West syndrome: causes, symptoms and treatment

West Syndrome is a medical condition characterized by the presence of epileptic spasms during early childhood. As with other types of epilepsy, West Syndrome follows a specific pattern during the spasms, as well as the age at which they occur.

Below we explain what West Syndrome is, what are the main symptoms and causes, how it is detected and what treatments are the most frequent.

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What is West Syndrome?

West Syndrome is technically defined as an age-dependent epileptic encephalopathy. This means that it is associated with epileptic seizures that originate in the brain, which at the same time contributes to its progression. It is said to be an age-dependent syndrome because it occurs during early childhood.

Broadly speaking, these are groups of rapid and abrupt movements that usually begin during the first year of life. Specifically between the first three and eight months. Less frequently it has also been presented in the course of the second year. For the same reason, the West Syndrome

It is also known as infantile spasm syndrome..

While it is characterized by the presence of repetitive compulsive patterns, and also by a determined electroencephalographic activity, has also been defined as an "epileptic syndrome electroclinical”.

This condition was first described in the year 1841, when the English-born surgeon William James West studied the case of his own son 4 months old.

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Main causes

Initially, the crises usually occur in isolation, with few repetitions and short duration. For the same reason it is common for West Syndrome to be identified when it has already advanced.

One of the main causes that have been found for West Syndrome is hypoxia-ischemicalthough this can vary. In any case, the causes of the syndrome have been associated with prenatal, neonatal and postnatal history.

1. prenatal

West Syndrome can be caused by different chromosomal abnormalities. Also for neurofibromatosis, some infections and metabolic diseases, as well as hypoxia-ischemic, among other causes that take place in the period before birth.

2. perinatal

It can also be caused by hypoxic-ischemic encephalopathy, selective necrosis or hypoglycemia, among other medical conditions that originate in the period from the 28th week of gestation to the seventh day of birth.

3. postnatal

West Syndrome has also been linked to different infections that occur after the child is born and in early childhood development, such as bacterial meningitis or brain abscesses. It has also been linked to bleeding, trauma and the presence of brain tumors.

most common symptoms

West Syndrome typically presents with sudden (forward) flexion, accompanied by body stiffness that affects the arms and legs on both sides equally (this is known as a "tonic form"). Sometimes it presents with the arms and legs throwing forward, which is called "extensor spasms."

If the spasms occur while the child is lying down, the typical pattern is forward flexion of the knees, arms, and head.

Although single spasms may occur, especially early in the development of the syndrome, epileptic seizures usually last a second or two. Then a pause may follow, and immediately one more spasm. In other words, they tend to occur repeatedly and frequently.

Diagnosis

this condition can affect the development of the baby in different areas, as well as cause a lot of impression and anguish to their caregivers, so it is important to know about their diagnosis and treatments. Currently there are several options to control spasms and improve the electroencephalographic activity of children.

The diagnosis is made by means of an electroencephalographic test that can demonstrate or rule out the presence of "hypsarrhythmia", which are disorganized patterns of electrical activity in the brain.

Sometimes these patterns can only be visible during sleep, so it is common for the electroencephalogram to be performed at different times and accompanied by other tests. For example, brain scans (magnetic resonance imaging), blood tests, urine tests, and sometimes cerebrospinal fluid tests, to help locate the cause of the syndrome.

Main treatments

The most common treatment is pharmacological. There are studies that suggest that West Syndrome usually responds favorably to antiepileptic treatment, such as Vigabatrin (known as Sabril). The latter inhibits the decrease in gamma-aminobutyric acid (GABA), the main inhibitor of the central nervous system. When the concentration of this acid decreases, electrical activity can be accelerated, so these drugs help regulate it. The same sense are used nitrazepam and epilim.

Likewise, corticosteroids can be used, such as adrenocorticotropic hormones, a treatment that turns out to be very effective in reducing crises and hypsarrhythmia. Both corticosteroids and antiepileptics are used with important medical control due to the high possibility of developing adverse effects important.

The treatments can be more or less prolonged depending on the way in which the syndrome is presented in each case. In the same sense, epileptic episodes can have different consequences, especially in the development of the nervous system.

The abilities related to psychomotor development and some cognitive processes can be affected differently. Likewise, it is possible that children develop other types of epilepsy in other periods of childhood. The latter can also follow a specific treatment depending on the way in which they present themselves.

Bibliographic references:

• Epilepsy Action (2018). West syndrome (infantile spasms). Retrieved June 29, 2018. Available in https://www.epilepsy.org.uk/info/syndromes/west-syndrome-infantile-spasms
• Arce Portillo, E., Rufo Campos, M., Muñoz Cabello, B, et. al (2011). West syndrome: etiology, therapeutic options, clinical evolution and prognostic factors. Journal of Neurology, 52(2): 81-99.
• Pozo Alonso, A., Pozo Lauzán, D. and Pozo Alonso, D. (2002). West syndrome: etiology, pathophysiology, clinical aspects and prognosis. Cuban Journal of Pediatrics. 74(2): 151-161.