West syndrome: causes, symptoms and treatment

Also known as infantile spasms syndrome, this condition affects infants as young as a few months old.

West syndrome is a medical condition characterized characterized by the presence of epileptic spasms during early infancy. As with other types of epilepsy, West syndrome follows a specific pattern during the spasms, as well as in 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 which treatments are the most frequent.

What is West syndrome?

The syndrome of West is technically defined as an age-dependent epileptic encephalopathy.. This means that it is associated with seizures originating 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, it consists of clusters of rapid, jerky movements that usually begin during the first year of life. Specifically between the first three and eight months. Less frequently, it has also occurred during the second year of life. For the same reason, West syndrome is also known as infantile spasms syndrome..

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

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

Main causes

In the beginning, seizures 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 may vary. In any case the causes of the syndrome have been associated with prenatal, neonatal and postnatal antecedents.

1. Prenatal

West syndrome can be caused by different chromosomal abnormalities. It may also be caused by neurofibromatosis, some infections and metabolic diseasesas well as by 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 neural necrosis or hypoglycemia. selective neural necrosis or due to hypoglycemiaamong 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 related 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 hemorrhage, trauma, and the presence of brain tumors.

Most common symptoms

West syndrome typically presents with sudden flexion (forward bending), accompanied by body stiffness that affects the arms and legs equally on both sides (this is known as a "forward bend"). (this is known as a "tonic form"). It sometimes 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 bending of the knees, arms and head.

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

Diagnosis

This condition can affect the baby's development in several areasIt is therefore important to know about its diagnosis and treatments. Currently there are several options to control spasms and improve the electroencephalographic activity of the little ones.

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 may be visible only during sleep, so it is common for EEGs 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, which may help to locate the cause of the syndrome.

Main treatments

The most common treatment is pharmacological. Studies suggest that West syndrome usually responds favorably to drug treatment. syndrome usually responds favorably to treatment with antiepilepticssuch 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 may be accelerated, so these drugs help to regulate it. Nitrazepam and epilim are used in the same way.

Corticosteroids can also be used, such as adrenocorticotropic hormones, a treatment that is very effective in reducing seizures and hypsarrhythmia. Both corticosteroids and antiepileptic drugs are used with important medical control due to the high possibility of developing significant adverse effects. effects.

Treatments may be more or less prolonged depending on the form in which the syndrome presents in each case. In the same sense, epileptic episodes can have different consequences, especially in the development of the nervous system.

Different skills related to psychomotor development and some cognitive processes may be affected.. It is also possible that children may develop other types of epilepsy in other periods of childhood. The latter may also follow a specific treatment depending on the form in which they present.

Bibliographic references:

• Epilepsy Action (2018). West syndrome (infantile spasms). Retrieved June 29, 2018. Available at 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 course and prognostic factors. Journal of Neurology, 52(2): 81-99.
• Pozo Alonso, A., Pozo Lauzán, D. and Pozo Alonso, D. (2002). West syndrome: Etiology, physiopathology, clinical and prognostic aspects. Cuban Journal of Pediatrics. 74(2): 151-161.