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https://www.readbyqxmd.com/read/28731661/-melkersson-rosenthal-syndrome-report-of-two-pediatric-cases
#1
Lucas Bordino, Mariana L Juchli, Marcelo Fernández, María de Los Ángeles Fitz Maurice, Andrea Martins, Zaida E Ramírez, Roxana Spini
Melkersson Rosenthal syndrome is a very infrequent disease of unknown etiology, chronic and progressive course, with neurocutaneous disease that affects the orofacial innervation and mucocutaneous tissues with non-caseating granulomatous infiltration. Clinically, it is characterized by the diagnostic triad: recurrent edema of lips and/or face; recurrent and alternating acute peripheral facial paralysis and scrotal or geographic tongue. The condition generally presents as oligosymptomatic or monosymptomatic form...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28726523/investigational-therapies-for-the-treatment-of-narcolepsy
#2
Stefano de Biase, Annacarmen Nilo, Gian Luigi Gigli, Mariarosaria Valente
Narcolepsy is a chronic sleep disorder characterized by a pentad of excessive daytime sleepiness (EDS), cataplexy, sleep paralysis, hypnagogic/hypnopompic hallucinations, and disturbed nocturnal sleep. While non-pharmacological treatments are sometimes helpful, more than 90% of narcoleptic patients require a pharmacological treatment. Areas covered: The present review is based on an extensive Internet and PubMed search from 1994 to 2017. It is focused on drugs currently in development for the treatment of narcolepsy...
July 20, 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28726134/non-functioning-parathyroid-carcinoma-a-case-report
#3
Nobuyasu Suganuma, Hiroyuki Iwasaki, Satoru Shimizu, Tatsuya Yoshida, Takashi Yamanaka, Izumi Kojima, Haruhiko Yamazaki, Soji Toda, Hirotaka Nakayama, Katsuhiko Masudo, Yasushi Rino, Kae Kawachi, Yohei Miyagi, Akio Miyake, Kenichi Ohashi, Munetaka Masuda
BACKGROUND: Non-functioning parathyroid carcinoma is a rare disease that is difficult to distinguish from other diseases based on the lack of hyperparathyroidism. This is a report of non-functioning parathyroid carcinoma diagnosed by reverse transcription polymerase chain reaction (RT-PCR) targeting parathyroid hormone (PTH) messenger RNA. CASE PRESENTATION: The patient is a 67-year-old male who visited our hospital for the chief complaint of hoarseness. A 5-cm mass was observed in the right lobe of the thyroid gland, and poorly differentiated thyroid carcinoma was suspected according to the fine-needle biopsy results...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28725179/fine-tuning-er-stress-signal-transducers-to-treat-amyotrophic-lateral-sclerosis
#4
Danilo B Medinas, Jose V González, Paulina Falcon, Claudio Hetz
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of motoneurons and paralysis. The mechanisms underlying neuronal degeneration in ALS are starting to be elucidated, highlighting disturbances in motoneuron proteostasis. Endoplasmic reticulum (ER) stress has emerged as an early pathogenic event underlying motoneuron vulnerability and denervation in ALS. Maintenance of ER proteostasis is controlled by a dynamic signaling network known as the unfolded protein response (UPR)...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28724781/muscle-disuse-caused-by-botulinum-toxin-injection-leads-to-increased-central-gain-of-the-stretch-reflex-in-the-rat
#5
Jessica Pingel, Hans Hultborn, Lui Naslund-Koch, Dennis Bo Jensen, Jacob Wienecke, Jens Bo Nielsen
Botulinum toxin (Btx) is used in children with cerebral palsy and other neurological patients to diminish spasticity and reduce the risk of development of contractures. Here, we investigated changes in the central gain of the stretch reflex circuitry in response to botulinum toxin injection in the triceps surae muscle in rats. Experiments were performed in 21 rats. 8 rats were in a control group and 13 rats were injected with 6 IU of Btx in the left triceps surae muscle. Two weeks after Btx injection larger monosynaptic reflexes (MSR) were recorded from the left (injected) than the right (non-injected) L4 + L5 ventral roots following stimulation of the corresponding dorsal roots...
