Pyridoxine-dependent and pyridoxine-responsive seizures

[Pyridoxine dependent seizures].

Pyridoxine dependent seizures is a rare autosomal recessive disorder. Its manifestations are intractable epilepsy leading to death in status epilepticus. Treatment with pyridoxine prevents the seizures and normalizes the EEG. Early diagnosis is important for the intellectual outcome. In Denmark, the disease has occurred in a child of healthy Tamil immigrants, who are first cousins. The child's ...

Epidemiology of pyridoxine dependent and pyridoxine responsive seizures in the UK.

OBJECTIVE To study the epidemiology of pyridoxine dependent seizures and other forms of pyridoxine responsive seizures. DESIGN Monthly notifications to the British Paediatric Surveillance Unit over two years. Questionnaire follow up. SETTING UK and the Republic of Ireland. PATIENTS Children aged 15 years or younger whose seizures respond to pyridoxine. INTERVENTIONS None. MAIN OUTCOME...

Neglected Atypical Pyridoxine Dependent Seizures

Omid Yaghini, MD; Mohammad-Amir Shahkarami*, MD; Somayeh Shamsaii, MD Pediatrics Department, Isfahan University of Medical Sciences, Isfahan, IR Iran Received: Nov 02, 2009; Accepted: Mar 13, 2010 Vitamin B6 (pyridoxine) dependent seizure (PDS) is an autosomal-recessively inherited disorder which starts within a few hours of birth or even earlier, and can cause intrauterine seizures[1,2]. It oc...

Pyridoxine responsive epilepsy: expanded pyridoxine dependency?

Pyridoxine dependent seizures--a wider clinical spectrum.

We report 4 infants with pyridoxine dependent seizures who had clinical features that led to diagnostic uncertainty. Their clinical course was unusual in 1 or more of the following: 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. This report illust...