Pyridoxine‐dependency seizure: Report of a rare presentation

Krishnamoorthy, K. S.

doi:10.1002/ana.410130123

Cited by 28 publications

(13 citation statements)

References 10 publications

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“…Epileptic myoclonus and partial seizures have been reported in other series,2 8 9 19 23 more often than spasms 78 Four patients in this series had clear spasms, and this pattern may be quite common, although rarely reported because of lack of consensus in the terminology of neonatal seizures.…”

Section: Discussionmentioning

confidence: 52%

Pyridoxine dependent epilepsy: a suggestive electroclinical pattern

Nabbout

Soufflet

Plouin

et al. 1999

Archives of Disease in Childhood - Fetal and Neonatal Edition

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Aims-To determine if there is an electroencephalographic pattern suggestive of pyridoxine dependent epilepsy that could be used to improve the chances of early diagnosis. Methods-A retrospective study was made of all the clinical records and electroencephalograms of neonates identified with pyridoxine dependent seizures between 1983 and 1994, at this hospital. Neonates whose seizures began after more than 28 days of life were excluded; in all, five patients from four families were studied. Follow up ranged from 2 to 10 years. Results-A history of miscarriage and neonatal death during an epileptic seizure had occurred in the siblings of two families. One mother reported rhythmic movements of her child during the last month of pregnancy. At birth, all babies were hypotonic; four had decreased visual alertness. All babies were agitated, irritable, jittery, hyperalert, and exhibited sleeplessness and a startle reaction to touch and sound. Age of onset of seizures varied from 30 minutes to 3 days. Seizures of various types were recorded in all cases on EEG tracings, including spasms, myoclonic seizures, partial clonic, and secondary generalised seizures. Burstsuppression patterns occurred in three cases, and a combination of continuous and discontinuous patterns in two others. Bilateral high voltage delta slow wave activity was observed in four patients. Psychomotor delay was severe in three patients, moderate in one, and mild in one. Conclusions-There is an identifiable EEG pattern that is highly suggestive of pyridoxine dependent epilepsy. Pyridoxine dependent epilepsy is probably underdiagnosed. (Arch Dis Child Fetal Neonatal Ed 1999;81:F125-F129)

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Section: Discussionmentioning

confidence: 52%

Pyridoxine dependent epilepsy: a suggestive electroclinical pattern

Nabbout

Soufflet

Plouin

et al. 1999

Archives of Disease in Childhood - Fetal and Neonatal Edition

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“…Atypical cases have been reported with late onset of seizures and seizure-free intervals of up to several months duration without pyridoxine supplementation [2,4,14,18,24,32,44]. A few patients required unusually high doses of pyridoxine to control the seizures [7,9,10].…”

Section: Introductionmentioning

confidence: 97%

Pyridoxine-dependent seizures, clinical and therapeutic aspects

1991

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Pyridoxine-dependency is a rare autosomal recessive disorder causing a severe seizure disorder of prenatal or neonatal onset, psychomotor retardation and death in untreated patients. Treatment requires life-long supplementation with pyridoxine (vitamin B6). The underlying defect is unknown, and there is no biological marker for the disease. Clinical diagnosis is often delayed and severe neurological sequelae are common. This article summarizes both clinical and therapeutic aspects.

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“…Several reports of unusual cases have been published: the initial episode of seizures being controlled by conventional anticonvulsants,3-5 the seizures developing relatively late in the newborn period,3,5,6 a long seizurefree period being observed without pyridoxine after diagnosis,3,5 untoward effects being noticed at initial pyridoxine administration,3,~-9 and a high dose of pyridoxine being required to control seizures. 11,12 In this article we report a Japanese girl with pyridoxine dependency who showed atypical clinical features and characteristic findings on magnetic resonance imaging (MRI) scans.…”

mentioning

confidence: 97%

Pyridoxine-Dependent Seizures: Report of a Case With Atypical Clinical Features and Abnormal MRI Scans

et al. 1992

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A Japanese girl with atypical pyridoxine-dependent seizures is reported. Until 9 months of age the seizures had been controlled by conventional anticonvulsants. The initial administration of pyridoxine was followed by a collapse; the suppression-burst pattern changed to an almost flat pattern in the EEG. T1- and T2-weighted magnetic resonance imaging (MRI) scans showed poor differentiation between white and gray matter, and T2-weighted MRI scans showed periventricular hyperintensity areas adjacent to the posterior horns of lateral ventricles. The findings in this patient indicate that pyridoxine should be given to infants with intractable epilepsy, regardless of the response to anticonvulsants, and that resuscitation facilities should be available during such a trial.

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Pyridoxine‐dependency seizure: Report of a rare presentation

Cited by 28 publications

References 10 publications

Pyridoxine dependent epilepsy: a suggestive electroclinical pattern

Pyridoxine dependent epilepsy: a suggestive electroclinical pattern

Pyridoxine-dependent seizures, clinical and therapeutic aspects

Pyridoxine-Dependent Seizures: Report of a Case With Atypical Clinical Features and Abnormal MRI Scans

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