Headache in Behçet's Disease.

PURPOSE OF REVIEW: Our objective is to describe the various neurologic manifestations of Behcet's syndrome with special attention to headache syndromes. RECENT FINDINGS: Most recent studies have focused on data collection to better characterize the relatively rare disorder of neuro-Behcets (NBD). In patients with Behcet's disease (BD) who are experiencing headache, most are primary headache disorders, specifically tension type or migraines. Worsening headache can, however, be a sign of increased Behcets disease activity. Behcet's disease is a chronic, systemic, inflammatory disorder of unknown etiology. The prevalence of BD is most notable in countries along the ancient Silk Road, particularly the Middle East. BD occasionally has neurologic manifestations, further categorized into parenchymal and non-parenchymal syndromes. Headache is the most frequently reported neurological symptom of BD. Primary headaches are the most common headache type among patients with BD with comorbid headache disorders with debate regarding whether migraine or tension-type headache is most prevalent. Of the secondary causes of headache in BD, cerebral venous thrombosis is the most common.

Correction to: Diving Headache.

The original version of this article incorrectly listed the second author's name. It should be Stephanie J. Nahas, not Stephanie J. Nahas-Geiger. The author name is corrected in this erratum.

Diving Headache.

This review will focus on the most recent information regarding the ICHD-3 definition of diving headache as well as other important causes of diving headache that are not listed in the ICHD-3 classification system. The paper will discuss etiology, diagnosis, and management of these disorders, focusing, when possible, on the newest research available. ICHD-3 diving headache is due to hypercapnia and is treated accordingly with oxygen. Other causes of diving headache range from decompression sickness to external compression headache to primary headache disorders, such as migraine. Correctly determining the underlying cause of the diving headache is critical to management and relies on history taking and physical exam. The pathophysiology of newly described types of diving headache, such as diving ascent headache, remains under investigation but may be related to other homeostatic headache causes, such as airplane headache. Further investigation may yield more information regarding management as well as possible insight into other headache disorders.

An Up to Date Review of Pseudotumor Cerebri Syndrome.

PURPOSE OF REVIEW: Idiopathic intracranial hypertension (IIH), pseudotumor cerebri syndrome (PTCS), and benign intracranial hypertension are all terms that have been used for a neurologic syndrome consisting of elevated intracranial pressure (ICP), headache and vision loss without mass lesion or underlying infection or malignancy. In this review article, categorization, diagnostic criteria, symptom management strategies, and disease treatment options for pseudotumor cerebri syndrome will be discussed. RECENT FINDINGS: The Idiopathic Intracranial Hypertension Treatment Trial has now proven that acetazolamide should be the first line therapy in primary PTCS, but other treatment options exist in patients who cannot tolerate acetazolamide or in selected cases, which requires surgical intervention for PTCS which acutely threatens vision. Headache has also been shown to require focused treatment beyond therapies that lower ICP, specifically targeting coexistent primary headache disorders and medication overuse. Advances in treatment and diagnostic modalities have improved understanding of PTCS types and their treatment. The pathophysiology of primary PTCS, however, remains incompletely understood, but continued evaluation of cerebrospinal fluid flow dynamics, aquaporins, hormones, natriuretic peptides, and the link with female gender and obesity may lead to future answers.

Recognizing CADASIL: a Secondary Cause of Migraine with Aura.

PURPOSE OF REVIEW: CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an important disease to consider in the differential diagnosis of migraine with aura. This review examines current literature regarding migraine in CADASIL, as well as diagnostic and treatment modalities. RECENT FINDINGS: Recent studies have shown that smoking is a modifiable risk factor for progression of CADASIL (Chabriat et al. in Stroke 47:4-11, 2015). Specific imaging changes and findings on clinical exam can predict disease progression. However, migraine symptoms often precede MRI changes (Guey et al. in Cephalalgia 36:1038-47, 2015). A recent paper on migraine treatment in CADASIL reevaluates the prevailing belief that vasoconstrictive abortive medications are contraindicated in these patients (Tan and Markus in PLoS ONE 11:e0157613, 2016). CADASIL is an autosomal dominantly inherited vasculopathy causing ischemic pathology in younger individuals due to a mutation in the NOTCH3 gene. The mutation results in impaired arterial contractility due to accumulation of granular osmiophilic extracellular material (GOM) in vascular smooth muscle cells (VSMCs). Clinical manifestations include migraine with and without aura, cognitive decline, ischemic events, and mood disorders. The presenting symptom is often migraine with aura. Characteristic MRI changes are often present. Genetic screening is available to confirm NOTCH3 mutation and pathognomic changes are often seen in skin biopsy. Treatment of migraine is similar to the general population, but with some notable and specific differences. Further studies in CADASIL, other small vessel arteriopathies, and migraine may help us understand more about the pathophysiology of these conditions and help with treatment development.