This is the current release of the guideline.

Definitions of the strength of the recommendations (A, B, C, U) and classification of the evidence (Class I through Class IV) are provided at the end of the "Major Recommendations" field.

Effectiveness of Adrenocorticotropic Hormone (ACTH) and Oral Corticosteroids in the Treatment of Infantile Spasms

1. ACTH is probably effective for the short-term treatment of infantile spasms and in resolution of hypsarrhythmia (level B).
2. There is insufficient evidence to recommend the optimum dosage and duration of treatment with ACTH for the treatment of infantile spasms (level U).
3. There is insufficient evidence that oral corticosteroids are effective in the treatment of infantile spasms (level U).

Effectiveness of Vigabatrin in the Treatment of Infantile Spasms

1. Vigabatrin is possibly effective for the short-term treatment of infantile spasms (level C, class III and IV evidence).
2. Vigabatrin is also possibly effective for the short-term treatment of infantile spasms in the majority of children with tuberous sclerosis (level C, class III and IV evidence).
3. Serious concerns about retinal toxicity in adults suggest that serial ophthalmologic screening is required in patients on vigabatrin. However, data are insufficient to make recommendations regarding the frequency or type of screening that would be of value in reducing the prevalence of this complication in children (level U, class IV studies).

Other Agents Evaluated for the Treatment of Infantile Spasms

1. There is insufficient evidence to recommend other treatments (valproic acid, benzodiazepines, pyridoxine, newer antiepileptic drugs, or other or novel therapies) for the treatment of infantile spasms (level U, class III and IV evidence).

Long-term Outcome

1. The data are insufficient to make any recommendations regarding the use of ACTH, corticosteroids, vigabatrin, valproic acid, and pyridoxine to improve the long-term outcomes (seizure freedom and normal development) of children with infantile spasms (level U, class III and IV evidence).
2. The data are insufficient to conclude that early initiation of treatment should be used to improve the long-term outcome of children with infantile spasms (level U, class III and IV evidence).

Definitions:

Rating of Recommendation

A = established as effective, ineffective, or harmful for the given condition in the specified population.

B = probably effective, ineffective, or harmful (or probably useful/predictive or not useful/predictive) for the given condition in the specified population.

C = possibly effective, ineffective, or harmful (or possibly useful/predictive or not useful/predictive) for the given condition in the specified population.

U = data inadequate or conflicting. Given current knowledge, treatment is unproven.

Translation of Evidence to Recommendations

Level A rating requires at least one convincing class I study or at least two consistent, convincing class II studies.

Level B rating requires at least one convincing class II study or at least three consistent class III studies.

Level C rating requires at least two convincing and consistent class III studies.

American Academy of Neurology Evidence Classification Scheme for a Therapeutic Article

Class I: evidence provided by a prospective, randomized, controlled clinical trial with masked outcome assessment, in a representative population. The following are required:

1. Primary outcome(s) is/are clearly defined.
2. Exclusion/inclusion criteria are clearly defined.
3. Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias.
4. Relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences.

Class II: evidence provided by a prospective matched group cohort study in a representative population with masked outcome assessment that meets a–d above or a randomized control trial in a representative population that lacks one criteria of a–d.

Class III: all other controlled trials (including well-defined natural history controls or patients serving as own controls) in a representative population, where outcome assessment is independent of patient treatment.

Class IV: evidence from uncontrolled studies, case series, case reports, or expert opinion.

None provided

The type of supporting evidence is identified and graded for each recommendation (see "Major Recommendations").

Not applicable: The guideline was not adapted from another source.

2004 May 25

American Academy of Neurology - Medical Specialty Society

American Academy of Neurology (AAN)

Quality Standards Subcommittee of the American Academy of Neurology

Practice Committee of the Child Neurology Society

American Academy of Neurology (AAN) Quality Standards Subcommittee Members: Gary Franklin, MD, MPH (co-chair); Gary Gronseth, MD (co-chair); Charles E. Argoff, MD; Stephen Ashwal, MD (ex-officio); Christopher Bever, Jr., MD; Jody Corey-Bloom, MD, PhD; John D. England, MD; Jacqueline French, MD (ex-officio); Gary H. Friday, MD; Michael Glantz, MD; Deborah Hirtz, MD; Donald J. Iverson, MD; Samuel Wiebe, MD; William J. Weiner, MD; Catherine Zahn, MD (ex-officio)

Child Neurology Society Practice Committee Members: Carmela Tardo, MD (chair); Bruce Cohen, MD (vice-chair); Elias Chalhub, MD; Roy Elterman, MD; Murray Engel, MD; Bhuwan Garg, MD; Brian Grabert, MD; Annette Grefe, MD; Michael Goldstein, MD; David Griesemer, MD; Betty Koo, MD; Edward Kovnar, MD; Leslie Ann Morrison, MD; Colette Parker, MD; Ben Renfroe, MD; Michael Shevell, MD; Shlomo Shinnar, MD; Gerald Silverboard, MD; Russell Snyder, MD; Dean Timmons, MD; Greg Yim, MD

Dr. Mackay has received honoraria from GlaxoSmithKline, and Janssen Cilag. Dr. Mackay has no equity, stock, or any other ownership interests in these companies. Dr. Pellock has received grants/research support in excess of $10,000 and is a paid consultant for Abbott Laboratories, Aventis, Carter Wallace (MedPointe), Elan Pharmaceuticals, GlaxoSmithKline, Ortho McNeil/Johnson & Johnson, and UCB Pharmaceuticals. Dr. Shinnar has received grants/ research support from Abbott Laboratories, Elan Pharmaceuticals, and Xcel Pharmaceuticals. He is a paid consultant for Abbott Laboratories, Cephalon, Inc., Elan Pharmaceuticals, Ovation, Pfizer Laboratories, and Xcel Pharmaceuticals. He also has received honoraria from Abbott Laboratories, Cephalon, Inc., Elan Pharmaceuticals, Pfizer Inc., the R.W. Johnson Pharmaceutical Research Institute, UCB Pharmaceuticals, Inc., and Xcel Pharmaceuticals. Dr. Shinnar has no equity, stock, or any other ownership interest in any of these companies. Dr. Shields participated in a study of vigabatrin that was partially supported by an unrestricted grant from Aventis.

American Epilepsy Society - Disease Specific Society

This is the current release of the guideline.

This NGC summary was completed by ECRI on August 17, 2004. The information was verified by the guideline developer on September 9, 2004.

This NGC summary is based on the original guideline, which is copyrighted by the American Academy of Neurology.