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Wilsons Disease
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West Syndrome

INFORMATION ON WEST SYNDROME:-
West syndrome may be defined as a rare neurological disorder that occurs in early childhood. The development of west syndrome is predominantly in the first year of life. Characteristic features of west syndrome is, Myoclonic seizures, hypsarrhythmia (abnormal, chaotic electroencephalogram), and mental retardation.

CAUSES OF WEST SYNDROME:-
The main factors which leads to the development of west syndrome are:-
1. Improper function of brain stem.
2. Due to some immunological abnormality.
3. Alteration in the brain-adrenal axis in patients with west syndrome.
4. Disturbance in genetic architecture.

SYMPTOMS OF WEST SYNDROME:-
1. Myoclonic seizures
2. Hypsarrhythmia (chaotic electroencephalogram)
3. Mental retardation.
4. Delay in mental development.
5. Learning disability.

DIAGNOSIS OF WEST SYNDROME:-
Clinically west syndrome can be diagnosed by:-
1. Electromyography.
2. Blood urine test.
3. Urine test.
4. CT scan.
5. MRI.
6. Lumber puncture.

TREATMENT OF WEST SYNDROME:-
The primary goal of treatment for infants suffering with West syndrome is to free infant from seizures, the fewest adverse effects from treatment, and the least number of medications.
Medications such as ACTH and conventional antiepileptic medications are the strongest and important point of therapy for infants with West syndrome. So far it’s our bad luck that no medical treatment gives 100 percent satisfaction in giving relief to all infants with West syndrome.
In some subjects a surgical procedure must be used called “resection”. Resection of a localized region can lead to freedom from seizures.
Neurosurgeons can assess whether the infant is a candidate for focal resection.
Dietitians can assist in the institution and maintenance of the ketogenic diet.

 

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