Spotlight on Migraine in childhood

Childhood migraine is common, affecting 4% of children. Migraine in children commonly causes bilateral or midfrontal headaches. The peak incidence for migraine in males of all ages is 10 to 14 years, and for females, it is 20 to 24 years. The biggest concerns parents have regarding the etiology of childhood headaches are brain tumors or vascular problems, particularly aneurysms. However, when the exam is normal and the headaches are episodic, these concerns are usually unwarranted. The CHAMP trial has led some clinicians to question the utility of pharmacological treatment for migraine. However, many pharmacological approaches are still warranted. In addition, alternative treatments, including acupuncture, biofeedback, and nutraceuticals, have not been adequately studied to show efficacy. The benefit of a healthy lifestyle to treat acute migraine and to forestall chronic migraine is increasingly recognized. For chronic migraine, one treatment to be considered is injection of the greater occipital nerve. In this article, Dr. Raymond Kandt of Johns Hopkins University reviews the clinical manifestations and discusses treatment strategies.

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Spotlight on Neurostimulation in sleep medicine

Sleep ventilatory disorders are prevalent and include, commonly, both obstructive and central sleep apnea. Positive airway pressure therapy, although widely used, has inherent drawbacks, clinically. Alternative treatment modalities have been approved and hold interest within certain spheres.

Dr. Richard Knudsen of the Pacific Sleep Tech in Aiea, Hawaii reviews the first-in-class, novel “electroceuticals” in this article. These “electroceuticals” rely on the pathway of neural signal modulation via products that regulate the firing of neural circuits, in contradistinction to prescription drugs or pharmaceuticals that harbor untoward, systemic, and generalized side effects.

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Spotlight on Primary CNS angiitis

Diffuse inflammation of small- and medium-sized blood vessels confined exclusively to the brain, meninges, or spinal cord is called primary angiitis of the central nervous system (PACNS). There have been significant advances in the understanding of this disorder due to the identification of pathological and clinical subsets, along with advances in the imaging modality of vessel wall abnormalities. A considerable gap still exists, however, particularly with respect to its pathophysiological mechanisms. Although various laboratory and neuroimaging findings may support the diagnosis of primary CNS angiitis, they are not highly specific, and neurologists often face a substantial challenge when diagnosing this disorder because they need to rule out a vast array of nonvasculitic conditions and secondary causes of central nervous system vasculitis. Therefore, a correct and timely diagnosis of primary CNS angiitis requires a high degree of suspicion coupled with knowledge of other diseases that can masquerade as primary CNS vasculitis.

In this article, Dr. Jorge Moncayo-Gaete of the International University of Ecuador and Dr. Julien Bogousslavsky of the Genolier Swiss Medical Network focus on the clinical and pathological findings, diagnostic work-up, differential diagnosis, and current therapeutic options of this still enigmatic and complex entity.

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Spotlight on Disequilibrium

In this article, Dr. Douglas Lanska of the University of Wisconsin School of Medicine and Public Health, the Medical College of Wisconsin, and IM Sechenov First Moscow State Medical University explains the clinical presentation, pathophysiology, diagnostic work-up, and management of disequilibrium.

Vestibular vertigo is a common problem, particularly in the elderly. Careful history and detailed examination, including focused evaluation of proprioception, vestibular function, directional bias of postural sway, ability to maintain stance with eyes open and closed (Romberg test), ability to maintain stance despite perturbations (eg, pull test), and gait will allow distinction of the major categories of disequilibrium in most cases and will often allow a specific etiologic diagnosis as well.

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Spotlight on Hexacarbon neuropathy

Hexacarbons are present in many solvents and glues, and exposure is most commonly occupational. The neuropathy associated with hexacarbon exposure results in giant axonal swellings and distal slowing of conduction velocity. Hexacarbon neuropathy may continue to worsen for some time after cessation of exposure (coasting).

In this article, Dr. Michael Pulley of the University of Florida, Jacksonville reviews the clinical features of this interesting toxic neuropathy, which was fairly common in the past, related to recreational glue sniffing, but is primarily occupational currently. The axonal swellings seen in hexacarbon neuropathy are similar to those seen in carbon disulfide and acrylamide neuropathy.

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Spotlight on Coma due to supratentorial and cerebellar lesions

Coma is characterized by the absence of arousal (wakefulness, vigilance) and awareness of self and environment, lasting for more than 1 hour. Comatose patients have closed eyes, do not communicate, and do not arouse to verbal, tactile, or noxious stimuli. The terminology and etiologies of coma have not changed historically since the seminal work by Plum and Posner. Current work-up starts with imaging to delineate anatomic from physiologic causes, which drives the ensuing investigation and treatment.

In this article, Dr. Richard Jackson of University of Vermont addresses the clinical manifestations, biological basis, and management of coma due to supratentorial and cerebellar lesions.

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Spotlight on Argininosuccinic acidemia

Argininosuccinic aciduria is an inherited urea cycle disorder caused by argininosuccinic acid lyase deficiency. Patients can present with hyperammonemic encephalopathy at any age, resulting in brain damage or even death if treatment is inadequate or delayed. Patients may also develop hepatomegaly, progressive liver fibrosis, and liver function abnormalities, but they can also present with intellectual disabilities only. The disease can be detected by tandem mass spectrometry-based newborn screening. Treatment consists of low-protein diet, nitrogen scavengers, and L-arginine. Liver transplantation prevents the production of toxic metabolites in the liver, but not in nonhepatic tissues.

In this article, Dr. Matthias Zielonka and Dr. Stefan Kolker of University Children’s Hospital Heidelberg discuss a novel model that predicts clinical severity using the in vitro residual enzyme activity of argininosuccinate lyase as biomarker.

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Spotlight on Headache associated with illicit drug use

In general, most physicians are not familiar with headache syndromes induced by the use of illicit drugs such as cocaine, marijuana, and opioids. Some patients may develop thunderclap headache and reversible cerebral vasoconstriction syndrome.

In this article, Dr. Shuu-Jiun Wang of the Brain Research Center, National Yang-Ming University and the Neurological Institute, Taipei Veterans General Hospital, provides an update on the underlying pathophysiology and reviews the clinical aspects of headache syndromes induced by illicit drugs in light of the criteria presented in the International Classification of Headache Disorders, 3rd edition (ICHD-3). Also discussed is the high frequency of illicit drug use in patients with cluster headache and the possible mechanism for this association.

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Spotlight on Benign paroxysmal positional vertigo

Benign paroxysmal positional vertigo is the most common vestibular disorder. Canalolithiasis of the posterior semicircular canal is now widely accepted as the biological basis for typical benign paroxysmal positional vertigo. Better understanding of its pathophysiological concepts has led to specific therapeutic strategies, which aim to clear the affected semicircular canal from mobile particles. In this article, Dr. Michael von Brevern and Dr. Thomas Lempert of Charité University Hospital added insight in the differentiation of benign paroxysmal positional nystagmus from central positional nystagmus.

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Spotlight on Progressive supranuclear palsy

Progressive supranuclear palsy remains an intractable and underrecognized neurodegenerative illness with a median survival of only 7 years. However, patients will benefit from accurate, early diagnosis that allows them to understand the prognosis, to avoid unnecessary tests and useless treatments, and to avail themselves of disease-specific support services. Advances in understanding the tau protein aggregation and mitochondrial dysfunction of progressive supranuclear palsy and the toxic and genetic insults underlying them are accruing swiftly.

In this article, Dr. Robert Fekete of New York Medical College describes the subtypes of progressive supranuclear palsy as well as advances in pathology, imaging, and genetics of the disorder.

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