Long-term outcome of patients with West syndrome

Riikonen, Raili

doi:10.1016/s0387-7604(01)00307-2

Cited by 168 publications

(132 citation statements)

References 9 publications

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“…Others have found that 3 to 5% of patients treated with ACTH die and that these patients tend to have received large doses of ACTH (Riikonen 2001;Hamano et al 2004). Most protocols of ACTH treatment for WS indicate that ACTH should be administered for more than 14 days and then tapered (Yanagaki et al 1999;Mackay et al 2004).…”

Section: Discussionmentioning

confidence: 99%

Cardiac Vagal Activation by Adrenocorticotropic Hormone Treatment in Infants with West Syndrome

Hattori

Hayano

Fujimoto

et al. 2007

Tohoku J. Exp. Med.

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West syndrome (WS) is a generalized epileptic syndrome of infancy and early childhood with various etiologies, and consists of a triad of infantile spasm, arrest or regress of psychomotor development and specific electroencephalogram (EEG) pattern of hypsarrhythmia. WS had been believed to be refractory, but recent evidence supports effectiveness of adrenocorticotropic hormone (ACTH) treatment. The ACTH treatment, however, has a problem that it is often accompanied by adverse autonomic symptoms. We therefore examined heart rate variability (HRV) for assessing cardiac autonomic functions in WS and prospectively observed the changes during ACTH treatment. We studied 15 patients with WS and 9 age-matched controls during sleep (EEG stage 2). Compared with controls, the patients with WS were greater in the low-frequency component (LF) of HRV, an index reflecting sympatho-vagal interaction ( p = 0.02), but were comparable for high-frequency component (HF) and LF-to-HF ratio (LF/HF), indices reflecting cardiac vagal activity and sympathetic predominance, respectively. During ACTH treatment, heart rate decreased ( p < 0.01), LF and HF increased ( p < 0.01), and LF/HF did not differ significantly. These results indicate that West syndrome might be accompanied by autonomic changes and that ACTH treatment enhances parasympathetic function and causes bradycardia. West syndrome; ACTH; heart rate variability; bradycardia; autonomic function

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Section: Discussionmentioning

confidence: 99%

Cardiac Vagal Activation by Adrenocorticotropic Hormone Treatment in Infants with West Syndrome

Hattori

Hayano

Fujimoto

et al. 2007

Tohoku J. Exp. Med.

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“…Combining these two studies revealed that high-dose ACTH stopped the spasms in 79 % of subjects compared with 76 % of subjects with low-dose ACTH [12]. An older, prospective, large Finnish study showed no difference in response rate (cessation of infantile spasms and resolution of hypsarrhythmia) or relapse rate comparing low-dose (20-40 IU/day) [N = 54] and high-dose (80-120 IU/day) [N = 97] regimens; however, long-term cognitive outcome was better with the low-dose regimen [29,30]. Recent evidence also suggests that low-dose ACTH is probably as effective as high-dose ACTH for short-term treatment of infantile spasms (Class I and II evidence) [7].…”

Section: What Is the Most Effective Treatment For Infantilementioning

confidence: 99%

Recent Advances in the Pharmacotherapy of Infantile Spasms

Riikonen

2014

CNS Drugs

Self Cite

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Adrenocorticotrophic hormone (ACTH), oral corticosteroids and vigabatrin are now first-line treatments for infantile spasms in the US and Europe. There is now increased knowledge regarding the role of ACTH, corticosteroids and vigabatrin (e.g. efficacy, doses, side effects, treatment in specific aetiological subtypes of infantile spasms), and other antiepileptic drugs (i.e. topiramate, valproate, zonisamide, sulthiame, levetiracetam, lamotrigine, pyridoxine, ganaxolone), as well as adjunctive flunarizine and novel drugs not yet in clinical use for infantile spasms (i.e. pulse rapamycin and melanocortin receptor agonists). The existence of a latent period, weeks to months following a precipitating brain insult, raises the possibility of preventive interventions. Recent experimental data emerging from animal models of infantile spasms have provided optimism that new and innovative treatments can be developed, and knowledge that drug treatment can affect long-term cognitive outcome is increasing. The aim of this article is to review recent developments in the pharmacotherapy of infantile spasms and to highlight the practical implications of the latest research.

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“…Infantile spasms, per se, do not usually persist into adulthood. 8 Approximately 36% of patients with a history of IS are seizure-free by adulthood 9 ; however, 50 -70% of patients develop other seizure types, and 18 -50% develop Lennox-Gastaut syndrome.…”

Section: Epidemiology Of Complex Partial Seizures and Infantile Spasmsmentioning

confidence: 99%

“…Between 70 and 90% exhibit learning difficulties or mental retardation, 8 and psychiatric symptoms are common in patients, occurring in 20 -40% of adults with a history of IS. 8 Among patients with a history of IS, the reported premature death rate ranges from 2 to 31%, [7][8][9] with 61% of deaths occurring at or before 10 years of age, and 10% occurring after the age of 20 years. …”

Section: Consequences Of Uncontrolled Seizuresmentioning

confidence: 99%

Vigabatrin

2007

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Summary: Refractory epilepsies such as infantile spasms (IS) and complex partial seizures (CPS) can have a severe negative impact on the neurological integrity and quality of life of affected patients, in addition to drastically increasing their risk of premature mortality. Early identification of potentially effective pharmacotherapy agents is important. Vigabatrin has been shown to be a generally well tolerated and effective antiepileptic drug (AED) in a wide variety of seizure types affecting both children and adults, particularly those with IS and CPS. A bilateral, concentric constriction of the peripheral visual field characterizes the visual field defect (VFD) associated with vigabatrin, well characterized by numerous studies. This peripheral VFD presents in 30 -50% of patients with exposure of several years; however, most of these patients are asymptomatic. In well-controlled studies, the earliest onset in patients with CPS is 11 months and at 5 months in infants, with average onsets being more than 5 years and 1 year, respectively. Patients with a peripheral VFD retain an average 65°of lateral vision (normal, 90°). The fact that many patients never develop the vigabatrin-related peripheral VFD, despite long-term exposure at high doses, may support the hypothesis that the injury is an idiosyncratic adverse drug reaction (as opposed to a strict dose-or duration-dependent toxicity). Effective testing methods are available to aid in the early detection and management of the peripheral VFD. This article discusses issues of importance to clinical decision-making in the use of vigabatrin to assist the physician and patient in assessing the benefits of vigabatrin therapy and understanding the potential risks of the VFD and uncontrolled seizures.

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Long-term outcome of patients with West syndrome

Cited by 168 publications

References 9 publications

Cardiac Vagal Activation by Adrenocorticotropic Hormone Treatment in Infants with West Syndrome

Cardiac Vagal Activation by Adrenocorticotropic Hormone Treatment in Infants with West Syndrome

Recent Advances in the Pharmacotherapy of Infantile Spasms

Vigabatrin

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