Pub Date : 2022-06-22DOI: 10.17161/rrnmf.v3i2.15925
Vernon D. Rowe, Lauren J. Peck
2) Complete el Formulario de autorización de servicios e inscripción al programa de Youth Guidance. Tenga en cuenta que para inscribir a su hijo/a, usted debe marcar “Sí” en en el punto #1 en la parte inferior del formulario. Sin embargo, puede marcar “No” en las otras opciones y su hijo/a aún tendrá la posibilidad de participar. a. Complete todos los campos del formulario. b. Si su hijo/a tiene 12 años o más, también le pediremos su permiso ("consentimiento") para participar en el programa de Youth Guidance.
{"title":"Youth","authors":"Vernon D. Rowe, Lauren J. Peck","doi":"10.17161/rrnmf.v3i2.15925","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i2.15925","url":null,"abstract":"2) Complete el Formulario de autorización de servicios e inscripción al programa de Youth Guidance. Tenga en cuenta que para inscribir a su hijo/a, usted debe marcar “Sí” en en el punto #1 en la parte inferior del formulario. Sin embargo, puede marcar “No” en las otras opciones y su hijo/a aún tendrá la posibilidad de participar. a. Complete todos los campos del formulario. b. Si su hijo/a tiene 12 años o más, también le pediremos su permiso (\"consentimiento\") para participar en el programa de Youth Guidance.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121666932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-06-22DOI: 10.17161/rrnmf.v3i2.18093
Dylan Mortimer
{"title":"Reflections On My Double Lung Transplant","authors":"Dylan Mortimer","doi":"10.17161/rrnmf.v3i2.18093","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i2.18093","url":null,"abstract":"","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"120900200","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-06-22DOI: 10.17161/rrnmf.v3i2.15904
A. Bhashyam, Salman F Bhai
Introduction Creatinine kinase (CK) is a common lab ordered by generalists and specialists that is often misinterpreted. Given its prevalent use, we highlight a case that outlines pitfalls of the test. The case is of a gentleman who is referred to a neuromuscular clinic for weakness and an elevated CK. However, during the COVID-19 pandemic, this was initially a video visit, which then serves to highlight the challenges of video visits. In this report, we primarily aim to highlight an algorithm to evaluate CK in the presence of weakness. Secondary objectives include reviewing common pitfalls of CK testing, especially with the rising trend of video visits. Practically, total CK enzyme activity (IU/L) is measured with a photometric assay utilizing the enzymatic rate method to calculate the rate of phosphate transfer from phosphocreatine to adenosine diphosphate per minute. Tissue isoform assays fractionate total CK using antibodies to CK-MM (skeletal muscle), CK-MB (cardiac muscle), or CK-BB (brain). Male sex, black race, younger age, and exercise are the most common reasons for normal physiologic increases in CK, possibly due to differences in muscle or total body mass and the permeability of the sarcolemma to CK.1–3 Exercise causes transient increases in CK over 24-48 hours, followed by return to baseline over 7+ days. Pathologic CK elevation is associated with myopathies or muscle injury, but can also occur in neurogenic disorders due to impaired muscle membrane integrity secondary to muscle degeneration from axonal loss.4–6 Other causes of CK elevation include race, medication use, systemic disorders (e.g. acute renal failure, malignancy, viral illness), and endocrine abnormalities.4, 5, 7 Of these, statin-induced CK elevation is most commonly observed.3 In clinical settings, assay manufacturers provide a CK reference range assuming a gaussian distribution (0-180 IU/L). This results in high false-positive rates as population CK distribution is skewed toward higher values.5 For this reason, recent practice guidelines recommend using a upper limit of normal (ULN) threshold at the 97.5th percentile rather than manufacturer-quoted ULN (Table 2).2,4,7,8 Using these guidelines, the prevalence of incidentally elevated CK in asymptomatic patients is 5.3%, with persistent unexplained elevation in 1.3%.9 CK elevation can vary based on sex. In a cohort of musculoskeletal patients with elevated CK 29% were female (F) and 44% were male (M). Sensitivity using the 97.5th percentile versus manufacturer’s guidelines was 29%(F)/60%(M) versus 50%(F)/80%(M) and sensitivity was 80%(F)/80%(M) versus 70%(F)/67%(M), respectively.4 When using a cutoff of 1.5xULN instead of the 97.5th percentile, sensitivity for diagnosing myopathy decreased by 37%. CK > 1000 IU/L had a high likelihood for myopathy (11.0).6,7 Thus, increasing the ULN improves specificity and decreases the false positive rate when evaluating CK elevation.1,6,7 The cost of total CK to Medicare is $6.51. To
