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Ceskoslovenska Neurologie a... Jul 1991The article is a review of contemporary knowledge of the so-called West syndrome. It deals with the definition, aetiology, clinical aspects, therapy and prognosis of... (Review)
Review
The article is a review of contemporary knowledge of the so-called West syndrome. It deals with the definition, aetiology, clinical aspects, therapy and prognosis of this disease. In addition to the clinical picture of West's syndrome the author describes also recently described units which may fuse with this syndrome or mutually. The author deals on purpose in more detail with treatment of West's syndrome, in particular steroid administration (specially ACTH and its synthetic derivatives) as among our neurologists there are still very controversial views as regards indication and control of steroid treatment, sometimes associated with certain not always rational prejudices.
Topics: Humans; Infant; Infant, Newborn; Prognosis; Spasms, Infantile
PubMed: 1751975
DOI: No ID Found -
Pediatric Neurology Aug 2009This study examines factors influencing the evolution of West syndrome to Lennox-Gastaut syndrome. The study population comprised 98 patients diagnosed with West...
This study examines factors influencing the evolution of West syndrome to Lennox-Gastaut syndrome. The study population comprised 98 patients diagnosed with West syndrome and monitored for at least 3 years. During follow-up, West syndrome evolved to Lennox-Gastaut syndrome in 48 of the 98 patients. Etiology analysis indicated that West syndrome was cryptogenic in 36 patients (36.7%) and symptomatic in 62 (63.3%). West syndrome was managed with antiepileptic drugs in 31 patients, ketogenic diets in 33 patients, hormonal therapy with prednisolone in 45 patients and with adrenocorticotropic hormone in 15 patients, epileptic surgery in 3 patients, and either no treatment or only herbal medication in 4 patients. The risk of developing Lennox-Gastaut syndrome was significantly lower in patients who were placed on a ketogenic diet, given prednisolone or adrenocorticotropic hormone, or treated with a combination of these two therapies (bivariate logistic regression analysis, P < 0.05). There was no relationship between the development of Lennox-Gastaut syndrome and age at West syndrome onset or disease etiology. In conclusion, a ketogenic diet and hormonal therapy may play key roles in preventing encephalopathy in patients with West syndrome.
Topics: Age of Onset; Child; Child, Preschool; Diet, Ketogenic; Disease Progression; Epilepsy; Female; Follow-Up Studies; Hormone Replacement Therapy; Humans; Infant; Infant, Newborn; Logistic Models; Male; Retrospective Studies; Risk Factors; Spasms, Infantile; Syndrome
PubMed: 19589458
DOI: 10.1016/j.pediatrneurol.2009.03.006 -
Advances in Experimental Medicine and... 2002Many cases of West's syndrome are inoperable, because there is no clear epileptic focus. In some cases, however, there is a structural abnormality which can be... (Review)
Review
Many cases of West's syndrome are inoperable, because there is no clear epileptic focus. In some cases, however, there is a structural abnormality which can be surgically removed. Dr. Hoffman discusses his experience with surgery for cortical dysplasia in West's syndrome.
Topics: Humans; Infant; Infant, Newborn; Neurosurgical Procedures; Spasms, Infantile
PubMed: 11993740
DOI: No ID Found -
Brain & Development Nov 2001Approximately 10-30% of patients with West syndrome respond to high-dose vitamin B(6) treatment. The response to vitamin B(6) is rapid; seizures disappear within the... (Review)
Review
Approximately 10-30% of patients with West syndrome respond to high-dose vitamin B(6) treatment. The response to vitamin B(6) is rapid; seizures disappear within the first 2 weeks of treatment. Mild side effects, such as gastrointestinal symptoms and liver dysfunction, are observed in 40-70%, but these resolve after discontinuation or a reduction of the dosage of vitamin B(6). High-dose vitamin B(6) treatment is useful as a first line agent in treating West syndrome.
Topics: Electrocardiography; Female; Humans; Infant, Newborn; Spasms, Infantile; Vitamin B 6
PubMed: 11701272
DOI: 10.1016/s0387-7604(01)00292-3 -
Neurosciences (Riyadh, Saudi Arabia) Jan 2011To determine Topiramate efficacy on treatment of infantile spasms and ancillary seizures, and whether there were any improvements on EEG.
