In Babies and more We have talked several times about diseases that affect babies and young children. On the issue of conditions related to childhood epilepsy, there are occasions when some types of epilepsy are misdiagnosed because the symptoms may go unnoticed, as in the case of west syndrome.
This disease is characterized by infantile spasms and psychomotor retardation, the latter being the reason why infants they lose the smile Because of this condition.
What is West syndrome?
He West syndrome or infantile spasm syndrome It is an epileptic encephalopathy of childhood, a disease that is serious but rare. It is characterized by three elements: epileptic spasms, retardation of psychomotor development and hipsarrhythmia in the electroencephalogram, although one of these may be absent.
This condition occurs approximately in 1 in 4,000 to 6,000 babies and predominates in the male sex. Children with West syndrome usually manifest the condition between 3 and 7 months old, although there have been cases in which it appears until after 2 years. Although there have been advances in its treatment, there have been cases in which it is not detected in time, especially when the symptoms are mild, since seizures can be confused with hiccups, cramps or abdominal pain.
AdvertisingThe causes that cause this syndrome can be prenatal, perinatal or postnatal. Prenatal causes are the most frequent and among them are the following:
- Cerebral dysplasia, is the most frequent cause. It includes: tuberous sclerosis, neurofibromatosis, Sturge-Weber syndrome, epidermal nevus syndrome with hemimegalencephaly, Aicardi syndrome, cortical focal dysplasia, among others.
- Chromosomal abnormalities like Down syndrome or Miller Dieker syndrome.
- Infection: cytomegalovirus, herpes simplex, rubella, toxoplasmosis or syphilis (when they affect the fetus).
- Metabolic diseases.
- Congenital syndrome such as Sjogren-Larsson syndrome, CHARGE syndrome, PEHO syndrome, Smith-Lemli-Optiz syndrome, Fahr disease, among others.
- Hypoxia or ischemia of prenatal cause: poroencephaly, hydranencephaly, periventricular leukomalacia.
The Perinatal causes are those that occur between the 28th week of pregnancy and the first seven days of life, and they include:
- Hypoxic-ischemic encephalopathy, selective neural necrosis, status marmoratus, parasagittal brain damage, periventricular leukomalacia, focal and multifocal ischemic necrosis (poroencephaly, multicystic encephalomalacia).
- Hypoglycemia.
The postnatal causes are the least frequent Among babies diagnosed with West syndrome and include:
- Infections such as bacterial meningitis (tuberculosis, meningococcus, pneumococcus), brain abscess, meningoencephalitis of viral etiology (measles, chicken pox, herpes simplex, enteroviruses, adenoviruses, cytomegalovirus, Epstein-Barr virus, among others).
- Hemorrhage and trauma: subdural and subarachnoid hemorrhage.
- Hypoxicischemic encephalopathy: cardiac arrest, among others.
- Brain tumor.
Steroid therapy and the use of drugs are usually effective in the treatment of this disease when detected at an early age, but in general, it has a poor prognosis. 90% of cases have a significant psychomotor delay, with motor limitations and autistic personality traits.
West syndrome has a mortality of 5%, and almost half of the cases can develop other epileptic syndromes, such as Lennox-Gastaut syndrome. Most children will have serious sequelae such as mental retardation and severe epilepsy, while about 10% will have a normal life.
Symptoms of West syndrome
As I mentioned previously, there are three elements that manifest the presence of the symptom in babies:
Epileptic spasms
Spasms are characterized by sharp, usually bilateral and symmetrical contraction of the muscles of the neck, trunk and extremities. They are usually accompanied by loss of consciousness.
There are 3 main types of spasms: in flexion (nodding or shrugging), extension (abrupt extension of the neck and trunk, with extension and abduction of the 4 limbs) and mixed.
Psychomotor delay
In babies with West syndrome, it is possible to detect a psychomotor delay even before childhood spasms begin to occur. In this delay there are loss of acquired abilities and neurological abnormalities.
This psychomotor delay manifests with the mood modification. The baby begins to be indifferent, showing loss of smile and reactions to sensory stimuli. They abandon the pressure of objects and eye tracking, they become very irritable, cry for no reason and sleep worse.
EEG alterations
During the performance of an electroencephalogram is presented hipsarrhythmia, which consists of the slowing and disorganization of brain electrical activity, in the form of a chaotic plot with a mixture of independent spikes and slow waves.
How to identify if your baby has childhood spasms
The most common characteristics of infantile spasms They are as follows:
- The typical pattern of spasms consists of sudden forward bending and stiffness of the body, arms and legs.
- Each attack lasts only a second or two, but they are usually in series.
- They are more common after waking up but rarely occur during sleep.
- They usually begin to manifest between 3 and 7 months of age.
