Reviews & Analysis

Incidentally identified demyelinating pathology within the CNS has been extensively documented in neuropathological studies, and is increasingly being reported during MRI-based premortem investigations. In this article, Okuda reviews data on unanticipated demyelinating pathology from both autopsy and imaging studies, and considers the clinical implications of these findings, including the recently introduced concept of the radiologically isolated syndrome.

A survey conducted in Canada examined the prevalence of perceived genetic discrimination against patients with Huntington disease. The respondents reported discrimination not only by insurance or mortgage companies, but also in family and social contexts. Discrimination was more frequently attributed to family history than to genetic test results.

The management of acute migraine in the emergency department presents a therapeutic challenge for physicians, who need to provide adequate pain relief while minimizing adverse events and relapses. New research suggests that phenothiazines are effective drug therapies for acute migraine, highlighting their potential use as first-line treatments for acute migraine in the hospital setting.

Clinical trials aimed at preventing hematoma expansion in patients with intraparenchymal hemorrhage have failed to show benefit from experimental intervention. Novel methods for identifying those patients at the highest risk of hemorrhage growth might enable better patient selection and, hence, increase the chance of demonstrating an improvement in clinical outcome.

A genome-wide association study has identified the R92Q variant of the TNFRSF1A gene as a new susceptibility locus for multiple sclerosis. This locus is of special interest because the R92Q substitution was previously detected in a group of multiple sclerosis patients who had additional symptoms compatible with the autoinflammatory syndrome TRAPS.

Nonmotor symptoms are assuming an increasingly important place in the constellation of clinical traits that characterize Parkinson disease. A multicenter survey of patients with Parkinson disease highlights the prevalence of these symptoms. The findings indicate that further research into the pathophysiology and treatment of nonmotor symptoms is warranted.

Complete brachial plexus injury leads to paralysis and loss of sensation in the affected arm and hand. Carlstedt et al. present a long-term follow-up assessment of a 9-year-old boy who underwent spinal cord surgery after sustaining such an injury in a motorcycle accident. The patient showed recovery of hand motor function without restoration of sensation.

Early detection of a poor treatment response to disease-modifying agents could be of great benefit to patients with relapsing–remitting multiple sclerosis. Such individuals could be switched to a potentially more effective treatment before too much neurological damage has occurred. In this Review, Río and colleagues examine the use of clinical measures, MRI, and pharmacogenomics in assessing and predicting the treatment response in patients with this condition.

Thrombolytic therapy is often used to treat arterial ischemic stroke in children, despite lack of FDA approval in this age group. The International Pediatric Stroke Study investigators have assessed the extent and nature of recombinant tissue plasminogen activator use in children in their registry, and have compared their findings with previously published case reports.

The ABCD system uses clinical features identifiable in primary or emergency care to reliably predict the risk of stroke in the acute phase following a transient ischemic attack. Further study is required to establish both the effectiveness of a refined system that includes carotid and brain imaging, and how such a refined system should be implemented in clinical practice.

Volunteer work in developing countries can be extremely rewarding for neurologists, from both a professional and a personal perspective. Sidney Rosenberg has worked extensively as a volunteer neurologist in hospitals in India, Cambodia and Botswana, and in this article he provides a personal account of his experiences.

Estimates of the incidence of cognitive dysfunction following ischemic stroke vary considerably in the medical literature. New research suggests that cognitive dysfunction might occur more frequently than was previously thought, emphasizing the importance of assessing such dysfunction beyond the standard deficits measured on most stroke severity scales.

Chinese investigators performed a well-designed, randomized trial to investigate the efficacy of true Chinese acupuncture compared with two different sham acupuncture approaches for the treatment of acute migraine attacks. Real acupuncture was superior to sham acupuncture, but the absolute pain relief was minimal.

Frontotemporal lobar degeneration often presents with distinct language impairments that are associated with different distributions of cortical atrophy. Two studies have now demonstrated that these clinical syndromes are associated with highly characteristic patterns of cortical thinning. Specific cortical atrophy profiles do not, however, seem to reliably predict histologically distinct frontotemporal lobar degeneration variants.

Chik et al. describe the case of a woman who developed a severe 'thunderclap' headache, followed by generalized tonic–clonic seizures, shortly after giving birth. A head CT scan revealed a subarachnoid hemorrhage, and an angiogram showed areas of vasoconstriction in the cerebral arteries that were consistent with a diagnosis of postpartum cerebral angiopathy.

The incidence of Parkinson disease (PD) increases with age, so age-related conditions are expected to feature prominently among the comorbidities associated with this disease. In this article, Nanhoe-Mahabier et al. explore a possible link between PD and cerebrovascular disease, discussing the pathophysiological mechanisms that could underlie such a relationship, and considering how comorbid cerebrovascular disease might affect the clinical presentation of PD.

Chronic kidney disease is a serious global health problem, and almost all patients with severe forms of the disease develop neurological complications, which can include cognitive impairment, cerebrovascular disease and peripheral neuropathies. Krishnan and Kiernan review the clinical features and pathophysiology of the most common neurological complications of chronic kidney disease, and highlight the currently available treatment options.

Clinical overlap between the various types of neurodegenerative parkinsonism, particularly in the early stages of disease, can make differential diagnosis problematic. Biological fluid biomarker assays are being explored as adjunctive tests to aid the diagnostic work-up of patients with neurodegenerative parkinsonism, and in this article Eller and Williams cast a critical eye over the currently available cerebrospinal fluid, plasma and urine biomarkers.

Thrombolytic therapy is an effective treatment for ischemic stroke, but only when delivered within a short period after the onset of the condition. Delay in patients being admitted to hospital is one of the main reasons why the therapeutic window is often missed. This Review examines the factors that affect prehospital delay, and describes strategies designed to overcome the problem.

Sudden unexpected death in epilepsy most frequently occurs in people with chronic epilepsy, and seems to be a seizure-related event. In this article, Surges et al. review the incidence of sudden unexpected death in epilepsy and the risk factors associated with this condition, before exploring the pathological mechanisms related to chronic epilepsy that could lead to sudden death.