Dystonia, or involuntary body movements. What are they caused by? What is the diet for dystonia?

Proven content

Dystonia is a disorder of the nervous system. It manifests itself in involuntary movements, e.g. tilting the head, waving the hand. Find out what the causes and types of the disease are, and how to prevent dystonia.

Dystonia – causes

The disease consists of involuntary movements of the body caused by tightening of the muscles. The cause is disorders of the nervous system at its beginning, that is, in the brain. The extrapyramidal system is the part of the brain that is responsible for maintaining proper body posture. During the onset of the disease, it is damaged.

The causes may be genetic, then it is said to be primary dystonia. Another type of causes are:

1. a brain tumor,
2. cerebral ischemia,
3. cerebral hemorrhage.

Dystonia can also be caused by other diseases such as Parkinson’s, Wilson’s disease, and mitochondrial disease.

Dystonia – types

Jeżeli mówimy o rodzajach dystonii, to na początku należy powiedzieć, że dzielą się one w zależności od: przyczyny; wieku, w którym wystąpił początek choroby; oraz w zależności od lokalizacji objawów.

Depending on the cause, we distinguish:

1. primary dystonia, which includes familial dystonia of known cause, and sporadic forms of unknown cause;
2. dystonia-plus syndrome, which is a group of conditions in which dystonia is the main symptom, but there are other symptoms such as parkinsonism or myoclonus;
3. secondary dystonia due to trauma or stroke, medication side effects, or other disorders of the central nervous system;
4. dystonia in neurodegenerative diseases, e.g. in Wilson’s, Huntington’s, Parkinson’s, and mitochondrial diseases.

Depending on the age, we can distinguish:

1. in the age range – from birth to 12 years, that is, dystonia of onset in childhood;
2. in the age range – from 12 to 20 years, that is, dystonia with onset in adolescence;
3. in the age range – over 20 years of age, i.e. dystonia with onset in adulthood.

Depending on the occupancy of the body, there are several types of dystonia.

Focal dystonia, i.e. one that occupies only one or more closely spaced muscles, e.g. the muscles of one limb. This form of dystonia is in turn divided into:

1. Cervical dystonia (torticollis, torticollis) – the muscles of the neck are involved in it, which in turn causes the head to be incorrectly positioned. Here, he distinguishes between: a one-way turn of the head (torticollis; występuje ona najczęściej), zgięcie głowy do boku (laterocollis), bending the head back (retrocollis), forward bend of the head (anterocollis), and mixed forms. In addition to the incorrect position of the head, the head also shakes.
2. Kurcz powiek (blepharospasm) – in this case, there is an abnormal, involuntary and excessive contraction of the facial muscles surrounding the eye. At first, you may notice frequent blinking, which over time turns into involuntary closing of the eyelids, which in turn leads to total or partial blindness (although the vision itself works fine).
3. Oromandibular dystonia – involves the muscles of the lower face, jaw, tongue and mouth.
4. Meige’s syndrome – in this case, blepharospasm and oromandibular dystonia coexist.
5. Half-facial spasm – although the condition is not classified as dystonia, it is cited for many of the same clinical features and treatment similarities. It consists in frequent, repeated and short-term contraction of the muscles of one half of the face. Also known as unilateral blepharospasm (where only the eye’s circular muscle is affected) or as segmental myoclonus.
6. Laryngeal dystonia – it affects the muscles of the larynx and vocal cords and occurs in two clinical forms (adduction and abduction type).
7. Writing’s cramp – this condition is associated with the performance of certain specific activities and affects the muscles of the hand and forearm (it occurs when you try to write). Another dystonia of this type is musician dystonia, which is the occurrence of dystonic disorders when playing various musical instruments.

Segmental dystoniainvolving many muscles of adjacent body parts, e.g. the neck and upper limb;

Dystonię połowicząwhere the upper and lower limbs of the same side of the body are affected;

Multifocal dystoniawhere the muscles of several different parts of the body are affected;

Generalized dystoniawhich is the most extensive of all types of dystonia and affects the muscles of most parts of the body (especially the extremities and torso).

Who gets dystonia?

