Category Archives: Content Spotlight

Spotlight on Clinically isolated syndrome

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A clinically isolated syndrome is a first symptomatic episode of central nervous system dysfunction due to inflammatory demyelination. Risk factors for conversion to clinically definite multiple sclerosis have been identified, and treatment of high-risk individuals may delay subsequent relapses. Individuals with a clinically isolated syndrome may demonstrate accelerated brain atrophy and mild cognitive impairments. Revisions to the diagnostic criteria for relapsing-remitting multiple sclerosis are associated with a reduction in time to diagnosis of clinically definite multiple sclerosis in some clinically isolated syndrome patients.

In this article, Dr. Elizabeth Hartman of CNOS in Dakota Dunes, South Dakota summarizes the diagnosis, evaluation, and prognostic implications of clinically isolated syndromes.

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Spotlight on Benign adult familial myoclonic epilepsy

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In this article, Dr. Pasquale Striano of the University of Genova, Istituto Gaslini discusses benign adult familial myoclonic epilepsy, which is an inherited epileptic syndrome characterized by cortical hand tremors, myoclonic jerks, and rare convulsive seizures. In most affected individuals, the disease takes a benign course; however, at advanced age, worsening of the tremor and myoclonus is common, and slight intellectual disability is present in a subset of patients. Prevalence is unknown but is estimated to be less than 1 out of 35,000. It is transmitted autosomal dominantly, and penetrance is high.

This is a well-delineated disease with remarkable features that clearly distinguish it from other forms of myoclonic epilepsies. Genetic studies of the families show heterogeneity, and different susceptible chromosomal loci have been identified. Diagnosis is based on clinical and electrophysiological findings. It must be differentiated from epilepsy syndromes with prominent myoclonus features. Valproate, levetiracetam, and benzodiazepines are the most beneficial treatments.

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Spotlight on Headache attributed to head trauma

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Headache is common following head injury. Most individuals recover within days, weeks, or months, but a minority of subjects may suffer from intractable posttraumatic headache despite treatment. Posttraumatic headache remains a subject of controversy concerning its pathophysiology. Litigation and medicolegal problems further complicate this issue.

In this article, Dr. Jr-Wei Wu of Taipei Veterans General Hospital attempts to summarize current understanding of posttraumatic headache, including a brief historical review of the disease, the classification and diagnostic criteria, the epidemiology, clinical manifestations, pathophysiology, diagnostic evaluation, and management.

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Spotlight on Blepharospasm

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Blepharospasm is a form of focal dystonia manifested by involuntary eye closure due to excessive contractions of the eyelids. In addition, contractions of orbicularis oculi (pretarsal, preseptal, and periorbital portions) adjacent muscles, including procerus and corrugator, as well as paranasal and other facial muscles may be involved. Blepharospasm is often misdiagnosed as “dry eyes” or “nervousness.”

In this article, Dr. Joseph Jankovic of Baylor College of Medicine reviews the clinical features, pathogenesis, and treatment of blepharospasm, focusing on the use of botulinum toxin.

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Spotlight on Chronic fatigue syndrome

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Chronic fatigue syndrome, also known as myalgic encephalomyelitis or chronic fatigue immune dysfunction syndrome, is a debilitating illness diagnosed by means of exclusion. The illness is often controversial; the proposed etiology, preferred method of treatment, and even the nomenclature varies widely from source to source. The symptoms are also diverse and fluctuating, making uniform studies and research difficult. Even diagnosing chronic fatigue syndrome is a challenge as reliable diagnostic tests and biological markers remain elusive.

Little is certain about this illness, but in this article, Dr. William Conte of The University of Chicago summarizes most of the current and widely accepted information about the subject.

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Spotlight on Bipolar disorder

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Bipolar disorder, or manic depression, is a serious mental illness that may drastically affect an individual’s ability to function because of mood instability. Given the unpredictability of severe high (mania) and severe low (depression) mood states, people with bipolar disorder may struggle with social and workplace settings. Bipolar disorder affects mood, sleep, concentration, and reasoning. Some individuals may experience psychosis or may struggle with suicidal thoughts. Although the underlying cause of bipolar disorder is not clear, research in neurobiology and genetics provides some insight into this complex illness. Treatment of the disorder is primarily with mood stabilizers but often requires additional adjunctive medications. Additionally, psychosocial support and therapy are essential for maintaining stability.

In this article, Dr. John Hammond and Dr. David Geldmacher of the University of Alabama at Birmingham aim to provide distinction between bipolar disorder subtypes and includes updates on the current research regarding the underlying pathology as well as guidance on the pharmacologic treatment of the illness.

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Spotlight on Angelman syndrome

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Angelman syndrome is a neurodevelopmental disorder characterized by intellectual disability, epilepsy, ataxia, and a unique behavioral phenotype. Diagnosing Angelman syndrome has important implications for prognosis, genetic counseling, health surveillance, and, in some instances, specific symptomatic therapies.

In this article, Dr. Ryan Lee of the John A Burns School of Medicine at the University of Hawaii discusses the diagnosis, prognosis, genetic counseling, and health surveillance of patients with Angelman syndrome. Also discussed are studies using models of Angelman syndrome, which provide insight into the pathoetiology and potential treatment of Angelman syndrome.

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Spotlight on Sleep, stroke, and vascular dementia

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In this article, Dr. Antonio Culebras of SUNY Upstate Medical University at Syracuse highlights the importance of obstructive sleep apnea as a risk factor for stroke and vascular dementia. Rehabilitation and recovery of stroke are less successful in the presence of sleep apnea. Habitual short and long sleep durations, long-standing night shift work, and periodic leg movements of sleep negatively affect cerebrovascular morbidity and mortality. Vascular dementia may be a complication of uncontrolled sleep apnea with hypoxemia.

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Spotlight on Hemorrhagic transformation of ischemic stroke

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Hemorrhagic transformation after ischemic stroke is an often underdiagnosed phenomenon. With increasing and widespread use of tPA and with improved imaging capabilities afforded by newer sequences on MRI, it’s now possible to predict which patients might be at increased risk of clinically significant hemorrhagic transformation.

In this article, Dr. Jorge Moncayo-Gaete of the International University of Ecuador and Dr. Julien Bogousslavsky of the Genolier Swiss Medical Network have added information about the risk of hemorrhagic transformation with tPA, the use of minocycline to decrease risk of hemorrhagic transformation, and the predictive value of hemorrhagic transformation in long-term prognosis.

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Spotlight on Normal-pressure hydrocephalus

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Normal-pressure hydrocephalus is characterized by enlarged ventricles and tightened parasagittal cortical sulci with characteristic clinical presentation of gait disorder, cognitive decline, and urinary incontinence. Shunt surgery is the only established effective treatment.

In this updated article, Dr. Peter Hedera of Vanderbilt University and Dr. Robert Friedland of the University of Louisville School of Medicine provide an overview of clinical features suggesting normal-pressure hydrocephalus as well as diagnostic approaches to establishing this diagnosis and selecting patients who have the highest likelihood of improvement after shunting. Although normal-pressure hydrocephalus is relatively rare, prompt recognition and surgical treatment of this condition can significantly reverse clinical features and improve the long-term outcome of these patients.

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