The condition is a type of epilepsy characterized by spasms and associated with the loss of developmental milestones. Understand what the main signals are
Seizures with spasms that affect intelligence and behavior, as well as developmental delays, may be more obvious signs of a serious condition known as West Syndrome. “It is a rare disease, but not that rare,” says pediatric neurologist Daniel Almeida do Valle, from the Pequeno Príncipe Hospital (PR). According to the specialist, it affects one in ten thousand children. The problem is slightly predominant in boysalthough both sexes can be affected.
Symptoms of West syndrome
West syndrome is a type of epilepsy. If your child makes repetitive movements, such as muscle spasms, flexing or extending the body, this may need to be evaluated. If these “seizures” are associated with the loss of developmental milestones, that is, if there is a delay in achieving expected skills – such as sitting, holding one’s neck and smiling – this may characterize the symptoms of the condition. The signs tend to begin between 6 months and 3 years of age.
OR diagnosis consists of the presence of at least two of the following three criteria:
- “Convulsive” seizures such as infantile spasms;
- EEG with hypsarrhythmia (a specific pattern of brain waves, measured during the exam);
- Developmental arrest or regression.
The causes of West syndrome
Several factors can lead to West syndrome. As an example, we can cite genetic changes, such as Down syndrome, channelopathies (abnormalities of cardiac cell proteins, which alter electrical activity) or Miller-Dieker syndrome (another rare disease genetics).
Furthermore, according to Daniel Almeida do Valle, the disease can result from inborn errors of metabolism and structural changes in the brain, which are the most common. “Especially pre- and perinatal lesions, such as perinatal hypoxia (deprivation of oxygen to the baby before or during birth), cerebrovascular accidents (CVA) and sequelae of meningitis. Other structural changes include brain malformations and tuberous sclerosis. The presence of these changes functions as a risk factor for the development of West syndrome,” says the neurologist.
Is there a cure?
Once the syndrome is diagnosed, It is important to seek help, because there is a cure and, according to the doctor, some patients can progress towards recovery. “Initial treatment involves high-dose corticosteroids and an anticonvulsant. If the response is unsatisfactory, treatment with ACTH (acetylcorticotropic hormone) may be necessary,” she points out.
The result can be influenced by several factors. It depends, for example, on starting treatment early and on the cause of the condition: structural, genetic or metabolic. The most important thing is to have the support of a specialist, to offer the best chance of an adequate assessment of the condition and the evolution of the condition.
Source: Terra
Ben Stock is a lifestyle journalist and author at Gossipify. He writes about topics such as health, wellness, travel, food and home decor. He provides practical advice and inspiration to improve well-being, keeps readers up to date with latest lifestyle news and trends, known for his engaging writing style, in-depth analysis and unique perspectives.
