What is Tardive Dystonia?
Extrapyramidal symptoms (EPS), which can be understood as a condition that affects your ability to control your movements, first appeared in the 1950s after a new type of drug called neuroleptics got discovered. These symptoms were formally recognized at a medical conference in Switzerland in 1953. The drugs were named “neuroleptic” because they seemed to help calm people down who were experiencing restlessness from mental health conditions.
Initially, it was believed that these symptoms would appear soon after taking the medication. However, it was later found that certain movement abnormalities might not emerge until later on.
In 1957, a doctor named Schoenecker reported the first case of a condition that would later be known as “tardive dyskinesia”. This condition was seen in a patient who had been on neuroleptic therapy for a long time, and they were having involuntary movements, especially around their mouth. This was different from the symptoms that occur immediately after taking the drug.
The term “tardive dyskinesia” officially came into use in medical language in 1964, thanks to Faurbye. Later, in 1973, the terms “dystonia tarda” were introduced following the story of a female patient who developed involuntary neck twisting.
The phenomenon was then thoroughly described in 1982, under the name “tardive dystonia”. Dystonias are movements that people can’t control; they can be distressing and often painful. They are characterized by twisting and repetitive postures because different muscle groups are contracting at the same time.
What Causes Tardive Dystonia?
The exact cause and process of tardive dystonia, a disorder which causes uncontrollable muscle contractions, is still unknown. However, it’s generally thought that tardive dystonia happens when a type of drug that blocks dopamine (a chemical messenger in the brain) leads to too much activity in response, resulting in these muscle contractions once the drug has been processed and broken down by the body. Some believe that these muscle contractions are simply due to too much blocking of dopamine.
Interestingly, either way, what is commonly agreed upon is that a part of the brain called the nigrostriatal tract is involved. It’s thought that unusual activity of dopamine within a region of the brain called the basal ganglia eventually leads to symptoms indicating damage to the nervous system (extrapyramidal symptoms).
The medications that are known to block the dopamine receptor and can cause this condition belong to a group called neuroleptics. These are primarily antipsychotics. But other types of drugs that block dopamine have also been observed to potentially cause tardive dystonia.
Risk Factors and Frequency for Tardive Dystonia
Acute and tardive dystonic reactions are side effects of certain medications, but they’re less common than symptoms similar to Parkinson’s disease or restlessness. However, there’s discussion among experts about the risk of developing tardive dystonia; studies suggest it to be less than 1 to 2% among patients taking antipsychotic drugs. Risk factors of these reactions can include age, being male, a past history of dystonic reactions, using cocaine, having electrolyte abnormalities, and being dehydrated. Most research agrees that young males are more likely to develop tardive dystonia. This difference by sex might be due to schizophrenia, which these drugs treat, typically starting earlier in males.
- Tardive and acute dystonic reactions are less common side effects of certain drugs compared to symptoms similar to Parkinson’s or restlessness.
- The risk of developing tardive dystonia from taking antipsychotic drugs is estimated at less than 1 to 2%.
- Younger males tend to experience tardive dystonia more often.
- Being older, male, having a past history of dystonic reactions, using cocaine, having irregular levels of electrolytes, and being dehydrated are all risk factors.
One study revealed that the time period between the start of the drug and the onset of tardive dystonia can range from four days to 23 years, with an average of about six years. These researchers also noticed that the younger the patient, the shorter the time from drug start to symptom onset. Similarly, people who developed dystonia at a younger age tended to see their symptoms worsen quicker.
Signs and Symptoms of Tardive Dystonia
Tardive dystonia is a condition that typically develops slowly and gets worse over time. It often appears sometime after the start of treatment with a certain type of medication that blocks dopamine receptors. The exact time it takes for symptoms to appear can vary widely – from days to years after the first dose of medicine. Some patients have reported unusual feelings or strange bodily sensations before the actual onset of the condition. A common early symptom is neck pain, which often happens before the development of cervical dystonia – a specific type of tardive dystonia.
Research shows that tardive dystonia usually starts as a focal dystonia, affecting specific areas. This condition most commonly manifests as abnormal neck posture (torticollis) or eye spasms (blepharospasm). Other areas it may affect include the throat (causing trouble swallowing), the jaws and mouth (leading to severe speech problems), and the lips and tongue.
Involvement of the arms, legs, and trunk is less common. Although it begins in one area, over time, the condition might spread and affect a wider area.
- Starts slowly and worsens over time
- Occurs days to years after starting dopamine-blocking medication
- Early symptoms include unusual feelings and neck pain
- Commonly manifests as neck posture issues or eye spasms
- Can affect the throat (causing swallowing problems), jaws and mouth (causing speech problems), and the lips and tongue
- Spread to the arms, legs, and trunk is less common
- May spread to wider areas over time
Testing for Tardive Dystonia
If you’re dealing with tardive dystonia, a movement disorder mainly affecting the face, neck and tongue, quick recognition and treatment are vital for your health. The healthcare professionals begin the evaluation process by looking at your main medical issues. They then describe any unusual and involuntary muscle movements you’re having, determine how severe your symptoms are and use this information to create a custom treatment plan.
During this process, your doctor will also review all your medical records and the medicines you’re taking. This is because certain medications that interfere with dopamine (a chemical messenger in your brain) can cause these movement disorders. Some of these medications are used to treat nausea and vomiting, like prochlorperazine, metoclopramide, and promethazine.
