Infantile spasms.

Appleton, Richard

doi:10.1136/adc.69.5.614

Cited by 32 publications

(30 citation statements)

References 40 publications

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“…Agents that have been employed in the treatment of infantile spasms include benzodiazepams (especially nitrazepam), sodium valproate, vigabatrin (VGB), corticosteroids, ACTH, a ketogenic diet, vitamin B6, intravenous gammaglobulin, a benzodiazepam-carbamazepine cocktail, topiramate and zonisamide [2,5]. …”

Section: Introductionmentioning

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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Section: Introductionmentioning

confidence: 99%

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

et al. 2010

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“…This study did not use the Walker criteria and so it is difficult to tell how definite the diagnosis of mitochondrial disease was in these patients. Overall, metabolic disorders are considered to be an uncommon cause of infantile spasms (Appleton, 1993; Aicardi, 1994) and have been reported in only 2.6% (Lombroso, 1983; Nolte et al. , 1988; Ohtahara et al.…”

Section: Discussionmentioning

confidence: 99%

Spasms in children with definite and probable mitochondrial disease*

Sadleir

Connolly²,

Applegarth

et al. 2004

Euro J of Neurology

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The diagnosis of mitochondrial encephalomyopathies is complex and a system for classification of the diagnosis as definite, probable, and possible has been proposed. The objective of this study was to explore the spectrum of epileptic disorders associated with probable and definite mitochondrial disease in children using this classification system. The patient population with mitochondrial disease and epilepsy was selected from a tertiary care children's hospital. Interictal electroencephalograms and video-EEG recordings were used to characterize seizure types. Ten children fulfilled the criteria for probable or definite mitochondrial disease and had epilepsy. Four had siblings with a similar clinical phenotype. Spasms were the most common seizure type and were the initial seizure type in seven patients and two siblings. Four patients had only partial seizures, with or without generalization, and one patient had seizures that were difficult to classify. Blood lactate concentrations were elevated consistently in patients with partial seizures alone but were occasionally normal in children with spasms. Spasms were the most common presenting seizure type in children with probable and definite mitochondrial disease.

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“…West syndrome presents most commonly at 4-9 months of age 26. The defining seizure type is a brief epileptic spasm, which may be flexor ('salaam attack'), extensor or flexor-extensor and may involve the whole body, limbs, or only the head and eyes.…”

Section: Treatmentmentioning

confidence: 99%

“…A specific underlying cause (e.g. tuberous sclerosis, or a history of perinatal hypoxic/ischaemic encephalopathy) is identified in about 75-80% of affected children 26 , 27. The prognosis (largely determined by the underlying cause) is poor.…”

Section: Treatmentmentioning

confidence: 99%

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Managing childhood epilepsy

2001

DTB

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An epileptic seizure is a paroxysmal, stereotyped disturbance of consciousness, motor function, sensation, perception, behaviour or emotion (occurring singly or in any combination), resulting from a cortical neuronal discharge. Epilepsy is a condition in which such seizures recur, usually spontaneously. Estimates suggest that 0.7-0.8% of school-aged children (corresponding to around 61, 000 in England, Wales and Scotland) have active epilepsy.1 Here, we review the diagnosis, investigation and treatment of children with common forms of epilepsy.

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Infantile spasms.

Cited by 32 publications

References 40 publications

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

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

Spasms in children with definite and probable mitochondrial disease*

Managing childhood epilepsy

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