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Neuropsychological assessment can identify deficits in brain function that assist in the diagnosis of patients with dementing illness. Fields et al. highlight the unique properties of neuropsychological measurements, including their role as biomarkers of dementia, in differentiating diseases with similar topographies, and in predicting the risk of dementia. Prediction of functional decline on the basis of these measurements will assist in planning of the future care requirements for patients at risk of dementia.
An improved understanding of the cellular events that lead to motor neuron injury in amyotrophic lateral sclerosis (ALS) could highlight promising new therapeutic strategies. Pamela Shaw and colleagues provide a comprehensive overview of the numerous molecular mechanisms that are involved in ALS, including oxidative stress, mitochondrial dysfunction and excitotoxicity. They discuss features specific to motor neurons that might render this cell type vulnerable to damage, and highlight important links between cellular events and clinical features of the disease.
Most patients who present with longitudinal extensive transverse myelitis (LETM) are diagnosed with neuromyelitis optica (NMO). Trebst et al. use case studies of patients without NMO who presented with spinal lesions to show the variety of different etiologies that can underlie LETM. The authors highlight the diagnostic indicators and difficulties encountered in making differential diagnoses in these patients.
Combination therapy is a rational strategy to optimize therapeutic benefits and minimize risks associated with treatment of patients with multiple sclerosis (MS). A recent study reporting negative results of combination therapy in MS should not discourage investigators from future attempts to identify optimal combinations for the treatment of this disease.
Clazosentan—a drug previously demonstrated to reduce angiographic vasospasm in patients with aneurysmal subarachnoid hemorrhage—had no significant effect on vasospasm-related morbidity and mortality in the CONSCIOUS-2 trial. A multifactorial cause for delayed ischemic neurological deficits might partly account for these negative findings.
A recent clinical trial showed no beneficial effects of statin treatment in patients with Alzheimer disease (AD) and normal cholesterol levels. Other studies show that the effects of statins can vary depending on cholesterol levels and stage of disease, so statins should not be ruled out as an AD therapy.
Rituximab is an immunosuppressive monoclonal antibody therapy that is used in some cases to help prevent relapses of neuromyelitis optica, an inflammatory CNS demyelinating disease. A new study provides evidence supporting efficacy and a strategy for optimizing the timing of repeated courses of treatment for the individual patient.
A recent study has identified mutations in the ubiquilin 2 gene in patients with dominant X-linked amyotrophic lateral sclerosis (ALS) and ALS–dementia. The results suggest that ubiquilin 2 inclusions and impairment of the protein degradation pathway contribute to the pathogenesis of ALS and possibly other neurodegenerative disorders.
Until the early 1990s, the prevailing view was that amyotrophic lateral sclerosis (ALS) was rarely familial, but subsequent genetic discoveries have overturned this idea. Andersen and Al-Chalabi document the rapidly changing genetic landscape in ALS, highlighting the lack of a clear distinction between heritable and apparently sporadic ALS, and providing recommendations for genetic counseling.