What is Hand (Upper Limb) Dystonia?
Hand dystonia is a movement disorder caused by abnormalities in the nervous system, characterized by involuntary muscle contractions, spasms, and abnormal postures. The most commonly affected areas are the fingers and wrists, although symptoms can also occur in the arms and shoulders. Due to the interconnected nature of the musculoskeletal system, even patients with only finger involvement may experience tightness in the upper arms and shoulders. Thus, the more inclusive term “Upper Limb Dystonia” better encompasses the diversity of symptoms.
Based on the specific task involved, upper limb dystonia can take various forms, such as writer’s cramp, musician’s dystonia, typist’s cramp, cobbler’s cramp, tailor’s cramp, barber’s cramp, and golfer’s cramp. Over time, symptoms may remain task-specific or may generalize to other movements. With the increasing use of electronic devices, tasks such as using smartphones, typing, and operating mice have become common triggers for upper limb dystonia.
The earliest documented case of upper limb dystonia was writer’s cramp. For centuries, there was a debate over whether writer’s cramp was a psychogenic or physiological condition. Writer’s cramp is now categorized as a type of upper limb dystonia, which is widely accepted to be caused by neurological abnormalities rather than purely psychological factors or muscle injuries.
Although the exact pathophysiology of dystonia remains unclear, advancements in neuroscience and neuroimaging have revealed its involvement with numerous brain functions and regions, including the basal ganglia, cerebellum, thalamus, supplementary motor area, and sensorimotor cortex. These brain alterations lead to impaired inhibition, maladaptive plasticity, and sensory-motor dysfunction.
This new understanding has opened up novel therapeutic possibilities. Dystonia is no longer considered incurable as it once was. Neuroplastic movement therapy that target the nervous system, brain function, and neural plasticity have emerged as promising treatment options.
Symptoms
The symptoms of upper limb dystonia exhibit a high degree of variability and are often related to the affected body part and the task being performed. The severity of symptoms depends on the number of affected areas and the severity of spasms, while the complexity of the task can increase symptom variability. Given the frequent use of the upper limbs in daily life, even mild symptoms can significantly impact a patient’s work and daily activities. In upper limb dystonia, the affected areas may include the hand, wrist, arm, and shoulder. Patients may experience symptoms in a single area or in a combination of areas.
Hand/ Wrist
The hand is the most commonly affected area. The hand includes the fingers, thumb, and palm, with the fingers being the most prominently affected. Common symptoms include finger splaying, rigidity, curling, and adduction, as well as mild tremors. In severe cases, fingers may form an involuntary fist. The thumb, essential for fine motor skills, is also frequently affected. The muscle groups of the thumb are quite complex, and mild symptoms may be difficult to detect but can significantly impact overall hand function. Common thumb symptoms include adduction and flexion. The palm, though often overlooked, plays a crucial role in hand function. Its size and shape influence the range of motion of the fingers and thumb. Involuntary inward curving is a common symptom of the palm. These three components form the foundation of grasping, and hand dystonia primarily affects this function. Any abnormality in the fingers, thumb, or palm can lead to limited hand function.
The wrist is commonly affected by flexion, meaning the palm bends towards the arm. While wrist flexion is necessary for many daily activities, but in dystonia, this movement exceeds the normal range of use, and is therefore considered an abnormal posture. This abnormal posture is commonly seen in writer’s cramp, where the wrist involuntarily flexes excessively when holding a pen. Additionally, many patients with hand dystonia also have mild ulnar deviation, where the palm deviates from the neutral position towards the little finger. While this deviation may be subtle, it can interfere with the performance of fine motor tasks.
Arm/ Shoulder
Common abnormal postures of the arm include forearm pronation (palm down) and humeral abduction (raising the upper arm outward), often accompanied by shoulder elevation. In addition to these abnormal postures, the arm may also experience tremors, twitching, and involuntary spasms. Patients often describe their arm as “getting stuck” in a certain position, making it difficult to perform actions smoothly, or experiencing increased tremors in specific postures.
A common abnormal posture of the shoulder is elevation, where one side of the shoulder involuntarily raises and is difficult to relax. Besides upper limb dystonia, this symptom is also commonly seen in patients with cervical dystonia.
Task
The manifestation of symptoms is closely tied to the task being performed. Patients may exhibit different symptoms based on the specific task, which is why upper limb dystonia has given rise to many names associated with occupational categories. Generally, symptoms in the hands and wrists are more commonly associated with fine motor tasks, while symptoms in the arms and shoulders are more often linked to gross motor tasks. The complexity of the task can also influence symptom presentation. For instance, esports athletes, who require high-speed and precise keyboard operations, may experience symptoms during intense gaming even if their dystonia is mild and undetectable during regular typing. Beyond specific tasks, many basic activities of daily living, particularly those involving grasping, such as picking up objects, using utensils, and dressing, can also be affected.
