About west syndrome

What is west syndrome?

West syndrome is a type of epilepsy characterized by spasms, abnormal brain wave patterns called hypsarrhythmia and sometimes mental retardation. The spasms that occur may range from violent jackknife or "salaam" movements where the whole body bends in half, or they may be no more than a mild twitching of the shoulder or eye changes. These spasms usually begin in the early months after birth and can sometimes be helped with medication. They can also occur rarely in older patients; if this happens, they are called "epileptic spasms" rather than infantile spasms. There are many different causes of West syndrome and if a specific cause can be identified, a diagnosis of symptomatic West syndrome can be made. If a cause cannot be determined, a diagnosis of cryptogenic West syndrome is made.

What are the symptoms for west syndrome?

Some children arch their backs while extending their arms and legs symptom was found in the west syndrome condition

Symptoms of the west syndrome

West syndrome is a constellation of symptoms characterized by epileptic/infantile spasms, Abnormal brain wave patterns called hypsarrhythmia, and intellectual disability. The Spasms that occur range from violent jackknife where the body bends in half or very mild twItching of the shoulder. They usually occur at an early age or can occur at an older age where they are called epileptic spasms.

1. The symptoms onset from the age of 6 months.
2. These Spasms that occur are due to electrical disturbances in the brain.
3. Each of these involuntary Spasms occurs suddenly and lasts for a few seconds and then occurs again and can last for 10 to 20 minutes.
4. These sudden episodes are characterized by involuntary contraction of the head, and neck and uncontrolled extension of the leg.
5. This duration, intensity, and muscle groups differ from infant to infant
6. The infants with this syndrome have very abnormal electroencephalograms (EEG) with high amplitude, and chaotic spike-wave patterns (hypsarrhythmia).
7. The skills that require the coordination of voluntary movements and muscles might be delayed in these infants.
8. There might be some metabolic disorders as well that take place or show up as symptoms.
9. Mental retardation is also one of the symptoms of the condition.
10. A third of children with west syndrome may develop recurrent epileptic seizures.
11. The last third to quarter of patients will have Spasms that resolve with time, especially in [patients who have no clear etiology.

Symptoms
The child's body may suddenly bend forward and the arms and legs stiffen,Some children arch their backs while extending their arms and legs
Conditions
Involuntary Muscle Spasms that occur due to episodes of uncontrolled electrical disturbances in the brain (seizures)
Drugs
Adrenocorticotropic hormone (ACTH),Prednisone,Vigabatrin,Pyridoxine

What are the causes for west syndrome?

Causes of west syndrome:

1. Usually, a specific cause is detected in 70 to 75 5 patients with the west syndrome.
2. From trauma to brain malformations, any other brain disorder can be a cause of the west syndrome.
3. Chromosomal abnormalities such as Down syndrome, neurocutaneous disorders such as tuberous sclerosis complex (TSC), Sturge Weber syndrome, and incontinentia pigmenti can be the cause of west syndrome to develop.
4. Different metabolic/genetic diseases such as pyridoxine deficiency, non-ketotic hyperglycemia, maple syrup urine disorder, phenylketonuria, mitochondrial encephalopathies, biotinidase deficiency, Otahara’s syndrome, and an abnormality (mutation) in the ARX gene or CDKL5 gene located on the X chromosome. are the cause of the west syndrome
5. TSCtuberous sclerosis complex is the most common disorder the cause of the west syndrome.
TSC is an autosomal genetic condition associated with seizures, eye, heart, and kidney tumors or skin conditions.
6. X-linked West syndrome can be caused by a mutation in the CDKL5 gene or the ARX gene in the X chromosome. X-linked genetic disorders are conditions caused by a non-working gene on the X chromosome and manifest mostly in males.
7. Females that have a non-working gene present on one of their X chromosomes are carriers for that disorder. Carrier females usually do not display symptoms because females have two X chromosomes and only one carries the non-working gene.
8. Males have one X chromosome that is inherited from their mother and if a male inherits an X chromosome that contains a non-working gene he will develop the disease.
9. Female carriers of an X-linked disorder have a 25% chance with each pregnancy to have a carrier daughter like themselves, a 25% chance to have a non-carrier daughter, a 25% chance to have a son affected with the disease, and a 25% chance to have an unaffected son.
10. If a male with an X-linked disorder can reproduce, he will pass the non-working gene to all of his daughters who will be carriers.

Symptoms
The child's body may suddenly bend forward and the arms and legs stiffen,Some children arch their backs while extending their arms and legs
Conditions
Involuntary muscle spasms that occur due to episodes of uncontrolled electrical disturbances in the brain (seizures)
Drugs
Adrenocorticotropic hormone (ACTH),Prednisone,Vigabatrin,Pyridoxine

What are the treatments for west syndrome?

