First-Line Treatment for Infantile Spasms
The first-line treatment for infantile spasms is ACTH (adrenocorticotropic hormone) or prednisolone (corticosteroids), with vigabatrin reserved as the preferred first-line agent specifically for infantile spasms associated with tuberous sclerosis. 1, 2
Treatment Algorithm
Standard First-Line Therapy
- ACTH or oral corticosteroids (prednisolone) are the recommended initial treatments for most cases of infantile spasms, based on guideline recommendations from the American Academy of Pediatrics and British Medical Journal 1
- ACTH (Acthar Gel) is FDA-approved for infantile spasms in children under 2 years of age and demonstrates high efficacy for both cessation of spasms and elimination of hypsarrhythmia 2, 3
- Treatment should be initiated within the first month of symptom onset to optimize neurodevelopmental outcomes 4
Etiology-Specific Considerations
- Vigabatrin is the treatment of choice when infantile spasms are associated with tuberous sclerosis, as these patients demonstrate superior response to this agent 5, 1
- For all other etiologies (hypoxic-ischemic injury, intracranial hemorrhage, infections, genetic disorders), ACTH or corticosteroids remain first-line 5
Critical Safety Monitoring
Cardiovascular Surveillance
- Cardiology consultation is mandatory for all children receiving steroid treatment due to the risk of cardiomyopathy 5, 1, 4
- This applies to both ACTH and oral corticosteroid therapy 1
Blood Pressure Monitoring
- Children receiving ACTH or steroids for infantile spasms are at high risk for hypertension and require frequent blood pressure monitoring during treatment 6
- This is particularly important given the young age of these patients and the intensity of hormonal therapy 6
Common Adverse Effects to Anticipate
ACTH/corticosteroid side effects include: 2, 7
- Infections (most common complication, occurring in 37% of patients) - including septic infections, pneumonias, urinary and gastrointestinal infections 7
- Arterial hypertension (documented in 11 patients in historical series) 7
- Electrolyte disturbances including hypokalaemic alkalosis 7
- Growth suppression 2
- Adrenal suppression requiring careful monitoring 2
Vigabatrin-specific concerns: 8
- Visual field constriction (often asymptomatic, requires perimetric testing to identify) 8
Treatment Response Criteria
- A responding patient is defined as having both complete cessation of spasms AND elimination of hypsarrhythmia on EEG 2
- Video-EEG monitoring is necessary to confirm both ictal activity cessation and resolution of the characteristic interictal pattern 4
Essential Multidisciplinary Care
Beyond seizure control, comprehensive management requires: 1
- Immediate referral to pediatric neurology upon diagnosis confirmation 4
- Early intervention services for developmental support 5, 1
- Neurology, speech/language evaluation, physical therapy, and occupational therapy referrals 1
- Ongoing developmental assessments alongside seizure management 5, 1
Common Pitfalls to Avoid
- Insufficient attention to developmental needs while focusing solely on seizure control 1
- Lack of coordination between multiple specialists involved in care, leading to fragmented management 1
- Delaying treatment beyond the first month of symptom onset, which worsens prognosis 4
- Failing to obtain cardiology clearance before initiating steroid therapy 1, 4
- Inadequate blood pressure monitoring during hormonal treatment 6
Second-Line and Alternative Agents
If first-line treatments fail, alternative options include valproic acid, topiramate, zonisamide, lamotrigine, levetiracetam, nitrazepam, pyridoxine, ketogenic diet, or surgical intervention for medically intractable cases 8, 9