The lowest effective ACTH dose, with fewest adverse effects, for the treatment of West syndrome (WS) was determined in a comparative, randomly assigned, controlled study involving 25 patients with cryptogenic (CWS, n=9) or symptomatic (SWS, n=16) WS, at the Department of Pediatrics, Tokyo Women’s Medical University, Tokyo, Japan.

Either low dose (0.005 mg/kg per day = 0.2 IU/kg per day) or high dose (0.025 mg/kg per day = 1 IU/kg per day) synthetic ACTH, was administered every morning for 2 weeks and tapered to zero over the subsequent 2 weeks. In the CPS group, infantile spasms and hypsarrhythmia were completely controlled in 3/4 given low-dose and 5/5 on high-dose ACTH. In the SWS group, spasms and hypsarrhythmia were controlled in 6/8 at each dose level. No significant differences were observed between low and high-dose ACTH for either type of WS. Long-term responses in the 17 responders followed for more than 1 year showed no significant differences among groups. Sleepiness and brain shrinkage estimated by CT scan were significantly milder in the low-dose group. Low-dose ACTH therapy may be equally effective as high-dose and is recommended in CWS and in SWS with cerebral atrophy. [1]

COMMENT. The controversy regarding the optimum dosage of ACTH for treatment of infantile spasms continues, the low-dose, short-duration regimen favored in Japan, the high-dose, extended treatment advocated by the majority in the United States and the UK. My own preference has been for smaller doses (10-20 units Acthar gel daily IM for 3 weeks), with relative avoidance of serious side-effects. The present report involving the use of synthetic ACTH confirms previous studies in Japan, using natural ACTH [2, 3, 4]. Proponents of high-dose ACTH include Snead OC et al. [5, 6]. See Progress in Pediatric Neurology I, PNB Publ, 1991;pp30-34, for further discussion of ACTH in treatment of infantile spasms.