Outcome of Hemispherectomy for Refractory Epilepsy

The clinical features, presurgical workup, and postoperative outcome of 39 patients with medically intractable epilepsy who underwent hemispherectomy from 1996 to 2005 are reported from University of Sao Paulo, Brazil.

COMMENT. The Johns Hopkins extensive series of hemispherectomies, 111 cases from 1975-2001, demonstrates a beneficial response in 86% (seizures controlled in 65% and occasional in 21%). The outcome was particularly favorable in children with Rasmussen syndrome and vascular disorders. Seizures due to migrational disorders were less responsive. (Kossof EH. Neurology 2003;61:887-890).
In the Sao Paulo study, seizures were associated with cortical maldevelopment in 10 patients; seizure control was 100% in 7 and 75% in 2. In 12 with Rasmussen syndrome, seizure control was 95-100% in 11 and 75% in 1. Contrary to the Hopkins data, outcome was not significantly correlated with seizure etiology.

LAMOTRIGINE AND SUDDEN UNEXPECTED DEATH IN EPILEPSY
Four consecutive cases of sudden unexpected death in epilepsy (SUDEP), occurring in non-hospitalized patients while treated with lamotrigine (LTG), between 1995 and 2005, are reported from Stavanger University Hospital, Norway. All were female gender, the epilepsy was idiopathic, age range was 16 to 37 years, and LTG was monotherapy. Seizures Pediatric Neurology Briefs 2007 were generalized tonic clonic, the EEG showed bilateral synchronous epileptiform activity, pulmonary edema was found at autopsy, and a terminal seizure was suspected but not witnessed. Low post-mortem levels of LTG indicated probable non-compliance in 2 patients. Four possible explanations for the SUDEP are proposed: 1) a fatal seizure resulting from LTG non-compliance; 2) LTG-induced ventricular cardiac tachyarrhythmia (torsade de pointes); 3) a combination of LTG effects and seizure; and 4) coincidental unrelated to LTG. (Aurlien D, Tauboll F, Gjerstad L. Lamotrigine in idiopathic epilepsy increased risk of cardiac death? Acta Neurol Scand March 2007;115:199-203

VALPROIC ACID-INDUCED HAIR CURLING
A 47-year-old white female patient treated with valproic acid (VPA) 500mg 3xd, at Utrecht University, the Netherlands, developed curling of the hair which gradually resolved over 5 years continuous therapy, with blood levels ranging from 97 to 146 mg/L. Thinning of the hair with spots of alopecia preceded the curling effect and persisted after curling resolved. She had dyed her hair long before the curling appeared, but she had never had a permanent wave hair styling. This report of a transient perming effect of VPA is considered unique. (Wilting I, van  COMMENT. VPA is known to cause alopecia, thinning of the hair, and hair color changes. Changes in hair texture are also reported, including curliness in 2% of 250 patients (Jeavons PM et al. Lancet 1977;1:359), but rarely transient.

ATTENTION DEFICIT DISORDERS LOW BIRTH WEIGHT AND ATTENTION DEFICIT DISORDER
The relation of low birth weight to the risk of attention deficit hyperactivity disorder was determined in a population-based sample of 1,480 twin pairs ascertained from the Swedish twin registry in the period 1985-1986. Eighteen twin pairs at 8-9 years of age and 10 twin pairs at 13-14 years were discordant for both birth weight and ADHD criteria. The child with ADHD was smaller in 11 of 18 pairs at 8-9 years and in 9 of 10 pairs in adolescence (P=0.011). The lighter twin had on average 13% higher ADHD symptom score at age 8-9 years (P=0.006) and 12% higher ADHD score at age 13-14 years (P=0.018) compared with Pediatric Neurology Briefs 2007