AAN Summary of Evidence-based Guideline for CLINICIANS
PRACTICE PARAMETER: MEDICAL TREATMENT OF INFANTILE SPASMS
This is a summary of the American Academy of Neurology (AAN) and Child Neurology Society (CNS) evidence-based guideline, which determines the current best practice for treatment of infantile spasms in children. The guideline summarizes the most effective therapies for infantile spasms, their safety, and whether successful treatment of infantile spasms leads to long-term improvement. This summary is based on a complete and critical analysis of the published studies to date. It is designed to provide a strategy to make decisions in patient care. Please refer to the full guideline for detailed findings, dosing, and supporting evidence at www.aan.com/professionals/practice/index.cfm
SHORT-TERM TREATMENT OF INFANTILE SPASMS
ACTH Good evidence supports ACTH is probably effective for the short-term treatment of infantile spasms and in resolution of hypsarrhythmia (Level B*).
Vigabatrin Weak evidence supports Vigabatrin is possibly effective for the short-term treatment of infantile spasms (Level C, Class III and IV**evidence). 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).
Oral Corticosteroids Evidence is insufficient to support or refute There is insufficient evidence that oral corticosteroids are effective in the treatment of infantile spasms (Level U).
ACTH There is insufficient evidence to recommend the optimum dosage and duration of treatment with ACTH for the treatment of infantile spasms (Level U).
Vigabatrin 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 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 ON INFANTILE SPASMS
ACTH, corticosteroids, vigabatrin, valproic acid, pyridoxine Evidence is insufficient to support or refute 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). 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).
View the following additional AAN child neurology guidelines at www.aan.com/professionals/practice/index.cfm Date February 2004 February 2003 January 2003 September 2000 August 2000 Title Diagnostic Assessment of the Child with Cerebral Palsy Evaluation of the Child with Global Developmental Delay Treatment of the Child with a First Unprovoked Seizure Evaluating a First Nonfebrile Seizure in Children Screening and Diagnosis of Autism
Copies of this summary and additional companion tools are available at www.aan.com/professionals/practice/index.cfm or through AAN Member Services at (800) 879-1960.
This guideline summary is evidence-based. The AAN uses the following definitions for the level of recommendation and classification of evidence. *Recommendation Level: "Level" refers to the strength of the practice recommendation based on the reviewed literature. Level A: Established as effective, ineffective or harmful for the given condition in the specified population. Level B: Probably effective, ineffective or harmful (or probably useful/predictive or not useful/predictive) for the given condition in the specified population. Level C: Possibly effective, ineffective or harmful (or possibly useful/predictive or not useful/predictive) for the given condition in the specified population. Level U: Data inadequate or conflicting; given current knowledge, treatment is unproven. **Class of evidence for therapy: "Class" refers to the quality of research methods employed in the reviewed literature. Class I: Evidence provided by a prospective, randomized, controlled clinical trial with masked outcome assessment, in a representative population. The following are required: (a) primary outcome(s) is/are clearly defined; (b) exclusion/inclusion criteria are clearly defined; (c) adequate accounting for drop-outs and crossovers with numbers sufficiently low to have minimal potential for bias; and (d) 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 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.
This is an educational service of the American Academy of Neurology. It is designed to provide members with evidence-based guideline recommendations to assist with decision-making in patient care. It is based on an assessment of current scientific and clinical information, and is not intended to exclude any reasonable alternative methodologies. The AAN recognizes that specific patient care decisions are the prerogative of the patient and the physician caring for the patient, based on the circumstances involved. Physicians are encouraged to carefully review the full AAN guidelines so they understand all recommendations associated with care of these patients.
1000 West County Road E • Suite 290 • St. Paul, MN 55126 www.childneurologysociety.org (651) 486-9447
1080 Montreal Avenue • St. Paul, MN 55116 www.aan.com • www.thebrainmatters.org (651) 695-1940