Infantile Spasm (West Syndrome) Treatment & Management

  • Author: Tracy A Glauser, MD; Chief Editor: Amy Kao, MD   more...
 
Updated: Jan 19, 2012
 

Approach Considerations

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 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.[11, 12] Alternative options for symptomatic spasms included ACTH and prednisone.[13]

Focal cortical resection

In some patients, resection of a localized region can lead to freedom from seizures.

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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.[14]

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.[15]

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.[16]

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Consultations

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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Contributor Information and Disclosures
Author

Tracy A Glauser, MD  Professor, Departments of Pediatrics and Neurology, University of Cincinnati College of Medicine; Director, Comprehensive Epilepsy Center, Co-Director, Genetic Pharmacology Service, Cincinnati Children's Hospital Medical Center

Tracy A Glauser, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society

Disclosure: Eisai Consulting fee Consulting; Eisai Honoraria Speaking and teaching; Lundbeck Honoraria DSMB member; Questcor None Stock; Ortho McNeil Pharmaceutical Consulting fee Consulting; ucb Pharma Consulting fee Consulting; ucb Pharma Speaking and teaching

Coauthor(s)

Diego A Morita, MD  Assistant Professor of Pediatrics and Neurology, Department of Pediatrics, Division of Neurology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine

Diego A Morita, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and American Medical Association

Disclosure: Nothing to disclose.

Karen Mary Stannard, MD, FRCPC  Fellow in Pediatric Epilepsy, Cincinnati Children's Medical Center

Karen Mary Stannard, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD  Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society

Disclosure: Nothing to disclose.

Additional Contributors

Robert J Baumann, MD Professor of Neurology and Pediatrics, Department of Neurology, University of Kentucky College of Medicine

Robert J Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, and Child Neurology Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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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.
 
 
 
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