Characterizing Vitamin B12 Deficiency in Neurology Outpatients: A Retrospective Observational Study.

OBJECTIVES: Clinical manifestations of vitamin B12 deficiency are varied and may result in missed or delayed diagnosis. This investigation explores the diverse clinical manifestations and demographic characteristics of vitamin B12 deficiency in neurology outpatients, aiming to enhance timely diagnosis and outcomes. METHODS: The severity of vitamin B12 deficiency was classified as absolute (≤150 pg/mL) or borderline deficiency (150-300 pg/mL). We conducted a retrospective analysis of 165 outpatients with vitamin B12 deficiency at the department of neurology between May 2020 and May 2021. RESULT: Absolute vitamin B12 deficiency was found in 23.0% of the patients. The most common age range was 50-60 years, the most common cause was vegetarianism, and the most common symptom was headache. Epileptiform symptoms were more likely to occur in younger patients (<20 years old) with vitamin B12 deficiency, whereas psychiatric symptoms were more likely to occur in older patients (>70 years old). Vegetarians, salivation, and nonmegaloblastic anemia were more obvious in patients with absolute vitamin B12 deficiency, whereas headaches often showed borderline B12 deficiency. CONCLUSIONS: The clinical characteristics of vitamin B12 deficiency are complex and nonspecific. The diagnosis should be based on multiple factors.

Correlation between benign joint hypermobility syndrome and headache in children and adolescents.

BACKGROUND: Benign Joint Hypermobility Syndrome (BJHS) is a most common hereditary connective tissue disorders in children and adolescents. This study aimed to investigate the prevalence and subtypes of headache in children with BJHS. METHODS: This observational-analytical study was conducted in a case-control setting on school children aged 7 to 16 years in 2021-2023 in Isfahan, Iran. Students were examined for BJHS using Beighton criteria by a pediatric rheumatologist. Headache disorder was diagnosed according to the Child Headache-Attributed Restriction, Disability, and Social Handicap and Impaired Participation (HARDSHIP) questionnaires for child and adolescent and International Classification of Headache Disorders (ICHD-III). RESULTS: A total of 4,832 student (mean age 10.3 ± 3.1 years), 798 patients with BJHS and 912 healthy children were evaluated. The probability of headache in children aged 7-11 with hypermobility was 3.7 times lower than in children aged 12-16 with hypermobility (P = 0.001). The occurrence of headache in children with BJHS was more than the control group (P = 0.001), and the probability of headache in children with BJHS was 3.7 times higher than in healthy children (P = 0.001). Migraine was the most common headache type reported of total cases. The probability of migraine in children with BJHS was 4.5 times higher than healthy children ( P = 0.001). CONCLUSION: This study showed a significant correlation between BJHS and headache (especially migraine) in children and adolescents.

Approach to the Diagnosis and Treatment of Headache.

Headache is consistently one of the most common complaints reported by patients in the medical setting worldwide. Headache can be a symptom of another condition or illness, secondary to the disruption of homeostasis, or can be a primary disorder with inherent variability and patterns. Headache disorders, whether primary or secondary, can cause significant disability and loss of quality of life for those affected. As such, it is important for primary care providers to feel confident evaluating and treating patients with headache, especially given the limited access to Headache Medicine subspecialists.

Headache in Children and Adolescents.

OBJECTIVE: This article reviews the assessment of children and adolescents presenting with headache, provides an overview of primary headache disorders, and reviews evidence-based management of headache in this age group. LATEST DEVELOPMENTS: In the last few years, new epidemiological data have shed light on less common pediatric headache disorders (eg, pediatric trigeminal autonomic cephalalgias) and psychosocial risk factors associated with primary headache disorders in children and adolescents. There has also recently been a substantial increase in interventions that target the calcitonin gene-related peptide pathway and that treat primary headache disorders using noninvasive neuromodulation. Although these interventions have primarily been studied in adults, there is emerging evidence of their use in the pediatric population. ESSENTIAL POINTS: Primary headache disorders are very common in youth, and the most commonly encountered headache diagnosis in neurology practice is migraine, which affects approximately 10% of children and adolescents. Diagnosing and effectively treating primary headache disorders before adulthood may have a sustained impact on the patient by improving long-term headache and mental health outcomes, thereby significantly reducing the burden of disability over time. There are several available and emerging acute and preventive interventions for youth with primary headache disorders, and treatment decisions should be made in the context of available evidence using a shared decision-making approach.