Symptom presentation varies from person to person. Some patients may only experience symptoms during specific tasks or in particular contexts, while others may experience symptoms throughout their daily lives. Symptoms may also change over time. As compensation increases, symptoms may spread from one body part to other parts, and task difficulty may increase, which are common phenomena.
Treatment
Writer’s cramp was the earliest recorded form of upper limb dystonia in medical literature. In the past, it was often misdiagnosed as a psychogenic disorder, leading patients to be referred to psychiatrists and receiving inappropriate treatments. This situation persisted until the 1970s and 1980s, when task-specific dystonia was finally recognized as a movement disorder. From this historical perspective, the treatment of dystonia is a relatively new field, dating back only a few decades. Given the uncertain pathophysiology, treatment approaches have been varied and experimental.
Currently, most hospitals treat dystonia with botulinum toxin injections. However, this is a symptomatic treatment that only provides temporary relief. Once the drug wears off (usually in three to four months), reinjections are necessary. Given the intricate and complex nature of hand muscles, and the need to preserve function for specific tasks, achieving precise and accurate dosing is extremely challenging. This is a primary reason why botulinum toxin injections have been relatively ineffective for hand dystonia. As for pharmacological treatments, there are currently no specific medications for dystonia. Neurologists frequently turn to medications for other movement disorders, yet the results are often unsatisfactory.
Given the wide spectrum of symptoms associated with dystonia, treatment plans must be highly individualized. At the two ends of the spectrum, patients with mild symptoms and those with severe spasticity require vastly different treatment approaches. A multifaceted approach is necessary, as a one-size-fits-all treatment is unlikely to be effective. Successful rehabilitation should be tailored to the individual patient. In recent decades, the treatment of dystonia has generally focused on the following approaches:
I. Movement Training and Biofeedback: Reshaping Brain Circuits
Combining movement training and biofeedback has become a cornerstone in the treatment of musician’s dystonia. This approach, inspired by Victor Candia’s constrained-induced movement therapy, involves using splints to limit compensatory movements and biofeedback devices to provide real-time feedback on physiological signals. This combination allows musicians to actively participate in the relearning process and promote neuroplasticity.
II. Psychological Interventions for Neurological Conditions
While the exact cause of dystonia remains unknown, psychological factors have been increasingly recognized as playing a significant role in its development and progression. Although dystonia is primarily a neurological disorder, improving mental health by reducing anxiety and stress can positively impact brain function and alleviate symptoms for some patients. In recent years, innovative therapeutic approaches such as Eye Movement Desensitization and Reprocessing (EMDR) have shown promise in treating dystonia.
III. Diverse Structural Therapies for Dystonia
A variety of structural therapies have been explored for the management of dystonia. These approaches aim to improve body alignment, reduce muscle tension, and promote neuromuscular balance. Techniques such as the Feldenkrais Method, Alexander Technique, chiropractic, Rolfing, and craniosacral therapy are commonly used. Additionally, the emerging field of TMJ adjustment offers a promising new avenue for dystonia treatment, highlighting the dynamic and evolving nature of this therapeutic field.
IV. Surgical or Brain Stimulation Interventions for Dystonia
Traditional surgical interventions for dystonia, such as thalamotomy and DBS, involve invasive procedures. The first is a stereotactic “thalamotomy”, which involves precise destruction of a specific region of the brain to control symptoms; and the second is deep brain stimulation (DBS), where electrodes are implanted into specific locations in the brain and a brain pacemaker is used to deliver electrical stimulation to modulate abnormal neural signals and improve motor symptoms. Additionally, focused ultrasound is an emerging treatment that uses precise heating to destroy specific neural tissue in the brain, modulating abnormal neural signals. This technique is still in the experimental phase. Regarding brain stimulation, some clinicians have explored the use of transcranial magnetic stimulation (TMS) and autonomic nervous system modulation in dystonia patients, though the efficacy of these approaches remains uncertain.
Farias Technique:
Pioneering Neural Plasticity Training for Primary Dystonia
Why Do We Need a New Treatment?
