with intermittent neck spasms on the left involving the left sternocleidomastoid muscle and part of the left trapezius muscle, became extremely distressed. Once home, Jai's physical condition continued to deteriorate. Despite the neck brace, Jai was unable to keep the head in midline, and his body also began to twist to the left (in the same direction as his neck). The intermittent spasms on the left side of Jai's neck became more frequent and more intense. These spasms were worse when the collar was removed, when Jai tried to sit straight, and when he moved his shoulder. During these spasm episodes, Jai would go white, shake in pain, and grit his teeth. Although he had initially been able to walk short distances in the house with support from his mother, he began to drag his left leg more and more, and began to experience difficulties in coordinating his legs when walking. By week 5 of his illness (one week prior to presentation to our team), Jai resisted anyone touching his collar, and he avoided any movement to his torso and shoulders because the resulting neck spasms were exceedingly painful. His body flexed more and more to the left, and eventually he dangled in a C-shape over the edge of the wheelchair, making it difficult for anyone to maneuver the wheelchair through doorways without Jai hitting his head (see Figure 1, Panel B). It became increasingly difficult for him to walk because the flexion of his body had worsened and because the movement exacerbated the neck spasms and associated pain. He ended up lying in his bed—in a curved position—for most of the day. As Jai's physical well-being deteriorated, it became more and more difficult for the family to manage his daily needs without a commode chair and a support chair in shower. Even during sleep, Jai's body retained the flexed position; he slept in a C-shape. And because of the pain and recurring spasms, he slept for maximum of four hours a night. In week 5 of Jai's illness, a rheumatologist (who was also a family friend) referred Jai to a pediatric rheumatologist at our tertiary care hospital for further medical evaluation. At our hospital, Jai was assessed and evaluated by seven different medical teams (see Appendix 1), and he had multiple other investigations (see Appendix 2). Because of Jai's bent position, the medical teams found the scans—two CT scans and one CT angiography—difficult to interpret. Among the varied medical hypotheses that needed clarification were the following: possible subluxation and fracture of the cervical spine; arthritis of the spine causing possible subluxation; and possible calcification/injury in the vertebral artery, causing dissection. But all of these diagnoses were ruled out by studies using magnetic resonance imaging and angiography (under general anesthesia to enable Jai's body to relax and straighten, resulting in more accurate interpretations of the images). At this point Jai was evaluated by two neurologists; except for the fixed neck dystonia, Jai's neurological examination was normal (see Appendix 3). What was noteworthy, however, was that Jai's trunk musculature revealed no increased tone—no torsional dystonia. The neurologists hypothesized that the C-shape of Jai's trunk reflected a pain-related reflex response that occurred alongside the dystonia in his neck and that was actually part of the dystonia presentation. The neurologist's diagnosis was FND, including fixed functional dystonia of the neck and functional motor loss and loss of coordination in the legs (see Text Box 1 for information about functional dystonia). Although chronic pain is technically a separate diagnostic category—a comorbid diagnosis—fixed dystonia of sudden onset is often accompanied by pain. Other types of dystonia tend not to be painful.5Text Box 1 Functional Dystonia Functional neurological disorders (FNDs) are among the most common causes of neurological disability and have an incidence of 4–12 per 100,000 population per year and a prevalence of 50 per 100,000 population based on a community registry.2 Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal movements, postures, or both. Functional dystonia, part of the spectrum of FND, is relatively uncommon in the adolescent population. The functional dystonia manifests in paroxysms or as a fixed, persistent muscle contraction that sets the body part in a fixed posture. In contrast to the other functional neurological presentations, the diagnosis of functional dystonia can be especially challenging because of the lack of clinical features (e.g., entrainment of tremor) that distinguish functional from organic dystonias. Sudden onset and the presence of pain are common in functional fixed dystonia and rare in organic dystonia with the exception of cervical dystonia. The key diagnostic findings include the following: sudden onset of dystonia, with the dystonia fixed at onset; presence of pain; and variable resistance to passive manipulation.3 Functional dystonia is usually very disabling and is one of the hardest functional symptoms to treat. One study with children and adults (n = 35) showed improvement in less than a fourth of patients, remission only in 6%, and a worsening of symptoms in a third.4 There is very little literature about treatment. Key recommendations include the following: effectively communicating the diagnosis; using a multidisciplinary treatment