INFORMATION ON STRIATONIGRAL DEGENERATION

Striatonigral Degeneration also known as Machado-Joseph disease is a rare hereditary disease which creates a weakness condition in muscles. Striatonigral Degeneration is characterized by clumsiness and weakness in the arms and legs.

SYNONYMS OF STRIATONIGRAL DEGENERATION

1. Autosomal Dominant Spinocerebellar Degeneration
2. Azorean Neurologic Disease
3. Machado Disease
4. Joseph Disease
5. MJD
6. Nigrospinodentatal Degeneration
7. Spinocerebellar Ataxia Type III

CAUSES OF STRIATONIGRAL DEGENERATION

The main factor which leads to the development of Striatonigral Degeneration is mutation in gene due to which degeneration of cells in an area of the brain called the hindbrain leads to deficits in movement.

SYMPTOMS OF STRIATONIGRAL DEGENERATION

1. Arm weakness
2. Leg weakness
3. Speech difficulty
4. Difficulty swallowing
5. Involuntary eye movements
6. Double vision
7. Frequent urination
8. Muscle contractions
9. Repetitive movements
10. Abnormal postures
11. Parkinson’s-like symptoms
12. Twitching face
13. Twitching tongue
14. Bulging eyes

DIAGNOSIS OF STRIATONIGRAL DEGENERATION

Clinically Striatonigral Degeneration can be diagnosed by:-

1. Genetic testing.
2. Blood test.
3. Biopsy.
4. MRI.

TREATMENT OF STRIATONIGRAL DEGENERATION

So far Striatonigral Degeneration is incurable, but some symptoms of the Striatonigral Degeneration can be treated. For the subjects who show Parkinson features, levodopa therapy can help for many years. Treatment with antispasmodic drugs, such as baclofen, can help to reduce spasticity. Botulinum toxin can also be helpful in treating severe spasticity as well as some symptoms of dystonia.