Rabu, 26 Maret 2014

Dystonia: Movement Disorders Can Appear In Children


Dystonia is a movement disorder in which sustained muscle contractions that continue to cause rotary motion and causes recurrent or abnormal posture .

The movement is not realized , and sometimes painful , may affect a single muscle , muscle group ( eg, muscular arms , legs or neck ) or the entire body .

In some patients , symptoms of dystonia appear in childhood ( 5-16 years ) , usually on the feet or hands .

Some other new patients show symptoms in late adolescence or early adulthood .

CAUSE

The experts believe that dystonia occurs because of abnormalities in several regions of the brain ( basal ganglia , thalamus , cerebral cortex ) , where several messages for orders processed muscle contraction .
Allegedly there is damage to the body's ability to process a set of chemicals that disebtu neurotransmitters , which help the cells in the brain to communicate with each other .

Dystonic symptoms can be caused by :
- Injuries at birth ( mainly due to lack of oxygen )
- Certain infections
- Reaction to certain drugs , heavy metal or carbon monoxide poisoning
- Trauma
- Stroke .

Approximately 50 % of cases have no connection with illness or injury , and is called idiopathic primary dystonia or dystonia .
The rest are offspring that are dominant dystonia .

Dystonia can also be a symptom of other diseases , some of which are derived ( eg Wilson's disease ) .

SYMPTOMS

Early symptoms are a decline in writing ( after writing a few lines kalima ) , leg cramps and kecenderunagn attraction of one leg up or leg dragging tendencies after walking or running at a certain distance .

Rotating the neck or interested people outside of awareness , especially when the patient feels tired .

Other symptoms are tremor and difficulty speaking or making a sound .

Initial symptoms can be very mild and is felt only after heavy exercise , stress or because of fatigue .
Over time the symptoms become more apparent and spreads as well as unbearable .

Classification of dystonia

Based on the affected body part :

1 . Generalized dystonia , the most or the entire body
2 . Focal dystonias , limited to a particular body part
3 . Multifocal dystonia , about 2 or more unrelated body parts
4 . Segmental dystonia , the two or more adjacent body parts
5 . Hemidistonia , involving the arm and leg on the same side of the body , often the result of a stroke .


Some patterns of dystonia have typical symptoms :

1 . Torsion dystonia , previously known as dystonia musculorum deformans or DMD .
Generalized dystonia is rare and can be lowered , usually begins in childhood and progressively get worse .
Patients may experience serious defects and had to sit in a wheelchair.

2 . Torticollis Spasmodic torticollis is a focal dystonia or most frequently found .
Attacking the muscles in the neck that control the position of the head , so the head spins and turned to one side . In addition , the head can be pulled forward or backward .
Torticollis can occur at any age , although most patients first experience symptoms in middle age . Often start slowly and usually will peak .
Approximately 10-20 % of patients experienced remission ( symptom -free periods ) spontaneous , but it did not last long .

3 . Blepharospasm an eyelid closure unconscious .
Initial symptoms can include loss of control of eye pengedipan .
At first only affects one eye , but eventually both eyes are usually affected .
Seizures cause the eyelids completely closed causing functional blindness , though the eyes and normal vision .

4 . Cranial dystonia is dystonia that affects the muscles of the head , face and neck .

5 . Dystonia oromandibuler attacked the muscles of the jaw , lips and tongue .
The jaws can be opened or closed and patients have difficulty speaking and swallowing .

6 . Spasmodic dysphonia melibatkanotot throat that control the process of speaking .
Also called spastic dysphonia or laryngeal dystonia , which causes difficulty in speaking or breathing .

7 . Meige syndrome is a combination of blepharospasm and dystonia oromandibuler , sometimes with spasmodic dysphonia .

8 . Cramps authors is that attacks muscle dystonia and sometimes hand down the front of the arm , only occurs during the hand used for writing .
Uga called dystonia same cramping and cramping musician piano player .

9 . Dopa - responsive dystonia is dystonia were successfully overcome with medication .
One important variant is Segawa dystonia
Starts during childhood or adolescence , such as difficulty in walking .
In Segawa dystonia , the symptoms have fluctuated throughout the day , ranging from the ability to move in the morning to an inability in the afternoon and evening , well after doing the activity .


Diagnosis

The diagnosis is based on symptoms.

TREATMENT

A number of actions taken to reduce or eliminate muscle spasms and pain :

1 . Drugs .
It has been used several types of drugs that help correct the imbalance of neurotransmitters .
Drugs are given a set of drugs that reduce levels of neurotransmitter acetylcholine , which is triheksifenidil , benztropine and procyclidine HCl .
Drugs that regulate the neurotransmitter GABA can be used in conjunction with the above drugs or given separately ( in patients with mild symptoms ) , namely diazepam , lorazepam , clonazepam and baclofen .
Other drugs have an effect on the neurotransmitter dopamine . Drugs that increase dopamine effect is levodopa / carbidopa and bromocriptine . Drugs that reduce the effects of reserpine or dopamine is tetrabenazin .
In order to control epilepsy are given anti-seizure medication carbamazepine .

2 . Poison botulinum .
A small amount of this toxin can be injected into the affected muscles to reduce focal dystonia .
At the beginning of this toxin is used to treat blepharospasm .
Toxins stop muscle spasms by inhibiting the release of neurotransmitters acetylcholine . Its effects last for several months before repeat injections done .

3 . Surgery and other treatments .
If drugs do not work or the side effects are too severe , then surgery .
Generalized dystonia advanced stages have been successfully treated with surgery that destroyed a portion of the thalamus . The risk of this surgery is a disorder of speech, because the thalamus is located near the brain structures that control the process of speaking .
In focal dystonia ( including blepharospasm , spasmodic dysphonia and torticollis ) surgery to cut or lift the nerve of the affected muscles .
Some patients with spasmodic dysphonia can undergo treatment by a speech-language pathologist . Physical therapy , splinting , stress management and biofeedback can also help people with certain types of dystonia .