West Syndrome is a medical condition which is characterized by the presence of epileptic spasms during early childhood. As occurs in other types of epilepsy, West Syndrome follows a specific pattern during spasms, as well as the age at which they occur.
Below we explain what West Syndrome is, what the main symptoms and causes are, how it is detected and what treatments are the most common.
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 occurred during the second year. For the same reason, West Syndrome It 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 the English-born surgeon William James West studied the case of his own son 4 months old.
Main causes
Initially, 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-ischemic, although this may 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 due to 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 neural 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 related to different infections that occur from the time the child is born and during early childhood development, such as bacterial meningitis or brain abscesses It has also been related to hemorrhages, trauma and the presence of brain tumors.
Most common symptoms
West Syndrome typically presents with a sudden bending (forward), accompanied by body stiffness that affects the arms and legs on both sides equally (this is known as a “tonic form”). Sometimes it occurs with the arms and legs thrusting forward, which is called “extensor spasms.”
If 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, epileptic seizures usually last one or two seconds A pause may then follow, and immediately one more spasm. In other words, they tend to occur repeatedly and frequently.
This condition can affect the baby’s development in different areas, as well as causing a lot of impression and distress 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 through 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 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, can 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 said acid decreases, electrical activity can accelerate, which is why these drugs help regulate it. Nitrazepam and epilim are used in the same sense.
Likewise, corticosteroids can be used, such as adrenocorticotropic hormones, a treatment that turns out to be very effective in reducing seizures and hypsarrhythmia. Both corticosteroids and antiepileptics are used with important medical monitoring due to the high possibility of developing adverse effects important.
Treatments may be more or less prolonged depending on the way in which the syndrome presents in each case. In the same sense, epileptic episodes can have different consequences, especially on the development of the nervous system.
Different skills related to psychomotor development and some cognitive processes may be affected Likewise, it is possible that children develop other types of epilepsy in other periods of childhood. The latter can also follow specific treatment depending on the way in which they present.
