Treatments for Dystonia

There are a variety of treatments for Dystonia – but it should be remembered that, as yet, there is no known cure.

The key to managing the symptoms is locating and having access to a movement disorder specialist. This is first step in Dystonia treatment; thus it is important for you to be receiving care for Dystonia from a movement disorder specialist - a neurologist with additional training in the area of movement disorders. Not all neurologists are have the knowledge base and skill in treating Dystonia. Begin by reading the following information.

Oral Medications

A number of oral medications (pills) have been used to treat and control symptoms of Dystonia. Unfortunately, a cure does not yet exist for Dystonia. Your health care provider will most likely begin your Dystonia treatment plan with one of these drugs. Sometimes, it takes a combination of several drugs to obtain a beneficial effect. None of these medications CURE Dystonia. Some of these medications at higher doses can have unpleasant side effects. One can also develop a tolerance to them over a period of time which leaves them ineffective resulting in no benefits for you. The following rules should make things easier for you when it comes to taking oral medications.

The use of oral medications is based on trial-and-error. Currently available oral medications benefit only a minority of patients, and the benefit is usually not complete. Therefore, you must not expect your Dystonia to resolve completely and "disappear". There is no cure for Dystonia but one can live well with it.

Common Oral Medications Used To Treat Dystonia (Generic, Trade Name):

BENZODIAZEPINES

CLONAZEPAM (Klonopin)
DIAZEPAM (Valium)
LORAZEPAM (Ativan)

SKELETAL MUSCLE RELAXANTS

LIORESAL (Baclofen)
CYCLOBENZAPRINE (Flexaril)
CARISOPRODOL (Soma)

ANTICHOLINERGICS

TRIHEXIPHENIDYL (Artane)
BENZTROPINE (Cogentin)
ETHOPROPAZINE (Parsidol)

DOPAMINE STIMULATION

LEVODOPA/CARDIDOPA (Sinemet)
BROMOCRIPTINE (Parlodel)
PERGOLIDE (Permax)
PRAMIPEXOLE (Mirapex)
REQUIP (Ropinerol)

DOPAMINE DEPLETION

TETRABENAZINE (Nitoman)
RESERPINE (Serapes)

Additional drugs that have been utilized in the treatment of Dystonia include ZANAFLEX, BENADRYL, MEXILITINE, TOPAMAX, DILANTIN, TEGRETOL, NEURONTIN and AMITRIPTYLINE (trade names).

The above list of medications are commonly used to treat Dystonia and its symptoms. These drugs have common side effects such as drowsiness, dizziness, weakness, fatigue and nausea. Like many drugs, they can be habit forming and should not be taken beyond the prescribed dosage. Avoid using over-the-counter medications, unless otherwise directed by your physician. In general, these drugs are used to help either activate or block receptors in the brain that induce over-activity of muscles. The goal of these drugs is to minimize and slow abnormal movements.

Take-home message

Follow the directions for using the medicine prescribed by your health care provider.
If you miss a dose, take it as soon as possible. However, if it is almost time for your next scheduled dose, skip the missed dose and go back to your regular dosing schedule.
Do not take 2 doses at once unless instructed to do so by your physician.
Do not suddenly stop taking any of these medications without checking with your physician FIRST.
Never take anyone else's medications.
When discussing medications on-line with others, do not follow their recommendations. Clarify these "recommendations" with your own personal physician.
The dosage of these medications varies from person to person. Not everyone responds to the same drug and dose in the same way. Everyone reacts to medication differently.
Your doctor can tell you the best way to gradually increase or reduce the dosage of your medication. Do not do this on your own.
Many of these medications cause fatigue, drowsiness, altered abilities to "think" at high doses. Plan your activities wisely.
Write out a "time sheet" to remind yourself when you are due for your medicine. Your physician can help you with this task.
Some of the drugs used in Dystonia will add to the effects of alcohol and other depressants.
Read up on all of your medications.
Never make any dosage changes on your own, even if you are feeling better. Changing doses on your own can be detrimental. If you have concerns, relay them to your health care provider/team.

Source: Demystifying Dystonia, care4Dystonia.org (http://www.care4dystonia.org/)

If you want to find information about the medication you are using on-line, simply use the trade name of the drug as the key search word. Another option is to use the URL address for the drug. For example, if you are searching for "botox®" simply write out www.botox.com. This will often lead you directly to the pharmaceutical company that produces the drug. You should find a wealth of information at that particular pharmaceutical industry site.

