Short-Duration ACTH Therapy for West Syndrome

Short-term developmental and seizure outcomes were assessed in 7 children, aged <12 months, with cryptogenic West syndrome treated with a short-duration (7-12 days) and low-dose synthetic ACTH at Nagoya City Hospital, Japan.

Cognition and electrical SE during sleep (ESES).The duration of ESES and the localization of interictal foci play a major role in the degree and type of cognitive dysfunction following continuous spike-wave activity.ESES interferes with slow-wave activity at the site of the epileptic focus, impairing learning and other cognitive functions.(Tassinari CA, Rubboli G. Epilepsia Nov 2006;47(Suppl 2):40-43).

SHORT-DURATION ACTH THERAPY FOR WEST SYNDROME
Short-term developmental and seizure outcomes were assessed in 7 children, aged <12 months, with cryptogenic West syndrome treated with a short-duration (7-12 days) and low-dose synthetic ACTH at Nagoya City Hospital, Japan.All patients had received a trial of one or two AEDs before ACTH.Daily single dose of ACTH was 0.022-0.027mg/kg/day (mean, 0.96 IU/kg), and total dose was 0.17-0.28mg/kg (mean, 9.0 IU/kg).Interval between onset of spasms and initiation of ACTH was 12-105 days (median 14 days).Tonic spasms were controlled in all patients with no serious side effects.Spasms disappeared within 1 week, and hypsarrhythmia resolved within 2 weeks.EEG abnormalities persisted in 2. IQ or developmental quotients of 6 patients were 79-110 at age 2 to 6 years.One patient with a long treatment lag had a developmental quotient of 60.

LONG-TERM USE OF THE KETOGENIC DIET
A retrospective chart review of 28 children (15 males, 14 females) treated with the ketogenic diet for epilepsy for more than 6 years was conducted at Johns Hospital Hospital.Response was documented by attendance at clinic and by telephone.Patients were aged 7 to 23 years at follow-up.Diet duration was 6 to 12 years.Seizure frequency was decreased in 90% patients.Side effects included kidney stones in 7 (25%), skeletal fractures in 6 (21%), and an increase in the number of children with height below the 10th centile from 10 at initiation of the diet to 23 at follow-up (p=0.001).Overall lipid profiles were generally within the normal range, and cholesterol and triglycerides did not increase significantly.(Groesbeck DK, Bluml RM, Kossof EH.Long-term use of the ketogenic diet in the treatment of epilepsy.COMMENT.In this report, the efficacy of the ketogenic diet in the treatment of childhood epilepsy was maintained after long-term use, but side effects, especially growth retardation, kidney stones, and fractures, need to be monitored and may be serious. Several previous reports of long-term use of the diet, or of adults, 40-50 years of age, previously treated with the ketogenic diet have been published, some from the above authors' institution (Livingston S, 1972) and earlier, from the Mayo Clinic where the diet was first introduced (Keith HM, 1963;Wilder RM, 1921).Livingston allayed concerns about the high fat diet and possible cardiac complications in later life; he found no increased evidence of arteriosclerosis, hypertension, ECG abnormalities, or elevated cholesterol levels in the adults examined.He referred to Haddow Keith, my former colleague and mentor regarding use of the diet, and his report of follow-up data on 530 patients with idiopathic epilepsy treated for periods varying up to 36 years.Of 32 followed for 25 to 30+ years, 24 were well and 8 improved.Of the total of 530 patients, 30% were completely free of attacks, 24% were improved, and 39% were not benefited; 34 (6%) had died, 4 in status epilepticus.

INFECTIOUS DISEASES INFLUENZA A AND FEBRILE SEIZURES
Predisposing factors and characteristics of febrile seizures in children with influenza A infection were studied in children hospitalized with febrile seizures between January and July 2005 at Tuen Mun Hospital, Hong Kong.Of 177 children confirmed with influenza A infection, 34 (19.5%) had febrile seizures.Ages ranged from 0.9 to 6 years (mean 2.69 years); 19 males and 15 females.Age-matched controls had influenza A but no febrile seizures (control 1; n=34), and children with febrile seizures who tested negative for influenza (control 2; n=34).Mean maximum body temperature of children with febrile (Hattori A, Ando N, Hamaguchi K, et al.Short-duration ACTH therapy for cryptogenic West syndrome with better outcome.Pediatr Neurol Dec 2006;35:415-418).(Respond: Dr Hattori, Department of Pediatrics, Nagoya City Hospital, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan).
COMMENT.Vitamin B6 and AEDs, including clonazepam, valproic acid, and zonisamide, are used before initiating ACTH, because of possible adverse effects of hormone therapy.Delay in intiation of ACTH may result in poor outcome, and a favorable response to very low dose-short duration therapy should favor a recommendation for early trial of Pediatric Neurology Briefs 2006 ACTH.Previous trials of ACTH using relatively low dose regimens have proven effective without serious side effects (Millichap JG, et al.JAMA 1962; 182:125; Ito M et al.Pediatr Neurol 1990;6:240-244), whereas high-dose, long-term regimens are associated with frequent serious side effects (Snead OC III.Pediatr Neurol 1990;6:147-150).