Both men and women are exposed to the disease. When the disorder is discovered in childhood, treatment can be started early, but it is also likely that the disease will affect most of the body. When diagnosed in adults, it usually involves a small part of the body.

According to the data of the Polish Association of People with Dystonia, around 500 people suffer from the disease across Europe, of which a small number are aware of this. It is worth mentioning that some forms of the disease are more common in specific geographical areas, eg Roma in Spain, Ashkenazi Jews, single families in Australia or Canada.

Dystonia – inheritance

It should be said that dystonia, which begins in childhood, mostly has a genetic basis associated with damage to one or more genes. Also segmental and generalized dystonia is hereditary and it is transmitted predominantly (if either parent has this type of dystonia, there is a chance that the disease will be passed on to the child). It should be noted, however, that a person inheriting the abnormal gene does not have to get sick. This is due to a very common phenomenon in dystonia, namely the so-called incomplete gene penetration.

It is not only childhood dystonia that may be hereditary, as is the case with a disease that develops in adulthood. However, this is usually quite difficult to diagnose, especially if other family members have mild dystonia or have not been screened for dystonia, or the diagnosis is incorrect.

Dystonia – diagnosis

The diagnosis is made on the basis of the results of the EMG test, i.e. electrocardiography (it allows to determine which muscles are affected) or on the basis of genetic tests.

In addition, in the diagnosis of dystonia, an interview conducted by a GP, who may, if necessary, refer the patient to a neurologist. During the visit, the patient will be asked by the doctor about the factors that cause the symptoms of the disease (e.g. exercise or stress) and relieve symptoms. The hallmark of dystonia is a brief decrease in muscle tension after a specific movement (e.g., touching the cheek with a hand).

It should also be remembered that if you experience symptoms of dystonia, please tell your doctor about all the medications you are currently taking. This makes it easier to determine if the symptoms of dystonia are caused by the medications you are taking.

Dystonia – what to do when we get sick?

Dystonia is a slowly developing condition that can appear at any age. If a person begins to feel pain in the muscles or a feeling of forced positioning of the head or other part of the body, they should see a GP as soon as possible, who may refer the person to a neurologist. It should be added that people who perform the same activities all the time (playing an instrument or writing) are more likely to develop dystonia. This is also important because the doctor will look for the cause of the disease (it is of primary origin or maybe it results from medications or another disease, e.g. multiple sclerosis).

Patients also need to pay attention to the strength of their contractions, as a dangerous dystonic state can develop as they become stronger. Sometimes the patient’s condition may turn out to be so serious that hospitalization in the intensive care unit will be necessary.

If your contractions are strong enough to make breathing difficult, call an ambulance as soon as possible and inform the doctors or paramedics about your dystonia (and its worsening).

Dystonia – treatment

Apply three treatments for dystonia:

1. surgical,
2. oral or intrathecal;
3. botulinum toxin.

Surgical treatment of dystonia (deep brain stimulation – DBS) consists in placing special electrodes from a part of the damaged brain. They are supposed to stimulate muscle tension with the help of neurotransmitters. This method is used when the disease takes a very severe form and does not respond to any other forms of treatment. More specifically, a part of the brain called the pale globule, which is responsible for the development of stiffness, is stimulated.

However, it is not the only operational method because there is still a possibility perform a procedure called a thalamotomy. It involves very precisely injuring a part of the brain called the thalamus to reduce tremors. It should be added that this procedure brings improvement in about 2 out of 3 patients undergoing this procedure.

Treatment for oral dystonia is similar to surgical treatment. The medications you take affect the nerve neurotransmitters. They are designed to prevent uncontrolled tensions. The drugs that are given for dystonia are, first, levodopa. If this drug does not bring the expected results, further drugs are administered, including benzodiazepines, baclofen or tizanidine. However, it should be added that oral treatment is often ineffective. When administering drugs intrathecally (in the lumbar spine), the doctor inserts a needle and then administers the drugs directly into the cerebrospinal fluid surrounding the spinal cord. The drug that is administered in this method is baclofen.

