28 results on '"Hani Kushlaf"'
Search Results
2. Patient preference for virtual versus in‐person visits in neuromuscular clinical practice
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Komal Hafeez, Hani Kushlaf, Husam Al‐Sultani, Anny‐Claude Joseph, Zoya Zaeem, Zaeem Siddiqi, Shannon Laboy, Michael Pulley, Ali A. Habib, Nathaniel M. Robbins, Sean Zadeh, Muhammad Ubaid Hafeez, Yessar Hussain, Alexandria Melendez‐Zaidi, Charles Kassardjian, Kristin Johnson, Holly Leonhard, Suur Biliciler, Jorge E. Patino Murillas, and Aziz I. Shaibani
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Cellular and Molecular Neuroscience ,Physiology ,Communication ,Surveys and Questionnaires ,Physiology (medical) ,Humans ,Patient Preference ,Neurology (clinical) ,Telemedicine - Abstract
It is unknown if patients with neuromuscular diseases prefer in-person or virtual telemedicine visits. We studied patient opinions and preference on virtual versus in-person visits, and the factors influencing such preferences.Telephone surveys, consisting of 11 questions, of patients from 10 neuromuscular centers were completed.Five hundred and twenty surveys were completed. Twenty-six percent of respondents preferred virtual visits, while 50% preferred in-person visits. Sixty-four percent reported physical interaction as "very important." For receiving a new diagnosis, 55% preferred in-person vs 35% reporting no preference. Forty percent were concerned about a lack of physical examination vs 20% who were concerned about evaluating vital signs. Eighty four percent reported virtual visits were sufficiently private. Sixty eight percent did not consider expenses a factor in their preference. Although 92% were comfortable with virtual communication technology, 55% preferred video communications, and 19% preferred phone calls. Visit preference was not significantly associated with gender, diagnosis, disease severity, or symptom management. Patients who were concerned about a lack of physical exam or assessment of vitals had significantly higher odds of selecting in-person visits than no preference.Although neither technology, privacy, nor finance burdened patients in our study, more patients preferred in-person visits than virtual visits and 40% were concerned about a lack of physical examination. Interactions that occur with in-person encounters had high importance for patients, reflecting differences in the perception of the patient-physician relationship between virtual and in-person visits.
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- 2022
3. Safety and efficacy of cipaglucosidase alfa plus miglustat versus alglucosidase alfa plus placebo in late-onset Pompe disease (PROPEL): an international, randomised, double-blind, parallel-group, phase 3 trial
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Sheela Sitaraman, Richard Roxburgh, Kristina Gutschmidt, Ela Stefanescu, Drago Bratkovic, Thomas Burrow, Kornblum Cornelia, Kristl Claeys, Miriam Freimer, Ozlem Goker-Alpan, Srilakshmi Kuchipudi, Alan Pestronk, Wolfgang Löscher, Francoise Bouhour, Maria Judit Molnar, Ans T. van der Ploeg, Halina Bartosik-Psujek, Mitchell Goldman, Robert D. Henderson, Stephanie Dearmey, Colin Quinn, Paula R. Clemens, Priya S. Kishnani, Jennifer B Avelar, Nicola Longo, Shahram Attarian, Robert Hopkin, Tomo Sawada, Blaž Koritnik, George Konstantinos Papadimas, Hideaki Shiraishi, Christopher Lindberg, Jin-Hong Shin, Ivaylo Tarnev, Tahseen Mozaffar, Heather Lau, Michel Tchan, Jozsef Janszky, Tobias Ruck, Sabrina Sacconi, Benedikt Schoser, Hashiguchi Akihiro, Patrick Deegan, Ernest Butler, Nuria Vidal-Fernandez, Antonio Toscano, Tarekegn Hiwot, Gee Kim, Emmanuelle Salort-Campana, Jeff Castelli, Pascal Laforet, Céline Tard, Crystal Eldridge, Aneal Khan, Stephan Wenninger, Simona Fecarotta, Jordi Díaz-Manera, Jorge Alonso-Pérez, Yin-Hsiu Chien, Mark Tarnopolsky, Olimpia Musumeci, Hiroshi Kobayashi, Helio Pedro, Jonathan Cauci, Agnes Sebok, Cynthia Bodkin, Hai Jiang, Julie Berthy, Vescei Laszlo, Derralynn Hughes, David Reyes-Leiva, Aleksandra Dominovic-Kovacevic, Mazen M. Dimachkie, Hernan Amartino, Hani Kushlaf, Barry J. Byrne, Giancarlo Parenti, Henning Andersen, Mark Roberts, Marie Wencel, Jaime Vengoechea, Schoser, B., Roberts, M., Byrne, B. J., Sitaraman, S., Jiang, H., Laforet, P., Toscano, A., Castelli, J., Diaz-Manera, J., Goldman, M., van der Ploeg, A. T., Bratkovic, D., Kuchipudi, S., Mozaffar, T., Kishnani, P. S., Sebok, A., Pestronk, A., Dominovic-Kovacevic, A., Khan, A., Koritnik, B., Tard, C., Lindberg, C., Quinn, C., Eldridge, C., Bodkin, C., Reyes-Leiva, D., Hughes, D., Stefanescu, E., SALORT-CAMPANA, E., Butler, E., Bouhour, F., Kim, G., Konstantinos Papadimas, G., Parenti, G., Bartosik-Psujek, H., Kushlaf, H., Akihiro, H., Lau, H., Pedro, H., Andersen, H., Amartino, H., Shiraishi, H., Kobayashi, H., Tarnev, I., Vengoechea, J., Avelar, J., Shin, J. -H., Cauci, J., Alonso-Perez, J., Janszky, J., Berthy, J., Cornelia, K., Gutschmidt, K., Claeys, K., Judit Molnar, M., Wencel, M., Tarnopolsky, M., Dimachkie, M., Tchan, M., Freimer, M., Longo, N., Vidal-Fernandez, N., Musumeci, O., Goker-Alpan, O., Deegan, P., Clemens, P. R., Roxburgh, R., Henderson, R., Hopkin, R., Sacconi, S., Fecarotta, S., Attarian, S., Wenninger, S., Dearmey, S., Hiwot, T., Burrow, T., Ruck, T., Sawada, T., Laszlo, V., Loscher, W., Chien, Y. -H., and Pediatrics
