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Journal of Child Neurology Oct 2012West syndrome (infantile spasms) is an epileptic encephalopathy that includes psychomotor deterioration. In rare cases, it is due to an inherited, progressive metabolic... (Review)
Review
West syndrome (infantile spasms) is an epileptic encephalopathy that includes psychomotor deterioration. In rare cases, it is due to an inherited, progressive metabolic disease. More than 25 inborn errors of metabolism have been considered etiologic or predisposing factors for infantile spasms. This is a review of the literature on reported cases of children diagnosed with a metabolic disease who developed infantile spasms. This article presents in brief the most frequent inborn errors of metabolism that have been associated with West syndrome and also illustrates the importance of screening for inborn errors of metabolism in infantile spasms.
Topics: Humans; Infant; Infant, Newborn; Metabolism, Inborn Errors; Spasms, Infantile
PubMed: 22832779
DOI: 10.1177/0883073812448532 -
Epilepsia 2006Cognitive and behavioral impairments in West syndrome are analyzed at the onset of the disease. Multimodal sensory impairments, namely of the visual function, that are... (Review)
Review
Cognitive and behavioral impairments in West syndrome are analyzed at the onset of the disease. Multimodal sensory impairments, namely of the visual function, that are usually associated with the acute stage of the disease, are stressed as possible mechanisms interfering with cognitive development. This notion could open a rehabilitation prospective for functional disorders. Long-term developmental outcome is dependent on various factors, ranging from the primary lesions to the epilepsy evolution and use of drugs, up to environmental factors such as the quality of the family or that of the community within which the infant grows up.
Topics: Age Factors; Cognition Disorders; Comorbidity; Follow-Up Studies; Humans; Incidence; Infant; Intellectual Disability; Mental Disorders; Prognosis; Spasms, Infantile
PubMed: 17105461
DOI: 10.1111/j.1528-1167.2006.00689.x -
Srpski Arhiv Za Celokupno Lekarstvo 2001West syndrome (WS) is an age dependent epileptic syndrome caused by various brain disorders. WS has been frequently classified in two groups, cryptogenic and... (Review)
Review
West syndrome (WS) is an age dependent epileptic syndrome caused by various brain disorders. WS has been frequently classified in two groups, cryptogenic and symptomatic. As symptomatic WS consists of patients with numerous types of brain lesions, the prognosis and evolutional changes may be different among the types of brain lesions. WS is resistant to treatment to most conventional antiepileptic drugs, and only valproic acid, benzodiazepines, adrenocorticotropic hormone (ACTH), corticosteroids and vigabatrin have been found efficacious. Benzodiazepine, notably nitrazepam, and less clonazepam had been effective in bringing spasms under control but emerging tolerance and significant side effects (hypotonia and drowsiness) precluded its wider use. ACTH has been the treatment of choice for infantile spasms ever since Sorel and Dusaucy-Bouloye described its effectiveness in 1958. Despite the empirical approach steroids were capable of controlling the spasms and normalizing EEG in about 60% of cases. Corticotropin (ACTH) was used in doses from 5 to 180 I.U. daily, prednizolone 2-10 mg/kg daily, hydrocortisone 5-10 mg/kg daily and dexamethason 0.3-0.5 mg/kg. However, poor consensus was defined regarding the best steroid molecule to use, the dosage, and the duration of treatment. Also frequent and sometimes serious side effects have occurred during ACTH therapy, notably serious infections and hypertension that promoted continuous search for alternative and safer drugs tolerated outpatient treatment, good tolerance and minor side effects. Recently a specific visual field loss has been reported in treated adults that raised concern about safety of vigabatrin. New reports in children claim that vigabatrin treatment of children and adolescents has a lower risk for visual field defect than in adults, because of reported reversibility. Vigabatrin paediatric advisory group recommend the trial of vigabatrin for 12-14 days as first treatment for WS and in the case of good clinical response continuation of therapy for six months. Other new antiepileptic drugs (lamotrigine, topiramate, felbamate and zonisamide) have shown significant efficacy in the treatment of resistant WS to previous medication. The current task is to determine risk/benefit ratios of these two drugs (vigabatine, ACTH) and to delineate the group of patients with WS where their use would be optimal.
