Epilepsies associated with inborn errors of metabolism (IEM) represent a major challenge. Seizures rarely dominate the clinical presentation, which is more frequently associated with other neurological symptoms, such as hypotonia and/or cognitive disturbances. Although epilepsy in IEM can be classified in various ways according to pathogenesis, age of onset, or electroclinical presentation, the...

OBJECTIVE We aimed to determine the clinical and electroencephalographic (EEG) characteristics of the patients with West syndrome (WS) in south Iran. MATERIALS & METHODS In this retrospective study, all patients with a clinical diagnosis of WS were recruited in the outpatient epilepsy clinic at Shiraz University of Medical Sciences between September 2008 and May 2012. Age, gender, age at seiz...

In some patients without vitamin B6 deficiency, epilepsy can not be controlled without an extra supplement of vitamin B6. The therapeutic role of pyridoxal phosphate (PLP), the active form of vitamin B6, may not be replaced with other forms of vitamin B6 sometimes. Until now, four inborn errors of metabolism are known to affect vitamin B6 concentrations in the brain. Three of them are hyperprol...

To The ediTor Recently, Lin and colleagues have reported a case of pyridoxine-dependent seizures in this journal 1. The diagnosis was based upon the clinical criteria as formulated by Baxter 2. In discussing the case the authors state that the underlying pathophysiology of the disorder is unknown and that no biochemical aids are available in establishing the diagnosis. Indeed, for 50 years pyri...

![Graphic][1]#### Notable in Neurology This Week issue features an article that investigates the clinical outcomes of postendovascular thrombectomy patients with acute stroke cancer; another determines cognitive lysine reduction therapies for pyridoxine-dependent epilepsy. A featured Review explores origins amyotrophic lateral sclerosis as either distal or cortical. ### Cognitive-Driven A...

A relation between pyridoxine and convulsive phenomena has been established over several years (Snyderman, Holt, Carretero, and Jacobs, 1953; Molony and Parmelee, 1954; Adams, Bessey, Bussey, and Hansen, 1954; May, 1954; Coursin, 1954, 1955). This led to a search for evidence of pyridoxine deficiency among children with cryptogenic epilepsy. Soon patients were discovered who, though receiving a...