Despite the multitude of treatment options available, the outcomes of dystonia treatments have been less than satisfactory. This may be attributed to several factors. Firstly, the underlying mechanisms of dystonia remain unclear, leading to an incomplete theoretical foundation. Secondly, most studies have small sample sizes, making it difficult to establish a comprehensive patient profile. A review of past treatments reveals that almost all approaches have been unidimensional, such as attributing “musician’s dystonia” solely to psychological factors or attributing the disorder simply to poor physical alignment. Such a narrow perspective may be one reason for the limited effectiveness of these treatments. Moreover, the small sample sizes of most studies, which often isolate “musician’s dystonia” as a separate category or even classify it by instrument, place excessive emphasis on skill recovery while neglecting the possibility of observing commonalities among dystonia patients. This makes it difficult to construct a complete patient profile. Therefore, we need a new treatment that is grounded in a more comprehensive theoretical framework and can provide a more complete picture of the patient.
What is the Farias Technique?
The Farias Technique is the first therapy specifically designed for primary dystonia. Based on Dr. Farias’ clinical experience with thousands of dystonia patients, this innovative treatment method was developed by establishing a comprehensive patient profile from a large sample size and incorporating knowledge from neuroscience. What sets this technique apart is its unique understanding of dystonia and its core concepts. It goes beyond addressing overt movement disorders by delving deeper into the impact of dystonia on brain function and the nervous system. As a result, the treatment is not limited to movement training but also includes exercises to improve brain function and balance the nervous system.
Movement-Based Training Utilizing Neuroplasticity
The Farias Technique is a movement-based therapy that differs from other approaches by leveraging neuroplasticity and neuroscience to enhance brain inhibition and restore motor control. Dr. Farias observed that specific nerves and muscles are often selectively affected in dystonia, and based on this observation, he identified characteristic movement patterns and developed simple yet effective exercises. These exercises, though basic in nature, serve as the foundation for long-term rehabilitation of dystonia.
Is this a task-specific treatment?
Dr. Farias initially focused on treating “musician’s dystonia” and later expanded to other body parts affected by dystonia. Although the manifestation of upper limb dystonia varies depending on the task being performed, there is an underlying pattern to this apparent variability. This is because human movement is grounded in specific movement patterns, such as fine motor control. In addition to basic movement pattern training, therapists familiar with the Farias technique design task-specific exercises to help patients gradually recover the ability to perform specific tasks.
Upper Limb Dystonia FAQ
Wondering the same thing?
Join us as we delve into the top questions patients have.
Is there a cure for dystonia?
When discussing diseases, we often use the terms “cure” and “recovery.” The former implies the complete elimination of a disease or condition, while the latter refers to the restoration of health or normal function from an illness or injury. For dystonia, “recovery” is a more appropriate term as most patients experience varying degrees of improvement, and some may even relapse after a full functional recovery. Therefore, it is not accurate to judge solely based on whether or not a cure is achieved. The extent of “recovery” is difficult to standardize and depends on the individual’s activity requirements. For instance, the keyboard manipulation speed required of an e-sports athlete far exceeds that of the average person. Consequently, their expectations for recovery are considerably higher.
How long does rehabilitation take?
The duration of rehabilitation is highly individualized and can vary significantly depending on a multitude of factors. Some patients can fully recover, while others may require a long and gradual recovery or learn to live with the condition. Generally, rehabilitation takes at least six months to a year, and most musicians require several years to gradually regain function.
Is this a disease caused by psychological factors?
The etiology of dystonia is multifaceted, with the underlying pathology residing in the dysfunction of the neural system, especially the motor control centers of the brain. While emotional factors may exacerbate symptoms, they are not the cause of the disease. Losing the ability to perform daily activities or maintain a career can be a significant blow for patients, and emotional distress is a common reaction. It is inappropriate to attribute the cause of dystonia to patients’ emotional problems.
Is this a disease caused by excessive repetition?
It is difficult to convincingly attribute the sole cause of dystonia to repetitive practice. Many professions, such as musicians and athletes, are inherently based on repetitive tasks. Why do most people who engage in repetitive practice not develop dystonia, while some individuals, even amateurs or those with limited repetitive practice, still develop the condition? Dr. Farias addresses this question in the chapter “The Monkey with Emotions” in his book, “Limitless” Interested readers may delve deeper into this topic.
Will rest cure it?
Generally, most patients do not experience symptom improvement with rest alone. For instance, some patients may immediately experience symptoms upon picking up an instrument, even after years of inactivity. However, a minority of patients may find relief through rest, especially those who initiate rest early in the disease process, before the establishment of aberrant neural pathways.
Is an abnormal movement a bad habit?
Dystonia is not a bad habit but rather a condition where the original movement pathways have been replaced by primitive reflexes that govern survival. Primitive reflexes are innate, involuntary responses controlled by the central nervous system, especially the brainstem and spinal cord. They bypass the cerebral cortex and are extremely fast. When the brain cannot effectively inhibit these primitive reflexes, this shortcut is prioritized, overriding the fine motor skills stored in the cerebral cortex. Therefore, the goal of dystonia rehabilitation is to suppress this shortcut and restore the previously stored movement pathways.