July 19, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28722232/rapidly-growing-cystic-vestibular-schwannoma-with-sudden-onset-facial-palsy-ten-years-after-subtotal-excision
#6
Manjunath Dandinarasaiah, Golda Grinblat, Sampath Chandra Prasad, Abdelkader Taibah, Mario Sanna
An elderly male patient diagnosed with a right-sided cystic vestibular schwannoma (CVS) at our center underwent a translabyrinthine approach with a subtotal excision to preserve the facial nerve (FN). The tumor grew slowly for the first 9 years but in the subsequent 2 years grew rapidly, with the patient developing a FN paralysis. Using the previous approach, a second surgery was done and the tumor was excised, leaving behind a sheath of tumor on the facial and lower cranial nerves. This case demonstrates that CVSs show unpredictable growth patterns and need to be followed up for a longer period of time...
July 19, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28721789/mindset-shift-on-pj-paralysis
#7
Brian Dolan
When I first created #EndPJparalysis, little did I know it would be adopted globally - and particularly across the UK - as a vehicle for enabling patients to get up, dressed and moving while in hospital.
July 19, 2017: Nursing Standard
https://www.readbyqxmd.com/read/28721219/two-rare-manifestations-of-primary-hyperparathyroidism-paralysis-and-peptic-ulcer-bleeding
#8
Maryam Heidarpour, Mehdi Karami, Pegah Hedayat, Ashraf Aminorroaya
Primary hyperparathyroidism revealed by thoracic spine brown tumor and peptic ulcer bleeding is rare. We presented a case of 33-year-old male patient who was admitted with paraplegia. Thoracic spine magnetic resonance imaging (MRI) showed extradural lesion at T4 level. He underwent surgical decompression in T4. According to histopathologic finding and elevated serum parathormone (PTH) and hypercalcemia (total serum calcium 12.1 mg/dL), the diagnosis of brown tumor was down. Ultrasonography of his neck showed a well-defined lesion of 26 × 14 × 6 mm...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28721012/comparison-of-neurotoxic-potency-between-a-novel-chinbotulinumtoxina-with-onabotulinumtoxina-incobotulinumtoxina-and-lanbotulinumtoxina-in-rats
#9
Ya Feng, Wuchao Liu, Lizhen Pan, Cong Jiang, Chengxi Zhang, Yuxuan Lu, Zhiyu Nie, Lingjing Jin
Four botulinumtoxin type A (BoNT/A) products, onabotulinumtoxinA (A/Ona), incobotulinumtoxinA (A/Inco), lanbotulinumtoxinA (A/Lan) and chinbotulinumtoxinA (A/Chin), are applied in the present study, among which A/Chin is newly produced. We aimed to compare the neurotoxic potency of these toxins by the gauge of muscle strength reduction. Furthermore, potential molecular and cellular mechanisms were also explored. According to our data, muscle strengths in the four toxin groups were all significantly decreased after injection for 1 week...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28720601/a-case-of-progressive-orthopnoea-and-shoulder-pain-secondary-to-bilateral-diaphragmatic-paralysis
#10
Anna Herbert, Waseem Asrar Khan, Salem Madi
A 45-year-old man with a raised body mass index presented with an acute history of right lower chest pain and increasing breathlessness. C reactive protein, D dimer and cardiac echocardiography were negative, with mild bibasal atelectasis the only positive reported finding from erect chest X-ray and CT pulmonary angiogram. He was discharged with antibiotics for a chest infection. He remained severely breathless and was referred to the sleep-related breathing disorders clinic. He described shoulder pain, daytime tiredness and sleeping upright due to a ââ'¬Ëœsuffocatingââ'¬â"¢ sensation...
