Professional and Linguistic Assessments Board (PLAB) Practice Exam

Which syndrome is characterized by infantile spasms and developmental regression?

1. Dravet syndrome

2. West's syndrome

3. Angelman syndrome

4. Rett syndrome

The correct answer is: West's syndrome

West's syndrome is the correct answer because it specifically refers to a type of epilepsy that begins in infancy, characterized by infantile spasms, developmental regression, and often a distinctive electroencephalogram (EEG) finding known as hypsarrhythmia. The spasms typically occur in clusters and can severely impact the child's development, often leading to cognitive impairment or delays in milestones. In contrast, the other syndromes listed have different primary characteristics. Dravet syndrome is a severe form of epilepsy that starts in the first year of life but does not primarily feature infantile spasms or a straightforward developmental regression pattern. Instead, it usually involves prolonged seizures and is associated with a range of developmental issues over time. Angelman syndrome is a genetic neurological disorder marked by developmental delays, speech impairment, and distinctive facial features, but it does not prominently feature infantile spasms as a primary symptom. Rett syndrome is an neurodevelopmental disorder that primarily affects girls and leads to a progressive loss of motor skills and speech, but it typically manifests later than the infancy period and does not include infantile spasms as a key clinical feature. Understanding these distinctions helps clarify why West's syndrome is the appropriate choice among the options presented.