2022
Reliability of patient self-reports to clinician-assigned functional scores of inclusion body myositis
Roy B, Zubair A, Petschke K, O'Connor KC, Paltiel AD, Nowak RJ. Reliability of patient self-reports to clinician-assigned functional scores of inclusion body myositis. Journal Of The Neurological Sciences 2022, 436: 120228. PMID: 35344793, DOI: 10.1016/j.jns.2022.120228.Peer-Reviewed Original ResearchConceptsIBM functional rating scaleInclusion body myositisBody myositisInterrater correlation coefficientSporadic inclusion body myositisFine motor skillsImpaired ambulationFleiss kappa statisticFunctional scoresHand functionDisease progressionWhite CaucasiansClinical practiceNatural history dataRating ScaleKappa statisticsMyositisScoresMotor skillsIndividual questionsSubstantial agreementCorrelation coefficient analysisReliable indicatorParticipantsAmbulationClinical Reasoning: A 73-Year-Old Woman With Episodic Dysarthria and Horizontal Binocular Diplopia
Bower AS, Fisayo A, Baehring JM, Roy B. Clinical Reasoning: A 73-Year-Old Woman With Episodic Dysarthria and Horizontal Binocular Diplopia. Neurology 2022, 98: 767-772. PMID: 35264421, DOI: 10.1212/wnl.0000000000200347.Peer-Reviewed Original ResearchConceptsEMG findingsHorizontal diplopiaHorizontal binocular diplopiaPeripheral nerve hyperexcitabilitySodium channel inhibitorsImpaired abductionTongue fasciculationsTransient dysarthriaElectrodiagnostic studiesTongue deviationSymptomatic reliefClinical presentationNerve hyperexcitabilityNeurologic examinationElectrographic seizuresRight eyeBinocular diplopiaInflammatory conditionsSymptom frequencyOlder womenTransient episodesAdditional episodesMyokymiaSpontaneous depolarizationsChannel inhibitors
2021
Electronic health record derived-impact of COVID-19 on myasthenia gravis
Roy B, Kovvuru S, Nalleballe K, Onteddu SR, Nowak RJ. Electronic health record derived-impact of COVID-19 on myasthenia gravis. Journal Of The Neurological Sciences 2021, 423: 117362. PMID: 33639420, PMCID: PMC7895699, DOI: 10.1016/j.jns.2021.117362.Peer-Reviewed Original Research
2020
Immunosuppression in chronic autoimmune neurological disorders during the COVID-19 pandemic
Kovvuru S, Nalleballe K, Onteddu SR, Sharma R, Jasti M, Kapoor N, Veerapaneni K, Yadala S, Dandu V, Archer R, Nowak RJ, Roy B. Immunosuppression in chronic autoimmune neurological disorders during the COVID-19 pandemic. Journal Of The Neurological Sciences 2020, 420: 117230. PMID: 33256952, PMCID: PMC7837234, DOI: 10.1016/j.jns.2020.117230.Peer-Reviewed Original ResearchConceptsAutoimmune neuromuscular disorderImmunosuppressive therapyMultiple sclerosisChronic autoimmune neuromuscular disorderNeuromuscular disordersCOVID-19Electronic medical record dataAutoimmune neurological disordersRisk of hospitalizationOverall infection riskMedical record dataRegistry-based dataCorona Virus Disease 2019Expert opinion guidelinesCOVID-19 databaseClinical research platformImmunosuppressed patientsImmunotherapy regimensAdditional physiciansDisease 2019Diseases-10International ClassificationPatientsNeurological disordersHospitalizationClinical Effects of the Self-administered Subcutaneous Complement Inhibitor Zilucoplan in Patients With Moderate to Severe Generalized Myasthenia Gravis