July 18, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28719996/efficacy-of-short-term-robot-assisted-rehabilitation-in-patients-with-hand-paralysis-after-stroke-a-randomized-clinical-trial
#11
Jorge H Villafañe, Giovanni Taveggia, Silvia Galeri, Luciano Bissolotti, Chiara Mullè, Grace Imperio, Kristin Valdes, Alberto Borboni, Stefano Negrini
BACKGROUND: We evaluated the effectiveness of robot-assisted motion and activity in additional to physiotherapy (PT) and occupational therapy (OT) on stroke patients with hand paralysis. METHODS: A randomized controlled trial was conducted. Thirty-two patients, 34.4% female (mean ± SD age: 68.9 ± 11.6 years), with hand paralysis after stroke participated. The experimental group received 30 minutes of passive mobilization of the hand through the robotic device Gloreha (Brescia, Italy), and the control group received an additional 30 minutes of PT and OT for 3 consecutive weeks (3 d/wk) in addition to traditional rehabilitation...
February 1, 2017: Hand: Official Journal of the American Association for Hand Surgery
https://www.readbyqxmd.com/read/28719756/medialization-laryngoplasty-in-the-elderly-outcomes-and-expectations
#12
Andrew Jay Bowen, Tiffany L Huang, Michael S Benninger, Paul C Bryson
Objective To describe the profile and outcomes of elderly patients undergoing medialization laryngoplasty for vocal cord paralysis. Study Design Case series with retrospective review. Setting Tertiary care hospital. Subjects and Methods Patients were included in the study if they were >65 years old at the time of medialization laryngoplasty between 2008 and 2015. Patient comorbidities, anticoagulation status, disease etiology, and physical examination findings were recorded with postoperative length of stay, complications, pre- and posttreatment voice outcomes with the Voice Handicap Index (at 6 weeks, 4 months, 8 months, and 1 year), and postprocedural interventions (revision injections, surgery, therapy)...
July 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28719515/anaesthetic-management-of-patients-with-myopathies
#13
Mark Schieren, Jerome Defosse, Andreas Böhmer, Frank Wappler, Mark U Gerbershagen
: The anaesthetic management of patients with myopathies is challenging. Considering the low incidence and heterogeneity of these disorders, most anaesthetists are unfamiliar with key symptoms, associated co-morbidities and implications for anaesthesia. The pre-anaesthetic assessment aims at the detection of potentially undiagnosed myopathic patients and, in case of known or suspected muscular disease, on the quantification of disease progression. Ancillary testing (e.g. echocardiography, ECG, lung function testing etc...
July 15, 2017: European Journal of Anaesthesiology
https://www.readbyqxmd.com/read/28717957/repellency-effect-of-an-imidacloprid-flumethrin-seresto-%C3%A2-controlled-release-polymer-matrix-collar-against-the-australian-paralysis-tick-ixodes-holocyclus-in-dogs
#14
Susan de Burgh, Kasia Hunter, Chrissie Jackson, Michael Chambers, Corinna Klupiec, Veronica Smith
No abstract text is available yet for this article.
July 17, 2017: Parasitology Research
https://www.readbyqxmd.com/read/28717033/vocal-fold-paralysis-on-positron-emission-tomography-ct
#15
Chelsea Ma, Zachary Ak Frosch, Beth Overmoyer, Avraham Z Cooper
No abstract text is available yet for this article.
July 17, 2017: Thorax
https://www.readbyqxmd.com/read/28716449/venom-derived-peptides-inhibiting-kir-channels-past-present-and-future
#16
REVIEW
Craig A Doupnik
Inwardly rectifying K+ (Kir) channels play a significant role in vertebrate and invertebrate biology by regulating the movement of K+ ions involved in membrane transport and excitability. Yet unlike other ion channels including their ancestral K + -selective homologs, there are very few venom toxins known to target and inhibit Kir channels with the potency and selectivity found for the Ca2+-activated and voltage-gated K+ channel families. It is unclear whether this is simply due to a lack of discovery, or instead a consequence of the evolutionary processes that drive the development of venom components towards their targets based on a collective efficacy to 1) elicit pain for defensive purposes, 2) promote paralysis for prey capture, or 3) facilitate delivery of venom components into the circulation...