Leczenie toksyną botulinową polega na podawaniu botoksu do organizmu. Hamuje on napięcie nerwowe, a jednocześnie zapobiega niekontrolowanym ruchom. Metodę tą stosuje się w przypadku kurczu powiek, kręczu karku, dystonii ust i żuchwy. Przed zabiegiem konieczne jest wykonanie badania elektromiograficznego (EMG) lub ultrasonograficznego (USG), dzięki czemu znane będzie dokładne miejsce, do którego należy wstrzyknąć toksynę botulinową. Należy dodać, że w przypadku tej metody, chorzy odczuwają ogromną różnicę i komfort, związane z powrotem do prawidłowego funkcjonowania. Efekty toksyny botulinowej utrzymują się do trzech miesięcy, a po tym czasie należy zabieg powtórzyć.

Bez względu na metodę osoby chore na dystonię powinny być objęte opieką rehabilitacyjną, pozwalającą na usprawnianie ruchowe.

Dystonia – treatment during pregnancy

Very little research has been done on the effects of drugs for dystonia and botulinum toxin injections in pregnant women, so to be careful, it is generally recommended that pregnant women stop treatment. In some cases, a woman may (gradually) stop taking oral medications completely, in others their dosage is reduced. Treating dystonia during pregnancy is very individual and each woman’s situation is different. There is no single recommendation for all patients and women should discuss their situation with an experienced specialist in movement disorders.

Dystonia – other treatments

Chiropractic

People with dystonia should exercise extreme caution when considering traditional chiropractic care to address symptoms of dystonia. Due to the nature of chiropractic treatments and the nature of dystonia, there is a clear possibility of harm being done. A gentle massage can temporarily relieve muscle tension, but before seeking help from a chiropractor or massage therapist (or other therapist), make sure the specialist fully understands dystonia and the consequences of a person’s individual symptoms.

Acupuncture

Although the published information is limited, anecdotal reports suggest that acupuncture may help relieve pain in people with dystonia. The benefits of acupuncture are usually cumulative and require more than one visit to the doctor before they become apparent. Acupuncture can be used in conjunction with traditional medications without negative interactions.

Hypnosis

Hypnosis is not aimed directly at dystonia, but regular relaxation techniques such as self-hypnosis can improve the patient’s well-being and quality of life.

Dystonia – Where to Find Support?

Due to the small number of people aware of their disease, the Polish Association of People with Dystonies took patronage over the support group. The founder is a sick person who needed contact with a human who would understand his problems. It is semi-formal because it is a Facebook group “Dystonia Polska group support”. There, patients can exchange their experiences and talk to people with similar ailments.

Dystonia – szanse na wyleczenie

When it comes to complete recovery from the disease, this is admittedly, about 1 in 10 patients resolve their symptoms of focal dystonia spontaneously, e.g. torticollis or eyelid torticollis. However, dystonia most often turns into a chronic form with paroxysmal symptoms or sustained spasm. Treatment consisting in administering drugs or injecting botulinum toxin gives good results in some patients. In the case of surgical treatment, this method increases the quality of life in 2 out of 3 patients. In patients whose body responds well to the treatment, the disease has little effect on the quality of life and the patient’s performance.

Niestety w przypadku dystonii pierwotnej, której pierwsze objawy wystąpiły w dzieciństwie, przyjmuje się, że in every fourth patient this disease leads to permanent disability. This is due to the transition of the disease into a generalized form accompanied by excessive contractions.

Dystonia – post-treatment management

Since most people with dystonia do not fully heal, they need to see a neurologist regularly, which is of great importance for people who are taking oral medications or undergoing botulinum toxin treatments. If these methods do not bring the expected result, the patient may be referred

In the case of a neurosurgeon, who in turn will decide on possible neurosurgical treatment. In addition, reporting for follow-up visits is also important for another reason, because the treatment of dystonia is selected individually for each patient and it is during the visits that the response of the patient’s organism to the therapy used at a given time is assessed. spontaneously resolving dystonia should be monitored for recurrence of symptoms. However, if these come back and the patient feels, for example, cramps, muscle pain or other symptoms that occurred earlier during the disease, he should contact a doctor as soon as possible, who will determine whether the disease has recurred.

Dystonia – prevention

In primary dystonia, it results from a genetic disorder that is beyond the control of the patient. As a result, there is no effective prevention of this form of dystonia.