approach involving psychological interventions and physical rehabilitation; and setting the primary goals as reestablishing movement as soon as possible and effectively controlling pain. A delay between the symptoms onset and treatment is associated with poor outcomes.Assessment with the Whole Family The family assessment involved all members of the immediate family—Mrs. P, Mr. P, and Jai's brother Kumar—and a multidisciplinary consultation-liaison team (comprising a child psychiatrist, clinical psychologist, and medical resident). Because the medical investigations had been completed only a few days earlier, Jai's parents communicated their sense of relief that the various medical diagnoses had been excluded. They also told the team, however, that the diagnosis of FND was difficult to accept because Jai came from a good, solid family whose members had experienced, as far as they knew, no significant stressors or traumas. Their sense of being puzzled was acknowledged, and the family and team agreed to proceed with the usual assessment by the psychological medicine team. The interview process used in the initial assessment is summarized in Kozlowka and colleagues (2013).6 Developmental History Jai's developmental history, as it emerged from the family assessment, was remarkable for the following: − Anxiety, including separation anxiety, was evident as early as Jai's second year of life. − Dyslexia, diagnosed in fifth grade, resulted in uneven academic performance and significant bullying at school. − In sixth grade Jai was diagnosed with irritable bowel syndrome, a functional gut disorder. − In seventh grade Jai became what he described as a "de facto counselor" for school friends who confided in him about self-harm and suicidal intent. − Jai had told his parents nothing about any of the stressful events at school, though the parents noted that they had observed his stress increase over the years. Jai's full developmental history and the case formulation as it emerged from Jai's story are presented in Appendix 4. Therapeutic Contract We offered Jai a three-week admission into the Mind-Body Program, the time available prior to the upcoming, two-week break for school holidays, with the option of a readmission thereafter. For further details of the therapeutic contract, see Appendix 5.7–14 Jai's parents were made aware that of all FND symptoms, functional dystonia was the most difficult to treat. As a specialist group, the mind-body team has had 15 years' clinical experience treating children with dystonia (see Table 1). The team's experience has been mixed: 11 of 13 children (85%) had recovered after one month to six years of treatment. One of 13 children continued to suffer from recurring episodes of dystonia in the right arm during the eight-year follow-up period. One of 13 had fixed dystonia in both feet and ongoing astasia-abasia and stocking distribution sensory loss—with some improvement in the severity of the dystonia and the degree of leg incoordination.Table 1: Outcomes of the 13 Patients Treated by the Mind-Body Team for Functional Dystonia in the Previous 15 YearsThe team involved in Jai's care included a child psychiatrist, two clinical psychologists, two residents, a physiotherapist, an occupational therapist, nursing staff on the adolescent medicine ward, and school staff in the adolescent classroom in the hospital school. The Mind-Body Program In this section we provide a brief outline of the key therapeutic interventions that made up Jai's treatment program within the Mind-Body Program's rehabilitation framework. Pain management: Withdrawal of opiates Following a consult with the pain team, we added pregabalin to Jai's medication regimen to help with chronic pain and opiate withdrawal.† Although Jai reported a reduction in pain with pregabalin, managing his pain after full withdrawal of oxycodone presented a challenge because Jai's level of pain rendered him unable to participate in physiotherapy sessions and affected his sleep. For details concerning the continued use of oxycodone (through week 14 of the admission), see sections below on sleep interventions and physiotherapy. The pregabalin was itself discontinued once Jai had returned full time to school, a year after the onset of his illness. Ongoing nursing interventions The ongoing nursing interventions are described in Appendix 6. Regulating sleep As previously mentioned, Jai had difficulties falling asleep when he was admitted to the Mind-Body Program. He would fall asleep between midnight and 2 a.m., and then wake up three or four hours later because of pain resulting from his neck spasms. Unable to manage the pain in his twisted position in bed, he would request nurses to transfer him back to his wheelchair. Jai's sleep difficulties were also complicated, as was gradually disclosed over a period of weeks, by a severe depression. The components of sleep intervention, which took three months to implement fully, were as follows: – Sleep hygiene. Jai and the team established a regular bedtime/waking schedule, including a ban on using electronics in late evening. The schedule was displayed on each day's timetable. – Pharmacotherapy. Jai was very keen to try any medication that could help him with his sleep. Melatonin (increased from 3 mg to 6 mg to 9 mg) helped somewhat with sleep initiation. Next, clonidine was added to help decrease