West syndrome is a medical condition which is characterized by the presence of epileptic spasms during infancy. As with other types of epilepsy, West syndrome follows a specific pattern during spasms, as well as at 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 most common.
What is West Syndrome?
West syndrome is technically defined as age-dependent epileptic encephalopathy. This means that it is associated with epileptic seizures that originate in the brain, which in turn contributes to its progression. It is said to be an age-dependent syndrome because it occurs during early childhood.
Basically, these are groups of quick, sharp movements that usually begin in the first year of life. More precisely between the first three and eight months. It was also presented less frequently in the second year. Likewise, West’s syndrome it is also known as infantile spasm syndrome.
As it is characterized by the presence of repetitive compulsive patterns, as well as by some electroencephalographic activity, it has also been defined as an “electroclinic epileptic syndrome”.
This condition was first described in 1841, when the English-born surgeon William James West studied the case of his own son 4 months.
the main reasons
Initially, seizures usually occur in isolation, with few recurrences and of short duration. For the same reason, it is common for West syndrome to be identified when it is already advanced.
One of the main causes of West syndrome is hypoxia-ischemic, Although this may vary. In all cases, the causes of the syndrome have been associated with an antenatal, neonatal and postnatal history.
West syndrome can be caused by different chromosomal abnormalities. Also for neurofibromatosis, certain infections and metabolic diseases, As well as hypoxia-ischemia, among other causes that occur in the period before birth.
It can also be caused by hypoxic-ischemic encephalopathy, for selective neural necrosis or hypoglycemiaAmong the other medical conditions that arise from the period from the 28th week of gestation to the seventh day of birth.
West’s syndrome has also been linked to various infections that occur from childbirth and 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’s syndrome usually presents with sudden (forward) flexion, accompanied by body stiffness that also affects the arms and legs on both sides (This is known as a “toned form”). He is sometimes presented with the arms and legs thrown forward, which is called “extensor spasms.”
If spasms occur while the child is lying down, the typical pattern is bending of the knees, arms and head forward.
Although unique spasms can occur, especially in the early stages of syndrome development, epileptic seizures usually last a second or two. Then there may be a break, and immediately another spasm. In other words, they often occur repeatedly and frequently.
this condition it can affect the development of the child in different areasIn addition to causing a lot of feelings and distress to your caregivers, it is therefore important to know their diagnosis and treatment. There are currently several options for controlling spasms and improving electroencephalographic activity in children.
The diagnosis is made by means of an electroencephalographic test which can demonstrate or exclude the presence of “hypsarhythmia”, which are disorganized patterns of electrical activity in the brain.
Sometimes these patterns may be visible only during sleep, so it is common for the EEG 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 help pinpoint the cause of the syndrome.
The most common treatment is pharmacological. There are studies that suggest West syndrome generally responds favorably to antiepileptic therapy, Such as Vigabatrin (known as Sabril). The latter inhibits the depletion of gamma-aminobutyric acid (GABA), the main inhibitor of the central nervous system. When the concentration of this acid decreases, it can speed up electrical activity, so these drugs help to regulate it. In the same sense, nitrazepam and epilim are used.
They may also use corticosteroids, such as adrenocorticotropic hormones, a very effective treatment for reducing seizures and hypsarrhythmia. Corticosteroids and antiepileptics are used with an important medical examination because of the high possibility of developing side effects important.
The treatments can be more or less prolonged depending on how the syndrome presents itself at home. Similarly, epileptic episodes can have different consequences, especially on the development of the nervous system.
Skills related to psychomotor development and certain cognitive processes may have different effects. Children may also develop other types of epilepsy at other times in childhood. The latter can also undergo a specific treatment depending on how they are presented.
- Action Against Epilepsy (2018). West syndrome (infantile spasms). Accessed June 29, 2018.Available at https://www.epilepsy.org.uk/info/syndromes/west-syndrome-infantile-spasms
- Arce Portillo, E., Rufo Camps, M., Muñoz Cabello, B, et. in 2011). West syndrome: etiology, treatment options, clinical course and prognostic factors. Journal of Neurology, 52 (2): 81-99.
- Pou Alonso, A., Pou Lauzán, D. and Pou Alonso, D. (2002). West syndrome: etiology, pathophysiology, clinical aspects and prognosis. Cuban Journal of Pediatrics. 74 (2): 151-161.