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education.field_of_study ,medicine.medical_specialty ,1-Deoxynojirimycin ,Adolescent ,Glycogen Storage Disease Type II ,business.industry ,Population ,alpha-Glucosidases ,Enzyme replacement therapy ,Placebo ,Treatment Outcome ,Double-Blind Method ,SDG 3 - Good Health and Well-being ,Internal medicine ,Miglustat ,medicine ,Clinical endpoint ,Humans ,Respiratory function ,Neurology (clinical) ,Adverse effect ,education ,business ,Alglucosidase alfa ,medicine.drug - Abstract
Summary Background Pompe disease is a rare disorder characterised by progressive loss of muscle and respiratory function due to acid α-glucosidase deficiency. Enzyme replacement therapy with recombinant human acid α-glucosidase, alglucosidase alfa, is the first approved treatment for the disease, but some patients do not respond, and many do not show a sustained benefit. We aimed to assess the safety and efficacy of an investigational two-component therapy (cipaglucosidase alfa, a novel recombinant human acid α-glucosidase, plus miglustat, an enzyme stabiliser) for late-onset Pompe disease. Methods We did a randomised, double-blind, parallel-group, phase 3 trial at 62 neuromuscular and metabolic medical centres in 24 countries in the Americas, Asia-Pacific, and Europe. Eligible participants were aged 18 years or older with late-onset Pompe disease, and had either been receiving alglucosidase alfa for at least 2 years or were enzyme replacement therapy-naive. Participants were randomly assigned (2:1) using interactive response technology software, stratified by 6-min walk distance and previous enzyme replacement therapy status, to intravenous cipaglucosidase alfa (20 mg/kg) plus oral miglustat or to intravenous alglucosidase alfa (20 mg/kg) plus oral placebo once every 2 weeks for 52 weeks. Patients, investigators, and outcome assessors were masked to treatment assignment. The primary endpoint was change from baseline to week 52 in 6-min walk distance, assessed using a mixed-effect model for repeated measures analysis for comparison of superiority in the intention-to-treat population (all patients who received at least one dose of study drug). This study is now complete and is registered with ClinicalTrials.gov , NCT03729362 . Findings Between Dec 3, 2018, and Nov 26, 2019, 130 patients were screened for eligibility and 125 were enrolled and randomly assigned to receive cipaglucosidase alfa plus miglustat (n=85) or alglucosidase alfa plus placebo (n=40). Two patients in the alglucosidase alfa plus placebo group did not receive any dose due to absence of genotype confirmation of late-onset Pompe disease and were excluded from analysis. Six patients discontinued (one in the alglucosidase alfa plus placebo group, five in the cipaglucosidase alfa plus miglustat group), and 117 completed the study. At week 52, mean change from baseline in 6-min walk distance was 20·8 m (SE 4·6) in the cipaglucosidase alfa plus miglustat group versus 7·2 m (6·6) in the alglucosidase alfa plus placebo group using last observation carried forward (between-group difference 13·6 m [95% CI −2·8 to 29·9]). 118 (96%) of 123 patients experienced at least one treatment-emergent adverse event during the study; the incidence was similar between the cipaglucosidase alfa plus miglustat group (n=81 [95%]) and the alglucosidase alfa plus placebo group (n=37 [97%]). The most frequently reported treatment-emergent adverse events were fall (25 [29%] patients in the cipaglucosidase alfa plus miglustat group vs 15 [39%] in the alglucosidase alfa plus placebo group), headache (20 [24%] vs 9 [24%]), nasopharyngitis (19 [22%] vs 3 [8%]), myalgia (14 [16%] vs 5 [13%]), and arthralgia (13 [15%]) vs 5 [13%]). 12 serious adverse events occurred in eight patients in the cipaglucosidase alfa plus miglustat group; only one event (anaphylaxis) was deemed related to study drug. One serious adverse event (stroke) occurred in the alglucosidase alfa plus placebo group, which was deemed unrelated to study drug. There were no deaths. Interpretation Cipaglucosidase alfa plus miglustat did not achieve statistical superiority to alglucosidase alfa plus placebo for improving 6-min walk distance in our overall population of patients with late-onset Pompe disease. Further studies should investigate the longer-term safety and efficacy of cipaglucosidase alfa plus miglustat and whether this investigational two-component therapy might provide benefits, particularly in respiratory function and in patients who have been receiving enzyme replacement therapy for more than 2 years, as suggested by our secondary and subgroup analyses. Funding Amicus Therapeutics.
