Spotlight on Irregular sleep-wake rhythm disorder

Irregular sleep-wake rhythm disorder (ISWRD) is characterized by the absence of circadian synchronization with an individual’s sleep-wake cycle. Irregular sleep-wake rhythm disorder is typically seen in children with neurodevelopmental disorders and patients with neurodegenerative disorders, such as Alzheimer dementia, or institutionalized elderly patients; however, it has also been observed in patients with traumatic brain injury. Increased exposure to circadian zeitgebers, such as bright light and structured activities, may minimize the disturbance. Pharmacologic therapy with melatonin was generally ineffective in randomized control trials despite encouraging results in small open-label studies.

In this article, Dr. Lynn Kataria of the Washington DC Veterans Affairs Medical Center and Dr. Bradley Vaughn of UNC Hospital Chapel Hill and University of North Carolina School of Medicine aim to provide a basic understanding of the circadian clock biology and its interactions with the sleep-wake cycle.

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Spotlight on Neurosurgical management of chronic pain

Chronic pain encompasses a spectrum of clinical scenarios involving both peripheral and central pain syndromes. In this article, Dr. Richard Polin of George Washington University discusses the progression of understanding about the pathophysiology of pain, culminating in the gait theory hypothesis.He addresses pain from peripheral nerve injury, spinal cord injury, and brain injury and stroke and discusses the basic science behind the rationale for current management schemes and pain treatments (both pharmacological and surgical).

Also discussed is cervical spinal cord stimulation and the success (or lack thereof) of surgical treatment modalities for chronic pain.

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Spotlight on Retinal migraine

In this article, Dr. Brian Grosberg and Dr. Seymour Solomon, both of Albert Einstein College of Medicine and Montefiore Headache Center, discuss clinical manifestations, biological basis, and prognosis of retinal migraine.

Retinal migraine is usually characterized by attacks of fully reversible monocular visual loss associated with migraine headache. Retinal migraine is most common in women of childbearing age who have a history of migraine with aura. In the typical attack, monocular visual features consist of partial or complete visual loss lasting less than 1 hour. Although the current diagnostic criteria for retinal migraine require fully reversible visual loss, our findings suggest that irreversible visual loss is part of the retinal migraine spectrum. Nearly half of reported cases with recurrent transient monocular visual loss subsequently experienced permanent monocular visual loss.

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Spotlight on Techniques of lumbar puncture and intrathecal and epidural injections

In this article, Dr. Tarakad Ramachandran of SUNY Upstate Medical University discusses the common procedures of lumbar punctures and epidural catheterizations. Failed procedures result in loss of diagnostic information or inadequate analgesia or anesthesia. With the available evidence, neither ultrasound nor the standard techniques have shown a significant effect in reducing the number of failed procedures to any significant extent. But ultrasound has been shown to reduce the risk of failed procedures in addition to reducing the risk of traumatic procedures and the number of needle insertions and reinsertions. The 4 p’s (position, preparation, projection, and puncture) continue to remain the very essence of these procedures.

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Spotlight on Psychogenic (functional) movement disorders

Psychogenic movement disorders represent a challenge for the clinical practitioner, as the diagnosis and treatment are usually difficult and the prognosis may be poor in some patients. Sometimes also referred to as “functional,” “conversion,” or “psychogenic,” these disorders must be differentiated from “organic” disorders. Psychogenic movement disorders may have several presentations, including tremor, dystonia, myoclonus, tics, parkinsonism, gait disorders, hemifacial spasm, opsoclonus, oculogyric crisis, painful legs, moving toes, etc. and are not uncommonly accompanied by psychogenic speech and voice disorders.

In this article, Dr. Jose Fidel Baizabal-Carvallo of Baylor College of Medicine discusses current advances in the diagnosis, pathophysiology, and treatment of psychogenic movement disorders.

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Spotlight on Crohn disease: neurologic manifestations

In this article, Dr. Jasvinder Chawla of Loyola University Medical Center and Chief of Neurology at Hines VA Hospital discusses Crohn disease, an inflammatory bowel disease that predominantly involves the terminal ileum and colon. A wide range of extraintestinal complications may also compose a portion of the clinical picture. Extraintestinal manifestations may precede the onset of gastrointestinal symptoms by several years. Neurologic disorders associated with inflammatory bowel disease are reported in 3% of patients, but they often represent an important cause of morbidity as well as a diagnostic challenge.

Besides the disease itself, the increasing use of immunosuppressant and other therapies may also play a crucial role in the development of neurologic disorders of different types and pathogenesis. Virtually all components of the nervous system, both central and peripheral, may become involved. Vigilance in recognizing neurologic dysfunction in the setting of Crohn disease, with prompt institution of appropriate treatment, can be important in avoiding irreversible consequences.

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Spotlight on Landau-Kleffner syndrome

Landau-Kleffner syndrome, or acquired epileptic aphasia, was first reported in 1957 and is now recognized as an epileptic encephalopathy. Many authors state that there is a spectrum, including this syndrome and the syndrome of continuous spikes-and-waves during slow sleep, and data supporting this concept are presented in this article by Dr. Natalio Fejerman of Hospital de Pediatria JP Garrahan. Dr. Fejerman also discusses an interesting new pathogenetic interpretation regarding seizures and language dysfunction in Landau-Kleffner syndrome.

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Spotlight on Cephaloceles

Cephaloceles are typically herniations of brain tissue with meninges (encephalocele) or meninges only (cranial meningocele) through a congenital skull defect. They are classified among “neural tube defects” and categorized according to the site of the skull defect. Cephaloceles are often sporadic, but the occipital form may be part of a genetic malformation syndrome.

In this article, Dr. Javad Towfighi of Penn State University reviews the various forms of cephaloceles (encephaloceles and cranial meningoceles) and their diverse clinical manifestations. He provides historical context, classification, and modern methods of diagnosis and treatment of these diseases. This article has been updated to include new approaches to the surgical management of such patients.

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Spotlight on Osmotic demyelination syndromes

Spastic quadriparesis, pseudobulbar palsy, and pseudobulbar affect occurring several days following a rapid rise in serum osmolality are the classical signs of osmotic demyelination syndrome. In this article, Dr. Nicholas Beimer of the University of Michigan reviews the classic clinical signs and pathophysiology of osmotic demyelination syndrome and includes updates from recent literature on methods of prevention and potential future treatment options for this infrequent, but preventable, iatrogenic disease.

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Spotlight on Stroke therapy

Stroke treatment is an emergency, and options for treatment have increased since the FDA approval of intravenous t-PA use in acute ischemic stroke in 1996. In this recently updated article, Dr. Carolyn Brockington, Director of the Stroke Center at St. Luke’s-Roosevelt Hospital, provides information about the scientific basis and adverse effects of acute stroke as well as prevention strategies and treatment guidelines. Also discussed is the use of combination antiplatelet therapy in patients with minor stroke or transient ischemic attack and the latest on blood pressure management in the setting of intracerebral hemorrhage.

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