RNA Interference Therapy and Huntington Disease
Harper Article provides insight into how reducing mutant huntingtin expression may offer a treatment for Huntington disease. Therapy with RNA interference (RNAi) has emerged as a powerful method to silence dominant disease genes. He indicates that RNAi may be a prospective therapy for Huntington disease, and discusses current progress and the challenges that remain.
Emerging Viral Infections of the Central Nervous System: Part 1
Tyler Article describes in a 2-part review the important emerging viral infections of the central nervous system. Mechanisms of emergence are reviewed including viruses spreading into new host ranges, as exemplified by West Nile virus, Japanese encephalitis virus, Toscana virus, and enterovirus 71.
A New Therapy for Human Neuropathy, Ataxia, and Retinitis Pigmentosa Mitochondrial Mutation Metabolism
Sgarbi and colleagues Article report that adenosine triphosphate synthase–deficient cells can be rescued by increasing mitochondrial substrate-level phosphorylation and suggest dietary or pharmacological therapeutic approaches based on the supplementation of α-ketoglutarate/aspartate to patients with impaired adenosine triphosphate synthase activity. Editorial perspective is provided by Ronald G. Haller, MD, and John Vissing, MD.Article
Breastfeeding and the Risk of Postpartum Relapses in Multiple Sclerosis
Langer-Gould et al Article find that exclusive breastfeeding and concomitant suppression of menses significantly reduce the risk of postpartum relapses in multiple sclerosis.
Neuromyelitis Optica IgG in Fulminant Inflammatory Demyelinating Disorders
Magaña and colleagues Article indicate that neuromyelitis optica IgG is a specific biomarker for neuromyelitis optica spectrum disorders and is not simply a marker of destructive central nervous system inflammatory demyelinating disease.
Pediatric Patients Have Higher Disease Burden and Activity on Brain Magnetic Resonance Images at the Time of Multiple Sclerosis Onset Than Adults
Waubant et al Article report that children with multiple sclerosis more frequently had T2-bright foci in the posterior fossa and enhancing lesions at onset than adults.
Genome-Wide Scan in Responders and Nonresponders to Interferon Beta in Multiple Sclerosis
Comabella and colleagues Article report that the allelic variants in this genome-wide association study support the polygenic nature that underlies the response to interferon beta treatment in multiple sclerosis and highlights the importance of the glutamatergic system in the response to interferon-beta.
Women With Epilepsy: Do Seizures During Gestation Affect Pregnancy Outcomes?
Chen et al Article find that preventing seizures during pregnancy is an important step in reducing the risk of adverse pregnancy outcomes including low-birth-weight infants, preterm delivery, and infants who are small for gestational age.
Periodic Epileptiform Discharges: Prognosis
San-juan Orta and colleagues Article indicate that in patients with periodic lateralized epileptiform discharges, the absence of clinical seizures at the time of detection and presumed acute etiology are associated with death, whereas a nonepileptic etiology was associated with good clinical outcome.
Seizures in Alzheimer Disease
Scarmeas et al Article show that unprovoked seizures are uncommon in Alzheimer disease but occur more frequently than in the general population. Younger age is a risk factor for seizures in Alzheimer disease.
Damage to the Optic Radiation in Multiple Sclerosis
Reich and colleagues Article report that fractional anisotropy and perpendicular diffusivity are potentially useful quantitative imaging biomarkers of optic radiation–specific damage in multiple sclerosis. Such damage is associated with retinal injury and visual disability.
Saillour et al Article confirm the homogeneity of the profile of patients with LIS1-related lissencephaly who demonstrate a large proportion of Dobyns lissencephaly grade 3a and the absence of correlation with LISI mutations.