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- 2021
4. Current status of clinical outcome measures in inclusion body myositis: a systematised review
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Bhaskar Roy, Matteo Lucchini, James B. Lilleker, Namita A. Goyal, Elie Naddaf, Brittany Adler, Lindsay N. Alfano, Georgia A. Malandraki, Kendrea L. (Focht) Garand, David Mochel, Umesh Badrising, Pedro M. Machado, Ruben Pagkatipunan, Leo Wang, Melissa C. Funaro, Jens Schmidt, Hani Kushlaf, Elena Schiopu, Kaila Stipancic, Neelam Goyal, Miriana d'Alessandro, Edoardo Conticini, Betsaida Cruz-Coble, and Thomas E. Lloyd
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Rheumatology ,Immunology ,Immunology and Allergy - Published
- 2022
5. Efficacy and safety of avalglucosidase alfa in participants with late-onset Pompe disease after 145 weeks of treatment during the COMET trial
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Priya S. Kishnani, Jordi Díaz-Manera, Hani Kushlaf, Shafeeq Ladha, Tahseen Mozaffar, Volker Straub, Antonio Toscano, Ans T. van der Ploeg, Paula R. Clemens, John W. Day, Sergey Illarioshkin, Mark E. Roberts, Shahram Attarian, Gerson Carvalho, Sevim Erdem-Özdamar, Ozlem Goker-Alpan, Anna Kostera-Pruszczyk, Kristina An Haack, Olivier Huynh-Ba, Swathi Tammireddy, Nathan Thibault, Tianyue Zhou, Mazen M. Dimachkie, and Benedikt Schoser
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Endocrinology ,Endocrinology, Diabetes and Metabolism ,Genetics ,Molecular Biology ,Biochemistry - Published
- 2023
6. Extracellular amyloid deposition in sporadic inclusion body myositis: Further insights
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Hani Kushlaf and Kariem Ezzat
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Pathology ,medicine.medical_specialty ,Amyloid beta-Peptides ,biology ,Physiology ,business.industry ,Amyloidosis ,Sporadic Inclusion Body Myositis ,medicine.disease ,Myositis, Inclusion Body ,Cellular and Molecular Neuroscience ,Transthyretin ,Amyloid deposition ,Physiology (medical) ,biology.protein ,medicine ,Extracellular ,Humans ,Neurology (clinical) ,Inclusion body myositis ,medicine.symptom ,business ,Myopathy - Published
- 2021
7. The applicability of the myasthenia gravis patient registry data to disease burden and medication changes in clinical practice
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Hani Kushlaf
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Cellular and Molecular Neuroscience ,Cost of Illness ,Physiology ,Physiology (medical) ,Myasthenia Gravis ,Humans ,Registries ,Neurology (clinical) ,Thymectomy - Published
- 2022
8. Steps in Inclusion Body Myositis
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Thomas Trevor and Hani Kushlaf
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General Medicine - Abstract
Not applicable. This is a poem.
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- 2021
9. Immune profiling beyond antibodies in double seronegative and acetylcholine receptor antibody positive myasthenia gravis: What do we learn?
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Hani Kushlaf
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medicine.diagnostic_test ,biology ,Physiology ,business.industry ,medicine.disease ,Article ,Myasthenia gravis ,Flow cytometry ,Immune profiling ,Cellular and Molecular Neuroscience ,Acetylcholine receptor antibody ,Physiology (medical) ,Myasthenia Gravis ,Immunology ,medicine ,biology.protein ,Humans ,Biomarker (medicine) ,Receptors, Cholinergic ,Neurology (clinical) ,Antibody ,business ,Autoantibodies - Abstract
INTRODUCTION/AIM: The immunopathology of autoimmune seronegative myasthenia gravis (SN MG) is poorly understood. Our objective was to determine immune profiles associated with a diagnosis of SN MG. METHODS: We performed high-dimensional flow cytometry on blood samples from SN MG patients (N=68), healthy controls (N=46), and acetylcholine receptor antibody (AChR+) MG patients (N=27). We compared 12 immune cell subsets in SN MG to controls using logistic modeling via a discovery-replication design. An exploratory analysis fit a multinomial model comparing AChR+ MG and controls to SN MG. RESULTS: An increase in CD19(+)CD20(-)CD38(hi) plasmablast frequencies was associated with lower odds of being a SN MG case in both the discovery and replication analyses (discovery p-value=0.0003, replication p-value=0.0021). IL-21 producing helper T cell frequencies were associated with a diagnosis of AChR+ MG (p=0.004). DISCUSSION: Reduced plasmablast frequencies are strongly associated with a SN MG diagnosis and may be a useful diagnostic biomarker in the future.