arousal at bedtime and to help Jai to fall asleep by 11 p.m. Next, because Jai continued to wake with pain and to remain awake in the early hours of the morning, quetiapine was added (starting at 25 mg and gradually increasing up to 75 mg) to help manage arousal, sleep quality, and duration of sleep, and to reduce pain-related agitation in the early morning hours.18 With all these interventions combined, Jai's sleep normalized (week 3 of the program and week 9 of his illness), and his capacity to participate in the physiotherapy and occupational therapy program increased. – Hypnosis. A self-hypnosis intervention was added to the nighttime sleep routine. Using it helped Jai get to sleep and to fall back to sleep if he woke in the middle of the night (for more on hypnosis, see later subsection on hypnosis). Trial of botulinum toxin Following a consult with the rehabilitation team, botulinum toxin was trialed. The administration of botulinum toxin improved Jai's capacity to engage in physiotherapy but had no impact on his fixed dystonia. For full description of this intervention, see Appendix 7. Arousal Children and adolescents with FND typically present in a state of increased arousal and motor activation.19 Jai's activated body state was evidenced by a chronic increase in respiratory rate (typically 20–24 breaths per minute in therapy sessions). Slow breathing—which Jai initially found very difficult—was introduced and continued. A range of other mind-body strategies—progressive muscle relaxation and imagery strategies—were introduced to help Jai work on decreasing his arousal and to manage pain. Jai had some difficulty utilizing scripted imagery but found that imagery based on music and positive memories of music was helpful and could assist with pain management. Depression By the third hospital week, Jai became more open about his mental state and, in particular, his thoughts of self-harm. Especially at first, however, any efforts to probe further led Jai to immediately shut down. At home during the two-week holiday break, Jai fell into a depressive funk‡ punctuated by intense, debilitating panic attacks in response to virtually any demand placed upon him. Throughout this period, Jai's mother and often Jai himself were in regular contact with the treatment team, mostly in an effort to stabilize the situation until Jai could return for treatment after the holiday. Over the course of Jai's second hospital stay, which lasted almost four more months, he became increasingly forthcoming in discussions of his mental state. At baseline, he felt deeply ashamed not only of his thoughts of self-harm (which he had mentioned prior to the holiday break) but of his long-standing low mood and intermittent suicidal ideation. To address his ongoing severe depression, we changed the quetiapine to the sedating antidepressant mirtazapine (starting at 7.5 mg and gradually increased to 22.5 mg). Despite significant sleep disruption during this change in medications, Jai's sleep eventually settled again (at the 22.5 mg dose), and his mood began to improve. Psychological intervention for depression and anxiety Jai's growing capacity to disclose the extent and severity of his depression, including suicidal ideation and compulsions to self-harm, broadened over time to include discussions of his very long-standing anxiety, including (more recently) his severe panic attacks. This act of talking about depression and anxiety, as well as the implementation of a safety plan, was therapeutic in and of itself. With time, and a lot of psychoeducation, Jai's sense of shame resolved, and he was able to talk about his symptoms in a more matter-of-fact way. With his therapist§ he went on to explore and establish alternative ways to cope with stress, pain, panic attacks, thoughts of self-harm, and periods of wakefulness at night. To manage Jai's compulsions to self-harm, distress-tolerance strategies were introduced (e.g., holding ice in hand), as were other sensory strategies (e.g., listening to music) and distraction techniques. Jai was able to utilize these strategies well; thoughts pertaining to self-harming ceased. He also demonstrated an increased ability to communicate when he was distressed—initially with the treating team, but increasingly also with his parents. He began to compose music and used his keyboard to play the music and manage periods of distress. Psychoeducation regarding panic attacks and avoidance helped Jai accept the graded exposure therapy (in particular, having Jai go outside, which had been a trigger for panic attacks) that was introduced into his schedule. Cognitive-behavioral strategies enabled him to challenge unhelpful thoughts during exposure tasks. Jai was also encouraged to "ride the wave" if he found himself in a panic attack, and to accept that he would not always be able to recognize the early warning signs of his panic attacks and to intervene in time. With continued exposure to triggering situations, Jai's panic attacks and anxiety reduced. Throughout his admission, however, managing panic attacks continued (by design) to be a challenge, for we added new challenges to his schedule on a weekly basis (e.g., car transfer practice prior to discharge). In psychology sessions Jai explored his tendency to catastrophize and get stuck in an "I can't do it" mind-set. As time went on, Jai showed an increased capacity