OBJECTIVE
To determine Topiramate efficacy on treatment of infantile spasms and ancillary seizures, and whether there were any improvements on EEG.
METHODS
A retrospective study of 18 patients with infantile spasms recruited from the Pediatric Unit at King Fahd Hospital of the University, Dammam University, Saudi Arabia was carried out between January 2004 and December 2008. Topiramate was used as treatment in 7 males and 11 females aged 2-14 months. The maximum dose was 12 mg/kg/day.
RESULTS
The etiology in 9 (50%) patients was cryptogenic, 6 (33%) symptomatic, and 3 (17%) idiopathic. After Topiramate treatment 6 (33%) were spasm free, 8 (44%) had ≥50% reduction, 2 (11%) had no change, and one (6%) had worsening of their spasms. Eight patients had ancillary seizures, 2 (25%) were seizure free, 2 (25%) had ≥50% seizure reduction, and 4 (50%) had no change in the ancillary seizure. The EEG showed hypsarrhythmia in 14 (78%). Post Topiramate, the EEG was normal in one (5%), improved in 3 (17%), showed persistent hypsarrhythmia in 8 (44%), and evolved to other features in 3 (17%). Three patients developed side effects such as weight loss and irritability, for which 2 patients stopped the medication.
CONCLUSION
Topiramate has a good effect on the clinical features of West syndrome, but not on the EEG. It was tolerated with minimal side effects.
Topics: Anticonvulsants; Electroencephalography; Female; Fructose; Humans; Infant; Male; Retrospective Studies; Spasms, Infantile; Topiramate; Treatment Outcome
PubMed: 21206445
DOI: No ID Found -
The Neurologist Mar 2010Infantile spasms (West syndrome) is an epilepsy condition affecting 1 in 2000 infants. Perhaps no more worrisome neurologic disorder exists because of its frequent... (Review)
Review
BACKGROUND
Infantile spasms (West syndrome) is an epilepsy condition affecting 1 in 2000 infants. Perhaps no more worrisome neurologic disorder exists because of its frequent association with delayed development and cognition at such a young age. Despite its existence in the literature since 1841, proven therapies are limited because of efficacy, tolerability, at times even availability.
REVIEW SUMMARY
In this review, the clinical features, electroencephalogram (EEG) findings (hypsarrhythmia), prognostic factors, and myriad of treatment options for this condition will be discussed. Guidelines, surveys, and practice parameters have judged adrenocorticotropin hormone and vigabatrin to be the most proven treatments, with the latter indicated for tuberous sclerosis. However, potentially helpful therapies with fewer side effects have recently emerged including high-dose oral prednisolone, ketogenic diet, and topiramate. Additionally, advances in the past several years include the creation of viable animal models for testing new treatments.
CONCLUSIONS
At no other time since its first description in 1841 has the field of infantile spasms research been so rapidly changing. For the thousands of infants faced with this potentially devastating disorder, there is no time like the present.
Topics: Animals; Humans; Infant; Spasms, Infantile
PubMed: 20220440
DOI: 10.1097/NRL.0b013e3181d1416c -
Epileptic Disorders : International... Dec 2019Benign spasms of infancy (BSI), previously described as benign non-epileptic infantile spasms or benign myoclonus of early infancy, are non-epileptic movements...
Benign spasms of infancy (BSI), previously described as benign non-epileptic infantile spasms or benign myoclonus of early infancy, are non-epileptic movements manifesting during the first year of life and spontaneously resolving in the second year of life. BSI are characterized by spasms typically lasting 1-2 seconds, involving, to varying degrees, the head, neck, trunk, shoulders and upper extremities. Ictal and interictal EEG recordings are normal. BSI are not associated with developmental regression and do not require treatment. Distinction between BSI and infantile epileptic disorders, such as epileptic spasms or myoclonic epilepsy of infancy, can be challenging given the clinical similarities. Moreover, interictal EEGs can be normal in all conditions. Epileptic spasms and myoclonic epilepsy require timely treatment to improve neurodevelopmental outcomes. We describe a six-month-old infant presenting with spasm-like movements. His paroxysms as well as a positive family history for epileptic spasms were in keeping with a likely diagnosis of West syndrome. Surprisingly, ictal video-EEG did not reveal epileptiform activity, and suggested a diagnosis of BSI. We emphasize that ictal video-EEG is the gold standard for classification of infantile paroxysms as epileptic or non-epileptic, thereby avoiding over-treatment for BSI and facilitating timely targeted treatment of infantile epilepsies. [Published with video sequences].
