West syndrome: causes, symptoms and treatment
West Syndrome is a medical condition which is characterized by the presence of epileptic spasms during early childhood. As in other types of epilepsy, West Syndrome follows a specific pattern during spasms, as well as at the age at which they occur.
Next, we explain what West Syndrome is, what are the main symptoms and causes, how it is detected and which treatments are the most frequent.
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What is West Syndrome?
West Syndrome is defined technically as an epileptic encephalopathy dependent on age . 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 start during the first year of life. Specifically between the first three and eight months. Less frequently, it has also been presented during the second year. For the same reason, West Syndrome it is also known as infantile spasms syndrome .
Insofar as it is characterized by the presence of repetitive compulsive patterns, and also by a determined electroencephalographic activity, it has also been defined as an "electroclinic epileptic syndrome".
This condition was described for the first time in the year 1841, when English-born surgeon William James West studied his own son's case 4 months old.
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Main causes
In the beginning, 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. Prenatals
West Syndrome can be caused by different chromosomal abnormalities. Also by neurofibromatosis, some infections and metabolic diseases , as well as hypoxia-ischemic, among other causes that take place in the period before birth.
2. Perinatals
It can also be caused by a hypoxic-ischemic encephalopathy, selective neural necrosis or hypoglycaemia , among other medical conditions that originate in the period from week 28 of pregnancy to the seventh day of birth.
3. Postnatal
The West Syndrome has also been related to different infections that occur after the child is born and in the development of early childhood, as bacterial meningitis or brain abscesses . It has also been linked to hemorrhages, traumatisms and the presence of brain tumors.
Most common symptoms
West Syndrome typically presents with sudden (forward) flexion, accompanied by body stiffness that equally affects the arms and legs on both sides (This is known as a "tonic form"). Sometimes it occurs with arms and legs thrown forward, which is called "extensor spasms."
If the spasms occur while the child is lying down, the typical pattern is the bending of knees, arms and head forward.
Although single spasms may occur, especially in the early stages of the syndrome, epileptic seizures usually last one or two seconds . Then you can follow a pause, and immediately a spasm more. In other words, they often occur repeatedly and frequently.
Diagnosis
This condition can affect the development of the baby in different areas , as well as cause a lot of shock 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 small children.
The diagnosis is made by means of an electroencephalographic test that can demonstrate or rule out the presence of "hypsarrhythmia", which are the disorganized patterns of electrical activity in the brain .
Sometimes these patterns can be visible only during sleep, so it is common for the electroencephalogram to be performed at different times and be accompanied by other tests.For example, brain scans (magnetic resonance imaging), blood tests, urine tests, and sometimes tests of cerebrospinal fluid, to locate the cause of the syndrome.
Main treatments
The most common treatment is pharmacological. There are studies that suggest that West Syndrome usually respond favorably to treatment for antiepileptic drugs , such as Vigabatrin (known as Sabril). The latter inhibits the decrease of 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 to regulate it. Nitrazepam and epilim are used in the same sense.
Likewise, corticosteroids can 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 adverse effects important
The treatments can be more or less prolonged depending on the way in which the syndrome occurs at home. 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. . There is also the possibility that children develop other types of epilepsy in other periods of childhood. The latter can also follow a specific treatment according to the way in which they are presented.
Bibliographic references:
- Epilepsy Action (2018). West syndrome (infantile spasms). Retrieved June 29, 2018. Available at //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, physiopathology, clinical aspects and prognoses. Cuban Journal of Pediatrics. 74 (2): 151-161.