Howard JF, Nowak RJ, Wolfe GI, Freimer ML, Vu TH, Hinton JL, Benatar M, Duda PW, MacDougall JE, Farzaneh-Far R, Kaminski HJ, Barohn R, Dimachkie M, Pasnoor M, Farmakidis C, Liu T, Colgan S, Benatar M, Bertorini T, Pillai R, Henegar R, Bromberg M, Gibson S, Janecki T, Freimer M, Elsheikh B, Matisak P, Genge A, Guidon A, David W, Habib A, Mathew V, Mozaffar T, Hinton J, Hewitt W, Barnett D, Sullivan P, Ho D, Howard J, Traub R, Chopra M, Kaminski H, Aly R, Bayat E, Abu-Rub M, Khan S, Lange D, Holzberg S, Khatri B, Lindman E, Olapo T, Sershon L, Lisak R, Bernitsas E, Jia K, Malik R, Lewis-Collins T, Nicolle M, Nowak R, Sharma A, Roy B, Nye J, Pulley M, Berger A, Shabbir Y, Sachdev A, Patterson K, Siddiqi Z, Sivak M, Bratton J, Small G, Kohli A, Fetter M, Vu T, Lam L, Harvey B, Wolfe G, Silvestri N, Patrick K, Zakalik K, Duda P, MacDougall J, Farzaneh-Far R, Pontius A, Hoarty M. Clinical Effects of the Self-administered Subcutaneous Complement Inhibitor Zilucoplan in Patients With Moderate to Severe Generalized Myasthenia Gravis. JAMA Neurology 2020, 77: 582-592. PMID: 32065623, PMCID: PMC7042797, DOI: 10.1001/jamaneurol.2019.5125.Peer-Reviewed Original ResearchConceptsGeneralized myasthenia gravisSecondary end pointsDaily living scoreTolerability profileEnd pointLiving scoreMyasthenia gravisClinical effectsPlacebo-controlled phase 2 clinical trialKey secondary efficacy end pointsKey secondary end pointSecondary efficacy end pointsSevere Generalized Myasthenia GravisQuantitative Myasthenia Gravis ScorePhase 2 clinical trialAcetylcholine receptor autoantibodiesEfficacy end pointMyasthenia gravis scoreMG activityComplement component 5Disease-specific variablesQuality of lifeComplete complement inhibitionBroader populationDaily SCExploring outcomes and characteristics of myasthenia gravis: Rationale, aims and design of registry – The EXPLORE-MG registry
Anil R, Kumar A, Alaparthi S, Sharma A, Nye JL, Roy B, O'Connor KC, Nowak RJ. Exploring outcomes and characteristics of myasthenia gravis: Rationale, aims and design of registry – The EXPLORE-MG registry. Journal Of The Neurological Sciences 2020, 414: 116830. PMID: 32388060, DOI: 10.1016/j.jns.2020.116830.Peer-Reviewed Original ResearchConceptsMyasthenia gravisMG patientsContemporary patient cohortKey clinical featuresMuscle-specific kinaseMG clinicClinical featuresGeneralized diseaseOutcomes RegistryPatient cohortPatient outcomesTreatment responsivenessTreatment outcomesTreatment strategiesRegistry dataEpidemiological dataInterim analysisData registryOcular diseasesPatientsCommon data elementsAcetylcholine receptorsRegistryPatient careDiseaseClinical characteristics of fibroblast growth factor receptor 3 antibody‐related polyneuropathy: a retrospective study
Kovvuru S, Cardenas Y, Huttner A, Nowak R, Roy B. Clinical characteristics of fibroblast growth factor receptor 3 antibody‐related polyneuropathy: a retrospective study. European Journal Of Neurology 2020, 27: 1310-1318. PMID: 32068339, DOI: 10.1111/ene.14180.Peer-Reviewed Original ResearchConceptsChronic inflammatory neuropathiesInflammatory neuropathiesSensory neuropathyFGFR3 antibodiesDistal lower extremity weaknessIntravenous immunoglobulin responseLower extremity paresthesiasCommon presenting symptomLower extremity weaknessRetrospective chart reviewPure sensory neuropathyHistory of cancerDistal sensationMotor findingsPresenting symptomChart reviewClinical characteristicsDemyelinating featuresNerve biopsySymptom onsetUnsteady gaitClinical presentationFoot dropRetrospective studyAutoimmune diseases
2019
Electrical impedance myography as a biomarker of inclusion body myositis: A cross-sectional study
Roy B, Rutkove SB, Nowak RJ. Electrical impedance myography as a biomarker of inclusion body myositis: A cross-sectional study. Clinical Neurophysiology 2019, 131: 368-371. PMID: 31865137, DOI: 10.1016/j.clinph.2019.10.030.Peer-Reviewed Original ResearchConceptsInclusion body myositisManual muscle testingIBM patientsElectrical impedance myographyHealthy controlsBody myositisClinical outcome measuresCross-sectional studyImpedance myographySimilar age groupLongitudinal validation studyPotential objective biomarkersBilateral deltoidHandgrip dynamometryWalk testMuscle testingTibialis anteriorObjective biomarkersMedial gastrocnemiusOutcome measuresPatientsAge groupsFinal analysisMyositisMyography