Cranial Neuralgias.

OBJECTIVE: The cranial neuralgias are relatively rare, but recognizing these syndromes and distinguishing among them is critical to reducing unnecessary pain and disability for affected patients. Despite their distinctive features, cranial neuralgias may go undiagnosed or misdiagnosed for several years. A notable proportion of cranial neuralgia presentations are due to secondary causes and require targeted treatment. The purpose of this article is to review the diagnosis and management of cranial neuralgias encountered in clinical practice. LATEST DEVELOPMENTS: In 2020, the International Classification of Orofacial Pain was released for the first time. Modeled after the International Classification of Headache Disorders, it includes updated terminology for cranial neuralgias. The underlying pathophysiology of the cranial neuralgias is currently believed to be rooted in both peripheral and central nociceptive systems. In addition, a growing number of familial cases are being identified. Recent therapeutic advancements include a better understanding of how to utilize older therapies and procedures more effectively as well as the development of newer approaches. ESSENTIAL POINTS: Cranial neuralgia syndromes are rare but important to recognize due to their debilitating nature and greater likelihood of having potentially treatable underlying causes. While management options have remained somewhat limited, scientific inquiry is continually advancing the understanding of these syndromes and how best to address them.

[Imaging in headache]. / Bildgebung bei Kopfschmerzen.

Headache is worldwide one of the leading reasons to consult a general practitioner or a neurologist. In addition to the medical history and results of laboratory parameters, imaging represents one of the most important diagnostic steps. As there is a myriad of possible causes, it is nearly impossible to cover the whole spectrum of this topic. This article summarizes the most important morphological imaging findings and their pitfalls.

Approach to the Patient With Headache.

OBJECTIVE: The evaluation of patients with headache relies heavily on the history. This article reviews key questions for diagnosing primary and secondary headache disorders with a rationale for each and phrasing to optimize the information obtained and the patient's experience. LATEST DEVELOPMENTS: The availability of online resources for clinicians and patients continues to increase, including sites that use artificial intelligence to generate a diagnosis and report based on patient responses online. Patient-friendly headache apps include calendars that help track treatment response, identify triggers, and provide educational information. ESSENTIAL POINTS: A structured approach to taking the history, incorporating online resources and other technologies when needed, facilitates making an accurate diagnosis and often eliminates the need for unnecessary testing. A detailed yet empathetic approach incorporating interpersonal skills enhances relationship building and trust, both of which are integral to successful treatment.

Indomethacin-Responsive Headache Disorders.

OBJECTIVE: This article describes the clinical features and treatment of the indomethacin-responsive headache disorders paroxysmal hemicrania and hemicrania continua. LATEST DEVELOPMENTS: Both paroxysmal hemicrania and hemicrania continua are treated with indomethacin at the lowest clinically useful dose. It has recently become clear that some patients with either condition may respond to treatment with noninvasive vagus nerve stimulation, which can be both indomethacin sparing and, in some cases, headache controlling. Given the lifelong nature of both paroxysmal hemicrania and hemicrania continua, brain imaging with MRI is recommended when the conditions are identified, specifically including pituitary views. ESSENTIAL POINTS: Paroxysmal hemicrania and hemicrania continua are indomethacin-responsive headache disorders that offer a rewarding and unique opportunity to provide marked clinical improvement when recognized and treated appropriately. These disorders share the final common pathway of the trigeminal-autonomic reflex, with head pain and cranial autonomic features, and are differentiated pathophysiologically by the pattern of brain involvement, which can be seen using functional imaging. They have distinct differential diagnoses to which the clinician needs to remain alert.

