Management of Childhood Migraine by Headache Specialist vs Non-Headache Specialists.

OBJECTIVE: This study aims to compare the management practices of a headache specialist with non-headache specialists in the treatment of children with migraine. The use of appropriate rescue medications and prophylactic agents, application of neuroimaging, and short-term outcomes are compared in children treated by the two groups of physicians. METHODS: A retrospective cohort study was conducted by utilizing the electronic medical records of children 3-18 years of age with migraine, who were evaluated at a tertiary care children's hospital from 2016 to 2018. RESULTS: Of the 849 patients who met the study criteria, 469 children were classified as having chronic migraine or high-frequency episodic migraine and were followed-up on at least 1 occasion by the neurologists. Imaging was obtained in 66.5% of all children with migraine. The headache specialist used 5-HT agonists ("triptans") for migraine management in 56.7% (76/135) of cases compared to non-headache specialists who prescribed them in 28.7% (96/334) of cases (P < .001). Of the children with chronic migraine, the headache specialist evaluated 135 patients while the non-headache specialists treated 334 children. Non-headache specialists prescribed prophylaxis in the form of natural supplements more frequently (63.8% of cases) compared to the headache specialist (38.5% of children) (P < .001). Moreover, prophylaxis with prescription drugs was utilized more often by headache specialist (66.7%) than non-headache specialists (37.4%) (P < .001). CONCLUSIONS: Imaging appears to be commonly recommended by both headache specialists and non-headache specialists in children with migraine. The headache specialist was more likely to use triptans as rescue medications for pediatric migraine. Outcomes in the short-term were not statistically different whether children were being managed by the headache specialist or the non-headache specialists.

Intravascular Papillary Endothelial Hyperplasia in the Coronary Artery: An Unusual Cause of Massive Myocardial Infarction in Hypoplastic Left Heart Syndrome.

Background: Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion that usually involves the head and neck or extremities. Involvement of the coronary arterial system is unreported. Case: A 1-month-old patient born with hypoplastic left heart syndrome died from a massive myocardial infarction shortly after first stage palliation with Norwood/Sano. Autopsy demonstrated a massive univentricular hemorrhagic infarction with complete occlusion of the left main coronary artery and its intramural branches by intraluminal papillary endothelial hyperplasia (IPEH). Immunostaining with CD34 and CD31 confirmed the diagnosis. The inferior and superior mesenteric artery branches also had IPEH. Conclusion: IPEH can involve the coronary vasculature, can be multifocal and can occur in the newborn.

Deep Brain Stimulation for Medication Refractory Tremor in Leber Optic Neuropathy Plus Syndrome.

Leber hereditary optic neuropathy (LHON) is a mitochondrial disorder that presents with acute to subacute onset of unilateral progressive optic neuropathy, with sequential involvement of the fellow eye months to years later. The condition may be accompanied by neurological symptoms, including tremors, dystonia, seizures, or psychosis, in which case, it is termed LHON-plus. Here, we present the case of a 53-year-old man who was initially diagnosed with essential tremor but was later found to have LHON-plus after the onset of bilateral visual loss and a genetic panel. His essential tremor was refractory to standard pharmacological therapies, including propranolol, primidone, and topiramate. As a result, he elected to undergo bilateral deep brain stimulation (DBS) of the bilateral ventral intermediate nucleus of the thalamus with a dramatic improvement in symptoms. To our knowledge, this is the first case of essential tremor presenting in the context of LHON-plus to be treated successfully with DBS. While DBS has been applied in LHON-plus presenting with dystonia with limited success, our outcome suggests that there is promise in this approach and that more research is needed to evaluate it.

When the Music Comes From the Brain: A Rare Case of Auditory Seizures Secondary to a Right Temporal Lobe Arteriovenous Malformation.

