Reviews & Analysis

This article describes the case of a 54-year-old man who experienced recurrent episodes of transient amnesia over a number of years. The patient was diagnosed with transient epileptic amnesia, and his case provides the first radiological evidence that this syndrome can result from seizure activity in the hippocampus.

This Case Study describes in detail a case of progranulin-associated frontotemporal lobar degeneration (FTLD). The patient was initially asymptomatic, but developed progressive brain atrophy and deficits on neuropsychological testing before the clinical onset of cognitive impairment. The authors use this case as a springboard to discuss advances in the genetics of FTLD with an emphasis on the importance of serial cognitive testing and neuroimaging.

In spite of recent advances in surgical and medical therapies, the prognosis for patients diagnosed with the high-grade glioma glioblastoma multiforme remains poor. In this Review, Das et al. present evidence that the resistance of these tumors to current therapies is attributable to a small subpopulation of cancer stem cells, and they consider potential new therapeutic strategies that specifically target this cell population.

Desynchronization of circadian rhythms is a common occurrence in individuals presenting with sleep disorders. In this Review, Pandi-Perumal et al. highlight the roles of light and endogenous melatonin in the synchronization of sleep–wake rhythms and rest–activity cycles with the light–dark cycle, and they discuss how light therapy and exogenous melatonin might be used to treat circadian rhythm sleep disorders.

Against the background of the debate about the best models of stroke unit care, Lenzi and Di Piero present their argument that patients with stroke have better outcomes if their stay in a dedicated stroke unit is extended, enabling prolonged and intensive monitoring for stroke complications.

This Viewpoint contributes to the topical debate on the therapeutic benefits of deep brain stimulation in Parkinson's disease (PD). The authors argue that there is more to this treatment approach than just providing symptomatic benefits, and suggest that it may actually become the first therapy proven to slow the progression of PD.

Regulatory T (T_REG) cells are emerging as key players in the pathogenetic scenario of CNS autoimmune inflammation. In this article, Zozulya and Wiendl discuss how T_REG-cell dysfunction might influence the disease course in multiple sclerosis and experimental autoimmune encephalomyelitis, highlighting the importance of interactions between T_REGcells and dendritic cells. The authors also assess the potential for therapeutic use of these cell types in patients with multiple sclerosis.

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.