for self-reflection and was able to openly discuss how his thinking patterns sometimes led him to feel "stuck" in terms of functional gains—contributing, in turn, to his depression, anxiety, and low motivation. In sessions with the occupational therapist and physiotherapist, Jai was able to implement, with prompting, cognitive strategies to challenge his counterproductive thinking patterns, but he continued to experience difficulty doing so independently. Hypnosis We routinely use hypnosis in our Mind-Body Program. Hypnosis is a top-down regulation strategy that some children are able to use to help in managing physiological arousal, pain, and anxiety.20–23 In Jai's case, the hypnotherapist quickly realized that Jai could readily enter a trance state. Hypnosis was therefore introduced into all of Jai's physical sessions—physiotherapy and occupational therapy—to manage his pain and dystonia during those interventions. Jai would go into trance at the beginning of each physical session. Suggestions were made about Jai being able to experience a state of deep relaxation; about disconnecting from his pain; about the flexibility of his body to move like a reed in the wind or like a tree; and so on. While Jai was in trance, the physiotherapist or occupational therapist would talk to Jai and implement that day's intervention (see below). Jai was able to use the hypnosis to disconnect from his pain and to allow the therapists to reposition his neck and body into a straighter position. A key challenge of using hypnosis was the process of coming out of trance. Despite strong, repeated hypnotic suggestions about staying calm, when Jai came out of trance—and when he became aware of his body being in a more upright position—he would perceive this upright position to be twisted and abnormal, and he would panic. In other words, Jai's brain was processing the C-shape position as straight and the straight position as twisted. Typically, in the early phase of the treatment program, each physical session consequently ended in panic, and usually a full-blown panic attack. Later on, as Jai got used to the discrepancy of what the mirror showed (Jai sitting more straight) and what his brain perceived (Jai as twisted), he began to panic less. With time he began to work on attaining the new position on his own throughout the day. Jai found the hypnosis less effective when he did it himself (self-hypnosis). At bedtime, using a hypnosis script that his hypnotherapist had recorded on her smartphone and then transferred over to his computer, Jai worked on putting himself in a trance state; this enabled the nursing staff to help Jai position himself in a straighter shape. Over time, Jai was able to establish and maintain a less curled C-shape, then an L-shape, and finally a straight sleep position throughout the night. Physiotherapy, occupational therapy, and hypnosis Physiotherapy, occupational therapy, and hypnosis need to be considered together because the three therapists closely collaborated to enable physical therapy sessions. In the first three weeks of the physiotherapy intervention—with no oxycodone available for the pain—Jai could not participate in the sessions at all; movement triggered muscle spasms accompanied by unbearable pain. Given the lack of progress, he was allowed to take oxycodone before physiotherapy sessions, which then enabled him and the therapist to make some small advances such as practicing transfers from wheelchair to bed, practicing standing with the assistance of a forearm frame, and practicing collar-free time, which was necessary for managing pressure sores on the back of Jai's neck. It was not until hypnosis was used to manage Jai's pain, however, that the physical work—the physiotherapy—began in earnest. The key components of this intervention included, in order of implementation: weaning of the collar; strengthening exercises; improving functional positions (better sitting position, better sleeping position); gait reeducation; using aids (walking frame, toilet support, shower support chair, walking sticks) for functional independence; and exercise and skill-based training that used games and hydrotherapy to improve mobility and safety, prevent secondary complications, and facilitate independence. Under hypnosis, Jai was able to disconnect from his pain, to attain a state of pleasant deep relaxation, and to visualize his body as bendable and flexible. In this state he allowed the therapist to work on repositioning him in his wheelchair—bit by bit, over time—into an upright posture. The occupational therapist gradually introduced a sideboard, supported by two pillows and a wedge, to help Jai sit upright. Eventually, Jai was able to start self-adjusting his position himself and to independently insert his postural supports. And as noted in the above subsection on hypnosis, he was, over time and with hypnosis, eventually able to main a straight sleep position on his own through the night. With the help of hypnosis, Jai was also able to transition progressively to increased use of a soft collar instead of the hard Miami J collar, and finally, he was able to keep his neck in a reasonable position without any collar. Three-and-a-half months into the hospital stay—week 20 of his illness—Jai was able to maintain his position without postural supports and was transitioned into a dynamic, self-propelled, manual