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 a preference for high-dose oral prednisolone due to its efficacy and lower cost compared to ACTH injections, as demonstrated in a recent retrospective review of 87 children 2.

Treatment Options

  • Adrenocorticotropic hormone (ACTH) is administered as intramuscular injections, usually at a dose of 150 units/m² per day divided into twice daily injections for 2 weeks, followed by a gradual taper over 2-4 weeks.
  • High-dose oral prednisolone can be used at 40-60 mg/day (or 4-8 mg/kg/day) for 2 weeks, followed by a similar tapering schedule.

Rationale

The choice of treatment should be based on the most recent and highest quality evidence, which suggests that high-dose oral prednisolone is a effective and readily available alternative to ACTH injections 2. Additionally, vigabatrin is an alternative first-line option, particularly for infantile spasms associated with tuberous sclerosis complex, as supported by earlier studies 3, 4.

Monitoring and Side Effects

During treatment, patients require close monitoring for side effects including hypertension, irritability, immune suppression, hyperglycemia, and electrolyte abnormalities. Blood pressure, blood glucose, and electrolytes should be checked regularly. Steroid therapy works by suppressing the abnormal electrical activity in the brain that causes infantile spasms, though the exact mechanism remains incompletely understood.

Treatment Response

Treatment response should be assessed within 2 weeks, with EEG monitoring to confirm resolution of hypsarrhythmia pattern. Earlier intervention is associated with better developmental outcomes, and treatment should be initiated as soon as possible after diagnosis.

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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