Neonatal seizures frequently accompany neonatal encephalopathies. Seizures occur in approximately 1.8-5/1,000 live births in this country and are caused by virtually any condition that affects neonatal brain function. This review provides a simple classification of seizures and emphasizes that many abnormal intermittent behaviors in this age group are not accompanied by ictal EEG patterns. Addi...

Vitamin B6 is closely associated with functions of the nervous, immune, and endocrine systems. Its deficiency may result in neurological disorders including convulsions and epileptic encephalopathy. Until today, this has only been reported in infants, children, and critically ill adult patients. We report a case of a 36year-old man with chronic alcoholism who presented with seizures after gastr...

Isoniazid is an effective antituberculosis drug. Isoniazid poisoning produces a characteristic clinical syndrome that occurs 30 to 120 minutes after ingestion and includes seizures, metabolic acidosis, and in severe cases, coma. Rhabdomyolysis is one of the reported complications of isoniazid poisoning, but relevant data are limited. Parenteral pyridoxine is the antidote of isoniazid. In this c...

OPINION STATEMENT Pyridoxine-dependent epilepsy (PDE) is a rare autosomal recessive disorder and is considered as a prototypical form of metabolic epilepsy. Characterized by recurrent seizures in the prenatal, neonatal, and/or postnatal periods that are resistant to conventional anti-epileptic drugs, PDE is responsive to pharmacological dosages of pyridoxine. Presently, however, there are no cl...

OBJECTIVE To assess the efficacy of various medications in the prevention of recurrent febrile seizures. STUDY DESIGN A meta-analysis of all published randomized, placebo-controlled trials of the preventive treatment of febrile seizures published in English; 45 articles were found, but only 9 trials were randomized and placebo-controlled--4 using phenobarbital; 3, diazepam; 1, pyridoxine; and...

Pyridoxine-dependent epilepsy (PDE) was first described in 1954. The ALDH7A1 gene mutations resulting in α-aminoadipic semialdehyde dehydrogenase deficiency as a cause of PDE was identified only in 2005. Neonatal epileptic encephalopathy is the presenting feature in >50% of patients with classic PDE. We report the case of a 13-month-old girl with profound neonatal hypoglycemia (0.6 mmol/L; refe...

BACKGROUND Pyridoxine dependent epilepsy (PDE) is characterized by neonatal epileptic encepahalopathy responsive to pharmacological doses of vitamin B6. Recently an autosomal recessive deficiency in Antiquitin (ALDH7A1), a gene involved in the catabolism of lysine has been identified as the underlying cause. CASE REPORT In 21 and 23 year-old sisters, who had presented with neonatal / early in...

AIM The long-term outcome of the Dutch pyridoxine-dependent epilepsy cohort and correlations between patient characteristics and follow-up data were retrospectively studied. METHOD Fourteen patients recruited from a national reference laboratory were included (four males, 10 females, from 11 families; median age at assessment 6y; range 2y 6mo-16y). The following data were retrieved: sex; age ...