What is the first-line treatment for infantile spasms, particularly using steroids like adrenocorticotropic hormone (ACTH)?

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From the FDA Drug Label

Acthar Gel is indicated as monotherapy for the treatment of infantile spasms in infants and children less than 2 years of age. The efficacy of Acthar Gel for the treatment of infantile spasms in infants and children less than 2 years of age was evaluated in a randomized, single blinded (video EEG interpreter blinded) clinical trial and an additional active control supportive trial [see Clinical Studies (14)] A responding patient was defined as having both complete cessation of spasms and elimination of hypsarrhythmia.

The first-line treatment for infantile spasms is adrenocorticotropic hormone (ACTH), specifically Acthar Gel, which is indicated as monotherapy for this condition in infants and children less than 2 years of age 1.

  • Key points:
    • Acthar Gel is used to treat infantile spasms
    • It is indicated for infants and children less than 2 years of age
    • The efficacy of Acthar Gel for infantile spasms was evaluated in clinical trials
  • Main consideration: The treatment of infantile spasms with Acthar Gel should be carefully managed due to potential adverse reactions, particularly in very young infants.

From the Research

The first-line treatment for infantile spasms is typically adrenocorticotropic hormone (ACTH) or high-dose oral prednisolone, with ACTH being the most commonly used treatment in the United States, as supported by the most recent study 2. The treatment should begin as soon as possible after diagnosis, as earlier intervention is associated with better developmental outcomes.

  • ACTH is administered as intramuscular injections, usually starting at 150 units/m² per day divided into twice daily dosing for 1-2 weeks, followed by a gradual taper over 2-4 weeks.
  • Alternatively, high-dose oral prednisolone can be used at 40-60 mg/day (or 4-8 mg/kg/day) for 2 weeks followed by a 2-week taper.
  • Vigabatrin is an alternative first-line option, particularly for infantile spasms associated with tuberous sclerosis complex, as shown in a study from 3. During treatment, patients require close monitoring for side effects including hypertension, irritability, immune suppression, and metabolic abnormalities, as noted in several studies 4, 5, 6.
  • Blood pressure, blood glucose, and electrolytes should be checked regularly.
  • The effectiveness of treatment is assessed by both clinical response (cessation of spasms) and normalization of EEG patterns, with follow-up EEG typically performed 2 weeks after initiating therapy. The response to ACTH is most often determined early in the treatment course, with a mean time to clinical remission of 5.8 days, as found in the study 2.
  • Given the importance of rapid remission, clinicians should consider adding or changing treatment if infantile spasms do not resolve within two weeks of ACTH initiation.

References

Research

The response to ACTH is determined early in the treatment of infantile spasms.

Epileptic disorders : international epilepsy journal with videotape, 2015

Research

Treatment of infantile spasms.

Current treatment options in neurology, 2006

Research

Current trends in the treatment of infantile spasms.

Neuropsychiatric disease and treatment, 2009

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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