Infantile spasms were originally described by West in 1841. West syndrome is the term employed when such spasms are concomitant with delayed psychomotor development and EEG hypsarrhythmia.
The present article discusses three cases of patients with Down syndrome who had infantile spams with psychomotor retardation but a nonhypsarrhythmic EEG pattern. Despite early diagnosis and early combination drug therapy, the condition persists, with seizures, psychomotor delay and abnormal EEG patterns. The sequence of events appears likely to develop into Lennox- Gastaut syndrome (LGS).
Several published case series report good progress in patients with Down syndrome and West syndrome.
We conclude that despite adequate treatment, patients with Down syndrome and infantile spasms with psychomotor delay and abnormal but non-hypsarrhythmic EEG may have poor disease progression, with persistence of seizures and severely impaired psychomotor development.