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- 2021
10. The avalglucosidase alfa phase 3 COMET trial in late-onset Pompe disease patients: Efficacy and safety results after 97 weeks
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Priya Kishnani, Jordi Díaz-Manera, Hani Kushlaf, Shafeeq Ladha, Tahseen Mozaffar, Volker Straub, Antonio Toscano, Ans T. van der Ploeg, Kenneth I. Berger, Paula R. Clemens, Yin-Hsiu Chien, John W. Day, Sergey Illarioshkin, Mark Roberts, Shahram Attarian, Gerson Carvalho, Young-Chul Choi, Sevim Erdem-Özdamar, Ozlem Goker-Alpan, Anna Kostera-Pruszczyk, Kristina An Haack, Nathan Thibault, Tianyue Zhou, Mazen M. Dimachkie, and Benedikt Schoser
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Endocrinology ,Endocrinology, Diabetes and Metabolism ,Genetics ,Molecular Biology ,Biochemistry - Published
- 2022
11. Update on the Diagnostic and Therapeutic Landscape of Sporadic Inclusion Body Myositis
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Hani Kushlaf
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Oncology ,medicine.medical_specialty ,Neurology ,business.industry ,Sporadic Inclusion Body Myositis ,Arimoclomol ,medicine.disease ,Clinical trial ,chemistry.chemical_compound ,chemistry ,Internal medicine ,Sirolimus ,medicine ,Neurology (clinical) ,Inclusion body myositis ,business ,Bimagrumab ,medicine.drug - Abstract
The purpose of this paper is to review the recent findings that pertain to the diagnosis and treatment of sporadic inclusion body myositis. New evidence highlighting lack of correlation between the presence of cytosolic 5′-nucleotidase 1A antibody with any of the clinical features, or laboratory findings in inclusion body myositis. New studies emphasizing heterogeneity and showing separation of inclusion body myositis patients into separate trajectories. The failure of bimagrumab and arimoclomol as potential treatments of inclusion body myositis and the mixed results of the sirolimus trial in sporadic inclusion body myositis. A significant gap exists in understanding the heterogeneity of sporadic inclusion body myositis. Recent evidence suggests that cytosolic 5′-nucleotidase 1A antibody does not provide significant clinical or laboratory differentiation between antibody-positive and negative patients. Despite recent failures in the clinical trials of arimoclomol and bimagrumab, sirolimus showed mixed results, and a larger definitive trial is needed.
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- 2021
12. Diving into the Heterogeneity of Inclusion Body Myositis
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Hani Kushlaf
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musculoskeletal diseases ,Clinical Research Article ,medicine.medical_specialty ,longitudinal modeling ,Physiology ,business.industry ,inclusion body myositis ,functional status ,medicine.disease ,dynamometry ,Myositis, Inclusion Body ,Clinical trial ,Cellular and Molecular Neuroscience ,Physiology (medical) ,Internal medicine ,parasitic diseases ,muscle strength ,medicine ,Humans ,trial outcomes ,Neurology (clinical) ,Inclusion body myositis ,business ,myositis ,Clinical Research Articles - Abstract
Introduction Objective information on longitudinal disease progression in inclusion body myositis (IBM) is lacking. Methods Longitudinal dynamometry and functional status data were collated from a cohort of IBM patients. Annual change was calculated by means of linear modeling. Trajectories of change in grip, knee extension, IBM Functional Rating Scale (IBM‐FRS) and Neuromuscular Symptom Score (NSS) were identified by means of latent growth mixture modeling. Results Data were collated from 75 IBM patients (348 person‐years follow‐up). Annual strength loss was greatest for pinch (−10%) and knee extension (−4%). Functional deterioration was greatest for males. Three distinct trajectory groups were identified. Rapid deterioration trajectory for grip strength was associated with younger diagnosis age. Rapid deterioration for knee extension strength was associated with older age of diagnosis. Discussion This study has quantified strength change in IBM and identified distinct trajectory groups, which will aid prognostication and stratification for inclusion into future clinical trials., See editorial on pages 7–9 in this issue.
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- 2020
13. How does rituximab fit in the treatment algorithm of myasthenia gravis?
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Yuebing Li and Hani Kushlaf
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medicine.medical_specialty ,Side effect ,Physiology ,business.industry ,medicine.disease ,Dermatology ,Myasthenia gravis ,Cellular and Molecular Neuroscience ,Physiology (medical) ,Myasthenia Gravis ,medicine ,Humans ,Receptors, Cholinergic ,Rituximab ,Neurology (clinical) ,business ,Algorithms ,medicine.drug - Published
- 2021
14. A rare overlap of statin-induced anti-3-hydroxy-3-methyl-glutaryl-coenzyme A necrotizing autoimmune myositis and dermatomyositis
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Priya Parikh, Nneoma Onuorah, Hani Kushlaf, and Priyanka Vashisht
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medicine.medical_specialty ,Statin ,business.industry ,medicine.drug_class ,Glutaryl-coenzyme A ,Dermatomyositis ,medicine.disease ,Gastroenterology ,Letter to the Editor (Case report) ,Autoimmune myositis ,Rheumatology ,Internal medicine ,medicine ,AcademicSubjects/MED00010 ,business - Published
- 2021
15. Toxin-Induced Channelopathies, Neuromuscular Junction Disorders, and Myopathy
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Hani Kushlaf and Jacqueline Janecek
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Ciguatera ,Ciguatoxin ,Bioinformatics ,03 medical and health sciences ,chemistry.chemical_compound ,0302 clinical medicine ,Muscular Diseases ,Medicine ,Humans ,Botulism ,030212 general & internal medicine ,Myopathy ,Toxins, Biological ,business.industry ,fungi ,food and beverages ,medicine.disease ,Neuromuscular Junction Diseases ,people.cause_of_death ,Botulinum toxin ,medicine.anatomical_structure ,chemistry ,Venomous snake ,Peripheral nervous system ,Tetrodotoxin ,Channelopathies ,Neurotoxicity Syndromes ,Neurology (clinical) ,medicine.symptom ,people ,business ,030217 neurology & neurosurgery ,medicine.drug - Abstract
Channelopathies, neuromuscular junction disorders, and myopathies represent multiple mechanisms by which toxins can affect the peripheral nervous system. These toxins include ciguatoxin, tetrodotoxin, botulinum toxin, metabolic poisons, venomous snake bites, and several medications. These toxins are important to be aware of because they can lead to serious symptoms, disability, or even death, and many can be treated if recognized ear.
