Spotlight on Reflex anoxic seizures

Reflex anoxic seizures are a common type of nonepileptic seizure mainly encountered in infants and younger children, but occasionally persisting into adulthood. They are dramatic and frightening to witness but in nearly all cases are benign and without sequelae. They are often confused with other forms of syncope. Exceptionally, they may trigger epileptic seizures. This phenomenon has been reported in adults as well as in children.

In this article, Dr. Colin Ferrie of Leeds Infirmary Hospital reviews their clinical manifestations, discusses their differential diagnosis (concentrating on the need to consider rare but potentially fatal conditions that may mimic reflex anoxic seizures, such as long QT syndromes), and considers the latest approaches to management.

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Spotlight on Posttraumatic sleep disturbance

Sleep disturbances are common after traumatic brain injury, affecting 30% to 70% of individuals, with varying degree of head injury. Not only can they negatively impact neurocognitive rehabilitation, but they also add to morbidity and slow recovery.

In this article, Dr. Muna Irfan of the University of Minnesota describes several sleep impairments, including insomnia, fatigue, and excessive daytime sleepiness, which are the most frequent complaints after head injury. Circadian rhythm dysregulation, sleep apnea (obstructive and/or central), restless leg syndrome, and parasomnias have also been reported after trauma. In addition, depression, anxiety, and pain are common comorbidities with substantial influence on sleep quality and course of recovery. Diagnosis of sleep disorders after traumatic brain injury may involve meticulous history, polysomnography, actigraphy, and multiple sleep latency testing.

Treatment is disorder-specific and may include pharmacotherapy, positive airway pressure, and behavioral modifications. Unfortunately, treatment of sleep disorders associated with traumatic brain injury have met with little success in improving clinical outcomes, mainly due to confounding psychiatric and neurobehavioral sequela of trauma. Nonetheless, some studies have demonstrated encouraging results, which highlight the need for further research and prospective studies to be able to standardize targeted management approach.

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Spotlight on Cluster headache

Cluster headache is a relatively common, very severe form of primary headache that belongs to the family of trigeminal autonomic cephalalgias. It involves dysfunction of central nervous system elements concerned with pain control and with links to circadian and circannual mechanisms. Acute cluster headache can be treated with oxygen, intranasal triptans (sumatriptan and zolmitriptan), and injected sumatriptan. Medicines or strategies useful in the preventive management of cluster headache include verapamil, lithium, corticosteroids, topiramate, and melatonin.

In this article, Dr. Peter Goadsby of King’s College London and the University of California, San Francisco summarizes the current understanding and management of cluster headache, including an update on the latest clinical trial data.

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Spotlight on Vestibular migraine

In this article, Dr. Thomas Lempert of Charité University Hospital discusses vestibular migraine, which presents with attacks of spontaneous or positional vertigo, head motion-induced vertigo, and visual vertigo lasting 5 minutes to 3 days. The diagnosis requires a history of migraine, temporal association of migraine symptoms with vertigo attacks, and exclusion of other causes. Because headache is often absent during acute attacks, other migraine features such as photophobia or auras have to be specifically inquired about. The pathophysiology of vestibular migraine is unknown, but several mechanisms link the trigeminal system, which is activated during migraine attacks, and the vestibular system. Treatment includes antiemetics for severe acute attacks, pharmacological migraine prophylaxis, and lifestyle changes.

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Spotlight on Neurocysticercosis

In this article, Dr. Larry Davis of the University of New Mexico School of Medicine, discusses neurocysticercosis, which continues to be the most common CNS parasite and is becoming increasingly identified in the United States. Most patients in the United States present with seizures (focal or generalized) or headaches and have come from Mexico or Latin America.

A new set of diagnostic criteria have been published. There are now 3 serological methods of diagnosing neurocysticercosis. A serum or CSF antibody assay, especially an enzyme-linked immunotransfer blot assay, is quite sensitive and specific, and is widely available. A new PCR-based assay to detect T. solium nucleic acid is more specific and is becoming more available. The third assay detects cysticercus antigen; this assay is the most valuable in diagnosing extraparenchymal neurocysticercosis in the ventricle or meninges and can be utilized to follow patient response to treatment.

It appears that the cysticercus cyst releases specific proteins that induce an anti-inflammatory host response to prevent the host from destroying viable cerebral cysts. Although some controversy continues regarding the necessity of treating single neurocysticercosis cysts in developing countries, studies find that albendazole treatment significantly hastens the disappearance of cysts and reduces the incidence of seizures with generalization.

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Spotlight on Agenesis of the corpus callosum

Agenesis of the corpus callosum may occur as an isolated event or as part of dozens of developmental and dysmorphic syndromes. Although affected patients may exhibit few obvious neurologic functional deficits, the absence of the corpus callosum, a structure responsible for interhemispheric connections, does carry consequences with respect to information processing and language functions.

In this article, Dr. Brian H Le of Reading Hospital discusses attempts to classify and characterize agenesis of the corpus callosum based on morphologic and molecular findings.

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Spotlight on Hospital-based neurologic care

Escalating economic pressures, a complex regulatory environment, and an ever-expanding scientific knowledge base are threatening to make traditional models for hospital-based neurologic care untenable. Similar to medical hospitalists nearly 20 years ago, neurohospitalists are an emerging group of inpatient subspecialists that have rapidly evolved as a way to bridge the widening gap between increasingly focused outpatient neurologists and the demands of inpatient care for acute neurologic disease.

In this article, Dr. James Greene of Emory University School of Medicine reviews advantages and disadvantages of novel models for hospital-based neurologic care and their potential impact on the future practice of neurology.

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Spotlight on Kearns-Sayre syndrome

Kearns-Sayre syndrome is a multisystem mitochondrial disease characterized by the obligate triad of onset before the age of 20 years, progressive external ophthalmoplegia, and pigmentary retinopathy plus at least one of the following: cardiac conduction block, cerebrospinal fluid protein greater than 100 mg/dl, and cerebellar ataxia. The disorder is usually caused by single large-scale deletions of mitochondrial DNA (mtDNA).

In this article, Dr. Thomas Klopstock of Ludwig Maximilian University of Munich reviews the clinical features and molecular pathogenesis of this unusual disease and places special emphasis on reports of the heterogeneous response to growth hormone therapy in Kearns-Sayre syndrome.

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Spotlight on Brachial neuritis

Brachial neuritis, also known as neuralgic amyotrophy, has a characteristic clinical presentation with sudden onset of pain in the shoulder or arm region followed by weakness and atrophy of shoulder girdle or arm muscles. Clinical variants may include upper limb mononeuropathies, lumbosacral plexus involvement, phrenic neuropathy with diaphragm dysfunction, and concomitant recurrent laryngeal or accessory nerve involvement. These variants are important to recognize to avoid unnecessary work-up or interventions and to direct appropriate treatment.

In this article, Dr. Nens van Alfen of the Radboud University Nijmegen Medical Center discusses typical and variant clinical features, diagnostic approach, and the role of immune therapy and targeted rehabilitation in patients with brachial neuritis.

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Spotlight on Brainstem gliomas in childhood

The management of brainstem gliomas remains problematic. Approximately 80% of children have diffuse, intrinsic pontine tumors, which carry a dismal prognosis. Children with cervicomedullary and tectal tumors have markedly better outcomes.

In this article, Dr. Roger Packer of George Washington University reviews outcomes after clinical trials and new biologic insights that open exciting innovative avenues of molecularly-targeted treatment.

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