Infantile Spasm (West Syndrome) Treatment & Management
- Author: Tracy A Glauser, MD; Chief Editor: Amy Kao, MD more...
The goals of treatment for infants with West syndrome 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, vigabatrin, and conventional antiepileptic drugs (AEDs) are the mainstay of therapy for infants with West syndrome. Unfortunately, no single medical treatment gives satisfactory relief for all infants with West syndrome.
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] Alternative options for symptomatic spasms included ACTH and prednisone.
Focal cortical resection
In some patients, resection of a localized region can lead to freedom from seizures.
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.
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.
A 2008 retrospective study showed a similar median time to seizure freedom in infants with new-onset infantile spasms treated with either the ketogenic diet or ACTH. There was delay of 2-5 months in normalization of the EEG in those patients treated with the diet, versus a delay of 1 month with ACTH. The side effects of the diet were less than those of ACTH. There was no difference in developmental outcome between the groups at 12 months’ follow-up.
Consultations with the following specialists can be beneficial:
Pediatric neuropsychologists - Can assess intellectual function and educational needs and advise on nonpharmacologic management of behavioral problems
Pediatric psychiatrists - Can advise on pharmacologic management of behavioral problems
Neurosurgeons - Can help to assess whether the infant is a candidate for focal resection
Dietitians - Can assist in the institution and maintenance of the ketogenic diet
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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