Headaches and Vasculitis.

Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. Unrecognized and therefore untreated, vasculitis of the nervous system leads to pervasive injury and disability making this a disorder of paramount importance to all clinicians. Headache may be an important clue to vasculitic involvement of central nervous system (CNS) vessels. CNS vasculitis may be primary, in which only intracranial vessels are involved in the inflammatory process, or secondary to another known disorder with overlapping systemic involvement. Primary neurologic vasculitides can be diagnosed with assurance after intensive evaluation that incudes tissue confirmation whenever possible.

The Postconcussion Syndrome and Posttraumatic Headaches in Civilians, Soldiers, and Athletes.

Posttraumatic headaches are one of the most common and controversial secondary headache types. After a mild traumatic brain, an estimated 11% to 82% of people develop a postconcussion syndrome, which has been controversial for more than 160 years. Headache is estimated as present in 30% to 90% of patients after a mild head injury. Most headaches are tension-type-like or migraine-like. Headaches in civilians, soldiers, athletes, and postcraniotomy are reviewed. The treatments are the same as for the primary headaches. Persistent posttraumatic headaches can continue for many years.

The Pseudotumor Cerebri Syndrome.

Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either primary (idiopathic intracranial hypertension, IIH) or secondary. A secondary cause is unlikely when adhering to the diagnostic criteria. Permanent visual loss occurs if undetected or untreated, and the associated headaches may be debilitating. Fulminant disease may result in blindness despite aggressive treatment. This study addresses the diagnosis and management of IIH including new insights into the pathobiology of IIH, updates in therapeutics and causes of overdiagnosis.

Headache due to Vascular Disorders.

Headache and cerebrovascular disease (CVD) are inextricably linked. Although in some cases headache complicating CVD may be little more than a symptomatic afterthought, in other cases, early recognition of headache's role in the CVD process is critical to effective management. In other words, headaches secondary to CVD span a spectrum, and in this article, we will review that spectrum.

Headaches Attributed to Disorders of Homeostasis.

Headaches attributed to disorders of homeostasis include those different headache types associated with metabolic and systemic diseases. These are headache disorders occurring in temporal relation to a disorder of homeostasis including hypoxia, high altitude, airplane travel, diving, sleep apnea, dialysis, autonomic dysreflexia, hypothyroidism, fasting, cardiac cephalalgia, hypertension and other hypertensive disorders like pheochromocytoma, hypertensive crisis, and encephalopathy, as well as preeclampsia or eclampsia. The proposed mechanism behind the causation of these headache subtypes including diagnostic criteria, evaluation, treatment, and overall management will be discussed.

Headache in Brain Tumors.

The prevalence of brain tumors in patients with headache is very low; however, 48% to 71% of patients with brain tumors experience headache. The clinical presentation of headache in brain tumors varies according to age; intracranial pressure; tumor location, type, and progression; headache history; and treatment. Brain tumor-associated headaches can be caused by local and distant traction on pain-sensitive cranial structures, mass effect caused by the enlarging tumor and cerebral edema, infarction, hemorrhage, hydrocephalus, and tumor secretion. This article reviews the current findings related to epidemiologic details, clinical manifestations, mechanisms, diagnostic approaches, and management of headache in association with brain tumors.

Cough, Exertional, and Sex Headaches.

In this article, the authors review the most common presentations of cough and exertional headaches and headaches associated with sexual activity. The authors elaborate on the most commonly described etiologies and identify those which are most critical to treat. The authors outline the recommendations for further evaluation and discuss effective treatment modalities for each headache type.

Cervicogenic Headaches: A Literature Review and Proposed Multifaceted Approach to Diagnosis and Management.