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- 2020
16. Cystinosis myopathy: Searching for optimal clinical outcome measures
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Hani Kushlaf
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Pediatrics ,medicine.medical_specialty ,Physiology ,business.industry ,Cystinosis ,Outcome measures ,medicine.disease ,Natural history ,Distal Myopathies ,Cellular and Molecular Neuroscience ,Muscular Diseases ,Physiology (medical) ,Outcome Assessment, Health Care ,medicine ,Humans ,Neurology (clinical) ,medicine.symptom ,Myopathy ,business ,Deglutition Disorders - Published
- 2020
17. Comparing Four Medicines to Treat Pain from Cryptogenic Sensory Polyneuropathy—The PAIN-CONTRoLS Study
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James Wymer, Tuan Vu, Yuebing Li, Pariwat Thaisetthawatkul, M Jacoby, Andrea Swenson, Aiesha Ahmed, Jau Shin Lou, Mamatha Pasnoor, Ted M. Burns, C Parks, Michael K. Hehir, David Walk, Stephen N. Scelsa, Shafeeq Ladha, Ghazala Hayat, Gordon Smith, Jaya Trivedi, Byron J. Gajewski, William Mallonee, Richard J. Barohn, P Shlemon, Omar Jawdat, Robert M. Pascuzzi, Matthew Wicklund, Tiyonnoh M. Cash, Noah Kolb, Sindhu Ramchandren, Jeffrey W. Ralph, L Brown, Paul Twydell, Hani Kushlaf, Gil I. Wolfe, Mazen M. Dimachkie, S Austin, Michael Pulley, Y Hussainn, David Saperstein, Stanley Iyadurai, Dianna Quan, T Liu, Chafic Karam, Amro M. Stino, D Heitzman, Anza B. Memon, Thomas H. Brannagan, A Tobon, Khema Sharma, M Ahmed, Kim S. Kimminau, Vera Bril, John T. Kissel, Christen Kutz, N Verma, M Bazant, Richard A. Lewis, Suur Biliciler, Alexandru Barboi, K Salajegheh, and Laura Herbelin
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medicine.medical_specialty ,business.industry ,Internal medicine ,Sensory polyneuropathy ,Medicine ,business - Published
- 2020
18. Needle electromyography practice patterns in patients taking novel oral anticoagulants: A survey‐based study
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Hani Kushlaf and Ikjae Lee
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medicine.medical_specialty ,Needle emg ,Physiology ,Facial Muscles ,Hemorrhage ,030204 cardiovascular system & hematology ,Risk Assessment ,Teaching hospital ,03 medical and health sciences ,Cellular and Molecular Neuroscience ,0302 clinical medicine ,Muscle nerve ,Physical medicine and rehabilitation ,Surveys and Questionnaires ,Physiology (medical) ,medicine ,Humans ,In patient ,Prospective Studies ,Muscle, Skeletal ,Needle electromyography ,Electromyography ,Practice patterns ,business.industry ,Anticoagulants ,Facial muscles ,Cross-Sectional Studies ,medicine.anatomical_structure ,Needles ,Neurology (clinical) ,business ,030217 neurology & neurosurgery ,Paraspinal Muscle - Abstract
INTRODUCTION This study sought to evaluate needle electromyography (EMG) practice patterns among electromyographers with patients taking novel oral anticoagulants (NOAC). METHODS A survey questionnaire was sent to members of the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) via email web link. Anonymous survey responses were collected through an online website. RESULTS Fifty-eight AANEM members responded, 28 (48%) of whom worked at a teaching hospital and 30 (52%) of whom worked in a private setting. Fifty-four (93%) responders perform needle EMG on patients taking NOACs. Twenty-nine (50%) responders examine paraspinal muscles, and 20 (40%) responders examine facial muscles. Among 14 responders who perform single-fiber EMG (SFEMG), 8 examine patients taking NOACs. DISCUSSION Although most of the electromyographers perform needle EMG on patients taking NOACs, they reported variable practice patterns in examination of the paraspinal and facial muscles and in performing SFEMG. A prospective risk-benefit study is required. Muscle Nerve 58: 307-309, 2018.
