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A large, retrospective analysis of patients with amyotrophic lateral sclerosis (ALS) and progressive muscular atrophy (PMA) indicates that a substantial proportion of PMA patients develop upper motor neuron signs indicative of an ALS diagnosis. The findings suggest that PMA should be considered as part of the ALS spectrum rather than as a distinct entity.
Substantial progress has been made over the past four decades in treating the symptoms of Parkinson disease, but treatments that slow its inexorable progression have remained tantalizingly out of reach. New research raises hopes that a means might have been found to safely and effectively slow progression of Parkinson disease and delay its clinical emergence.
Ganglioglioma is a rare form of low-grade glioma that mainly affects children and young adults. The low incidence of this tumor has precluded prospective studies of available treatment strategies. A large retrospective study has now examined the effectiveness of these strategies, specifically the influence of postoperative radiotherapy on patient outcome.
The first practice parameters published on amyotrophic lateral sclerosis (ALS) by the American Academy of Neurology (AAN) enhanced the care of people with this condition. These guidelines have now been updated on the basis of new evidence and will continue to provide the impetus for new research and better care; however, harmonization of the AAN and European guidelines is desirable.
Microglia rapidly adopt an activated phenotype in response to brain injury and disease, which suggests that they could act as diagnostic markers of neurodegenerative disease onset or progression. In this article, Perry et al. review the relationship between activated microglia and neurodegenerative diseases and discuss the interactions between microglial phenotype, systemic inflammation and neurodegenerative disease progression.
Aphasia is a disorder caused by damage to the brain regions that support the ability to comprehend and produce speech, and is a common occurrence after stroke. At present, clinicians cannot reliably predict the extent to which a patient with stroke is likely to recover from aphasia. In this article, Priceet al. introduce PLORAS (Predicting Language Outcome and Recovery After Stroke), a new data-led system that is designed to predict language outcome on the basis of lesion site.
TAR DNA-binding protein 43 (TDP-43) inclusions are the main histopathological feature of amyotrophic lateral sclerosis (ALS) and many cases of frontotemporal lobar degeneration (FTLD). In this article, Chen-Plotkinet al. examine the TDP-43 pathology found in these and other neurodegenerative diseases. They also argue for ALS and FTLD with TDP-43 inclusions to be considered as two ends of a continuum of disease that is characterized by TDP-43-mediated neurodegeneration.
Blindness in adults is frequently caused by acquired optic neuropathies, which can have vascular, inflammatory, toxic, nutritional, compressive, infiltrative or traumatic etiologies. Diagnosis of these neuropathies depends on accurate and precise examination of optic nerve head morphology. O' Neill et al. describe the morphological features of the optic nerve head in the various acquired optic neuropathies and explore the roles of new imaging modalities in the assessment of these disease entities.
