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Titolo:
CSF glutamate/GABA concentrations in pyridoxine-dependent seizures: etiology of pyridoxine-dependent seizures and the mechanisms of pyridoxine actionin seizure control
Autore:
Goto, T; Matsuo, N; Takahashi, T;
Indirizzi:
Keio Univ, Sch Med, Dept Pediat, Shinjuku Ku, Tokyo 1608582, Japan Keio Univ Tokyo Japan 1608582 Pediat, Shinjuku Ku, Tokyo 1608582, Japan
Titolo Testata:
BRAIN & DEVELOPMENT
fascicolo: 1, volume: 23, anno: 2001,
pagine: 24 - 29
SICI:
0387-7604(200103)23:1<24:CGCIPS>2.0.ZU;2-J
Fonte:
ISI
Lingua:
ENG
Soggetto:
GAMMA-AMINOBUTYRIC-ACID; ION-EXCHANGE CHROMATOGRAPHY; CEREBROSPINAL-FLUID; LIQUID-CHROMATOGRAPHY; REFERENCE VALUES; GYRATE ATROPHY; MICE LACKING; CLEFT-PALATE; AMINO-ACIDS; GABA LEVELS;
Keywords:
pyridoxine-dependent seizures; glutamate decarboxylase; glutamate; gamma-aminobutyric acid; pyridoxine; vitamin B-6;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
39
Recensione:
Indirizzi per estratti:
Indirizzo: Takahashi, T Keio Univ, Sch Med, Dept Pediat, Shinjuku Ku, Shinanomachi 35, Tokyo 1608582, Japan Keio Univ Shinanomachi 35 Tokyo Japan 1608582 1608582, Japan
Citazione:
T. Goto et al., "CSF glutamate/GABA concentrations in pyridoxine-dependent seizures: etiology of pyridoxine-dependent seizures and the mechanisms of pyridoxine actionin seizure control", BRAIN DEVEL, 23(1), 2001, pp. 24-29

Abstract

Several lines of evidence suggest that the binding affinity of glutamate decarboxylase (GAD) to the active form of pyridoxine is low in cases of pyridoxine-dependent seizures (PDS) and that a quantitative imbalance between excitatory (i.e. glutamate) and inhibitory (i.e. gamma -aminobutyric acid, GABA) neurotransmitters could cause refractory seizures. However, inconsistent findings with GAD insufficiency have been reported in PDS. We report a case of PDS that is not accompanied by an elevated cerebrospinal fluid (CSF)glutamate concentration. Intravenous pyridoxine phosphate terminated generalized seizures which were otherwise refractory to conventional anti-epileptic medicines. No seizure occurred once oral pyridoxine (13.5 mg/kg per day) was started in combination with phenobarbital sodium (PB, 3.7 mg/kg per day). The electroencephalogram (EEG) normalized approximately 8 months afterpyridoxine was started. The patient is gradually acquiring developmental milestones during the 15 months follow-up period. The CSF glutamate and GABAconcentrations were determined on three separate occasions: (1) during status epilepticus; (2) during a seizure-free period with administration of pyridoxine and PB; and (3) 6 days after suspension of pyridoxine and PB and immediately before a convulsion. The CSF glutamate level was below the sensitivity of detection (<1.0 <mu>M) on each of the three occasions; the CSF GABA level was within the normal range or moderately elevated. The CSF and serum concentrations of vitamin B-6-related substances, before pyridoxine supplementation, were within the normal range. We suggest that (1) PDS is not a discrete disease of single etiology in that insufficient activation of GAD may not account for seizure susceptibility in all cases and (2) mechanism(s) of anti-convulsive effect of pyridoxine, at least in some cases, may beindependent of GAD activation. (C) 2001 Elsevier Science B.V. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/03/20 alle ore 01:33:51