Key Point

With all oral medication trials, the rules of thumb are to (1) start at the lowest possible dose and increase the dose slowly, and (2) use the lowest effective dose for maintenance therapy.

Source: care4Dystonia.org (http://www.care4dystonia.org/)

Botulinum Toxin

Botulinum toxin was initially used in the early 1980s to treat strabismus and blepharospasm. The toxin is a neuromuscular junction blocking agent produced by the bacteria, Clostridium botulinum, which in large doses induces common food poisoning. It is one of the most potent biologic substances known today. There are 7 distinct serotypes or strains of the toxin: A, B, C, D, E, F and G. Each differs in their molecular size, structure, potency, duration of action, and cellular target sites. Both types A and B have been shown to be safe and effective in multiple clinical research trials in the treatment of Dystonia, specifically focal Dystonia (cervical, hemifacial ,blepharospasm etc.). Both A and B differ in dosages and are marketed under various names.

Both botulinum toxins A and B received FDA-approval for use in 2000-1. Botulinum toxin undergoes a series of purification and production to make it suitable for injection. At its basic structural level, the toxin has little effect on the neuromuscular junction. But when formulated in either a basic or acidic solution the toxin acts on the neuromuscular junction (through binding) preventing release of acetylcholine at nerve endings (located near muscles). Disruption of acetylcholine release and thus its transmission results in temporary weakening of the injected muscle.

NORMAL MUSCLE CONTRACTION:

Brain sends signal to muscle to contract to nerves...acetylcholine is released at the nerve-muscle junction...muscle contracts...movement occurs...

DYSTONIC MUSCLE CONTRACTION:

Brain sends too many signals to muscles..misfiring...recurrent muscle contractions occur...abnormal movements result...leading to dystonic symptoms...

DYSTONIA AND BOTULINUM TOXIN:

Brain sends multiple signals to muscles via nerves to contract...over-activity of muscles occurs...excessive muscle contraction develops...botulinum toxin is injected at select muscle sites... selective binding of toxin occurs at these nerve ending sites...acetylcholine is blocked at these neuromuscular junctions...acetylcholine disruption occurs...weakening of muscles develops...leading to temporary Dystonia relief...

Simply said, botulinum toxin interrupts faulty messages via nerves allowing over-active muscles to relax and return to a more normal state. This leads to less involuntary movements, tremors, postures, and pain.

Eventually, new nerve endings develop connections to new muscle sites and the toxin begins to wear off and dystonic spasms return. Sometimes, they change after treatment. Correct selection and targeting of dystonic muscles is crucial. Your health care provider may use electromyography (EMG) to help identify involved muscles. The EMG utilizes an electrode needle that records muscle activity. EMG machines record the sound of muscle activity through a speaker. You may hear static-like noises coming from the machine. The louder the noises, the more muscle over-activity exists in that area. You should expect botulinum toxin to be injected in those areas.

The degree of your clinical improvement is related to the extent of the local intramuscular botulinum toxin blockade--which is affected by the number, size, selection and location of muscles used as well as the dose and type of toxin used. Some people experience more pain during the injection process. Topical anesthetic (ex. EMLA cream), relaxation, music therapy may help minimize the pain during the process. Other side-effects include a stinging sensation, bruising, difficulty swallowing, excess muscle weakness and, rarely, a flu-like syndrome. All of the side-effects are dependent on the location where the toxin is given, it's dose, the size of needle used, and are short-lived in nature.

Other medical conditions that have responded positively to Botulinum Toxin Therapy

Nondystonic excessive muscle contraction

Back spasm, Bladdler detrusor-sphincter dyssynergia, Bruxism and temporomandibular joint pain, Gastrointestinal: achalasia, anismus (constipation), cricopharyngeal spasm, lower esophageal sphincter spasm, rectal spasm, anal fissure, anal and duodenal sphincter, Hemifacial spasm, Congenital nystagmus, Spasticity: Stroke, Cerebral palsy, Multiple sclerosis spasticity, Muscle contraction headaches, Pelvirectal spasms (vaginismus), Stuttering

Other Involuntary Movements

Other involuntary movements Parkinson's disease Essential tremor Hereditary chin tremor Palatal myoclonus, Tics.

