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https://www.readbyqxmd.com/read/28228434/sagittal-sinus-thrombosis-in-jak2-v617f-mutation-without-overt-myeloproliferative-disorder
#1
Prashant Ramesh Bhand, Supriya Ramesh Karde, Nagabathula Ramesh
We describe a case of a man aged 41 years who presented with a history of generalised headaches for 1 week and an acute episode of tonic-clonic seizures. He had no history of medical illness, was non-smoker, with an extensive family history of haematological disorders. Initial CT brain with contrast did not show any abnormalities in brain parenchyma. X-ray and CT right shoulder showed shoulder dislocation secondary to trauma associated with seizure prior to hospital admission. Subsequently, MRA and MRV brain was arranged, which revealed extensive expansible occlusive filling defect dominating the middle of superior sagittal sinus with left frontal cortical and subcortical acute oedema...
February 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28220286/characteristics-of-hypertrophic-pachymeningitis-in-patients-with-granulomatosis-with-polyangiitis
#2
Hyun Ah Choi, Mi Ji Lee, Chin-Sang Chung
Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients...
February 20, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28220020/randomized-phase-ii-study-of-ramucirumab-or-icrucumab-in-combination-with-capecitabine-in-patients-with-previously-treated-locally-advanced-or-metastatic-breast-cancer
#3
Linda T Vahdat, Rachel Layman, Denise A Yardley, William Gradishar, Mohamad A Salkeni, Anil Abraham Joy, Agustin A Garcia, Patrick Ward, James Khatcheressian, Joseph Sparano, Gladys Rodriguez, Shande Tang, Ling Gao, Rita P Dalal, John Kauh, Kathy Miller
BACKGROUND: Icrucumab (ICR) and ramucirumab (RAM) bind vascular endothelial growth factor (VEGF) receptors 1 and 2 (VEGFR-1 and -2), respectively. This open-label, randomized phase II study evaluated their efficacy and safety in combination with capecitabine (CAP) in patients with previously treated unresectable, locally advanced or metastatic breast cancer. METHODS: Patients were randomly assigned (1:1:1) to receive CAP (1,000 mg/m(2) orally twice daily, days 1-14) alone or in combination with RAM (10 mg/kg intravenously [IV], days 1 and 8) (RAM + CAP) or ICR (12 mg/kg IV, days 1 and 8) (ICR + CAP) every 21 days...
February 20, 2017: Oncologist
https://www.readbyqxmd.com/read/28216232/safety-and-efficacy-of-a-nav1-7-selective-sodium-channel-blocker-in-patients-with-trigeminal-neuralgia-a-double-blind-placebo-controlled-randomised-withdrawal-phase-2a-trial
#4
Joanna M Zakrzewska, Joanne Palmer, Valerie Morisset, Gerard Mp Giblin, Mark Obermann, Dominik A Ettlin, Giorgio Cruccu, Lars Bendtsen, Mark Estacion, Dominique Derjean, Stephen G Waxman, Gary Layton, Kevin Gunn, Simon Tate
BACKGROUND: Current standard of care for trigeminal neuralgia is treatment with the sodium channel blockers carbamazepine and oxcarbazepine, which although effective are associated with poor tolerability and the need for titration. BIIB074, a Nav1.7-selective, state-dependent sodium-channel blocker, can be administered at therapeutic doses without titration, and has shown good tolerability in healthy individuals in phase 1 studies. We therefore assessed the safety and efficacy of BIIB074 in patients with trigeminal neuralgia in a phase 2a study...
February 16, 2017: Lancet Neurology
https://www.readbyqxmd.com/read/28215937/long-term-treatment-with-evolocumab-added-to-conventional-drug-therapy-with-or-without-apheresis-in-patients-with-homozygous-familial-hypercholesterolaemia-an-interim-subset-analysis-of-the-open-label-taussig-study
#5
Frederick J Raal, G Kees Hovingh, Dirk Blom, Raul D Santos, Mariko Harada-Shiba, Eric Bruckert, Patrick Couture, Handrean Soran, Gerald F Watts, Christopher Kurtz, Narimon Honarpour, Lihua Tang, Sree Kasichayanula, Scott M Wasserman, Evan A Stein
BACKGROUND: Homozygous familial hypercholesterolaemia is a genetic disorder characterised by substantially raised LDL cholesterol, reduced LDL receptor function, xanthomas, and cardiovascular disease before age 20 years. Conventional therapy is with statins, ezetimibe, and apheresis. We aimed to assess the long-term safety and efficacy of the proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitor evolocumab in a subset of patients with homozygous familial hypercholesterolaemia enrolled in an open-label, non-randomised phase 3 trial...