- Published
- 2018
19. The evidence is stacked against thymectomy in MuSK myasthenia gravis
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Hani, Kushlaf and Yuebing, Li
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Myasthenia Gravis ,Humans ,Receptor Protein-Tyrosine Kinases ,Receptors, Cholinergic ,Thymectomy - Published
- 2019
20. Genetic Modifiers of Hereditary Neuromuscular Disorders and Cardiomyopathy
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Sakthivel Sadayappan, Hani Kushlaf, Mashhood Kakroo, John G. Quinlan, Sholeh Bazrafshan, and Richard C. Becker
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Adult ,Male ,muscular dystrophy ,Cardiomyopathy ,Disease ,neuromuscular disorders ,030204 cardiovascular system & hematology ,Gene mutation ,Bioinformatics ,Article ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,genotype–phenotype relationships ,Muscular dystrophy ,Myopathy ,lcsh:QH301-705.5 ,Aged ,SGCA ,Genes, Modifier ,business.industry ,Bethlem myopathy ,Neuromuscular Diseases ,General Medicine ,Middle Aged ,medicine.disease ,lcsh:Biology (General) ,Female ,next-generation sequencing ,medicine.symptom ,Cardiomyopathies ,business ,cardiomyopathy ,030217 neurology & neurosurgery ,Comparative genomic hybridization - Abstract
Novel genetic variants exist in patients with hereditary neuromuscular disorders (NMD), including muscular dystrophy. These patients also develop cardiac manifestations. However, the association between these gene variants and cardiac abnormalities is understudied. To determine genetic modifiers and features of cardiac disease in NMD patients, we have reviewed electronic medical records of 651 patients referred to the Muscular Dystrophy Association Care Center at the University of Cincinnati and characterized the clinical phenotype of 14 patients correlating with their next-generation sequencing data. The data were retrieved from the electronic medical records of the 14 patients included in the current study and comprised neurologic and cardiac phenotype and genetic reports which included comparative genomic hybridization array and NGS. Novel associations were uncovered in the following eight patients diagnosed with Limb-girdle Muscular Dystrophy, Bethlem Myopathy, Necrotizing Myopathy, Charcot-Marie-Tooth Disease, Peripheral Polyneuropathy, and Valosin-containing Protein-related Myopathy. Mutations in COL6A1, COL6A3, SGCA, SYNE1, FKTN, PLEKHG5, ANO5, and SMCHD1 genes were the most common, and the associated cardiac features included bundle branch blocks, ventricular chamber dilation, septal thickening, and increased outflow track gradients. Our observations suggest that features of cardiac disease and modifying gene mutations in patients with NMD require further investigation to better characterize genotype–phenotype relationships.
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- 2021
21. Efficacy and safety results of the avalglucosidase alfa phase 3 COMET trial in late-onset Pompe disease patients
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John W. Day, Tianyue Zhou, Mazen M. Dimachkie, Joao Lindolfo Borges, Sevim Erdem-Ozdamar, Anna Kostera-Pruszczyk, Shafeeq Ladha, Ozlem Goker-Alpan, Yin-Hsiu Chien, Tahseen Mozaffar, Shahram Attarian, Ans T. van der Ploeg, Priya S. Kishnani, Jordi Díaz-Manera, Kristina An Haack, Young Chul Choi, Olivier Huynh-Ba, Paula R. Clemens, Volker Straub, Mark Roberts, Françoise Bouhour, Hani Kushlaf, Antonio Toscano, Benedikt Schoser, and Sergey Illarioshkin
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medicine.medical_specialty ,Endocrinology ,business.industry ,Endocrinology, Diabetes and Metabolism ,Internal medicine ,Comet ,Genetics ,medicine ,Late onset ,business ,Molecular Biology ,Biochemistry ,Gastroenterology - Published
- 2021
22. Patient Assisted Intervention for Neuropathy: Comparison of Treatment in Real Life Situations (PAIN-CONTRoLS)
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Suur Biliciler, Chafic Karam, Alexandru Barboi, Michael K. Hehir, Ghazala Hayat, Paul Twydell, Gil I. Wolfe, Alexandra R. Brown, Pariwat Thaisetthawatkul, Dianna Quan, Moiz Ahmed, Mazen M. Dimachkie, Darryl Heitzman, Alejandro Tobon, Yuebing Li, Andrea Swenson, Jau Shin Lou, William Mallonee, Richard A. Lewis, John T. Kissel, Ted M. Burns, Mark Jacoby, Noah Kolb, Robert M. Pascuzzi, Jaya Trivedi, Tiyonnoh M. Cash, Thomas H. Brannagan, Jeffrey W. Ralph, Vera Bril, Amro M. Stino, Sindhu Ramchandren, Michael Pulley, Christen Kutz, Khema Sharma, Richard J. Barohn, Anza B. Memon, David Saperstein, Matthew Wicklund, Stanley Iyadurai, Navin Verma, Shafeeq Ladha, Gordon Smith, Kim S. Kimminau, Laura Herbelin, Dinesh Pal Mudaranthakam, Byron J. Gajewski, Mark Bazant, Hani Kushlaf, Mamatha Pasnoor, Aiesha Ahmed, Kian Salajegheh, Yessar Hussain, Sara Austin, Omar Jawdat, Tuan Vu, James Wymer, David Walk, and Stephen N. Scelsa
- Subjects