Topics: Diagnosis, Differential; Epilepsies, Myoclonic; Humans; Infant; Male; Spasms, Infantile
PubMed: 31843733
DOI: 10.1684/epd.2019.1116 -
Ryoikibetsu Shokogun Shirizu 2002
Review
Topics: Diagnosis, Differential; Humans; Hyperglycinemia, Nonketotic; Infant; Infant, Newborn; Menkes Kinky Hair Syndrome; Metabolism, Inborn Errors; Mitochondrial Diseases; Phenylketonurias; Prognosis; Pyruvate Carboxylase Deficiency Disease; Pyruvate Dehydrogenase (Lipoamide); Spasms, Infantile
PubMed: 12483882
DOI: No ID Found -
Brain & Development Nov 2001The prevalence of West syndrome in Thailand had not been studied. During January 1997 to December 1999, at the Department of Pediatrics, Ramathibodi Hospital, Bangkok,...
The prevalence of West syndrome in Thailand had not been studied. During January 1997 to December 1999, at the Department of Pediatrics, Ramathibodi Hospital, Bangkok, Thailand, of the total 628 first seen epileptic patients, aged 0-15 years, 31 patients (4.9%) with West syndrome were encountered. Sixteen were males. The mean age at presentation was 9.7 months (range 4-32 months) and at seizure onset was 5.7 months (range 2-11 months). Twenty-two patients had typical spasms, four each had generalized tonic seizures and focal seizures prior to the onset of infantile spasms, and another patient had myoclonic seizures. Nineteen patients were classified as symptomatic. Electroencephalogram revealed hypsarrhythmia in six patients, multifocal independent epileptiform discharges in 24 patients, and focal epileptiform discharges in four patients. Abnormal brain-imaging findings were demonstrated in six of the 11 patients investigated. Vigabatrin was given to 17 patients whereas one patient was given valproate. Others who had been severely retarded were given phenobarbital in combination with benzodiazepine. Six patients were lost to follow-up. Of the remaining patients, the duration of follow-up ranged from 7 to 44 months (mean 28 months). Infantile spasms disappeared in 11 patients, persisted in six patients, and changed to other form in eight patients. The developmental outcome was apparently normal in five, slightly delayed in four, moderately delayed in ten, and severely delayed in eight patients.
Topics: Adolescent; Age Distribution; Anticonvulsants; Child; Child, Hospitalized; Child, Preschool; Data Collection; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Male; Prevalence; Spasms, Infantile; Thailand
PubMed: 11701264
DOI: 10.1016/s0387-7604(01)00294-7 -
Current Neurology and Neuroscience... Apr 2018The care of children with infantile spasms is full of areas of ambiguity, despite practice parameters and randomized trials. This review is to update the current care of... (Review)
Review
PURPOSE OF THE REVIEW
The care of children with infantile spasms is full of areas of ambiguity, despite practice parameters and randomized trials. This review is to update the current care of children with infantile spasms.
RECENT FINDINGS
Several recent studies have generated new data to guide management including a randomized trial supporting combination therapy of hormone treatment and vigabatrin as an initial treatment for infantile spasms. Studies have suggested that we are not consistent in our recognition of hypsarrhythmia, while additional studies suggest that the presence or absence of this pattern may not have any predictive value. Additionally, the use of appropriate medications as well as an early diagnosis has the most impact on short-term outcomes. Children with infantile spasms benefit most from early diagnosis and early treatment with appropriate standard medications such as hormonal therapy (ACTH or prednisolone) or vigabatrin.
Topics: Adrenocorticotropic Hormone; Animals; Anticonvulsants; Child; Child, Preschool; Combined Modality Therapy; Electroencephalography; Glucocorticoids; Humans; Infant; Prednisolone; Spasms, Infantile; Treatment Outcome; Vigabatrin
PubMed: 29671077
DOI: 10.1007/s11910-018-0832-8