February 16, 2017: Lancet Diabetes & Endocrinology
https://www.readbyqxmd.com/read/28209309/cluster-headache-and-macroprolactinoma-case-report-of-a-rare-but-potential-important-causality
#6
Lukas Andereggen, Marie-Luise Mono, Frauke Kellner-Weldon, Emanuel Christ
While headache is not an uncommon symptom in patients suffering from pituitary adenomas, cluster headache (CH) has rarely been reported in such cases. Headache associated with hyperprolactinemia has been reported to be responsive to dopamine agonists (DA agonists) in many patients. We report on a patient with refractory CH secondary to a macroprolactinoma who showed immediate and permanent clinical and radiologic recovery following medical treatment with DA agonists. Measurement of prolactin levels in addition to cranial magnetic resonance imaging might be considered in patients with refractory CH, until the significance of this potential causality becomes clearer...
February 10, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28203571/evolutionary-history-of-multiple-dural-fistula
#7
Braulio Martinez-Burbano, Edgar Patricio Correa Diaz, Carolina Jácome Sánchez
Intracranial dural arteriovenous fistulas (DAVFs) are abnormal communications between arteries and veins or dural venous sinuses, which sit between the sheets of the dura. They represent 10% to 15% of intracranial vascular malformations. Clinical manifestations and prognosis depend on the pattern of venous drainage and location. The clinical presentation of DAVF may be mistaken for vascular or nonvascular brain pathologies. For that reason, within the differential diagnosis come a wide range of conditions, such as secondary headaches, encephalopathies, dementias including those with rapid progression, neurodegenerative diseases, inflammatory processes, or tumors typically at the orbital level or in the cavernous sinus...
October 2016: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28203278/efficacy-of-granulocyte-and-monocyte-apheresis-for-antibiotic-refractory-pouchitis-after-proctocolectomy-for-ulcerative-colitis-an-open-label-prospective-multicentre-study
#8
Takayuki Yamamoto, Toshiaki Tanaka, Tadashi Yokoyama, Takahiro Shimoyama, Hiroki Ikeuchi, Motoi Uchino, Toshiaki Watanabe
BACKGROUND: Granulocyte and monocyte apheresis (GMA) has shown therapeutic efficacy in active ulcerative colitis (UC). We thought that in patients with pouchitis after proctocolectomy for UC, GMA might produce immunological effects in the intestinal mucosa, and improve clinical symptoms. This prospective study was to evaluate the efficacy of GMA for antibiotic-refractory pouchitis after proctocolectomy for UC. METHODS: A total of 13 patients with pouchitis disease activity index (PDAI) > 7 unresponsive to 2 weeks of antibiotic therapy were included...
February 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/28202701/chronobiology-differs-between-men-and-women-with-cluster-headache-clinical-phenotype-does-not
#9
Nunu Lund, Mads Barloese, Anja Petersen, Bryan Haddock, Rigmor Jensen
OBJECTIVE: To describe differences between the sexes in the phenotype of cluster headache (CH) in a large, well-characterized clinical CH population. METHODS: Patients from the Danish CH survey aged 18-65 years, diagnosed with CH according to International Classification of Headache Disorders, second edition, completed questionnaires and structured interviews. RESULTS: A total of 351 patients with CH participated, with a male:female ratio of 2:1...
February 15, 2017: Neurology
https://www.readbyqxmd.com/read/28197843/copeptin-for-risk-stratification-in-non-traumatic-headache-in-the-emergency-setting-a-prospective-multicenter-observational-cohort-study
#10
Claudine Angela Blum, Bettina Winzeler, Nicole Nigro, Philipp Schuetz, Silke Biethahn, Timo Kahles, Cornelia Mueller, Katharina Timper, Katharina Haaf, Janina Tepperberg, Margareth Amort, Andreas Huber, Roland Bingisser, Peter Stephan Sándor, Krassen Nedeltchev, Beat Müller, Mira Katan, Mirjam Christ-Crain
BACKGROUND: In the emergency setting, non-traumatic headache is a benign symptom in 80% of cases, but serious underlying conditions need to be ruled out. Copeptin improves risk stratification in several acute diseases. Herein, we investigated the value of copeptin to discriminate between serious secondary headache and benign headache forms in the emergency setting. METHODS: Patients presenting with acute non-traumatic headache were prospectively enrolled into an observational cohort study...
