One survey indicated that two-thirds of providers utilize ACTH as first-line treatment for patients with IS.1 Recent studies show that ACTH is not only successful at treating spasms, but is also effective at eliminating hypsarrhythmia in both cryptogenic and symptomatic cases of IS as well.2 Although there is limited data on the efficacy and the appropriate dosing regimen of corticosteroids, there has been an increase in the use of these medications for the treatment of IS recently.1 Prednisolone is often used due to its availability and ease of administration. Vigabatrin is the preferred treatment option in patients with IS and tuberous sclerosis. Although vigabatrin has been shown to be less effective than ACTH, studies indicate that it has an efficacy of >90% in patients with tuberous sclerosis.

The overall prognosis for patients with IS is poor.1 It has been found that only 15–25% of patients with IS are considered to have normal or near normal development throughout their lifetime. In addition, 50% of patients with this condition suffer from epilepsy, 15–33% experience autism, and mortality at 3 years of age is as high as 10%. Fortunately, some evidence suggests that initiating treatment within one month of spasm onset can increase the likelihood of controlling spasms and may improve outcomes, however it is still unclear whether or not prompt treatment truly alters long-term outcomes for patients. Etiology of the patient’s disease has been shown to be the greatest factor that should be considered when determining a patient’s prognosis. Patients with cryptogenic IS are considered to have improved outcomes and a better overall prognosis compared to those with symptomatic IS. Table 1 details the prognosis for each IS category

IS is a rare and devastating condition that occurs in infants and involves clusters of myoclonic-tonic seizures. Diagnosis of IS can be determined by completing a history and physical examination and an EEG. Once diagnosis of IS is established, etiology of the patient’s disease should be determined, as it will aid in treatment decision-making and affects a patient’s prognosis. Although generally poor, a patient’s prognosis is dependent on the etiology of IS as well as a timely diagnosis and treatment.  Guidance on the treatment of IS is limited, however, ACTH, corticosteroids, and vigabatrin are first-line options in the management of IS. Recent studies have indicated ACTH is not only effective at treating spasms, but also at eliminating hypsarrhythmia as well.


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References

1.       Nelson GR. Management of infantile spasms. Transl Pediatri. 2015 Aug 28; 4: 260-270.

2.        The Power To Help Stop Infantile Spasms. Neurology Center for Healthcare Professionals Web site. https://www.actharneurology.com/infantile-spasms/overview. Published 2016. Accessed June 6, 2016.

3.       Infantile spasms. DynaMed. EBSCOhost. Available at http://www.dynamed.com/home/. Accessed June 6, 2016.