Cervicogenic headaches are a secondary headache disorder attributable to cervical spine dysfunction resulting in head pain with or without neck pain. Diagnosis of this condition has been complicated by varied clinical presentations, causations, and differing diagnostic criteria. In this article, we aim to clarify the approach to diagnosing cervicogenic headaches by providing an overview of cervicogenic headaches, clinical case examples, and a practical diagnostic algorithm based on the most current criteria. A standardized approach will aid in confirmation of the diagnosis of cervicogenic headaches and facilitate further research into this condition.

Temporomandibular Disorders, Bruxism and Headaches.

Temporomandibular disorders (TMDs) and headache disorders are highly prevalent in the population. TMDs can present headache symptoms as a secondary headache and, in addition, be comorbid with primary headache disorders. This overlap has significant clinical implications for which it is essential for the physician to be aware, and they should screen for the potential presence of TMDs in a headache patient. Bruxism is a parafunctional behavior also prevalent in the population which has a role in TMDs and may influence headache symptomatology, but it is still necessary to clarify this relationship.

Other Secondary Headaches: Odontogenic Pain and Other Painful Orofacial Conditions.

This article discusses extremely common odontogenic pain conditions, which may occasionally present to the neurology clinic mimicking headache, and other uncommon orofacial pain conditions, which may do the same. Typical presentations, investigative strategies, and management are discussed, as well as highlighting key diagnostic criteria and the importance of involving oral or dental specialists where diagnostic uncertainty exists.

Are the ICHD-3 criteria for headache attributed to idiopathic intracranial hypertension valid? Headache phenotyping and field-testing in newly diagnosed idiopathic intracranial hypertension.

BACKGROUND: Headache burden is substantial in idiopathic intracranial hypertension. The classification of idiopathic intracranial hypertension headache by the International Classification of Headache Disorders (ICHD) is an important tool for research and clinical purposes. METHODS: We phenotyped headaches and tested sensitivity and specificity of the ICHD-3 criteria for idiopathic intracranial hypertension headache in a prospective cohort of patients suspected of idiopathic intracranial hypertension at two tertiary headache centers. RESULTS: Sensitivity was 93% and specificity was 100% of ICHD-3 criteria for idiopathic intracranial hypertension-related headache validated in idiopathic intracranial hypertension (n = 140) and patients in whom idiopathic intracranial hypertension was suspected but disproven (n = 103). The phenotype of new/worsened headaches related to idiopathic intracranial hypertension suspicion was equally migraine-like (p = 0.76) and tension-type-like (p = 0.08). Lumbar puncture opening pressure was higher (p < 0.0001) and pulsatile tinnitus more frequent (p < 0.0001) in idiopathic intracranial hypertension patients, but neither improved the applicability of the headache criteria, nor did papilledema. CONCLUSION: Headache phenotype is not distinct in idiopathic intracranial hypertension. ICHD-3 criteria for idiopathic intracranial hypertension headache are sensitive and specific, but simplicity can be improved without compromising accuracy. We propose that a new or worsened headache temporally related to active idiopathic intracranial hypertension is a sufficient criterion for idiopathic intracranial hypertension headache regardless of headache phenotype or accompanying symptoms, and that elements of idiopathic intracranial hypertension diagnostics (papilledema and opening pressure) be segregated from headache criteria.Trial Registration: ClinicalTrials.gov Identifier: NCT04032379.

Diversity, Equity, and Inclusion in Headache Care and Research.

ABSTRACT: This article reviews the disparities faced by individuals who experience headache disorders and discusses potential solutions to deliver equitable care. Disparities exist in the diagnosis and treatment of headache disorders with regard to race, ethnicity, sex, gender, sexual orientation, geography, and socioeconomic status. Furthermore, research in the realm of headache disparities is inadequate, and the clinical trial representation of patients from underserved communities is poor. Many barriers exist to optimizing care for underserved communities and this article addresses these barriers and presents ways to combat them.