December 2017: Journal of Headache and Pain
https://www.readbyqxmd.com/read/28195428/blood-brain-barrier-breakdown-in-reversible-cerebral-vasoconstriction-syndrome-implications-for-pathophysiology-and-diagnosis
#11
Mi Ji Lee, Jihoon Cha, Hyun Ah Choi, Sook-Young Woo, Seonwoo Kim, Shuu-Jiun Wang, Chin-Sang Chung
OBJECTIVE: Diagnosis of reversible cerebral vasoconstriction syndrome (RCVS) is currently based on luminographic findings of vasoconstriction. In addition to vasoconstriction, the blood-brain barrier (BBB) breakdown has been postulated as a central mechanism of RCVS. Our aim was to document BBB breakdown in patients with RCVS and its role for the pathophysiology-based diagnosis of RCVS. METHODS: We prospectively recruited 72 consecutive patients with thunderclap headache who did not have aneurysmal subarachnoid hemorrhage from April 2015 to July 2016 at the Samsung Medical Center...
February 13, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28192114/sirolimus-and-autophagy-inhibition-in-lam-results-of-a-phase-i-clinical-trial
#12
Souheil El-Chemaly, Angelo Taveira-Dasilva, Hilary J Goldberg, Elizabeth Peters, Mary Haughey, Don Bienfang, Amanda M Jones, Patricia Julien-Williams, Ye Cui, Julian A Villalba, Shefali Bagwe, Rie Maurer, Ivan O Rosas, Joel Moss, Elizabeth P Henske
BACKGROUND: Animal and cellular studies support the importance of autophagy inhibition in lymphangioleiomyomatosis (LAM). In a cohort of subjects with LAM, we tested the hypothesis that treatment with sirolimus and hydroxycholoroquine (an autophagy inhibitor) at 2 different dose levels is safe and well tolerated. Secondary endpoints included changes in lung function. METHODS: This 48-week, two-center Phase I trial evaluated the safety of escalating oral hydroxychloroquine doses (100-200 mg) given twice a day in combination with sirolimus to eligible patients ≥18 years old with LAM...
February 10, 2017: Chest
https://www.readbyqxmd.com/read/28188907/safety-pharmacokinetic-and-pharmacodynamic-study-in-healthy-subjects-of-oral-neo6860-a-modality-selective-trpv1-antagonist
#13
William Brown, Richard L Leff, Andrew Griffin, Stuart Hossack, Roxane Aubray, Philippe Walker, Dan A Chiche
: Most previous TRPV1 antagonist programs have been put on hold, mainly due on-target adverse events: hyperthermia and impaired noxious heat sensation. NEO6860 is a TRPV1 antagonist, blocking capsaicin activation of the target, with little or no effect against pH or heat activation. The hypothesis is that this pharmacological profile will translate into analgesia without undesired effects on the body temperature or heat-pain threshold. This phase I, double blind, placebo controlled, ascending dose study, included 64 subjects...
February 7, 2017: Journal of Pain: Official Journal of the American Pain Society
https://www.readbyqxmd.com/read/28188618/paroxysmal-hemicrania-like-headache-secondary-to-phosphodiesterase-inhibitors-administration-a-case-report
#14
Arens Taga, Marco Russo, Antonio Genovese, Gian Camillo Manzoni, Paola Torelli
No abstract text is available yet for this article.
February 11, 2017: Headache
https://www.readbyqxmd.com/read/28187915/effect-of-ileal-bile-acid-transporter-inhibitor-gsk2330672-on-pruritus-in-primary-biliary-cholangitis-a-double-blind-randomised-placebo-controlled-crossover-phase-2a-study
#15
Vinod S Hegade, Stuart F W Kendrick, Robert L Dobbins, Sam R Miller, Douglas Thompson, Duncan Richards, James Storey, George E Dukes, Margaret Corrigan, Ronald P J Oude Elferink, Ulrich Beuers, Gideon M Hirschfield, David E Jones
BACKGROUND: Up to 70% of patients with primary biliary cholangitis develop pruritus (itch) during the course of their disease. Treatment of pruritus in primary biliary cholangitis is challenging and novel therapies are needed. Ursodeoxycholic acid, the standard first-line treatment for primary biliary cholangitis, is largely ineffective for pruritus. We investigated the efficacy and safety of GSK2330672, a selective inhibitor of human ileal bile acid transporter (IBAT), in patients with primary biliary cholangitis with pruritus...
