About Dystonia

Dystonia is a neurological movement disorder, in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures. The disorder may be hereditary or caused by other factors such as birth-related or other physical trauma, infection, poisoning (e.g., lead poisoning) or reaction to pharmaceutical drugs, particularly neuroleptics. Treatment is difficult and has been limited to minimizing the symptoms of the disorder, since there is no cure available.

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Early-onset generalized dystonia

Early-onset generalized dystonia is characterized by the twisting of the limbs, specifically the foot and leg or hand and arm. The spasms may spread to involve twisting contractions of other parts of the body.

Early onset dystonia can be broadly divided into two major categories: DYT1 early onset generalized dystonia and non-DYT1 early onset dystonia. The distinction is that DYT1 generalized dystonia is known to be caused by a specific mutation in the DYT1 gene. However, not all primary generalized dystonias that begin in childhood are caused by this specific mutation. These forms are simply referred to as non-DYT1 generalized dystonia.

DYT1 dystonia is primary. Non-DYT1 dystonia may be primary or secondary.

Terms used to describe DYT1 generalized dystonia include: Oppenheim’s dystonia, primary torsion dystonia, early onset dystonia, childhood onset dystonia, idiopathic torsion dystonia. Lesser-used terms include: dystonia musculum deformans

Terms used to describe non-DYT1 generalized dystonia include: Primary torsion dystonia, early onset dystonia, childhood onset dystonia, idiopathic torsion dystonia. Lesser-used terms include: dystonia musculorum deformans

Historically, early-onset generalized dystonia has also been referred to as idiopathic torsion dystonia and dystonia musculorum deformans.

Symptoms
Symptoms of early-onset generalized dystonia can include:

  • Twisted postures, for example in the torso or limbs
  • Turning in of the foot or arm
  • Muscle spasms, with or without pain
  • Unusual walking with bending and twisting of the torso
  • Rapid, sometimes rhythmic, jerking movements (often myoclonic jerks)
  • Progression of symptoms leading to areas of the body remaining in sustained or fixed postures

Symptoms often begin in one area of the body and spread to other areas. Factors such as age and the part of the body where symptoms begin play a significant role in the progression. The younger the age of onset, the more likely the dystonic symptoms will begin in one of the legs, spread upward to other areas, and possibly become generalized. However, if symptoms begin in the arm or neck, the progression to other body areas may be less likely. The age of onset varies, but the peak period is between the ages of seven and ten years with symptoms progressing, then stabilizing, within a five-year period.

Symptoms commonly begin with a specific action and are not present at rest. For example, if symptoms begin in one leg, they may only be present when walking and disappear when the individual runs or walks backwards.

In generalized dystonia that begins in the arm, symptoms may be task-specific, i.e. apparent only during specific activities such as writing or playing a musical instrument. If the disorder progresses, the symptoms of arm dystonia may appear when another part of the body is engaged in voluntary motor activity. If the dystonia spreads to involve parts of the body other than then limb of onset, it will first move to adjacent segments of the body and then outward to areas farther from the site where symptoms began.

Dystonia is usually present continually throughout the day whenever the affected body part is in use and may disappear with sleep.

Features such as cognition, strength, and the senses, including vision and hearing, are normal. While is a chronic disorder, it is not fatal. dystonia-foundation.org

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