News & Views in 2011

Despite the emergence of genetic and molecular approaches, disease categorization on the basis of clinical features remains an appealing approach, especially where the molecular mechanisms are poorly understood. A new study supports distinct clinical subtypes of Parkinson disease that could have implications for understanding the etiology of this condition.

One of the main challenges associated with late-onset genetic neurodegenerative diseases is predicting when the condition begins and how it progresses over time. In a new study, Tabrizi et al. have used a range of imaging, clinical and neuropsychiatric measures to assess the progression of Huntington disease.

Statin use in patients with cerebral hemorrhage may not be without risk. A recent article analyzing existing statin data in a mathematical model suggests that statins might need to be avoided after intracerebral hemorrhage, particularly in patients with a lobar hemorrhage.

Quality improvement in medicine has largely focused on conditions managed in primary care rather than specialist care. The American Academy of Neurology has introduced process-focused quality metrics for neurologists managing patients with Parkinson disease with the goal of informing evaluation of clinical performance. These metrics might eventually inform pay-for-performance decisions.

Acute exacerbations of myasthenia gravis (MG) need effective and urgent treatment because of life-threatening hypoventilation. Plasma exchange and intravenous immunoglobulin (IVIg) are both effective for acute MG. A new study suggests that these treatments produce similar patient outcomes and complications, with IVIg perhaps being superior from an economic perspective.

Focal cortical dysplasia (FCD) is a commonly encountered neuropathology in drug-resistant focal epilepsy; the bizarre pathological appearance of FCD reflects molecular disorder bridging neurodevelopment and neurodegeneration. A new classification stands to resolve discrepancies in FCD categorization and accelerate advances in fundamental understanding and clinical management of drug-resistant epilepsy.

Vigabatrin is an efficacious new-generation antiepileptic drug, but visual field loss (VFL) has strongly limited its use. A systematic review has established that vigabatrin increases the risk of VFL fourfold, with age and exposure as predicting factors. For infantile spasms, the benefits of vigabatrin seem to outweigh the VFL risk.

Patients with transient ischemic attack (TIA) at high short-term risk of stroke can be identified. Evidence-based treatments can decrease this risk; however, organization of care of TIA patients is suboptimal. The National Stroke Association provides recommendations for improving TIA-related care delivery, but symptom recognition and treatment response remain a challenge.

The occurrence of subarachnoid hemorrhage (SAH) in patients with acute headache is strongly associated with the presence of several clinical variables. Nevertheless, the absence of such variables should not discourage physicians from investigating for SAH in individuals with headache peaking in intensity within a few minutes of onset.

Decisions regarding resumption of anticoagulation are difficult in patients who present with a warfarin-associated intracranial hemorrhage and also have atrial fibrillation or a prosthetic heart valve. Current guidelines suggest waiting 1–2 weeks on average, but new research suggests that the optimal time to restart anticoagulation could be much later.

Observational studies suggest that high homocysteine levels are a risk factor for brain atrophy and Alzheimer disease, but intervention studies have been inconclusive. New evidence that B vitamin therapy can slow brain atrophy will allow re-examination of elevated homocysteine levels as a potentially treatable risk factor for this disease.

Mutations in six genes have been unequivocally linked to Parkinson disease (PD). A recent study found that among 953 patients with the early-onset form of PD, ≈17% harbored a mutation in one of these genes. This finding raises important issues concerning genetic testing and genetic counseling for early-onset PD.

The development of disease-modifying therapies for amyotrophic lateral sclerosis (ALS) is a priority for researchers in this field. Two studies have independently identified plasma and cerebrospinal fluid biomarkers for ALS, and these biomarkers could provide a means of testing the efficacy of novel ALS drug treatments in future clinical trials.