Infantile Spasms

Bobele, Gary B.

doi:10.1016/s0733-8619(18)30339-6

Cited by 21 publications

(9 citation statements)

References 36 publications

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“…Intramuscular corticotropin is generally considered the preferred mode of treatment, although oral prednisone has also been advocated.8 As additional backup drugs, valproic acid and clonazepam are frequently used.8 Overall outcome of infantile spasms has been poor despite treatment because a substantial number of cases are symptomatic epilepsies associated with conditions that in themselves have unfavorable outcomes, such as Down syndrome, untreated phenylketonuria, and congenital brain malformations. 6,8,9 However, an idiopathic subgroup exists that is felt to have a more favorable outcome if the seizure activity comes under good control.…”

mentioning

confidence: 99%

The Use of Felbamate to Treat Infantile Spasms

Hurst

Rolan

1995

J Child Neurol

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Based on the initial successful use of felbamate for infantile spasms in an infant with tuberous sclerosis, three additional infants with infantile spasms of different etiologies who had failed conventional therapies were treated with felbamate. Three of the four patients have shown complete resolution of infantile spasms. All responding patients did so within 1 week of starting felbamate. The one treatment failure had an initial reduction of seizure frequency and severity but has not maintained that response long term. Controlled studies are needed to firmly establish that felbamate is both safe and effective for the treatment of infantile spasms. As these cases document, felbamate is currently available for use in infantile spasms, and the frequent conversion of infantile spasms to Lennox-Gastaut syndrome, for which felbamate is approved, makes its use in infantile spasms logical.

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confidence: 99%

The Use of Felbamate to Treat Infantile Spasms

Hurst

Rolan

1995

J Child Neurol

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“…In the latter group, the patients have no discernible brain lesion and development is normal prior to seizure onset. The symptomatic group generally demonstrates evidence of neurodevelopmental abnormalities prior to onset of spasms and includes diverse etiologies such as tuberous sclerosis (~20%), congenital infections, cerebral malformations, hypoxic-ischemic encephalopathy, cerebral hemorrhage, and metabolic disorders such as phenyhetonuria [28,29,32]. The role of pertussis immunization is unclear, and since largely replaced by an acellular vaccine, though most authorities feel immunization is rarely, if ever, the cause of infantile spasms [32,33].…”

Section: From Sheridan and Jacobs [9]mentioning

confidence: 99%

“…In the treatment of neonatal seizures, two treatable metabolic disorders need to be considered, pyridoxine-dependent seizures and biotinidase deficiency. The diagnosis is established if seizures are controlled during administration of 100mg of pyridoxine during EEG monitoring [28]. Ongoing pyridoxine treatment may result in normal development.…”

Section: From Sheridan and Jacobs [9]mentioning

confidence: 99%

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Overview of Childhood Epilepsy and Epileptic Syndromes and Advances in Therapy

Morton¹,

Pellock²

2000

CPD

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Seizures have a variety of etiologies and manifestations. Descriptions of various epiletic seizures as well as electroencephalographic findings have led to a unifying international classification of epileptic seizures and epilepsy syndromes. The development of this classification system and the emergence of several new antiepiletic drugs have led to progress in the refractory pediatric patient particularly disorders which are traditionally difficult to treat such as infantile spasms and the Lennox-Gastaut Syndrome. However, there is limited data regarding optimal use in children. The childhood epilepsy syndromes are reviewed as well as the newer antiepileptic drug treatments - felbamate, gabapentin, lamotrigine, levetiracetam, oxcarbazepine, tiagabine, topiramate, and zonisamide. Efficacy data and toxicity are discussed from both the adult, and when available, pediatric data.

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“…Physical deformities and clinical signs of the lesion may be found (5,6). IS may be related to different genetic diseases of autosomal recessive mode of inheritance (7)(8)(9)(10) or of autosomal dominant nature (1). It has also been associated with diverse translocations (11)(12)(13) to X chromosomelinked disorders (14)(15)(16) and to metabolic alterations that have fixed genetic patterns of inheritance (1).…”

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confidence: 99%

Contribution of the MHC Class II Antigens to the Etiology of Infantile Spasm in Mexican Mestizos

Suastegui¹,

Rosa²,

Carranza³

et al. 2001

Epilepsia

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Summary Purpose: Infantile spasms (ISs) are age‐dependant epileptic seizures, which may be flexor, extensor, lightning or nods, or mixed. The aim of this study was the analysis of genetic factors within the human leukocyte antigen (HLA) complex associated with ISs. Methods: Sixty‐five patients diagnosed according to the established international criteria were compared with 229 healthy individuals; all of them were Mexican Mestizos. Five families were also analyzed (seven affected and five healthy sibs); HLA class I and class II antigens were typed using the standard microlymphocytotoxicity methods. Results: The findings showed female gender preference (2:1). Two thirds were symptomatic, and prevalent seizures were of mixed type (67%). A strong association with HLA‐DR17 was detected in the IS group (pc <0.01; OR = 3.6; EF = 0.20). DR17 was also found increased in the symptomatic patients (p = 0.009; OR = 3.16) and in those with other types of seizures (p = 0.001; OR = 2.0). Conversely, HLA‐DQ6 was significantly decreased (pc <0.002; PF = 0.37) in the total and in the symptomatic groups (p <0.01). Haplotype linkage was not confirmed in the families; however, those with more than one affected sib shared at least one haplotype. Conclusions: These findings suggest the contribution of DR locus to the susceptibility and the participation of DQ region in the resistance to IS. Severity seems also to be influenced by HLA‐DR17, and therefore class II typing may be a helpful tool for disease prognosis.

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Infantile Spasms

Cited by 21 publications

References 36 publications

The Use of Felbamate to Treat Infantile Spasms

The Use of Felbamate to Treat Infantile Spasms

Overview of Childhood Epilepsy and Epileptic Syndromes and Advances in Therapy

Contribution of the MHC Class II Antigens to the Etiology of Infantile Spasm in Mexican Mestizos

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