medicine.medical_specialty ,Randomization ,Diabetic neuropathy ,business.industry ,Pregabalin ,Interim analysis ,medicine.disease ,law.invention ,03 medical and health sciences ,chemistry.chemical_compound ,0302 clinical medicine ,Randomized controlled trial ,chemistry ,law ,Internal medicine ,Neuropathic pain ,Medicine ,Duloxetine ,030212 general & internal medicine ,Neurology (clinical) ,Nortriptyline ,business ,030217 neurology & neurosurgery ,medicine.drug - Abstract
Importance Cryptogenic sensory polyneuropathy (CSPN) is a common generalized slowly progressive neuropathy, second in prevalence only to diabetic neuropathy. Most patients with CSPN have significant pain. Many medications have been tried for pain reduction in CSPN, including antiepileptics, antidepressants, and sodium channel blockers. There are no comparative studies that identify the most effective medication for pain reduction in CSPN. Objective To determine which medication (pregabalin, duloxetine, nortriptyline, or mexiletine) is most effective for reducing neuropathic pain and best tolerated in patients with CSPN. Design, Setting, and Participants From December 1, 2014, through October 20, 2017, a bayesian adaptive, open-label randomized clinical comparative effectiveness study of pain in 402 participants with CSPN was conducted at 40 neurology care clinics. The trial included response adaptive randomization. Participants were patients with CSPN who were 30 years or older, with a pain score of 4 or greater on a numerical rating scale (range, 0-10, with higher scores indicating a higher level of pain). Participant allocation to 1 of 4 drug groups used the utility function and treatment’s sample size for response adaptation randomization. At each interim analysis, a decision was made to continue enrolling (up to 400 participants) or stop the whole trial for success (80% power). Patient engagement was maintained throughout the trial, which helped guide the study and identify ways to communicate and disseminate information. Analysis was performed from December 11, 2015, to January 19, 2018. Interventions Participants were randomized to receive nortriptyline (n = 134), duloxetine (n = 126), pregabalin (n = 73), or mexiletine (n = 69). Main Outcomes and Measures The primary outcome was a utility function that was a composite of the efficacy (participant reported pain reduction of ≥50% from baseline to week 12) and quit (participants who discontinued medication) rates. Results Among the 402 participants (213 men [53.0%]; mean [SD] age, 60.1 [13.4] years; 343 White [85.3%]), the utility function of nortriptyline was 0.81 (95% bayesian credible interval [CrI], 0.69-0.93; 34 of 134 [25.4%] efficacious; and 51 of 134 [38.1%] quit), of duloxetine was 0.80 (95% CrI, 0.68-0.92; 29 of 126 [23.0%] efficacious; and 47 of 126 [37.3%] quit), pregabalin was 0.69 (95% CrI, 0.55-0.84; 11 of 73 [15.1%] efficacious; and 31 of 73 [42.5%] quit), and mexiletine was 0.58 (95% CrI, 0.42-0.75; 14 of 69 [20.3%] efficacious; and 40 of 69 [58.0%] quit). The probability each medication yielded the highest utility was 0.52 for nortriptyline, 0.43 for duloxetine, 0.05 for pregabalin, and 0.00 for mexiletine. Conclusions and Relevance This study found that, although there was no clearly superior medication, nortriptyline and duloxetine outperformed pregabalin and mexiletine when pain reduction and undesirable adverse effects are combined to a single end point. Trial Registration ClinicalTrials.gov Identifier:NCT02260388
- Published
- 2021
23. Neurologic and neuroscience education: Mitigating neurophobia to mentor health care providers
- Author
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Kathryn S. Nevel, Jimmy V. Berthaud, Pritha Ghosh, Hani Kushlaf, Miguel Chuquilin, Rachel Gottlieb-Smith, Sneha Mantri, Daniel L. Menkes, Neil Masangkay, Logan Schneider, Harini Sarva, Stefano Sandrone, and Jacquelyne Cios
- Subjects
medicine.medical_specialty ,Neurology ,business.industry ,Attitude of Health Personnel ,Mentors ,Neurosciences ,Primary care ,United States ,Phobic disorder ,03 medical and health sciences ,0302 clinical medicine ,Phobic Disorders ,Health care ,Medicine ,Humans ,030212 general & internal medicine ,Neurology (clinical) ,business ,Neuroscience ,Delivery of Health Care ,030217 neurology & neurosurgery - Abstract
Neurologic disorders are among the most frequent causes of morbidity and mortality in the United States. Moreover, the current shortfall of neurologists is expected to worsen over the coming decade. As a consequence, many patients with neurologic disorders will be treated by physicians and primary care providers without formal neurologic training. Furthermore, a pervasive and well-described fear of neurology, termed neurophobia, has been identified in medical student cohorts, residents, and among general practitioners. In this article, members of the American Academy of Neurology A.B. Baker Section on Neurological Education review current guidelines regarding neurologic and neuroscience education, contextualize the genesis and the negative consequences of neurophobia, and provide strategies to mitigate it for purposes of mentoring future generations of health care providers.
