Approach Considerations
The goals of treatment for infants with infantile spasms are the best quality of life (with no seizures), the fewest adverse effects from treatment, and the lowest number of medications.
Medications such as ACTH, oral corticosteroids, vigabatrin, and conventional antiepileptic drugs (AEDs) have all been used for infantile spasms. Unfortunately, no single medical treatment gives satisfactory relief for all infants with infantile spasms, and what is considered the best approach for treatment has been a debated and evolving topic.
In 2007, an expert survey concluded that 1-3 trials of monotherapy should be implemented before considering epilepsy surgery. In patients with tuberous sclerosis or symptomatic infantile spasms, vigabatrin was the drug of choice. [13, 14] Publications regarding specific treatments are reviewed in the Medication section, however, findings from a multicenter prospective database of infants with new diagnosis of infantile spasms compared "standard therapy" with ACTH, oral steroids, or vigabatrin to all other medications and found that 46% of patients treated with standard therapy had remission of spasms and resolution of hypsarrhythmia sustained at 3 months after initiation of the treatment, versus 9% of patients treated with "nonstandard" therapy. [15]
Focal cortical resection
In some patients, resection of a localized region can lead to freedom from seizures.
Diet
The ketogenic diet has been used successfully to treat a variety of seizure types. Studies have shown that it can be considered for the medical management of infantile spasms. [16]
A 2006 retrospective study showed that the ketogenic diet in a ratio of 4:1 was effective in a small patient population with intractable infantile spasm previously treated with combinations of vigabatrin, topiramate, other AEDs, or prednisolone. A greater than 90% reduction in seizures was seen in 63% of children, and 40% were seizure free at 6 months. Side effects, including gastrointestinal disturbance, infection, and renal stones, were transient. [17]
One center prospectively followed 104 patients with infantile spasms, treated with the ketogenic diet; these patients included both patients with new onset infantile spasms and those that were unsuccessfully treated with other therapies. Of the total patients, 37% became spasm-free for at least 6 months within a median 2.4 months of starting the ketogenic diet, and 64% had a greater than 50% improvement in spasms. [18]
Consultations
Consultations with the following specialists can be beneficial:
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Pediatric neuropsychologists - Can assess intellectual function and educational needs and advise on nonpharmacologic management of behavioral problems
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Pediatric psychiatrists - Can advise on pharmacologic management of behavioral problems
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Neurosurgeons - Can help to assess whether the infant is a candidate for focal resection
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Dietitians - Can assist in the institution and maintenance of the ketogenic diet
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Cardiologists - Can be consulted at the initiation of therapy with ACTH or steroids; they can look for potential side effects of these therapies, evaluating cardiac function (by looking for signs of hypertrophic cardiomyopathy) and aiding in the management of hypertension
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Mountainous, chaotic, disorganized rhythms with superimposed multifocal spikes demonstrating hypsarrhythmia in a boy aged 8 months with infantile spasms and developmental delay. Courtesy of E Wyllie.
