Spotlight on Autism spectrum disorder

Autism spectrum disorder is a neurodevelopmental condition characterized by impairment in social communication, repetitive behaviors, restricted interests, and hyper- or hyposensitivity to sensory stimuli. It is estimated that 1 in 68 children (1 in 42 boys and 1 in 189 girls) have autism spectrum disorder. Autism spectrum disorder is complex with no single or precise etiology. Early identification and behavioral treatment lead to better outcomes. Pharmacological treatment targets problem behaviors, such as hyperactivity, impulsivity, aggression, and perseverative or anxious behaviors.

In this article, Dr. Caroline DiBattisto of the University of South Carolina School of Medicine describes the clinical features of autism spectrum disorder and summarizes recent research regarding etiology, pathogenesis, genetics, diagnosis, and management.

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Spotlight on Basal ganglia hemorrhage

Basal ganglia intracerebral hemorrhage remains among the most deadly of stroke types. Intracerebral hemorrhage in the basal ganglia is usually caused by hypertension. Patients presenting within the first few hours have a high risk of hemorrhage expansion and neurologic deterioration. Prognosis is based on multiple factors, including volume and location of hemorrhage, age, level of consciousness, presence of intraventricular hemorrhage, and warfarin use.

This update highlights some important clinical trial results on intracerebral hemorrhage, including blood pressure management and surgical interventions. Dr. Adrian Marchidann of SUNY Downstate Medical Center discusses these advances and updates on the epidemiology and pathophysiology of intracerebral hemorrhage.

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Spotlight on Lennox-Gastaut syndrome

In this article, Dr. Mary Spiciarich and Dr. Solomon Moshe of Albert Einstein College of Medicine discuss Lennox-Gastaut syndrome, which is diagnosed in individuals with intractable epilepsy characterized by multiple seizure types, slow spike-wave pattern on EEG, and cognitive deterioration typically after first seizure onset. Seizure types include tonic seizures that mainly occur in sleep, atonic seizures, atypical absences, and myoclonic seizures. The condition may follow West syndrome but has also been associated with various genetic and neurocutaneous syndromes, metabolic diseases, and early infectious or ischemic insults or may have an unknown etiology. Numerous interventions as well as medications have been studied in treating this condition. Clobazam has been studied in controlling drop attacks and cannabidiol is being researched as an adjunctive antiepileptic agent in patients with Lennox-Gastaut syndrome and other intractable epilepsy syndromes.

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Spotlight on Neuropathic pain: treatment

Treatment of neuropathic pain is an ongoing challenge for clinicians. In this article, Dr. Yi Pan of St. Louis University and Dr. Florian Thomas of Seton Hall-Hackensack Meridian School of Medicine and Hackensack University Medical Center summarize pharmacological management based on published clinical trials. Not all medications mentioned in this article have been investigated in placebo-controlled, double-blind, randomized trials. The aim of this article is to provide a variety of updated information so clinicians can choose an optimal treatment for an individual patient.

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Spotlight on Nystagmus

Nystagmus is the term used to describe rhythmic involuntary oscillations of the eyes. This examination finding is commonly encountered in clinical practice. To the untrained eye, it may be difficult to distinguish among the types of nystagmus.

In this article, Dr. Joome Suh and Dr. Sashank Prasad of Brigham and Women’s Hospital in Boston, Massachusetts provide an overview of various forms of physiologic and pathologic nystagmus, their differentiating characteristics, and associated findings. The authors discuss the common pathologies associated with each type of nystagmus and strategies for work-up. And finally, they discuss available therapeutics for specific types of nystagmus.

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Spotlight on Pregnancy: CNS complications

Given the acuity of possible outcomes, as well as the added clinical dimension of an unborn child, the pregnant patient can prove to be a challenge for the treating neurologist. As well, the postpartum period carries with it significant and unique neurologic risks. In this article, Dr. Adrian Marchidann of SUNY Downstate Medical Center reviews the common central neurologic complications seen in pregnancy and the postpartum period. The key presenting features of eclampsia are discussed as well as ischemic and hemorrhagic cerebrovascular events.

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Spotlight on Anencephaly

Anencephaly is a severe and uniformly lethal malformation, resulting from incomplete closure of the anterior neural tube, in which fetuses or infants present with absent calvarial and cerebral structures. In this article, Dr. Joseph Siebert of the University of Washington discusses the etiology, pathogenesis, genetic contribution, and epidemiology of anencephaly as well as approaches toward the diagnosis and prevention of this and other neural tube defects.

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Spotlight on Executive dysfunction

In this article, Dr. Alfredo Ardila of Florida International University discusses “executive function,” which includes the ability to filter interference, engage in goal-directed behaviors, anticipate the consequences of one’s actions, and exhibit the adaptive concept of mental flexibility. The analysis of executive functions represents one of the most important research areas in contemporary neurosciences. It is partially related to frontal lobe activity. Executive disorders have been documented in a diversity of conditions. Executive defects early in dementia predict subsequent behavior disturbances, functional decline, and mortality. In elders, intellectual tasks and everyday activity programs may benefit executive function abilities.

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Spotlight on Inclusion-body myositis

Inclusion-body myositis is the most frequent and disabling myopathy seen in patients over 50 years of age. The distinct clinical features that lead to correct diagnosis and inclusion-body myositis mimics are highlighted in this article. Inclusion-body myositis has a complex pathogenesis in which autoimmune and inflammatory features coexist with elements of degeneration and abundant accumulations of various stressor proteins.

In this article, Dr. Marinos Dalakas of the National and Kapodistrian University of Athens Medical School in Greece and Thomas Jefferson University, Philadelphia discusses the pitfalls in diagnosis, the diagnostic markers, and the role of inflammation and degeneration in the pathogenesis of the disease, including the interaction between these processes. The latest trends in therapeutic strategies are also presented.

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Spotlight on Movement disorders associated with infection

In this article, the author reviews the wide spectrum of movement disorders associated with infectious agents. Although these complications tend to be more frequent in parts of the world plagued by endemic infections, they are also seen in association with sporadic cases of infections. With better control of HIV (human immunodeficiency virus) infection, there has been a decline of movement disorders observed in these patients. There is a comprehensive discussion of Sydenham chorea and other problems related to beta-hemolytic group A streptococcus. New data have fueled the possibility that streptococcus infection may be associated with neuropsychiatric findings outside the context of Sydenham chorea. Other infections, such as Japanese encephalitis, remain an important cause of movement disorders in some parts of the world. The pathogenesis of Parkinson disease might be related to changes in gut microbiota leading to mutation of alpha-synuclein. Finally, epidemiological data suggest there is a relationship between hepatitis C virus and increased risk of development of Parkinson disease.

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