Reviews & Analysis

Bulbar symptoms such as impaired swallowing and speech impairment are frequent features of amyotrophic lateral sclerosis and can markedly reduce life expectancy and quality of life. In this article, Kühnlein et al. highlight the importance of early detection, as well as appropriate treatment and management, of bulbar symptoms in patients with amyotrophic lateral sclerosis, emphasizing the benefits of a multidisciplinary approach.

Central retinal artery occlusion (CRAO) is an acute stroke of the eye that can result in profound visual impairment. Standard noninvasive therapies do not markedly alter the natural history of CRAO, and this has prompted investigations into new treatment strategies. As Chen and Lee discuss in this Review, thrombolytic therapy—especially local intra-arterial fibrinolysis—is showing considerable promise for the acute management of acute CRAO.

The future of the Wada test as a predictor of verbal memory function following epilepsy surgery has been debated for some time. Memory mapping based on functional MRI is emerging as a potential alternative. Here, Andres Kanner examines where functional MRI currently stands in this setting.

This Case Study describes a 54-year-old man who developed anarthria and quadriplegia in the context of a brainstem stroke. Normal performance on neuropsychological measures administered nonverbally and failure to activate auditory cortices on functional MRI led to a diagnosis of locked-in-syndrome complicated by central deafness.

Long-term immunosuppression is often necessary in chronic autoimmune conditions such as myasthenia gravis. In this article, Sivakumar Sathasivam examines the mechanisms of action of steroids and other immunosuppressants, reviews randomized and nonrandomized evidence for their efficacy in generalized myasthenia gravis, and provides recommendations regarding the use of each immunosuppressant in the treatment of this condition.

This Case Study describes a 70-year-old immunocompetent male with rapidly-progressing dementia. Creutzfeldt–Jakob disease was suspected, but a diagnosis of enterovirus encephalitis was confirmed by brain biopsy. The authors discuss the diagnostic challenges presented by this case, and briefly review enteroviral infections of the brain.

This Case Study describes a 64-year-old woman who presented with a 2-year history of itch on her right dorsal forearm. The patient was diagnosed with brachioradial pruritus caused by cervical disc herniation, and the symptoms resolved after surgery to decompress the sixth cervical nerve root. Various alternative treatment options for brachioradial pruritus are discussed.

Itch is a common symptom of inflammatory skin disorders, but it can also occur as a result of damage to the itch-mediating nervous system, in which case it is known as neuropathic itch. In this Review, Binder et al. describe the mechanisms underlying itch generation, introduce a classification system for itch, and propose a mechanism-based management approach to chronic neuropathic itch. The topic of neuropathic itch is explored further in an accompanying Case Study in this issue.

For the practicing physician, the recognition of pain in patients with dementia can represent a challenge because these patients are often unable to verbally report their pain. Can facial expressions of pain be used as an alternative pain indicator in this population? Elliott Ross considers this question by examining relevant neurobiological research.

Signal detection theory was developed during the Second World War as a means to improve the ability of radar operators to spot enemy bombers. In this article, Oliver et al. explain how an overworked neurologist might use signal detection theory in the clinic to sift out important information from the often bewildering array of visual, verbal and auditory cues that constitute a patient's history and examination.

Sleep disturbances, such as insomnia, rapid eye movement sleep disorders and excessive daytime sleepiness, are symptoms that are commonly associated with movement disorders. This article discusses the prevalence and underlying mechanisms of sleep disturbances in patients with Parkinson's disease or atypical parkinsonism, and proposes therapeutic approaches that could benefit both the patient and their sleeping partner.

Currently, idiopathic Parkinson's disease can only be diagnosed definitively by the demonstration of intracellular Lewy body inclusions on histological specimens of brain tissue—an approach that is impractical in living individuals. In this Review, David Brooks discusses recent advances in noninvasive imaging approaches that are providing important insights into the structural, pathophysiological and pharmacological changes associated with Parkinson's disease, and could have major implications for the future diagnosis and management of this condition.

Immunotherapy seems to alter the natural course of multiple sclerosis (MS), so there is a general view that treatment should be initiated as soon as a diagnosis is made. In this Viewpoint, Giancarlo Comi makes a case for extending the early treatment approach to patients with a first neurological event suggestive of MS.

Multiple sclerosis (MS) is a complex disease with a high degree of clinical and neuropathological variability between patients. In this Viewpoint, Aksel Siva argues that such a heterogeneous disease demands a heterogeneous approach to treatment and questions the practice of initiating long-term immunotherapy before the MS diagnosis has become definite.

This article describes the case of a female patient who presented with transient right-sided weakness, and went on to develop further transient neurological signs and symptoms. A diagnosis of amyloid-β-related angiitis was made at autopsy following a sudden, fatal intracerebral hemorrhage. The authors highlight the importance of considering this diagnosis in patients with difficult-to-localize transient neurological symptoms.

Neuromyelitis optica (NMO) is a rare neuroinflammatory disorder that predominantly affects the optic nerves and spinal cord. NMO was originally considered to be a subtype of multiple sclerosis, but the recent discovery of NMO-specific serum autoantibodies has caused it to be reclassified as a distinct disease. In this article, Jariuset al. review clinical, serological and pathological findings in NMO, and discuss the evidence supporting an antibody-mediated mechanism in its pathogenesis.

This Case Study describes a 63-year-old woman with a history of metastatic breast cancer who presented with rapidly progressing myelopathic symptoms and an elevated titer for the neuromyelitis optic antibody. The appearance of myelopathy correlated with recurrence of the patient's breast cancer on two separate occasions, and the authors suggest this case represents a possible paraneoplastic syndrome associated with the neuromyelitis antibody.

Mandatory generic substitution of antiepileptic drugs is an increasingly common practice that has many physicians and patients concerned because of the potential for breakthrough seizures and adverse effects. Do the same concerns apply to the treatment of newly diagnosed epilepsy? Peter Wolf investigates.

Although there have been substantial innovations in antiepileptic drug therapy over the past 15 years, the proportion of patients with medically refractory epilepsy has remained largely unchanged, highlighting the need for new therapeutic strategies. In this article, Stacey and Litt review recent progress in the development of implantable devices designed to detect, predict and extinguish seizures, and consider the challenges that lie ahead, including improvements in seizure prediction and personalized control.

Over the past century, the pathophysiology of ischemia in the borderzones between the large cerebral arteries has been the topic of considerable debate. Two seemingly mutually exclusive mechanisms—hemodynamic failure and microembolism—have been proposed to explain this phenomenon. As Förster et al. discuss in this article, a new model of borderzone infarction, incorporating both hypoperfusion and microembolism, is now emerging.