DystoniaSymptoms and Treatments
Dystonia is a neurological condition that causes muscles to contract involuntarily and out of proper sequence. It can occur anywhere in the body, including the muscles of the arms, legs, trunk, or face, and appears as repetitive, twisting movements and unnatural posture. About 50% of MSA patients experience dystonia1.
Not an early symptom of MSA, dystonia tends to manifest as the disease progresses and occur more commonly in MSA-P than MSA-C2. Though botulinum toxin injection is the first-order treatment for dystonia, non-drug treatment options, such as physical and occupational therapy, often improve results when combined with botulinum treatment. In some instances, neurosurgery may also be an option.
Common Forms of Dystonia in MSA
Dystonia of the cervical spine is common in MSA patients, though some experts contend that it is not an actual dystonia, but a form of Parkinsonian-type muscle rigidity1. Also referred to as torticollis, it causes a severe forward or backward bending of the head. Because it affects the orientation of the head in space, cervical dystonia can contribute to balance and gait problems6. MSA patients also often experience dystonia of the muscles of the mouth and face that alters speech, resulting in high-pitched sounds1. Dystonia of the vocal cords can occur and contribute to obstructive sleep apnea. Dystonia of the trunk muscles alters posture and gait in some MSA patients.
Non-Pharmacological Treatment Options
Physical and Occupational Therapy
Physical and occupational therapists can help in the management of dystonia by mobilizing joints to maintain range of motion and stretching muscles to minimize contracture, or permanent shortening of the muscle3. Targeted sensory stimulation via certain orthopedic devices can help inhibit overactive muscles and activate weak muscles to achieve more balanced muscle function around a joint or body part3. A form of movement-retraining therapy called constraint-induced therapy is used in dystonia of the hands. This approach involves taping or splinting non-dystonic fingers as a way to isolate the dystonic fingers and encourage them to function3.
Physical therapy for dystonia of the neck may include a form of motor retraining in which the patient repeatedly moves his or her neck in the opposite direction to the dystonic movement5. Passive and active stretching techniques help mobilize the cervical spine. Additionally, low-level electrical stimulation on the skin surface can activate the non-dystonic muscles. This causes a reflex that inhibits and relaxes the overactive, dystonic muscles5. In a study that compared physical therapy combined with botulinum treatment to botulinum treatment alone, severity of dystonia decreased in both groups, but only the group that received the combination of treatments experienced a decrease in pain levels5. Additionally, mental health, vitality, and sociability improved in the group that received physical therapy but not in the botulinum-only group5.
For dystonia of the trunk muscles, one case-report study used botulinum toxin injections along with a combination of active and resisted movement exercises, stretching, athletic taping, functional rehabilitation, and sensory maneuvers.4 The results included improved posture and function, decreased pain, and lower doses of botulinum toxin needed.
While a mild stooped posture is a common characteristic of Parkinsonian conditions, some MSA patients with trunk muscle dystonia develop an extremely forward-bent posture known as camptocormia. Standing and walking worsens camptocormia symptoms, but when some patients with camptocormia lie down, the back straightens.10 Though its cause is not fully understood, it is thought to be either a form of trunk-muscle dystonia11, unbalanced muscle rigidity, or a muscular disease.10 A type of orthotic device comprised of two braces, one around the lower ribcage and one around the pelvis, connected by a rigid spacing bar has been found helpful in patients with camptocormia that resolves in the lying-down position9.
Touching a part of the face or head can help some patients reorient their posture6. The technique, known as geste antagonistique, was found, in one study, to be effective for correcting head position in half or more of patients7. Simply thinking of the sensory cue is effective for some patients. Also, wearing a device that creates continuous contact with geste antagonistique points has been found helpful.
Electrode sensors placed over involved muscles can give the dystonia patient a continuous stream of cues, or feedback, about the activity of those muscles8. In a typical biofeedback session, information from the electric sensors is projected onto a computer screen and the patient is prompted to alter his or her position or movements in accordance with the information on the screen. Biofeedback has been found to offer similar benefits to some forms of physical therapy.
In severe cases of dystonia, surgery may be considered. Surgery for dystonia involves severing the peripheral nerve or nerves that supply the affected muscles. For cervical dystonia, this procedure works well on a limited number of patients with specific patterns of dystonia, particularly those that are purely rotational3. Adverse effects of the surgery tend to be temporary, but can include balance problems and swallowing difficulties6.
