Liposteroid (Dexamethasone Palmitate) Therapy for West Syndrome: A Comparative Study With ACTH Therapy

Yamamoto, Hitoshi; Asoh, Masako; Murakami, Hiroshi; Kamiyama, Noriko; Ohta, Chikako

doi:10.1016/s0887-8994(98)00003-4

Cited by 27 publications

(19 citation statements)

References 24 publications

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“…Five studies were identified in the 2004 parameter: 2 RCTs (1 Class II, 15 1 Class III 16 ) using prednisone; 2 Class III prospective, open-label studies using prednisolone 17 and prednisone 18 ; 1 Class III prospective cohort series using liposteroid 19 ; and 1 Class IV retrospective case series using prednisone. 20 Oral corticosteroid dose ranged from 2 to 3 mg/kg/day.…”

Section: Other Forms Of Corticosteroids Are Other Forms Of Corticostmentioning

confidence: 99%

Evidence-based guideline update: Medical treatment of infantile spasms [RETIRED]

Mackay²,

Weiss³

et al. 2012

Neurology

300

277

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Objective:To update the 2004 American Academy of Neurology/Child Neurology Society practice parameter on treatment of infantile spasms in children.Methods: MEDLINE and EMBASE were searched from 2002 to 2011 and searches of reference lists of retrieved articles were performed. Sixty-eight articles were selected for detailed review; 26 were included in the analysis. Recommendations were based on a 4-tiered classification scheme combining pre-2002 evidence and more recent evidence. Results:There is insufficient evidence to determine whether other forms of corticosteroids are as effective as adrenocorticotropic hormone (ACTH) for short-term treatment of infantile spasms. However, low-dose ACTH is probably as effective as high-dose ACTH. ACTH is more effective than vigabatrin (VGB) for short-term treatment of children with infantile spasms (excluding those with tuberous sclerosis complex). There is insufficient evidence to show that other agents and combination therapy are effective for short-term treatment of infantile spasms. Short lag time to treatment leads to better long-term developmental outcome. Successful short-term treatment of cryptogenic infantile spasms with ACTH or prednisolone leads to better long-term developmental outcome than treatment with VGB.Recommendations: Low-dose ACTH should be considered for treatment of infantile spasms.ACTH or VGB may be useful for short-term treatment of infantile spasms, with ACTH considered preferentially over VGB. Hormonal therapy (ACTH or prednisolone) may be considered for use in preference to VGB in infants with cryptogenic infantile spasms, to possibly improve developmental outcome. A shorter lag time to treatment of infantile spasms with either hormonal therapy or VGB possibly improves long-term developmental outcomes. Neurology ® 2012;78:1974-1980 GLOSSARY AAN ϭ American Academy of Neurology; ACTH ϭ adrenocorticotropic hormone; AE ϭ adverse effect; AED ϭ antiepileptic drug; BD ϭ Breslow-Day; CI ϭ confidence interval; ERG ϭ electroretinogram; FDA ϭ Food and Drug Administration; IVIg ϭ IV immunoglobulin; LEV ϭ levetiracetam; NZP ϭ nitrazepam; OR ϭ odds ratio; RCT ϭ randomized controlled trial; TPM ϭ topiramate; TRH ϭ thyrotropin-releasing hormone; TSC ϭ tuberous sclerosis complex; UKISS ϭ United Kingdom Infantile Spasms Study; VABS ϭ Vineland Adaptive Behavioral Scale; VGB ϭ vigabatrin; VPA ϭ valproic acid; ZNS ϭ zonisamide.

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Section: Other Forms Of Corticosteroids Are Other Forms Of Corticostmentioning

confidence: 99%

Evidence-based guideline update: Medical treatment of infantile spasms [RETIRED]

Mackay²,

Weiss³

et al. 2012

Neurology

300

277

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“…Zonisamide, 55 IV immunoglobulin (IVIG), 56 liposteroid, 57 the ketogenic diet, 58 thyrotropin-releasing hormone (TRH), 59 and topiramate 60,61 all have been administered for the treatment of infantile spasms. All except one class III study 57 provided class IV evidence, either in small uncontrolled prospective open-label studies or in retrospective case series.…”

