Dyssynergia cerebellaris myoclonica

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(Redirected from Primary dentatum atrophy)

Dyssynergia cerebellaris myoclonica is a rare neurological disorder characterized by a combination of myoclonus, ataxia, and other cerebellar symptoms. This condition is also known as Ramsay Hunt syndrome type 1 and is distinct from the more commonly known Ramsay Hunt syndrome type 2, which involves facial paralysis and ear rash due to varicella zoster virus infection.

Symptoms[edit | edit source]

The primary symptoms of Dyssynergia cerebellaris myoclonica include:

  • Myoclonus: Sudden, involuntary muscle jerks.
  • Ataxia: Lack of muscle coordination affecting voluntary movements.
  • Dysarthria: Difficulty in articulating words due to muscle control issues.
  • Nystagmus: Rapid, involuntary eye movements.

Causes[edit | edit source]

The exact cause of Dyssynergia cerebellaris myoclonica is not well understood. It is believed to be a genetic disorder, although specific genetic mutations have not been conclusively identified. The condition may be inherited in an autosomal dominant or autosomal recessive manner.

Diagnosis[edit | edit source]

Diagnosis of Dyssynergia cerebellaris myoclonica typically involves:

Treatment[edit | edit source]

There is no cure for Dyssynergia cerebellaris myoclonica, and treatment focuses on managing symptoms. Options may include:

Prognosis[edit | edit source]

The prognosis for individuals with Dyssynergia cerebellaris myoclonica varies. Some may experience a slow progression of symptoms, while others may have a more rapid decline in motor function. Early intervention and supportive therapies can help improve quality of life.

See also[edit | edit source]

References[edit | edit source]

External links[edit | edit source]

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Contributors: Prab R. Tumpati, MD