What if the symptoms spread to other activities?
It’s not unusual for dystonia symptoms to manifest in daily activities. This often occurs when the brain’s inhibitory mechanisms are overwhelmed. While this can be distressing, it’s important to remember that there are treatment options available. Consult your therapist to develop a personalized treatment plan.
Why do some patients find wearing gloves helpful?
Wearing gloves can enhance sensation, allowing the brain to better perceive and control movement. Many patients with dystonia experience varying degrees of agnosia. When sensory nerve signals are weak, motor control can be affected. Therefore, enhancing sensation through touch or sensory tricks is a strategy that patients can try.
How can I improve my brain and nervous system?
Practices such as meditation, deep breathing, exercise, and nature immersion can enhance brain function and overall nervous system health. Engaging in activities like dance, drumming, tai chi, and yoga can also contribute to a greater sense of mind-body balance. Individual responses may vary, so it’s essential to explore different activities to find what resonates with you.
Tips
Will stress make my symptoms worse?
Stress management is a crucial component of dystonia rehabilitation, as increased stress can exacerbate symptoms. Stress tolerance varies from person to person, making it important for dystonia patients to learn how to identify their stress limits and develop effective stress management techniques.
Rehabilitation Tips
For Upper Limb Dystonia
Diagnose
When you suspect you may have dystonia, it is imperative to consult with a healthcare professional for a proper diagnosis. Dystonia is a symptom that can be associated with many different conditions, including Parkinson’s disease and medication side effects. The specific treatment for dystonia depends on its underlying cause, making an accurate diagnosis essential for effective management.
Neurologist
Understanding
Our understanding of a disease significantly influences how we approach it. Previously viewed as an incurable condition, dystonia is now seen through a new lens. A personalized rehabilitation plan is best developed with a thorough understanding of the condition. To explore dystonia from the perspectives of movement, brain function, and the nervous system, we recommend Dr. Farias’ book, ‘Limitless’.
Treatment
1. Prioritize non-invasive, safe, and harmless therapies.
2. Seek out a highly experienced therapist. A lack of specialized knowledge or observational skills, or solely rely on general rehabilitation methods, can be counterproductive.
3. Treatment plans should be adjusted based on the stage of the condition. Regular consultations with a therapist are essential for adjusting treatment plans as the symptoms of dystonia fluctuate and compensatory mechanisms develop.
4. A comprehensive approach is essential for effective treatment. A one-size-fits-all approach is not suitable for all patients. Successful rehabilitation requires individualized treatment plans.
Make an appointment with Dr. Monica Chen
Tips
1. Precision over Repetition: In dystonia training, accuracy is more important than repetition. We’re training neural pathways, not just muscles.
2. Consistency is Key: Consistent practice is essential for dystonia rehabilitation, rather than constantly changing methods.
3. Break Down Practice Sessions: Divide long practice sessions into shorter, more frequent ones.
4. Practice during periods of minimal symptoms: Many patients report minimal symptoms upon waking. Taking advantage of these symptom-free periods for practice, or even stopping before symptoms worsen, can be a beneficial strategy.
5. Manage Stress and Life: Higher stress correlates with more symptoms. Understanding your stress tolerance is crucial.
6. Rest is a practice strategy: Incorporating adequate rest and relaxation into your practice regimen is just as important as the practice itself. Overtraining can be counterproductive.
7. Walking Helps: Walking is beneficial for upper limb dystonia. Brisk walking promotes brain hemisphere coordination and dopamine release. Swinging your arms activates shoulder, chest, and back muscles.
8. Detach from negative feeling: When practicing, try to detach yourself from the psychological discomfort and simply focus on the physical movements. Alternatively, trying to shift your attention can also be a helpful strategy.
9. Keep Breathing: Many patients tend to hold their breath during exercises, which can disrupt the balance between the sympathetic and parasympathetic nervous systems. Maintaining natural breathing can help us stay calm and better manage our symptoms.
10. Avoid Excessive Expectations: The recovery process for dystonia is often ups and downs. As long as you’re making progress in the long run, that’s what matters. Every day is different, so just observe and adapt.
Psycological Support
Dystonia patients often experience significant psychosocial challenges, including social stigma and uncertainty about their treatment. The lack of a standardized treatment protocol can be particularly distressing. Seeking support from mental health professionals or joining support groups can provide emotional support, coping strategies, and a sense of community, enhancing overall well-being.
TESTIMONY
