West syndrome
West Syndrome
West Syndrome (pronounced: /wɛst ˈsɪndroʊm/), also known as Infantile Spasms, is a rare and severe form of epilepsy that occurs in infants and young children. The syndrome is named after the English physician, Dr. William James West, who first described it in the 19th century.
Etymology
The term "West Syndrome" is derived from the name of Dr. William James West, who first described the condition in a letter to the editor of The Lancet in 1841. He reported the case of his own son, who had "salaam seizures" or "jackknife seizures", now known as infantile spasms.
Definition
West Syndrome is characterized by a specific type of seizure known as infantile spasms, along with a specific pattern on electroencephalogram (EEG) testing called hypsarrhythmia and developmental regression. The seizures typically consist of a sudden bending forward of the body with stiffening of the arms and legs; some children arch their backs as they extend their arms and legs.
Symptoms
The primary symptom of West Syndrome is a specific type of seizure known as an infantile spasm. These seizures typically begin in the first year of life, usually between 4 and 8 months of age. Other symptoms may include developmental regression, where a child loses previously acquired skills, and a specific pattern on EEG testing known as hypsarrhythmia.
Treatment
Treatment for West Syndrome typically involves antiepileptic drugs (AEDs), such as vigabatrin and corticosteroids. In some cases, a ketogenic diet, which is high in fats and low in carbohydrates, may be recommended. If a specific cause for the seizures can be identified, such as a brain tumor, surgery may be considered.
Prognosis
The prognosis for children with West Syndrome varies. Some children may experience a reduction in seizures and improvement in developmental outcomes with treatment, while others may continue to have ongoing seizures and significant developmental delays.
See Also
External links
- Medical encyclopedia article on West syndrome
- Wikipedia's article - West syndrome
This WikiMD dictionary article is a stub. You can help make it a full article.
Languages: - East Asian
中文,
日本,
한국어,
South Asian
हिन्दी,
Urdu,
বাংলা,
తెలుగు,
தமிழ்,
ಕನ್ನಡ,
Southeast Asian
Indonesian,
Vietnamese,
Thai,
မြန်မာဘာသာ,
European
español,
Deutsch,
français,
русский,
português do Brasil,
Italian,
polski