Section: Other Agents What Other Agents Have Been Evaluated For the mentioning

confidence: 99%

Practice Parameter: Medical Treatment of Infantile Spasms

Weiss²,

et al. 2004

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Abstract-Objective:To determine the current best practice for treatment of infantile spasms in children. Methods: Database searches of MEDLINE from 1966 and EMBASE from 1980 and searches of reference lists of retrieved articles were performed. Inclusion criteria were the documented presence of infantile spasms and hypsarrhythmia. Outcome measures included complete cessation of spasms, resolution of hypsarrhythmia, relapse rate, developmental outcome, and presence or absence of epilepsy or an epileptiform EEG. One hundred fifty-nine articles were selected for detailed review. Recommendations were based on a four-tiered classification scheme. Results: Adrenocorticotropic hormone (ACTH) is probably effective for the short-term treatment of infantile spasms, but there is insufficient evidence to recommend the optimum dosage and duration of treatment. There is insufficient evidence to determine whether oral corticosteroids are effective. Vigabatrin is possibly effective for the short-term treatment of infantile spasm and is possibly also effective for children with tuberous sclerosis. Concerns about retinal toxicity suggest that serial ophthalmologic screening is required in patients on vigabatrin; however, the data are insufficient to make recommendations regarding the frequency or type of screening. There is insufficient evidence to recommend any other treatment of infantile spasms. There is insufficient evidence to conclude that successful treatment of infantile spasms improves the long-term prognosis. Conclusions: ACTH is probably an effective agent in the short-term treatment of infantile spasms. Vigabatrin is possibly effective. NEUROLOGY 2004;62:1668 -1681 West syndrome 1 is a unique, age-specific epilepsy of early infancy. Infantile spasms are distinct from myoclonic and tonic seizures.2 They are characterized by an initial contraction phase followed by a more sustained tonic phase. They can be divided into three types (flexor, extensor, and mixed flexor-extensor spasms), and they can also be asymmetrical.2 The EEG characteristically demonstrates hypsarrhythmia, and onset of spasms is frequently associated with neurodevelopmental regression. The incidence of infantile spasms is

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“…Infantile spasms and hypsarrhythmia on EEG disappeared in all 5 patients in the liposteroid therapy group within 4 doses; in the ACTH therapy group, infantile spasms and hypsarrhythmia on EEG similarly disappeared during treatment in all 5 patients, but infantile spasms reappeared 2 months after therapy in 2 patients. No notable adverse reactions occurred in the liposteroid group, but transient dysfunction of the thyroid and anterior pituitary gland and increased levels of serum cortisol were experienced in the ACTH group 96. In another study, a single intravenous injection of liposteroid (0.25 mg/kg) was administered 12 times in 1 month (total dosage 3.0 mg/kg) to 4 patients with West syndrome.…”

Section: Liposteroidmentioning

confidence: 95%

“…Dexamethasone palmitate (liposteroid) was used for the treatment of West syndrome and compared with adrenocorticotropic hormone (ACTH) therapy 96. A single intravenous injection of liposteroid (0.25 mg/kg) was administered seven times in 3 months (total dosage = 1.75 mg/kg) to 5 symptomatic patients with West syndrome, aged 4 to 11 months.…”

Section: Liposteroidmentioning

confidence: 99%

Current trends in the treatment of infantile spasms

Tsao¹

2009

NDT

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Infantile spasms are an epilepsy syndrome with distinctive features, including age onset during infancy, characteristic epileptic spasms, and specific electroencephalographic patterns (interictal hypsarrhythmia and ictal voltage suppression). Adrenocorticotropic hormone (ACTH) was first employed to treat infantile spasms in 1958, and since then it has been tried in prospective and retrospective studies for infantile spasms. Oral corticosteroids were also used in a few studies for infantile spasms. Variable success in cessation of infantile spasms and normalization of electroencephalograms was demonstrated. However, frequent significant adverse effects are associated with ACTH and oral corticosteroids. Vigabatrin has been used since the 1990s, and shown to be successful in resolution of infantile spasms, especially for infantile spasms associated with tuberous sclerosis. It is associated with visual field constriction, which is often asymptomatic and requires perimetric visual field study to identify. When ACTH, oral corticosteroids, and vigabatrin fail to induce cessation of infantile spasms, other alternative treatments include valproic acid, nitrazepam, pyridoxine, topiramate, zonisamide, lamotrigine, levetiracetam, felbamate, ganaxolone, liposteroid, thyrotropin-releasing hormone, intravenous immunoglobulin and a ketogenic diet. Rarely, infantile spasms in association with biotinidase deficiency, phenylketonuria, and pyridoxine-dependent seizures are successfully treated with biotin, a low phenylalanine diet, and pyridoxine, respectively. For medically intractable infantile spasms, some properly selected patients may have complete cessation of infantile spasms with appropriate surgical treatments.

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Liposteroid (Dexamethasone Palmitate) Therapy for West Syndrome: A Comparative Study With ACTH Therapy

Cited by 27 publications

References 24 publications

Evidence-based guideline update: Medical treatment of infantile spasms [RETIRED]

Evidence-based guideline update: Medical treatment of infantile spasms [RETIRED]

Practice Parameter: Medical Treatment of Infantile Spasms

Current trends in the treatment of infantile spasms

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