Topics: Humans; Infant; Spasms, Infantile
PubMed: 15637997
DOI: No ID Found -
Brain & Development Nov 2001To clarify the pathophysiology of tonic spasms, 21 patients with West syndrome were analyzed using ictal and interictal single photon emission computed tomography... (Review)
Review
To clarify the pathophysiology of tonic spasms, 21 patients with West syndrome were analyzed using ictal and interictal single photon emission computed tomography (SPECT). We focused on whether ictal perfusion changes were observed in the focal cortical region. Eight of the patients studied showed definite focal cortical ictal hyperperfusion, indicating that there is a unique subset of West syndrome that can be classified as infantile localization-related epilepsy. Of those eight patients, only two showed asymmetric spasms, suggesting that seizure symptomatology in infants gives only limited information on the localization-related nature of epilepsy. Furthermore, the activation of subcortical structures by focal cortical regions might be attributable to the symmetric seizure phenomena. Thirteen patients showed a diffuse pattern in their ictal SPECTs; this probably included patients with diffuse hyperperfusion and those with no changes. The following have yet to be determined: (1) whether West syndrome is divided into subgroups based on the origin of spasms, in that some patients have the origin in the cortical hemisphere and some have the origin in structures other than the cortical hemisphere, such as the brain stem; (2) whether differences in ictal SPECT patterns reflect a unique nature of tonic spasms in West syndrome, where tonic spasms appear in clusters and the interval of each spasm is different among each patient.
Topics: Brain; Humans; Infant; Spasms, Infantile; Tomography, Emission-Computed, Single-Photon
PubMed: 11701244
DOI: 10.1016/s0387-7604(01)00298-4 -
Indian Journal of Pediatrics Aug 2023To study the course of West syndrome (WS) and coronavirus disease-19 (COVID-19) in children with WS who contracted SARS-CoV-2 infection.
OBJECTIVES
To study the course of West syndrome (WS) and coronavirus disease-19 (COVID-19) in children with WS who contracted SARS-CoV-2 infection.
METHODS
This ambispective study was conducted at a tertiary-care center in North India between December 2020 and August 2021 after approval from the Institute Ethics Committee. Five children with WS, positive for COVID-19 based on RT-PCR, fulfilled the inclusion criteria.
RESULTS
One child with COVID-19 during the first wave was retrospectively included while four children (of the 70 children screened) were prospectively enrolled. The median age at onset of epileptic spasms was 7 mo (2 boys), and that at presentation with COVID-19 was 18.5 mo. Three had underlying acquired structural etiology. Three were in remission following standard therapy, while two had ongoing spasms at the time of COVID-19 illness. During the illness, two of those in remission continued to be in remission while one child had a relapse. The children with ongoing epileptic spasms had variable course [one had persistent spasms and other had transient cessation lasting 3 wk from day 2 of COVID-19 illness, but electroencephalography (on day 8 of COVID-19 illness) continued to show hypsarrhythmia]. Fever was the most typical symptom (and sometimes the only symptom) of COVID-19, with a duration ranging from 1-8 d. Two children had moderate COVID-19 illness requiring hospitalization, while the rest had a mild illness. All the affected children had complete recovery from COVID-19.
CONCLUSION
The severity of COVID-19 illness in children with WS is often mild, while the subsequent course of WS is variable.
Topics: Male; Humans; Child; Spasms, Infantile; COVID-19; Retrospective Studies; SARS-CoV-2; Spasm
PubMed: 35708881
DOI: 10.1007/s12098-022-04201-4 -
Ryoikibetsu Shokogun Shirizu 2002
Review
Topics: Cosyntropin; Diagnosis, Differential; Electroencephalography; Humans; Infant; Infant, Newborn; Prognosis; Spasms, Infantile; Valproic Acid; Vitamin B 6
PubMed: 12483836
DOI: No ID Found -
Indian Pediatrics Jul 2008To assess the seizure and developmental outcome in children with West syndrome with respect to treatment lag.
OBJECTIVES
To assess the seizure and developmental outcome in children with West syndrome with respect to treatment lag.
METHODS
Twenty-six children satisfying inclusion criteria of West syndrome i.e., infantile spasms, psychomotor retardation and abnormal EEG pattern were prospectively evaluated. Response to treatment was assessed based on seizure control, EEG, developmental assessment and parental observations.
RESULTS
The time lag from onset of seizures to appropriate treatment was <1 month in 11 children; 1-6 months in 8 children; and >6 months in 7 children. Children with treatment lag <1 month fared better than those who had a large treatment lag (P<0.05). Children with good seizure control also showed better developmental improvement (P<0.05).
CONCLUSION
Children with West syndrome have better seizure control and development, if the treatment is started within 1 month of onset of symptoms.
Topics: Adrenocorticotropic Hormone; Anticonvulsants; Brain; Child, Preschool; Cognition Disorders; Electroencephalography; Female; Humans; Infant; Male; Neuropsychological Tests; Prospective Studies; Severity of Illness Index; Spasms, Infantile; Valproic Acid
PubMed: 18695274
DOI: No ID Found -
Medicina 2018West syndrome or infantile spasms is an epileptic encephalopathy, classified as generalized epilepsies and syndromes. There are multiple reports of the evolution from...