- Published
- 2018
24. The evidence is stacked against thymectomy in MuSK myasthenia gravis
- Author
-
Yuebing Li and Hani Kushlaf
- Subjects
Physiology ,business.industry ,medicine.medical_treatment ,Receptor Protein-Tyrosine Kinases ,Pharmacology ,medicine.disease ,Myasthenia gravis ,Thymectomy ,Cellular and Molecular Neuroscience ,Physiology (medical) ,Medicine ,Cholinergic ,Neurology (clinical) ,business ,Receptor - Published
- 2019
25. New onset of seropositive generalized myasthenia gravis following intravesical bacille Calmette-Guerin treatment for bladder cancer: A case study
- Author
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Hani Kushlaf and Long Davalos
- Subjects
Male ,0301 basic medicine ,medicine.medical_specialty ,Physiology ,Antineoplastic Agents ,Bacille Calmette Guerin ,Gastroenterology ,New onset ,03 medical and health sciences ,Cellular and Molecular Neuroscience ,0302 clinical medicine ,Physiology (medical) ,Internal medicine ,Myasthenia Gravis ,medicine ,Humans ,Generalized myasthenia ,Aged ,Bladder cancer ,business.industry ,medicine.disease ,Myasthenia gravis ,Administration, Intravesical ,030104 developmental biology ,Urinary Bladder Neoplasms ,030220 oncology & carcinogenesis ,BCG Vaccine ,Intravesical bcg ,Immunotherapy ,Neurology (clinical) ,business ,Follow-Up Studies - Published
- 2018
26. Ultrasound diagnosis of bony nerve entrapment: Case series and literature review
- Author
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Giuseppe Granata, Mauro Giannini, Simon Podnar, Lisa D. Hobson-Webb, Carlo Martinoli, Giovanna Liotta, Fraser J. Leversedge, Hani Kushlaf, Luca Padua, and Carmen Erra
- Subjects
medicine.medical_specialty ,Physiology ,business.industry ,Callus formation ,Radiographic imaging ,Ultrasound ,Bone fracture ,Nerve entrapment ,medicine.disease ,Surgery ,Cellular and Molecular Neuroscience ,Peripheral nerve ,Physiology (medical) ,medicine ,Neurology (clinical) ,Ultrasonography ,business ,Complication - Abstract
Introduction: Nerve entrapment due to osseous callus formation is a rare complication after bone fracture. Electrodiagnostic studies and routine radiographic imaging often fail to demonstrate the pathology. The diagnosis is difficult and is often made incidentally upon surgical exploration. Nerve ultrasonography has not been used routinely to assess such lesions. Methods: We report 5 cases of nerve entrapment in osseous callus after fractures that occurred in 2011 and 2012. The diagnosis was made by ultrasound (US). We then performed a review of the relevant literature. Conclusions: US is becoming an invaluable tool for diagnosing peripheral nerve entrapments. The current cases suggest that nerve US should be strongly considered as an adjunctive diagnostic tool for nerve palsies developing after trauma. Muscle Nerve 48: 445–450, 2013
- Published
- 2013
27. Emerging toxic neuropathies and myopathies
- Author
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Hani Kushlaf
- Subjects
medicine.medical_specialty ,Prescription Drugs ,Street drugs ,Neuromuscular Junction ,Antineoplastic Agents ,Pharmacology ,Diagnosis, Differential ,chemistry.chemical_compound ,Polyneuropathies ,Muscular Diseases ,medicine ,Humans ,Myopathy ,Cisplatin ,Bortezomib ,business.industry ,Ixabepilone ,Imatinib ,Hydroxychloroquine ,Dermatology ,Oxaliplatin ,Psychiatry and Mental health ,Neuropsychiatric systemic lupus erythematosus ,chemistry ,Toxicity ,Neurotoxicity Syndromes ,Neurology (clinical) ,Differential diagnosis ,medicine.symptom ,Hydroxymethylglutaryl-CoA Reductase Inhibitors ,business ,Immunosuppressive Agents ,medicine.drug - Abstract
There is a daunting list of toxins that can affect the peripheral nervous system, with new drugs and chemicals added to this list every year. This article focuses on some of the more recent toxic neuropathies and myopathies that have emerged from the medical literature. Among these are toxic myopathies caused by statins, daptomycin, imatinib, hydroxychloroquine, and highly active antiretroviral therapy; neuromuscular junction toxicity caused by tandutinib; toxic peripheral neuropathies caused by bortezomib, angel's trumpet, cisplatin, oxaliplatin, tumor necrosis factor α antagonists, cobalt-chromium, and ixabepilone; and a unique syndrome reported in workers exposed to aerosolized porcine neural tissue.
- Published
- 2011
28. Proximal median neuropathy caused by surgical manipulation of Masson tumor
- Author
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E. Wayne Massey, Hani Kushlaf, and Lisa D. Hobson-Webb
- Subjects
medicine.medical_specialty ,Physiology ,business.industry ,Median Neuropathy ,Hyperplasia ,medicine.disease ,Ultrasonography doppler ,Median nerve ,Cellular and Molecular Neuroscience ,Proximal Median Neuropathy ,Text mining ,Surgical Manipulation ,Physiology (medical) ,medicine.artery ,medicine ,Neurology (clinical) ,Radiology ,Brachial artery ,business - Published
- 2014
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