Focal seizures with subjective auditory phenomena, known as auditory seizures, are uncommon and can include simple to complex auditory hallucinations. We present a case of a 59-year-old man who presented with motor and non-motor seizures. He had a four-month history of hearing things resembling continuous metallic sounds, pennies dropping into a bank, persistent music after radio cessation, and the sound of a passing train. Brain MRI showed multiple serpiginous flow voids in the right temporal lobes, consistent with an arteriovenous malformation that was confirmed eventually with a diagnostic brain angiogram. The etiology of the seizures was related to a structural lesion in the setting of a right temporal arteriovenous malformation (AVM). Treatment with 2000mg of levetiracetam twice daily and 300mg of oxcarbazepine twice daily improved symptoms, and subsequent stereotactic radiosurgery ablation successfully treated the AVM. Post-treatment MRI showed reduced visibility of parasitized vessels, with controlled generalized seizures but partial control of auditory seizures.

When Blood Cell Counts Matter: Hypereosinophilic Syndrome as a Rare Cause of Ischemic Strokes.

Hypereosinophilic syndrome (HES) is a rare condition characterized by elevated eosinophil counts (>1.5 x 109 on two consecutive measurements), which are of myeloid clonal in origin or are driven by excess cytokines. One subtype of HES exhibits the Fip1-like 1-platelet-derived growth factor receptor alpha (FIP1L1-PDGFRA) fusion gene, a gain-of-function mutation resulting in a hyperactive tyrosine kinase. HES, especially the FIP1L1-PDGFRA variant, exhibits an excellent response to chemotherapy with imatinib. In this report, we present a 38-year-old patient with no contributory past medical history who experienced sudden-onset fatigue, ataxia, visual changes, and headaches. He was found to have multiple small acute infarcts in his cerebrum and cerebellum. A stroke work-up, including transthoracic echocardiogram (TTE), transesophageal echocardiogram (TEE), and computed tomography angiography (CTA), did not yield insight into the origin of his infarcts. On CBC, he was consistently hypereosinophilic, and a bone marrow biopsy revealed hypercellularity and the FIP1L1-PDGFRA fusion gene, confirming the diagnosis of HES. The patient was treated first with methylprednisolone and then imatinib with excellent response. It appears that, in our patient, strokes were not of a thromboembolic nature but rather due to hypercoagulability. In this report, we advocate for considering HES and emphasize the importance of revisiting basic laboratory studies such as a CBC if the standard stroke workup fails to elucidate the mechanism behind ischemic strokes with an embolic pattern.

An Approach for Cognitive Evaluation and Management of Grade 5 Arteriovenous Malformation-Associated Thalamic Dementia: A Case Report and Literature Review.

The clinical approach to managing high-grade arteriovenous malformations (AVMs) has been challenging due to its various presentations, surgical risk of complications, and impact on patients' quality of life. We report a case of a 57-year-old female who experienced recurrent seizures and progressive cognitive decline secondary to a grade 5 cerebellar AVM. We reviewed the patient's presentation and clinical course. We also searched the literature for studies, reviews, and case reports involving the management of high-grade AVMs. We outline our recommendations on how to approach these cases after a review of the currently available treatment options.

A Cross-Country Network Analysis of Adolescent Resilience.

PURPOSE: In situations of adversity, young people draw on individual, relational, and contextual (community and cultural) resources to foster their resilience. Recent literature defines resilience as a capacity that is underpinned by a network of interrelated resources. Although empirical studies show evidence of the value of a network approach, little is known regarding how different country contexts influence which resources are most critical within a resource network and how resources interact for adolescent resilience. METHODS: Network analysis was conducted with data from studies that had used the Child and Youth Resilience Measure. Regularized partial correlation networks of 17 resources were estimated for 14 countries (Botswana, Canada, China, Colombia, Equatorial Guinea, India, Indonesia, Italy, Jordan, New Zealand, the Philippines, Romania, South Africa, and Syrian refugees living in Jordan). The sample size was 18,914 (mean age = 15.70 years, 48.8% female). RESULTS: We observed mostly positive associations between the resources of interest. The salience and strength of associations between resources varied by country. The most central resource across countries was having supportive caregivers during stressful times because this resource had the most and strongest positive associations with other resources. CONCLUSIONS: This study gives first empirical evidence from multiple countries that an interplay of social-ecological resources (such as individual skills, peer, caregiver and community support, and educational aspirations and opportunities) matter for adolescent resilience. Across countries, caregiver support appears to be most central for adolescent resilience. Future resilience interventions might apply this network approach to identify important, contextually relevant resources that likely foster additional resources.

