Pyridoxine dependent seizures--a wider clinical spectrum.

Bankier, Agnes; Turner, M; Hopkins, I. J.

doi:10.1136/adc.58.6.415

Cited by 106 publications

(63 citation statements)

References 15 publications

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“…Our patient did not have seizures for a few months after its discontinuation. Recently some atypical cases of pyridoxine-dependent seizures have been reported (Bankier et al 1983, Goutières and Aicardi 1985, Haenggeli et al 1991, Coker 1992, Baxter et al 1996. Those atypical cases have unusual findings: later onset of initial seizures; a seizure-free period after taking of anticonvulsants, but before taking of pyridoxine; a long remission after withdrawal of pyridoxine; and atypical seizure type.…”

Section: Discussionmentioning

confidence: 99%

“…The diagnosis should be made as early as possible to prevent subsequent severe encephalopathy. However, the diagnosis may not be easy as a wider spectrum of pyridoxine-dependent seizures has recently been suggested (Bankier et al 1983, Goutières and Aicardi 1985, Haenggeli et al 1991, Coker 1992, Baxter et al 1996. We report on a patient with pyridoxine-dependent seizures who has presented with some distinctive clinical features during the last 31 years of observation.…”

Section: *Correspondence To First Author At Department Of Childmentioning

confidence: 97%

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Long-term follow-up of an individual with vitamin B6-dependent seizures

Ohtsuka¹,

Hattori²,

Ishida³

et al. 1999

Dev Med Child Neurol

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Section: Discussionmentioning

confidence: 99%

Section: *Correspondence To First Author At Department Of Childmentioning

confidence: 97%

Long-term follow-up of an individual with vitamin B6-dependent seizures

Ohtsuka¹,

Hattori²,

Ishida³

et al. 1999

Dev Med Child Neurol

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“…Seizures recurred 10 days after. Long seizure-free periods with or without antiepileptic drugs and without pyridoxine have been observed in pyridoxine dependency (Bankier et al 1983, Goutières and Aicardi 1985, Gospe et al1994, which could be considered as an explanation for the seizure-free period in this patient from 30 to 80 days of age. However, the association between the introduction of the vitamin complex containing pyridoxine, at about 30 days of age, the disappearance of seizures, and their recurrence 10 days after its withdrawal, became clear retrospectively.…”

Section: Discussionmentioning

confidence: 99%

“…Affected infants develop an encephalopathy with prominent seizures, usually beginning during the first days of life, although later onset of seizures has been reported (Bankier et al 1983, Goutières and Aicardi 1985, Coker 1992, Bass et al 1996. Seizures are controlled with pharmacological doses of pyridoxine.…”

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confidence: 99%

Pyridoxine‐dependent seizures responding to extremely low‐dose pyridoxine

Grillo

Silva

Barbato

2001

Develop Med Child Neuro

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We report on a male infant with pyridoxine dependency and seizures from birth, controlled with pharmacological doses of pyridoxine at 4 months of age. Seizures stopped between 30 and 80 days of age when very‐low doses of pyridoxine were given in a multivitamin supplement. Daily dose was 0.5 mg that corresponded to 0.08 to 0.16 mg/kg/day when weight gain is considered. In previous reports doses have ranged from 0.2 to 30 mg/kg/day. Another distinctive feature was that this infant went into a coma and developed hypotonia and irregular breathing when pyridoxine was given by enteral tube which has usually been reported when the vitamin is given intravenously. Use of low doses of pyridoxine in multivitamin supplements could be a confounding factor for early diagnosis and appropriate treatment of pyridoxine‐dependent seizures.

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“…Another possible mechanism of TLE involves mutations leading to a highly localized failure of γ-aminobutyric acid (GABA)-mediated inhibition. There is compelling evidence to suggest that GABA neuron dysfunction plays a causal role in seizures and status epilepticus since baby food deficient in GABAsynthesizing enzyme cofactor pyridoxine caused seizures which can be terminated by pyridoxine replacement, compounds that interfered with GABA synthesis produced status epilepticus in normal animals [10], and status epilepticus initiated by excitatory chemoconvulsants and electrical stimulation decreased dentate granule cell inhibition and overcame paired pulse inhibition just before epileptiform activity emerged [9,11].…”

Section: Introductionmentioning

confidence: 99%

Effects of Propofol on Epileptiform Activity and Hippocampal Morphology in Febrile Convulsions and Pilocarpine Induced Seizures

Bican¹

2012

J Clinic Experiment Pathol

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Epidemiological and recent prospective analyses of long Febrile Seizures (FS) support the idea that such seizures can provoke Temporal Lobe Epilepsy (TLE) in some children. Because of the high prevalence of these seizures, if epilepsy was to arise as their direct consequence, this would constitute a significant clinical problem. Animal studies have revealed that exposure of hippocampal neurons to FS early in life, particularly prolonged or frequently repetitive FS, or together with brain malformation, may lead to sustained dysfunction of these cells including long-term memory impairment or epileptogenesis, in spite of the absence of neuronal damage. We established a hyperthermia model of febrile convulsions in young adult rats, and studied the effects of propofol treatment general anesthetic acting via GABA-A receptor on epileptiform activity and the morphological features of medial temporal lobe.We found statistically significant neuronal losses in the CA1, CA3 and dentate gyrus regions of hyperthermiaapplied rats as compared to the control rats. We also observed that propofol administration suppressed epileptic discharges in EEG and prevented clinical seizures behaviors. It took a median of 11 (range, 6-40) minutes for propofol to stop seizures. We conclude that prevention of epilepsy-related damage can be achieved via epileptogenesis-based clinical approaches, and that propofol is an effective agent in the hyperthermia-induced status epilepticus model.

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Pyridoxine dependent seizures--a wider clinical spectrum.

Cited by 106 publications

References 15 publications

Long-term follow-up of an individual with vitamin B6-dependent seizures

Long-term follow-up of an individual with vitamin B6-dependent seizures

Pyridoxine‐dependent seizures responding to extremely low‐dose pyridoxine

Effects of Propofol on Epileptiform Activity and Hippocampal Morphology in Febrile Convulsions and Pilocarpine Induced Seizures

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