The most commonly approved treatment of west syndrome is medication.

1. ACTH, Vitamin B, Steroids, and Vigabatrin are some of the medications a doctor can prescribe.
2. Benzodiazepines, rufinamide, topiramate, Valproic acid, and Zonisamde are some of the other medications that one can try as the above medication like vigabatrin have side effects on a child.
3. The ketogenic diet is one thing your doctor might suggest, with low carbohydrates and high fats.
4. You might still be breastfeeding but with the use of the ketogenic formula for yourself.
5. Observing the side effects on the little one is very important during the treatment.
6. The baby’s brain might need a surgery called the corpus callosotomy if there are any seizures found in the baby's brain.
7. Resection might work for the baby if cancerous tumors are growing in the baby's brain.
8. The developmental delays can be worked around with other types of therapies for the child.

Symptoms
The child's body may suddenly bend forward and the arms and legs stiffen,Some children arch their backs while extending their arms and legs
Conditions
Involuntary muscle spasms that occur due to episodes of uncontrolled electrical disturbances in the brain (seizures)
Drugs
Adrenocorticotropic hormone (ACTH),Prednisone,Vigabatrin,Pyridoxine

What are the risk factors for west syndrome?

Risk factors of west syndrome:

1. Usually, a specific cause is detected in 70 to 75 5 patients with the west syndrome.
2. From trauma to brain malformations, any other brain disorder can be a cause of the west syndrome.
3. Chromosomal abnormalities such as Down syndrome, neurocutaneous disorders such as tuberous sclerosis complex (TSC), Sturge Weber syndrome, and incontinentia pigmenti can be the cause of west syndrome to develop.
4. Different metabolic/genetic diseases such as pyridoxine deficiency, non-ketotic hyperglycemia, maple syrup urine disorder, phenylketonuria, mitochondrial encephalopathies, biotinidase deficiency, Otahara’s syndrome, and an abnormality (mutation) in the ARX gene or CDKL5 gene located on the X chromosome. are the cause of the west syndrome
5. TSCtuberous sclerosis complex is the most common disorder the cause of the west syndrome.
TSC is an autosomal genetic condition associated with seizures, eye, heart, and kidney tumors or skin conditions.
6. X-linked West syndrome can be caused by a mutation in the CDKL5 gene or the ARX gene in the X chromosome. X-linked genetic disorders are conditions caused by a non-working gene on the X chromosome and manifest mostly in males.
7. Females that have a non-working gene present on one of their X chromosomes are carriers for that disorder. Carrier females usually do not display symptoms because females have two X chromosomes and only one carries the non-working gene.
8. Males have one X chromosome that is inherited from their mother and if a male inherits an X chromosome that contains a non-working gene he will develop the disease.
9. Female carriers of an X-linked disorder have a 25% chance with each pregnancy to have a carrier daughter like themselves, a 25% chance to have a non-carrier daughter, a 25% chance to have a son affected with the disease, and a 25% chance to have an unaffected son.
10. If a male with an X-linked disorder can reproduce, he will pass the non-working gene to all of his daughters who will be carriers.

Symptoms
The child's body may suddenly bend forward and the arms and legs stiffen,Some children arch their backs while extending their arms and legs
Conditions
Involuntary muscle spasms that occur due to episodes of uncontrolled electrical disturbances in the brain (seizures)
Drugs
Adrenocorticotropic hormone (ACTH),Prednisone,Vigabatrin,Pyridoxine

Is there a cure/medications for west syndrome?

There is no cure for the west syndrome. However, there are treatments and medications. The most commonly approved treatment of west syndrome is medication.

1. ACTH, Vitamin B, Steroids, and Vigabatrin are some of the medications a doctor can prescribe.
2. Benzodiazepines, rufinamide, topiramate, Valproic acid, and Zonisamde are some of the other medications that one can try as the above medication like vigabatrin have side effects on a child.
3. The ketogenic diet is one thing your doctor might suggest, with low carbohydrates and high fats.
4. You might still be breastfeeding but with the use of the ketogenic formula for yourself.
5. Observing the side effects on the little one is very important during the treatment.
6. The baby’s brain might need a surgery called the corpus callosotomy if there are any seizures found in the baby's brain.
7. Resection might work for the baby if cancerous tumors are growing in the baby's brain.
8. The developmental delays can be worked around with other types of therapies for the child.

Symptoms
The child's body may suddenly bend forward and the arms and legs stiffen,Some children arch their backs while extending their arms and legs
Conditions
Involuntary muscle spasms that occur due to episodes of uncontrolled electrical disturbances in the brain (seizures)
Drugs
Adrenocorticotropic hormone (ACTH),Prednisone,Vigabatrin,Pyridoxine

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