alternative therapy infantile spasms west syndrome epilepsy seizures hypsarrhythmia eeg childhood epilepsy infant seizures developmental delay information myclonic baby acth

Alternative Therapys

Pyridoxine (vitamin B6)

dependency is a very rare cause of infantile spasms. A trial of 100-mg pyridoxine given intravenously should be administered if diagnosis remains in doubt after the history, examination, and MRI scan have been performed (33). An immediate normalization of the EEG suggests pyridoxine-dependent epilepsy. However, chronic oral administration of high doses of pyridoxine also may be effective for some patients who do not have pyridoxine-dependent seizures (34, 35). In Japan, high-dose pyridoxine is considered the initial drug of choice by many pediatric neurologists (36), with reports that approximately 15% respond. While this response rate is clearly inferior to either ACTH or vigabatrin, pyridoxine is their first choice based on the safety profile. Side effects include loss of appetite, irritability, and vomiting—all of which are relatively common but modest compared with those associated with ACTH or vigabatrin. Pyridoxine has not found favor outside of Japan and a few other epilepsy centers. But, given the low risk associated with its use, it seems reasonable to give patients a 1 to 2 week trial of 100 to 400-mg pyridoxine before starting other medications.

Ketogenic Diet

The high fat, low-protein, and low-carbohydrate diet for treating children with epilepsy who do not respond to or cannot tolerate drugs. This diet mimics the biochemical changes associated with starvation and induces, among other changes, production of ketone bodies (mainly beta hydroxybutyrate, and to lesser extent, acetoacetate and acetone), which has been implicated in the mechanisms of seizure control.

Before the historic first announcement of results with the ketogenic diet by the Mayo Clinic [Wilder 1921], bromides and Phenobarbital were the only effective options for antiepileptic therapy. Fasting as a method for controlling epilepsy had been reported sporadically long before this time. However, it was only with such reports in the clinical journals did other
medical centers begin adopting the ketogenic diet as an effective treatment for intractable cases of epilepsy.

Still, the diet remained generally under-utilized, being utilized mainly at institutions such as the Mayo Clinic and Johns Hopkins, until the 1990s when a national television program aired a report on a child whose epilepsy was cured by the ketogenic diet. Later, the Charlie Foundation (named after the young patient) was formed and a television movie recounting Charlie’s success with the ketogenic diet was produced. It also prompted a flood of inquiries to pediatric neurologists and epileptologists about this treatment option.

"We decided to review our experience at Johns Hopkins using the ketogenic diet to treat infantile spasms before medications were tried and compare this to our use of ACTH over the same time period," says Eric Kossoff, M.D, a pediatric neurologist at Johns Hopkins Hospital and lead author of the study. "We knew that the ketogenic diet worked well for difficult-to-control infantile spasms, so we thought it would also be effective earlier." 

If the diet stopped the spasms, infants were kept on it for usually 6 months. The diet worked in 8-of-13 infants within approximately one week. Only 1-of-8 had recurring spasms, and that infant was controlled again with the addition of topiramate to the diet. Side effects were fewer than ACTH in this series and the recurrence rate was also lower with the diet. In the 5 patients in which the diet did not work, ACTH was started immediately; it worked quickly in 4 of the 5 infants. ACTH did lead to a normal EEG quicker, but long-term developmental outcomes were identical.

As a result of the findings, the ketogenic diet is now one of the typically-offered first-line therapies for new-onset infantile spasms at Johns Hopkins. Other hospitals are beginning to use the ketogenic diet similarly. The researchers hope this novel use of the ketogenic diet may be the first step in finding another treatment to control new-onset infantile spasms. 

High-dose intravenous immunoglobulin

has been reported to be helpful in a variety of seizure disorders. Ariizumi et al. reported that all six children in their study who had cryptogenic infantile spasms achieved complete remission, but only one of five symptomatic patients responded (55). Intravenous immunoglobulin doses ranged from 100 to 200 mg/kg/dose administered every 2 to 3 weeks to 400 mg/kg/day for five consecutive days. Although the data are extremely limited, intravenous immunoglobulin could be considered a possible therapeutic option in patients who have failed other medical therapies, but the actual efficacy is unclear and the most appropriate dosing and duration have not been defined.

Thyrotropin-releasing hormone (TRH)

Intractable seizures remain a significant therapeutic challenge despite current advances in the treatment of epilepsy. Thyrotropin-releasing hormone, the first neuroendocrine releasing factor to be isolated and fully characterized, was also the first releasing factor investigated as a possible neurotransmitter/neuromodulator outside the hypothalamus. Basic and clinical research has revealed a distinct neuroanatomic distribution and a neurochemical role for thyrotropin-releasing hormone in seizure modulation.

  Thyrotropin-releasing hormone and selected analogs were reported to have antiepileptic effects in several animal seizure paradigms, including kindling and electroconvulsive shock. Clinically, thyrotropin-releasing hormone treatment has been reported to be efficacious in such intractable epilepsies as infantile spasms, Lennox-Gastaut syndrome, myoclonic seizures, and other generalized and refractory partial seizures. Herein, we review evidence that suggests that thyrotropin-releasing hormone and selected thyrotropin-releasing hormone analogs may represent a new class of novel antiepileptic drugs, namely, antiepileptic neuropeptides and provide insights into potential new treatments for the intractable epilepsies.

Cortical Resection

The final non drug therapeutic option is cortical resection and should be considered for patients who have failed ACTH and Vigabatrin or both, and have evidence of localizable abnormalities, such as cortical dysplasia, porencephaly, or tuberous sclerosis. Not many years ago, infantile spasms was considered to be a generalized seizure disorder, and thus not surgically remediable. It has become clear in the last several years that in spite of the generalized nature of the seizures, an area of cortical abnormality can often be discovered and that its removal may lead to control of seizures (56, 57) as well as possibly to improved developmental outcome (58, 59). The majority of patients who have cortical resection have evidence of focal cortical abnormalities prior to surgical evaluation (60), and for these patients, surgery should be considered early in the course rather than waiting for months or years. Selecting the appropriate candidates for surgery is usually more difficult in infantile spasms than for other types of epilepsy because of the generalized nature of the EEG abnormalities. A careful review of the history (especially history of partial seizures that preceded or accompanied infantile spasms) and the presence of cortical disturbances on MRI scan and of localized EEG abnormalities that suggest a localized cortical defect, all should lead to referral to a pediatric epilepsy surgery center for further evaluation.

Alternative Therapys

alternative therapy infantile spasms west syndrome epilepsy seizures hypsarrhythmia eeg childhood epilepsy infant seizures developmental delay information myclonic baby acth

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