Infantile Spasms Health Article

Treatment Team

The treatment team usually includes pediatric neurologists, neurosurgeons, nurses specializing in epilepsy care, and dietitians. In addition to conventional therapies, the team provides the latest in diagnostic and therapeutic approaches, including such innovations as the ketogenic diet, diagnostic video telemetry, and epilepsy surgery for intractable seizures. New epilepsy studies focus in investigating promising new drugs and other novel therapies.

Treatment

Due to the poor prognosis of infantile spasms, treatment is usually initiated quickly and aggressively after diagnosis, often at the risk of serious side effects, with the hope of changing the natural history of the disease. Antiepileptic medications are the mainstay of therapy for infants with infantile spasms. Unfortunately, no one medical treatment gives satisfactory relief for all patients. In most open-label or retrospective studies, adrenocorticotrophic hormone ACTH or prednisone induces a reduction or complete cessation of spasms, as well as an improvement in the EEG, in approximately 50–75% of patients. This effect is usually achieved within a couple weeks. Patients unresponsive to ACTH may respond to prednisone and vice-versa. A large variety of ACTH doses have been used, but there is no evidence that larger doses (150 units/day) are more effective than lower doses (20–30 units/day). While relapses occur in about one-third to one-half of patients, a second course of ACTH is often effective.

Among conventional anti-seizure drugs, valproate and nitrazepam have been shown to be effective as first-line therapy. In addition to medication, there are some potential surgical options for infantile spasms, although they may only be applicable to a small percentage of patients. Although in most patients the precise source of the spasms in the brain cannot be localized, there is a small minority of patients who have secondarily generalized spasms from lesions in the brain that can be surgically removed.

Newer anti-seizure medicines such as Vigabatrin, although not yet approved in the United States, have shown promise in reducing the frequency of infantile spasms by increasing the brain's available amount of GABA, a neurotransmitter that helps transmit information as it bridges the gaps between nerve cells.

Recovery and rehabilitation

Infantile spasms usually cease spontaneously by age five, but are often replaced by seizures of other types. Therefore, emphasis is placed on lifelong seizure prevention rather than recovery. Maintaining control of seizures in infancy can sometimes reduce developmental delays and mental retardation, although most infants will already have significant neurological impairment before the onset of symptoms.

Clinical trials

Although as of early 2004, there were no ongoing clinical trials for infantile spasms, the National Institutes of Health (NIH) sponsors research related to many seizure disorders. Information on the numerous current clinical trials for the study and treatment of seizure disorders can be found at the NIH website: <http://clinicaltrials.gov/search/term=Seizure+Disorder>.

Prognosis

Infantile spasms usually resolve with or without treatment in the majority of patients, generally by mid-childhood. However, other seizure types arise in 50–70% of patients. Similarly, on long-term follow-up, chronic intractable (unable to respond to treatment) epilepsy is present in approximately 50% of patients with a history of infantile spasms.

Mental retardation occurs in 70–90% of persons with infantile spasms, usually involving severe to profound retardation. Other neurological deficits, such as cerebral palsy, may be seen in about 30–50% of patients. By far, the most important factor in predicting neurological prognosis, including developmental outcome and long-term epilepsy, is the underlying cause of the seizures.

Factors that have been associated with a good prognosis include normal neurological exam and development at onset, absence of other seizure types at onset, older age of onset, short duration of spasms, and early effective treatment of spasms (reported with ACTH).

Special concerns

Once infants begin to have infantile spasms, they often fail to meet new milestones and may even regress, losing mental or physical skills previously learned. When the seizures begin, parents may notice a loss of interest in people and objects in the child's environment. Social interaction may diminish, smiling may cease, sleep may become disrupted, and the child may seem irritable or indifferent to surroundings. A child who had learned to sit may stop sitting or even lose the ability to roll over; a child who had been babbling happily may become silent or fussy.

BOOKS

Frost, James D., Jr., and Richard A. Hrachovy. Infantile Spasms: Diagnosis, Management and Prognosis. New York: Kluwer Academic Publishers, 2003.

PERIODICALS

Shields, W. D. "West's syndrome." J. Child Neurol 17 (2002): S76–79.

West, W. J. "On a peculiar form of infantile convulsions." Lancet (1840–1841) I: 724–725.

OTHER

National Institute of Neurological Disorders and Stroke. NINDS Infantile Spasms Information Page. (April 5, 2004). <http://www.ninds.nih.gov/health_and_medical/disorders/infantilespasms.htm>.