West syndrome: a university hospital based study from Oman

Koul, Roshan; Chacko, Alexander; Cherian, Elsa

doi:10.1016/s0387-7604(01)00286-8

Cited by 11 publications

(13 citation statements)

References 21 publications

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“…In other studies also this has been a signiﬁcant clinical abnormality, it has been suggested that it denotes the involvement of brain parenchyma even before the onset of infantile spasm (21). Visual impairment has been found in approximately 25% of cases (21) which is closed to our ﬁnding.…”

Section: Discussionmentioning

confidence: 90%

Clinical Profile of Infants with Hypsarrhythmia

Khreisat

2011

Acta Inform Med

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Objective:The present study was done in order to obtain a baseline profile of infantile spasms and associated neurological disorders.Patient and methods:The study included 50 patients with infantile spasm in Queen Rania Hospital for children in Jordan. The following data were obtained: sex, age at onset of spasms, details of seizure, family history of epilepsy, significant pre-/peri/ post-natal insults, Electroencephalography and detailed neuro imaging evaluation , detailed neurological, neuro developmental ,assessment were done by. Broad categories of possible etiologies were used the results were recorded for further study.Results:Age of onset of infantile spasms ranged from 1month to 1 year and 6 months , (mean 4.8 months). The mean time of presentation was 9.4 months . A male preponderance was noted (74 %). flexor spasms (52%) was the commonest . Other types of seizures also accompanied infantile spasm in 44% children . (84%) were born of normal delivery, History of birth asphyxia was obtained in 48%, 3 (6%) had positive family history Developmental delay was recognized prior to onset of spasms in 52%, microcephaly was the commonest associated problem, Imaging studies of the brain revealed abnormality in 18 patients. 78% patients were classified as symptomatic and 22 % as cryptogenic.Conclusion:the pattern of infantile spasm in our country do not differ from that of developed countries, further researches is required to prevent both chronic epilepsy and psychomotor retardation and .preventive measurement to prevent birth asphyxia is recommended.

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Section: Discussionmentioning

confidence: 90%

Clinical Profile of Infants with Hypsarrhythmia

Khreisat

2011

Acta Inform Med

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“…A prospective study reports that most children with epileptic syndromes on vigabatrin cannot complain of their eye problems, hence 3-6 monthly ophthalmic follow up is strongly advised, along with regular electroretinography, electro-oculography, and visual evoked potentials if possible [33]. A study from Oman reports one death in a patient on vigabatrin therapy due to aspiration pneumonia [34]. Currently, the benefits of treating infantile spasms with vigabatrin monotherapy seem to outweigh the risks, but further prospective studies and follow-up of children receiving treatment are needed to evaluate the place of vigabatrin in this indication [35].…”

Section: Discussionmentioning

confidence: 99%

Clinical profile and treatment of infantile spasms using vigabatrin and ACTH - a developing country perspective

et al. 2010

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BackgroundInfantile spasms represent a serious epileptic syndrome that occurs in the early infantile age. ACTH and Vigabatrin are actively investigated drugs in its treatment. This study describes the comparison of their efficacy in a large series of patients with infantile spasms from Pakistan.MethodsAll patients with infantile spasms who presented to Aga Khan University Hospital, Karachi, Pakistan from January, 2006 to April, 2008 were included in this study. Inclusion criteria were clinical symptoms of infantile spasms, hypsarrythmia or modified hyparrythmia on electroencephalography, at least six months of follow-up period and receipt of any of the two drugs mentioned above. The type of drug distribution was random according to the availability, cost and ease of administration.ResultsFifty six cases fulfilled the inclusion criteria. 62.5% were males. Mean age at onset of seizures was 5 ± 1.4 months. Fifty two (92.8%) patients demonstrated hypsarrythmia on electroencephalography. 64.3% cases were identified as symptomatic while 19.6% were cryptogenic and 16.1% were idiopathic. Eighteen patients received ACTH while 38 patients received Vigabatrin as first line therapy. Initial response to first line therapy was similar (50% for ACTH and 55.3% for Vigabatrin). Overall, the symptomatic and idiopathic groups responded better to Vigabatrin. The relapse rate was higher for ACTH as compared to Vigabatrin (55.5% vs. 33.3%) when considering the first line therapy. Four patients evolved to Lennox-Gastaut variant; all of these patients had initially received Vigabatrin and then ACTH.ConclusionVigabatrin and ACTH showed no significant difference in the initial treatment of infantile spasms. However, patients receiving ACTH were 1.2 times more likely to relapse as compared to the patients receiving Vigabatrin when considering monotherapy. We suggest that Vigabatrin should be the initial drug of choice in patients presenting with infantile spasms. However, larger studies from developing countries are required to validate the therapeutic trends observed in this study.

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“…Psychomotor delay is seen often in these children, related to the underlying brain insult. [1] Symptomatic infantile spasms is the most common type. Brain malformations, are often associated with symptomatic infantile spasms.…”

Section: Infantile Spasms Associated With Lissencephaly Pachygyria Inmentioning

confidence: 99%

“…Sir, Biotinidase deficiency is a disorder of multiple carboxylase defi ciency with an autosomal recessive mode of inheritance [1] and was fi rst described by Wolf and colleagues in 1983. [2] The reported incidence in various neonatal screening programs worldwide is 1:60,000 live births.…”

Section: Biotin Responsive Seizures and Encephalopathy Due To Biotinimentioning

confidence: 99%