Take Home Message

Botulinum toxins are neurotoxins produced by the anaerobic bacterium Clostridium botulinum and are the most deadly human neurotoxins known. They block neuromuscular transmission by inhibiting the release of acetylcholine at the neuromuscular junction, resulting in a flaccid paralysis of the muscles that are affected. Currently, two botulinum toxin type A (Botox) are FDA-approved and commercially available in the United States. Botulinum toxin type B is known as Myobloc.

Botulinum toxin is the treatment of choice for most forms of focal Dystonia, including blepharospasm, torticollis, spasmodic dysphonia, and jaw Dystonia. The botulinum toxin is injected directly into a hyperactive muscle, partially or completely paralyzing that muscle. By adjusting the dosage, it is possible to weaken the muscles enough to relieve involuntary contractions while possibly leaving some voluntary muscle control. Initially, you may note too much weakness of the muscles injected, but as the botulinum toxin wears off, you may have a period of decreased muscle activity. The chemodenervation lasts only a few months. Thereafter, voluntary activity gradually returns, and eventually your symptoms reoccur. The duration of benefit is usually 3 to 6 months but may vary from treatment to treatment

FAQ's

Question: I've heard about Toxin F; is it being used for Dystonia?

Answer: Toxin F has been used at Columbia-Presbyterian Hospital in NYC as well as a few other medical centers. The clinical duration of this toxin is about 6 weeks. Few have used it due to its limiting lasting effect. However, it may be worth investigating if you have developed antibodies to both Botox and MyoBloc.

Question: Are there clinical tests that determine the presence or absence of toxin antibodies? Answer: Yes, your doctor can determine the presence of antibodies by drawing a sample of blood from you. The sample is then sent to a laboratory. Results of the test take up to 2 weeks or so.

Question: I've heard about botulinum toxin C being used in Italy? Answer: This form of botulinum toxin is ineffective in humans therefore not available for use in humans.

Question: Is Botox the same thing as Myobloc? Answer: No. Both are two different serotypes-strains of botulinum toxin thus each toxin is a bit different in binding action and dosing.

Question: Are there average set doses for either toxin? Answer: Average dose for Botox is 250 units; the maximum dose is 400 units. Myobloc's average dose is 10,000 to 15,000 units. A maximum dose for Myobloc has not been identified yet. It has been reported that several patients received up to 25,000 units during the initial clinical trials of Myobloc.

Question: The toxin worked better the last time. Why not this time? Answer: The degree of your clinical improvement is related to the extent of the local intramuscular botulinum toxin blockade--which is affected by the number, size, selection and location of muscles used as well as the dose and type of toxin used.

Question: Do Syringes matter? Answer: Today there are a number of choices in syringe NEEDLE LENGTH and GAUGE, a measurement of needle thickness.

Needle gauges are measured in 28 gauge, 26 gauge, 24 gauge etc.: the higher the gauge, the thinner the needle.

Then there are short vs long needles :1/2 in. vs 5/16in. etc.

Keep the above points in mind during your next botulinum toxin injection.

Needle gauge and length, as well as Depth of the injection can affect the rate and amount of botulinum toxin absorption and degree of blockade.

Question: Should an EMG always be used ? Answer: Dystonic muscles are located either clinically by visual inspection and palpation or with the use of electromyography (EMG). Injections of BTX-A toxin can be performed with or without EMG. Use of EMG can help identify deep muscle involvement as is the case in cervical Dystonia.

Question: The 'shots' hurt. What can be done to minimize the pain I experience during the injection process?

Answer: About 10 % of adults in the US have a needle phobia.

There are 2 safe and effective topical local anesthetics available: 1) EMLA - a cream containing 2.5% lidocaine and 2.5% prilocaine
and
2) ELA-MAX - a 4% lidocaine cream.

EMLA is a prescription anesthetic, an emulsion cream applied to the skin and covered with a bandage for at least 60 minutes before a procedure. One hour is usually sufficient but 2 to 3 hours may be needed for intramuscular injections.

Botulinum toxin goes into muscle- therefore intramuscular.

EMLA should be applied in a thick layer and should extend a 1/2 inch beyond the area needing anaesthesia.

ELA-MAX is an over-the-counter (OTC) topical anaesthetic that works in about 15 to 30 minutes without a bandage according to the manufacturer.

Neither of these two creams should be ingested or applied to the EYES.

Both may help reduce needle phobia and minimize pain associated with the toxin injection process.

Neither should be used on a continuous basis to resolve or relieve ST pain.