However, the situation is different in the case of secondary dystonia, which is caused by external factors, which in turn makes it possible to avoid the disease. Prophylaxis then consists of avoiding harmful factors such as manganese, methanol, cyanides and carbon monoxide. In addition to them, drugs such as neuroleptics (i.e. antipsychotics) or metoclopramide should also be avoided.

It should be added, however, that neuroleptics are used in the treatment of serious psychiatric diseases and the doctor who decides to treat the patient with them, weighs the benefits and risks associated with their use each time. This is because these drugs may, in some cases, be necessary to control a psychiatric disease.

Dystonia – diet

The correlation between dystonia and diet is unknown. In general, people with dystonia should eat a nutritious diet with enough calories given the amount of muscle activity that occurs throughout the day. Stimulants such as nicotine and caffeine can make symptoms worse. In some cases, alcohol may reduce (or worsen) symptoms temporarily.

Overall, dietary supplements and vitamins have not been shown to dramatically improve movement disorders. Substances that make a person more relaxed, calm, or sleepy may alleviate symptoms nonspecifically. It is important for individuals to discuss their vitamins and supplements with their specialist in movement disorders to guard against potential interactions or safety concerns. Be aware that certain foods may interfere with the absorption of certain medications.

Dystonia – effect on bones

Dystonia does not primarily affect bones, but due to abnormal posture that results from dystonic contractions, bones can be subjected to unusual mechanical stress. For example, if someone has severe dystonia that requires a longer duration in one position, the ligaments and tendons may shorten so that the joint becomes “contracted” and can no longer move freely through the full range of motion. Over time, it is expected to cause excessive wear and tear on the affected bones.

Even without contracture, some bones may wear excessively due to such abnormal mechanical stresses. However, bone changes are usually not symptomatic in people with dystonia. We are increasingly concerned about the effects of dystonia on muscles and related support tissues as they affect posture.

Dystonia – Muscle Effects

Dystonia attacks muscles that can be controlled voluntarily, mainly skeletal muscles. Dystonia does not affect smooth muscles such as the heart. However, dystonia can affect breathing in several ways. Severe neck dystonia can cause breathing difficulties if the upper respiratory tract is affected. Vocal cord dystonia can potentially cause breathlessness when the vocal cords tighten strongly, but pressure generally occurs mainly when speaking. Breathing involves the muscles between the ribs and a large muscle called the diaphragm.

Dystonia może powodować sztywność mięśni między żebrami i może powodować uczucie duszności. Czasami może to również dotyczyć przepony. Wreszcie, gdy osoba z dystonią ma zajęty kręgosłup, skręcenie tułowia może ograniczyć rozszerzanie się płuc podczas oddychania, co może potencjalnie powodować duszność.

The bladder consists of smooth muscles and is therefore not affected by dystonia. However, very rarely, the muscles around the bladder opening (external sphincter) may become involved, making it difficult to pass urine.

Dystonia and Parkinson’s disease

Dystonia is persistent or repeated muscle contractions that can occur at different times of the day and at different stages of Parkinson’s disease. Dystonia is a common early symptom of Parkinson’s disease.

Osoby z chorobą Parkinsona najczęściej skarżą się na bolesną dystonię stopy po stronie bardziej dotkniętej chorobą. Zwykle dzieje się to z samego rana, kiedy poziom dopaminy jest najniższy, gdy leki stosowane na noc przestają działać. Ten bolesny skurcz zwykle ustępuje po pierwszej dobowej dawce leków na chorobę Parkinsona. Rzadziej dystonia może wpływać na inne części ciała i pojawiać się i odchodzić w ciągu dnia, nie mając żadnego związku z czasem przyjmowania leków. Może powodować gwałtowne ruchy skrętne, które na przykład mogą wygiąć rękę osoby chorej za jej plecy lub wyciągnąć głowę na bok, czy w kierunku klatki piersiowej. Ruchy te różnią się od płynnych, wijących się ruchów dyskinezy, które zwykle nie są bolesne.

Although people with Parkinson’s disease sometimes have dystonia, the movement disorder is not necessarily the result of the condition. People can have dystonia without Parkinson’s disease. Whether dystonia is part of Parkinson’s disease or not, it is often treated with the same medications.