wheelchair. Toward the end of his admission, Jai's goals were entirely focused on his daily-living skills, and his mother became part of the team supporting him. The occupational therapist—working closely with the physiotherapist and hypnotherapist—took on a prominent role at this stage. Examples of the challenges facing Jai include the following: − In order to go home, Jai needed to be able to travel by car. But at first, the mere idea of getting into a car would trigger a panic attack, and his body and neck would begin to twist painfully. It actually took a month or more of practice for Jai to get into a car and maintain a position that was considered safe. − He needed to be able to walk up a flight of stairs (see Figure 1, Panel C). − He did his land-based physiotherapy outside in order to practice walking on rough ground. − He practiced his toileting and showering independently so that he could continue to implement such daily-living skills at home. Jai initially experienced mounting pressure in these preparations to return home, but the hypnotherapist continued to have an important role in helping him manage these new tasks. The management of Jai's fatigue was also a challenge because he found the preparations for home to be exhausting, as was the management of his anxiety and mounting fear (and the family's fear) about what would happen when he went home. Hospital school intervention The hospital school intervention is described in Appendix 8. Family intervention For details of the family intervention, see Appendix 9.4 Discharge When a discharge date was proposed, Jai experienced an increase in anxiety—including a renewed need to wear his Miami J neck collar, even though he had not been using a collar for many weeks. Jai was able to use his psychology sessions to understand that the hospital had become his "safe place"; he was acutely worried about adjusting to being at home again. Jai's worries were acknowledged, and he was able to work with the team to implement limitations and rules that would come into play in the home setting; he would, as a consequence, be able to adjust to those projected new constraints in advance of going home. As an example, Jai noted that parental restrictions on screen time would make going home difficult, and he came up with a plan of how limits that approximated home could be implemented in hospital. During this time of increased anxiety, his progress in physical therapy became patchy: although he continued to make progress with car transfers, his progress with mobilizing on the frame and crutches stalled. In the last days of his admission, Jai was in a positive frame of mind. With his psychologist he had formulated some future-oriented plans with regard to visiting the hospital when he was well, including walking into the hospital independently, challenging his physiotherapist to basketball, and taking the occupational therapist for a drive once he got his driver's license. At discharge Jai was able to shower himself and use the commode chair; he needed assistance, however, with transferring from the wheelchair to the bathroom, with preparing the bath, and with drying himself. He was able to sit in a car for up to 40 min prior to discharge. Post-discharge Progress On discharge (week 30 of Jai's illness), Jai was referred to local health services—a child and adolescent mental health team and physiotherapy—to continue the multidisciplinary intervention that had begun in the hospital setting. A few weeks after discharge (week 34 of Jai's illness), in a telephone call with the team, Jai reported that the local physiotherapist was not pushing him enough. The team suggested that Jai had all the physiotherapy experience he needed and that he could push himself to make progress with his walking and with getting himself out of the wheelchair. Five weeks following discharge (week 35 of Jai's illness), Jai stood upright independently, without any support. His body felt "weird" and "different," and he felt some panic. In a phone conversation the team reminded Jai that when he had begun to sit up straight, his brain had also tricked him then; it had perceived the C-shape as being straight and the straight position as twisted. The team also reminded Jai that it had taken six weeks to adjust to the change. The team suggested that he slow down his attempts to walk. We suggested that on standing up, he use the grounding imagery of a tree and that he take time to feel his feet on the floor, to help his brain readjust to the new straight standing position (see Figure 2). Within a week (week 36 of Jai's illness), Jai was walking independently.Figure 2: Jai, just recovered from his illness, poses for a photo with the two pediatric residents who were part of the mind-body team that worked with him during his illness.Jai's regaining his physical function did not deter the family from following through on the various psychological and physical interventions that had been started in the hospital setting. Jai joined a gym, where he continued his fitness program with the help of an exercise physiologist and a personal trainer; he continued with psychology sessions for managing his anxiety and now resolved depression; and the family pursued ongoing work with a family therapist.** QUESTIONS TO THE CONSULTANTS − What are the primary components of treatment for patients with functional