West syndrome or infantile spasms is an epileptic encephalopathy, classified as generalized epilepsies and syndromes. There are multiple reports of the evolution from West to Lennox-Gastaut syndrome of 25 up to 60%, without a specific cause is determined. It has been reported that they may be only an epileptic entity age dependent that it would be in relation to the degree of brain immaturity. In this retrospective review of 130 cases of West syndrome, only 14 (10.7%) evolved to Lennox-Gastaut. Having received in all cases vigabatrin as a treatment, makes us suppose that the low incidence could be related to the use of this drug. Given that vigabatrin has a gabaergic action and increased levels of ACTH, may explain this relationship but this must be confirmed with the best knowledge of the intimate mechanisms of these serious epileptic encephalopathies.
Topics: Anticonvulsants; Disease Progression; Electroencephalography; Female; Humans; Infant; Lennox Gastaut Syndrome; Magnetic Resonance Imaging; Methylprednisolone; Retrospective Studies; Spasms, Infantile; Syndrome; Vigabatrin
PubMed: 30199357
DOI: No ID Found -
Brain & Development Nov 2001Thyrotropin-releasing hormone (TRH) has been successfully used for treating children with neurologic disorders including epilepsy. The effectiveness of TRH and a TRH... (Review)
Review
Thyrotropin-releasing hormone (TRH) has been successfully used for treating children with neurologic disorders including epilepsy. The effectiveness of TRH and a TRH analog has been reported in West syndrome, Lennox-Gastaut syndrome, and early infantile epileptic encephalopathy that were intractable to anticonvulsants and adrenocorticotrophic hormone (ACTH). However, the peptide has not been widely studied as a treatment of intractable epilepsy outside Japan. TRH is safe in children and effective in some cases of West syndrome and Lennox-Gastaut syndrome. TRH is considered as a possible new strategy for treating West syndrome and Lennox-Gastaut syndrome prior to ACTH therapy, especially for the patient with an infection, immunosuppression, or severe organic lesions in the brain. The mechanisms of its antiepileptic action may differ from those of other antiepileptic drugs. One possibility is that TRH may act as an antiepileptic through a kynurenine mechanism, considering that kynurenic acid acts as an antagonist on the N-methyl-D-aspartate receptor complex.
Topics: Child; Epilepsy; Humans; Infant; Kynurenine; Spasms, Infantile; Thyrotropin-Releasing Hormone
PubMed: 11701274
DOI: 10.1016/s0387-7604(01)00303-5 -
BMC Pediatrics Jul 2012Infantile spasms (IS; West syndrome) is a severe form of encephalopathy that typically affects infants younger than 2 years old. Pediatricians, pediatric neurologists,... (Review)
Review
BACKGROUND
Infantile spasms (IS; West syndrome) is a severe form of encephalopathy that typically affects infants younger than 2 years old. Pediatricians, pediatric neurologists, and other pediatric health care providers are all potentially key early contacts for families who have an infant with IS. The objective of this article is to assist pediatric health care providers in the detection of the disease and in the counseling and guidance of families who have an infant with IS.
METHODS
Treatment guidelines, consensus reports, and original research studies are reviewed to provide an update regarding the diagnosis and treatment of infants with IS. Web sites were searched for educational and supportive resource content relevant to providers and families of patients with IS.
RESULTS
Early detection of IS and pediatrician referral to a pediatric neurologist for further evaluation and initiation of treatment may improve prognosis. Family education and the establishment of a multidisciplinary continuum of care are important components of care for the majority of patients with IS. The focus of the continuum of care varies across diagnosis, initiation of treatment, and short- and long-term needs. Several on-line educational and supportive resources for families and caregivers of patients with IS were identified.
CONCLUSIONS
Given the possibility of poor developmental outcomes in IS, including the emergence of other seizure disorders and cognitive and developmental problems, early recognition, referral, and treatment of IS are important for optimal patient outcomes. Dissemination of and access to educational and supportive resources for families and caregivers across the lifespan of the child with IS is an urgent need. Pediatric health care providers are well positioned to address these needs.
Topics: Adrenocorticotropic Hormone; Anticonvulsants; Continuity of Patient Care; Directive Counseling; Humans; Infant; Infant, Newborn; Parents; Patient Education as Topic; Pediatrics; Spasms, Infantile; Vigabatrin
PubMed: 22830456
DOI: 10.1186/1471-2431-12-108