Long‐term Cognitive Outcomes of a Cohort of Children with Cryptogenic Infantile Spasms Treated with High‐dose Adrenocorticotropic Hormone

• Published in: Epilepsia (Copenhagen) 2004-03, Vol.45 (3), p.255-262
• Main Authors: Kivity, Sara, Lerman, Pinchas, Ariel, Raya, Danziger, Yardena, Mimouni, Marc, Shinnar, Shlomo
• Format: Article
• Language: English
• Subjects: ACTH; Administration, Oral; Biological and medical sciences; Brain - pathology; Child; Child, Preschool; Cognition Disorders - diagnosis; Cognition Disorders - etiology; Cohort Studies; Cosyntropin - administration & dosage; Cosyntropin - therapeutic use; Cryptogenic infantile spasms; Delayed-Action Preparations; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy; Humans; Infant, Newborn; Magnetic Resonance Imaging; Male; Medical sciences; Nervous system (semeiology, syndromes); Neurology; Neuropsychological Tests; Prednisone - therapeutic use; Prognosis; Prospective Studies; Severity of Illness Index; Spasms, Infantile - complications; Spasms, Infantile - diagnosis; Spasms, Infantile - drug therapy; Time Factors; Treatment Outcome
• ISSN: 0013-9580; 1528-1167
• DOI: 10.1111/j.0013-9580.2004.30503.x

Purpose: To evaluate the outcome of children with cryptogenic infantile spasms treated with high‐dose synthetic adrenocorticotropic hormone (ACTH) and the relation between early treatment, within 1 month of onset, and outcome. Methods: We assessed the long‐term cognitive and seizure outcomes of 37 patients with cryptogenic infantile spasms (onset, age 3 to 9 months) receiving standardized treatment regimen of high‐dose tetracosactide depot, 1 mg IM every 48 h for 2 weeks, with a subsequent 8‐ to 10‐week slow taper and followed by oral prednisone, 10 mg/day for a month, with a subsequent slow taper for 5 months or until the infant reached the age of 1 year, whichever came later. Development was assessed before treatment. Seizure outcomes were followed up prospectively. Cognitive outcomes were determined after 6 to 21 years and analyzed in relation to treatment lag and pretreatment regression. Results: Twenty‐two infants were treated within 1 month of onset of infantile spasms, and 15 after 1 to 6.5 months. Normal cognitive outcome was found in all 22 (100%) patients of the early‐treatment group, and in 40% of the late‐treatment group. Normal cognitive outcome was found in all 25 (100%) patients who had no or only mild mental deterioration at presentation, including four in the late‐treatment group but in only three of the 12 patients who had had marked or severe deterioration before treatment. Conclusions: Early treatment of cryptogenic infantile spasms with a high‐dose ACTH protocol is associated with favorable long‐term cognitive outcomes. Once major developmental regression lasts for a month or more, the prognosis for normal cognitive outcome is poor. Further studies are needed on the optimal treatment regimen for this disorder.