July 14, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28716327/surgical-management-of-neurogenic-lower-urinary-tract-dysfunction
#17
REVIEW
Ronak A Gor, Sean P Elliott
Surgery for patients with neurogenic urinary tract dysfunction (nLUTD) is indicated when medical therapy fails, to correct conditions affecting patient safety, or when surgery can enhance the quality of life better than nonoperative management. Examples include failure of maximal medical therapy, inability to perform or aversion to clean intermittent catheterization, refractory incontinence, and complications from chronic, indwelling catheters. Adults with nLUTD have competing risk factors, including previous operations, obesity, poor nutritional status, complex living arrangements, impaired dexterity/paralysis, and impaired executive and cognitive function...
August 2017: Urologic Clinics of North America
https://www.readbyqxmd.com/read/28715529/changes-in-peak-airflow-measurement-during-maximal-cough-after-vocal-fold-augmentation-in-patients-with-glottic-insufficiency
#18
Gregory R Dion, Efstratios Achlatis, Stephanie Teng, Yixin Fang, Michael Persky, Ryan C Branski, Milan R Amin
Importance: Compromised cough effectiveness is correlated with dysphagia and aspiration. Glottic insufficiency likely yields decreased cough strength and effectiveness. Although vocal fold augmentation favorably affects voice and likely improves cough strength, few data exist to support this hypothesis. Objective: To assess whether vocal fold augmentation improves peak airflow measurements during maximal-effort cough following augmentation. Setting, Design, Participants: This case series study was conducted in a tertiary, academic laryngology clinic...
July 13, 2017: JAMA Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/28714989/correction-of-a-splicing-defect-in-a-mouse-model-of-congenital-muscular-dystrophy-type-1a-using-a-homology-directed-repair-independent-mechanism
#19
Dwi U Kemaladewi, Eleonora Maino, Elzbieta Hyatt, Huayun Hou, Maylynn Ding, Kara M Place, Xinyi Zhu, Prabhpreet Bassi, Zahra Baghestani, Amit G Deshwar, Daniele Merico, Hui Y Xiong, Brendan J Frey, Michael D Wilson, Evgueni A Ivakine, Ronald D Cohn
Splice-site defects account for about 10% of pathogenic mutations that cause Mendelian diseases. Prevalence is higher in neuromuscular disorders (NMDs), owing to the unusually large size and multi-exonic nature of genes encoding muscle structural proteins. Therapeutic genome editing to correct disease-causing splice-site mutations has been accomplished only through the homology-directed repair pathway, which is extremely inefficient in postmitotic tissues such as skeletal muscle. Here we describe a strategy using nonhomologous end-joining (NHEJ) to correct a pathogenic splice-site mutation...
July 17, 2017: Nature Medicine
https://www.readbyqxmd.com/read/28714203/posterior-cordotomy-in-bilateral-vocal-cord-paralysis-using-monopolar-microelectrodes-and-radiofrequency-in-18-patients
#20
J Basterra, Y Castillo-Lopez, R Reboll, E Zapater, C Olavarria, F Krause, R Hernandez, M Torres, F Tocornal
Posterior cordotomy is an option in the surgical treatment of bilateral vocal cord paralysis in adduction. We present results obtained in 18 patients with bilateral vocal cord paralysis in adduction treated using microelectrodes terminal with tips in several angles, and a radiofrequency generator from 2008 to 2013. The functional outcome was evaluated using postoperative spirometry, the decannulation time, and swallowing. All patients except one were successfully decannulated and showed an increase in mid-inspiratory flow rates...
July 17, 2017: Clinical Otolaryngology
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