Dystonia


DYSTONIA

What is Dystonia?
The dystonia disorders are related to movement and are characterised by sustained muscle contractions that cause repetitive twisting movements or abnormal postures. The movements, which are involuntary and sometimes painful, may affect a single muscle, for example; or a group of muscles such as those in the arms, legs, or neck; or the entire body. Those with dystonia usually have normal intelligence and no associated psychiatric disorders.

 

Symptoms of Dystonia

 

Early-onset isolated Dystonia:

  • In most instances muscle contraction starts in the leg and moves to the trunk and then other parts of the body

Cervical Dystonia/spasmodic torticollis:

  • In most instances the muscle contractions affect the neck and shoulder
  • Pain
  • Head tremor

Blepharospasm:

  • Muscle contractions occur in the eyelid
  • Increased blinking
  • Eye spasms

Oromandibular and facial dystonia:

  • Muscle contractions occur in the face and throat
  • Clenching of jaw
  • Jaw may stay open
  • Jaw may be crooked
  • Tongue rolls out of mouth
  • Difficulty speaking
  • Difficulty swallowing

Spasmodic Dysphonia:

  • Muscle contractions occur in the voice box
  • Strained sounding voice
  • When speaking only able to whisper

Arm Dystonia:

  • Muscle contractions occur in the arm and hand
  • Hand or arm may stay in odd position

 

What causes Dystonia?
The exact causes of dystonia are unknown, but these may influencing be factors:

  • Communication between nerve cells may be altered
  • Genetic changes
  • May be a symptom of another disorder (e.g. Parkinson's disease, brain tumour, Wilson's disease)

 

Diagnosis of Dystonia
There is no specific test to diagnosis Dystonia, but a doctor can form diagnosis based on the symptoms experienced. Blood and radiology tests may be done to rule out any other diagnoses.

 

Treatment
Dystonia only affects the body's movements and not one's thinking or understanding, currently there is no cure but management is aimed at symptomatic treatment. Treatment is based on the type of Dystonia.

 

Treatments available

 

Botulinum toxic: these injections relax the muscle and are most effective for focal dystonia. The effect sets in a few days after receiving injection and can last for a few months before next injection is required.

 

Medication: Drugs used affect the different neurotransmitters by:

  • blocking the effects of the neurotransmitters
    OR
  • regulating the neurotransmitter GABA
    OR
  • by working on the neurotransmitter Dopamine to relax muscles.

 

Deep brain stimulation: This form of treatment is considered when medication does not work or the side effects of medication are severe. It involves sending electrical signals to the area that generates the dystonia symptoms via electrodes to block or interfere with electric impulses that cause the symptoms of dystonia.

 

Self-care:

  • Comply with the medication regime and/or treatment plan as prescribed by your doctor.
  • Collect your medication on time.
  • Report side effects to your doctor and take appropriate action (as indicated by doctor).
  • Maintain all follow-up visits with your doctor.
  • Discuss the condition and fears with your doctor.
  • Ensure that the people who make up your support system are educated about your condition.

 

References
1. MAYO CLINIC. 2012. , Dystonia. , 2012
2. NATIONAL INSTITUTES OF HEALTH (NIH). ,2015.  Dystonias Fact Sheet,. 2015
3. UPTODATE, PATIENT INFORMATION. : Dystonia (The Basics), Topic 83332, Version 3.0.