Abstract
The clinical characteristics, EEG abnormalities, response to therapy, and outcome of 14 patients with infantile spasms and Down syndrome were studied at the Hopital Saint Vincent de Paul, Paris (9 cases); Universita Degli Studi de Pisa, Italy (2 cases); and Hopital de La Timone, Marseille, France (3 cases).
Highlights
INFANTILE SPASMS IN DOWN SYNDROME The clinical characteristics, EEG abnormalities, response to therapy, and outcome of 14 patients with infantile spasms and Down syndrome were studied at the Hopital Saint Vincent de Paul, Paris (9 cases); Universita Degli Studi de Pisa, Italy (2 cases); and Hopital de La Timone, Marseille, France (3 cases)
Spasms began between 4 and 18 months, development was delayed before seizure onset, and visual contact deteriorated after seizure onset
A delay in diagnosis may contribute to a worsening of cognitive dysfunction, and parents of Down syndrome children should be alerted to the possible development of spasms in the first year
Summary
INFANTILE SPASMS IN DOWN SYNDROME The clinical characteristics, EEG abnormalities, response to therapy, and outcome of 14 patients with infantile spasms and Down syndrome were studied at the Hopital Saint Vincent de Paul, Paris (9 cases); Universita Degli Studi de Pisa, Italy (2 cases); and Hopital de La Timone, Marseille, France (3 cases). Spasms began between 4 and 18 months (mean 8 months), development was delayed before seizure onset, and visual contact deteriorated after seizure onset. Interictal EEGs showed typical hypsarrhythmia with no focal abnormality. Hydrocortisone (15 mg/kg/day for 2 weeks, and discontinuation over 2 weeks) in 10, and vigabatrin, valproate, or pyridoxine in 4 patients, controlled spasms and hypsarrhythmia within 6 months.
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