February 7, 2017: Lancet
https://www.readbyqxmd.com/read/28180053/isolated-spontaneous-midbrain-hemorrhage-in-a-14-year-old-boy
#16
Kim-Long R Nguyen, Hyunyoung Kim, Laszlo Nagy
Isolated spontaneous midbrain hemorrhages are rare because they are usually secondary to hemorrhages from inferior structures such as the pons and cerebellum, or superior structures such as the thalamus and putamen. While the etiologies are largely unidentified, the most common ones are vascular malformations and bleeding diathesis with hypertension being relatively uncommon. We report midbrain hemorrhage in a 14-year-old boy with a long-standing history of frequent migraine headaches and attention deficit/hyperactivity disorder (ADHD)...
January 2017: Journal of Neurological Surgery Reports
https://www.readbyqxmd.com/read/28168240/cracked-tooth-syndrome-mimicking-trigeminal-autonomic-cephalalgia-a-report-of-four-cases
#17
Noboru Noma, Kohei Shimizu, Kosuke Watanabe, Andrew Young, Yoshiki Imamura, Junad Khan
BACKGROUND: This report describes four cases of cracked tooth syndrome secondary to traumatic occlusion that mimicked trigeminal autonomic cephalalgias. All patients were referred by general practitioners to the Orofacial Pain Clinic at Nihon University Dental School for assessment of atypical facial pain. CLINICAL PRESENTATION: Case 1: A 51-year-old woman presented with severe pain in the maxillary and mandibular left molars. Case 2: A 47-year-old woman presented with sharp, shooting pain in the maxillary left molars, which radiated to the temple and periorbital region...
February 6, 2017: Quintessence International
https://www.readbyqxmd.com/read/28165764/gravity-assisted-valve-gav-systems-to-prevent-low-pressure-headaches-in-patients-with-lumboperitoneal-shunts
#18
Mohammed Akbar Hussain, Roger David Strachan
OBJECTIVES: Low-pressure symptoms after lumboperitoneal (LP) shunting for idiopathic intracranial hypertension (IIH) remain a significant problem. Gravity-assisted valves (GAV) operate at a higher pressure in a vertical position and therefore aim to reduce postural over-drainage. We audited patients with GAV valves inserted in their shunt system to assess their efficacy in reducing low-pressure symptoms and ascertain whether the additional cost of such device can be justified. METHOD: Using a standard proforma, we reviewed patient medical notes and recorded indications and post-operative outcomes in symptom control...
February 6, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/28153622/case-report-of-a-primary-pituitary-abscess-and-systematic-literature-review-of-pituitary-abscess-with-a-focus-on-patient-outcomes
#19
REVIEW
Justice O Agyei, Lindsay J Lipinski, Jody Leonardo
BACKGROUND: Pituitary abscess is a rare but potentially life-threatening condition with an incidence of 0.2-1.1% of operative pituitary lesions. Diagnosis can be challenging, as this disorder shares many similarities with other pituitary lesions in terms of symptomatology and radiographic findings. Most pituitary abscesses are categorized as secondary, arising from pre-existing pituitary lesions or in conjunction with transsphenoidal surgery, sepsis, meningitis, or sinusitis. To date, there have been only a handful of reports of primary pituitary abscess, which occurs without any of the aforementioned risk factors...
January 30, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28152528/post-operative-cluster-headache-following-carotid-endarterectomy
#20
Thijs H T Dirkx, Peter J Koehler
BACKGROUND: Secondary cluster headache following carotid endarterectomy (CEA) is a rare condition and may help us understand the pathophysiology of primary cluster headache. SUMMARY: We describe 2 patients diagnosed with cluster headache, fulfilling the ICHD-IIIB criteria, following CEA. Neither of the patients had headache prior to surgery. They both responded to treatment with oxygen and verapamil. Recent medical literature does not describe any definite cases of cluster headache following CEA...
February 3, 2017: European Neurology
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