Pharmacological Treatment Options
Injections of botulinum toxin, trade name Botox, particularly in the deeper muscles of the neck, have been found helpful for cervical dystonia12. Botulinum has also been used successfully and with significant pain reduction in dystonias of the mouth, jaw, and tongue, as well as in dystonias of the trunk muscles14. However, dystonias of the legs may not respond as well to botulinum therapy as cervical and arm dystonias13. A review of previously published studies found that botulinum toxin offers significant improvement, particularly for tremor associated with dystonia, compared to drug therapies, including anticholinergic drugs, levodopa, clonazepam, beta blockers, and primidone, which offer limited benefits for dystonia15. Side effects of botulinum toxin therapy, including dry mouth and swallowing difficulties, occur in up to 19% of patients14.
The compound levodopa, sold commonly under the trade name Sinemet, may be helpful for alleviating dystonia in a minority of MSA patients. In one case-report study, researchers noted that camptocormia, a dystonia characterized by an extremely forward-flexed posture that occurs when standing but goes away when the patient lies down, was alleviated with levodopa in an MSA-P patient16. Side effects of levodopa can include nausea, movement disorders, sleepiness, orthostatic hypotension, and cognitive impairment17.
A category of drugs known as anticholinergics is often used to treat dystonia. These drugs work by blocking acetylcholine, the neurotransmitter that activates muscles. Examples include benztropine (Cogentin), biperiden (Akineton), procyclidine, and scopolamine (Scopace or Maldemar)17. However, because anti-cholinergic drugs need to be taken in high doses, side effects are common and may include sleepiness, cognitive impairment, constipation, urinary retention, insomnia, blurred vision and dry mouth17.
This drug, sold as which lowers dopamine levels, is primarily used to treat Huntington’s disease and has also shown some effectiveness for dystonia17. Side effects of tetrabenazine, trade name Xenazine, include sleepiness, parkinsonism, cognitive impairment, depression, orthostatic hypotension, and insomnia18. Tetrabenazine has demonstrated effectiveness in treating cervical dystonia in Parkinson’s patients19.
This drug, trade name Lioresal, raises levels of the calming neurotransmitter GABA and is helpful for some dystonia patients17. Side effects may include sleepiness, nausea, cognitive impairment, dizziness, and muscle weakness. Baclofen use causes dependency, and stopping or suddenly lowering the dose can result in seizures and delirium17.
This category of drugs, including clonazepam (Klonopin), diazepam (Valium), and lorazepam (Ativan), increase GABA activity and may be helpful for some forms of dystonia. However, these drugs can cause dependence, resulting in seizures and delirium if they are suddenly stopped or if the dose is lowered too quickly17.
A GABA-enhancing drug called zolpidem, trade name Ambien, primarily used for insomnia, has also been shown to improve dystonia symptoms20. In one study, 37% of participants responded well to zolpidem20. In the same study, 25% experienced improvement with clonazepam and 19% improved with baclofen. Zolpidem has been found to be helpful for hand and face dystonias but less helpful for cervical dystonia. The most common side effect of this drug is drowsiness21. Other potential side effects of zolpidem include headache, muscle aches, stuffy or runny nose, constipation or diarrhea, indigestion, double vision, and movement difficulties22.
This broad range of drugs that includes some already mentioned, such as baclofen and benzodiazepines, as well as others, including carisoprodol (Soma), cyclobenzaprine (Flexeril), metaxalone (Skelaxin), and methocarbamol (Robaxin), can be helpful for managing pain of pulled or sore muscles that can occur often in dystonia17. Side effects of muscle relaxants are common and include drowsiness, dizziness, urinary retention, and dry mouth. These drugs also can be addictive23.
MSA - What You Need to Know
- MSA Overview
- Types and Symptoms
- Treatment of MSA
- Prognosis and Outlook
- Differential Diagnosis
- Evaluation Methods
- Neurogenic Orthostatic Hypotension (nOH)
- Neurogenic Bladder
- MSA-P (Parkinsonian)
- MSA-C (Cerebellar Ataxia)
- Breathing Disorders
- REM Sleep Behavior Disorder
- Depression and Cognitive Impairment
- Neuroprotective Diet
- Advanced Planning
- What is the ANS
- History of MSA
- What First Responders Need to Know About MSA