Mast cells in neuropathic pain: an increasing spectrum of their involvement in pathophysiology.

Mast cells are immunological cells that are diversely distributed in different parts of the body. Their role in various pathological conditions such as hypersensitivity, atherosclerosis, pulmonary hypertension, and male infertility has been reported by different scientists. Apart from these, a number of studies have shown their important role in pathogenesis of neuropathic pain of diverse aetiology. They have been found to release active mediators, primarily histamine and serotonin on degranulation in response to different stimuli including chemical, nerve damage, toxin or disease-related conditions. The mast cells stabilizer has shown pain attenuating effects by preventing degranulation of mast cells. Similarly, compound 48/80 (first dose 200 µg/100 g and after 6-h interval, second dose of 500 µg/100 g) caused the degranulation of the accumulated endoneurial histamine and 5-HT antagonists have shown pain relieving effects by attenuating the effects of histamine and serotonin, respectively. On the other hand, the mast cell degranulator compound 48/80 has shown dual action depending on its time of administration. The present review discusses the critical role of mast cells in the generation and maintenance of neuropathic pain in experimental models.

Pharmacological investigations on mast cell stabilizer and histamine receptor antagonists in vincristine-induced neuropathic pain.

The present study was designed to investigate the role of mast cells and mast cell-derived histamine in vincristine-induced neuropathic pain. Neuropathic pain was induced by administration of vincristine (100 µg/kg, i.p.) over a period of 10 days, with a break of 2 days, and pain behavioural estimations including pin prick, hot plate and acetone spray tests were performed to assess mechanical and heat hyperalgesia and cold allodynia, respectively, on days 0, 14 and 28. Mast cell stabilizer, sodium cromoglycate, H1 receptor antagonist promethazine and H2 receptor antagonist ranitidine were administered over a period of 12 days. Administration of vincristine resulted in significant development of heat and mechanical hyperalgesia as well as cold allodynia. Furthermore, the pain observed was markedly elevated on the 28th day in comparison to the 14th day. Administration of sodium cromoglycate, promethazine and ranitidine significantly reduced mechanical and heat hyperalgesia and cold allodynia. However, the pain-attenuating effects of ranitidine were significantly less as compared to sodium cromoglycate and promethazine, which suggests that H1 receptors play a more important role than H2 receptors in vincristine-induced neuropathic pain. It may be concluded that vincristine may degranulate mast cells to release inflammatory mediators, particularly histamine which may act through H1 (primarily H1) and H2 receptors to induce neuropathic pain.

Neuropathic pain attenuating effects of perampanel in an experimental model of chronic constriction injury in rats.

The present study explores the pain attenuating effect of perampanel, AMPA receptor antagonist, in chronic constriction injury-induced neuropathic pain. Chronic Constriction Injury was performed by putting four loose ligatures around the sciatic nerve. Pain was assessed by determining mechanical hyperalgesia, cold allodynia and heat hyperalgesia on 7th and 14th day post surgery. Perampanel (3mg and 6mg/kg, p.o.) was administered 30min before pain assessment test on 14th day post-surgery. CCI led to significant development of pain and peak symptoms were observed on 14th day. Perampanel significantly attenuated CCI-induced mechanical hyperalgesia, cold allodynia and heat hyperalgesia, at different time intervals 30, 60, 90, 120min, with more substantial effect observed at dose of 6mg/kgNaloxone was administered in CCI subjected rats before perampanel treatment to explore the potential role of opioids in anti-nociceptive effects of perampanel. Naloxone decreased the pain attenuating effects of perampanel significantly, indicating a critical role of opioid system in anti-nociceptive potential of perampanel. Perampanel has pain attenuating potential in CCI-induced neuropathic pain, which may be due to partly mediated through the opioid system.