Treatment Options for Tardive Dystonia
Tardive dystonia refers to a movement disorder characterized by involuntary muscle contractions. If identified correctly, it’s recommended to change or completely stop taking the medication causing the issue. It is up to your doctor to decide whether this change should be carried out slowly or abruptly. Some experts recommend switching to a different kind of medication like clozapine, which has a lower risk of causing tardive dystonia.
The cause of drug-induced tardive dystonia is thought to be a blockade of specific pathways in the brain that deal with the neurotransmitter dopamine. This imbalance leads to an increase in cholinergic activity compared to dopaminergic activity. Therefore, medications that decrease cholinergic activity and increase dopamine functions in the brain might help balance things out.
A study found varying levels of relief for patients using different treatments. Roughly half of the patients reported at least some benefit after being administered tetrabenazine, anticholinergic agents, benzodiazepines, or baclofen. The most significant improvement, with 83% of patients reporting considerable relief, followed the administration of botulin toxin (Botox). Other possible treatments to consider include L-dopa, dopamine agonists, amantadine, beta-blockers, anticonvulsants, choline, and vitamin E.
What else can Tardive Dystonia be?
Diagnosing a condition called tardive dystonia can be quite tricky because there are many possible causes. This can include ‘primary’ conditions that exist on their own, or ‘secondary’ conditions which are caused by something else. For example, secondary causes can include metabolic problems, harmful substances, deterioration of brain cells, or physical damage to the brain.
One way to pinpoint if the dystonia has been caused by certain medications is by checking if the patient has been using drugs for treating mental disorders, if there’s no family history of primary dystonia, and if there are no other associated neurological signs. Interestingly, patients who have been on these mental health drugs for a long time may also show signs of ‘tardive syndromes’, which can complicate the diagnosis. Some people may even have more than one of these syndromes at the same time.
These tardive syndromes include:
- Tardive dyskinesia, characterized by uncontrolled, awkward movements
- Tardive dystonia, marked by repetitive muscle spasms that can cause twisting and turning
- Tardive akathisia, where the person experiences a uncomfortable restlessness or unease.
Interestingly, tardive dyskinesia generally appears later in life compared to tardive dystonia. The average age of onset observed in a research group for tardive dyskinesia was 57 years, while for tardive dystonia it was 37 years.
What to expect with Tardive Dystonia
Once it starts, dystonia, a condition that causes uncontrollable muscle contractions, tends to worsen gradually over several months to years and may affect additional parts of the body. Research shows that such progression usually reaches its highest intensity within two years from the onset of symptoms. It can progress more quickly in people who are younger when the condition begins. Tardive syndromes, a group of and movement disorders, can cause significant distress and disability. These syndromes may even become permanent, not improving even after stopping the treatment that may have initially caused the problem.
A study tried to group patients based on the resolution of symptoms after treatment. In this study of 107 patients being treated for tardive dystonia, 14% went into remission, 39% improved, and 50% reported either no improvement or worsening symptoms. Among these patients, those who stopped taking their neuroleptic drugs, used to treat mental health conditions, were more likely to have their symptoms go away. Specifically, those who stopped taking antipsychotic medications more quickly had a higher chance of recovery. On average, recovery was reached within about 2.4 years after stopping all neuroleptic medications. However, dystonia seems to be irreversible in those who have been exposed to neuroleptics for more than 10 years.
Possible Complications When Diagnosed with Tardive Dystonia
Tardive dystonia can vary in severity, from almost unnoticeable to extremely debilitating. When it’s mild, it might cause muscle tightness and cramps that make chewing and talking uncomfortable. When it’s more severe, it can lead to complications like a severe eye condition called oculogyric crisis, trouble swallowing (dysphagia), speech impairments (dysarthria), and life-threatening breathing difficulties (respiratory stridor). Sometimes, severe dystonic reactions might even stop your breathing, which can be lethal. Less common complications can affect your axial, limb, and trunk muscles, causing conditions like Pisa syndrome, camptocormia, and opisthotonus.
Effects can include:
- Muscle tightness and cramps
- Chewing and talking discomfort
- Oculogyric crisis, severe eye condition
- Dysphagia, trouble swallowing
- Dysarthria, speech impairments
- Respiratory stridor, life-threatening breathing difficulties
- Apnea, a condition that might stop your breathing and become lethal
- Affecting your axial, limb, and trunk muscles
- Pisa Syndrome
- Camptocormia
- Opisthotonus
Preventing Tardive Dystonia
Doing well after experiencing EPS (extrapyramidal symptoms, which are movement disorders) largely depends on how quickly the symptoms are noticed and treated. Long-term management of these symptoms can also be improved by taking lower doses of a type of medication called neuroleptics, which are used to treat mental health conditions. Additionally, certain antipsychotic medications that don’t block the actions of dopamine (a chemical in the brain) as strongly may be used. This approach has led to the development of new medications that block dopamine less, helping reduce the side effects.
Because these new types of antipsychotics, also known as atypical antipsychotics, interact less with the dopamine receptors in the brain, they tend to be favored over the stronger first-generation antipsychotics. For instance, a study found that a first-generation antipsychotic called haloperidol was four times more likely to cause movement disorder symptoms compared to atypical antipsychotics. Therefore, some doctors decide to give preventive treatment with anticholinergic agents (medications that block certain nerve impulses), along with the neuroleptics, to prevent potential EPS.