{"title":"Creatinine Kinase: A test done from muscle memory or clinical reasoning?","authors":"A. Bhashyam, Salman F Bhai","doi":"10.17161/rrnmf.v3i2.15904","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i2.15904","url":null,"abstract":"Introduction Creatinine kinase (CK) is a common lab ordered by generalists and specialists that is often misinterpreted. Given its prevalent use, we highlight a case that outlines pitfalls of the test. The case is of a gentleman who is referred to a neuromuscular clinic for weakness and an elevated CK. However, during the COVID-19 pandemic, this was initially a video visit, which then serves to highlight the challenges of video visits. In this report, we primarily aim to highlight an algorithm to evaluate CK in the presence of weakness. Secondary objectives include reviewing common pitfalls of CK testing, especially with the rising trend of video visits. Practically, total CK enzyme activity (IU/L) is measured with a photometric assay utilizing the enzymatic rate method to calculate the rate of phosphate transfer from phosphocreatine to adenosine diphosphate per minute. Tissue isoform assays fractionate total CK using antibodies to CK-MM (skeletal muscle), CK-MB (cardiac muscle), or CK-BB (brain). Male sex, black race, younger age, and exercise are the most common reasons for normal physiologic increases in CK, possibly due to differences in muscle or total body mass and the permeability of the sarcolemma to CK.1–3 Exercise causes transient increases in CK over 24-48 hours, followed by return to baseline over 7+ days. Pathologic CK elevation is associated with myopathies or muscle injury, but can also occur in neurogenic disorders due to impaired muscle membrane integrity secondary to muscle degeneration from axonal loss.4–6 Other causes of CK elevation include race, medication use, systemic disorders (e.g. acute renal failure, malignancy, viral illness), and endocrine abnormalities.4, 5, 7 Of these, statin-induced CK elevation is most commonly observed.3 In clinical settings, assay manufacturers provide a CK reference range assuming a gaussian distribution (0-180 IU/L). This results in high false-positive rates as population CK distribution is skewed toward higher values.5 For this reason, recent practice guidelines recommend using a upper limit of normal (ULN) threshold at the 97.5th percentile rather than manufacturer-quoted ULN (Table 2).2,4,7,8 Using these guidelines, the prevalence of incidentally elevated CK in asymptomatic patients is 5.3%, with persistent unexplained elevation in 1.3%.9 CK elevation can vary based on sex. In a cohort of musculoskeletal patients with elevated CK 29% were female (F) and 44% were male (M). Sensitivity using the 97.5th percentile versus manufacturer’s guidelines was 29%(F)/60%(M) versus 50%(F)/80%(M) and sensitivity was 80%(F)/80%(M) versus 70%(F)/67%(M), respectively.4 When using a cutoff of 1.5xULN instead of the 97.5th percentile, sensitivity for diagnosing myopathy decreased by 37%. CK > 1000 IU/L had a high likelihood for myopathy (11.0).6,7 Thus, increasing the ULN improves specificity and decreases the false positive rate when evaluating CK elevation.1,6,7 The cost of total CK to Medicare is $6.51. To","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130213659","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-06-22DOI: 10.17161/rrnmf.v3i2.16304
Mazen M. Dimachkie
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous, acquired autoimmune neurological disorder affecting peripheral nerves. CIDP is characterized by progressive weakness, reduced or absent tendon reflexes and impaired sensory function in the lower and upper limbs. CIDP diagnosis is mainly based on clinical, laboratory and electrophysiologic criteria and there are currently no diagnostic or prognostic biomarkers. First-line treatment options include corticosteroids, intravenous immunoglobulin (IVIg) and plasma exchange (PLEX). While IVIg and corticosteroids are the most common therapies administered for CIDP, there are challenges associated with their use, including systemic adverse events (AEs), some of which can be serious. Studies have shown that subcutaneous immunoglobulin (SCIg) may be associated with improved quality of life, which is attributed partially to the patients’ freedom to administer SCIg at home and at their convenience. While AEs with SCIg mostly consist of local site reactions, SCIg is associated with fewer systemic AEs compared with IVIg, and these are commonly mild, though severe reactions may rarely occur. A number of studies in the last decade have assessed SCIg in CIDP. One of these studies, the Polyneuropathy and Treatment with Hizentra® (PATH) study, was a global phase 3, double-blind, randomized, placebo-controlled trial that assessed the efficacy, safety, and tolerability of SCIg treatment in patients with CIDP. Based on the results of the PATH study, the US Food and Drug Administration (FDA) approved SCIg as a maintenance treatment for CIDP in 2018. This review summarizes and discusses the results of the PATH study and its open-label extension (OLE) study and provides an overview of the April 2021 update to the Hizentra® FDA-approved U.S. package insert based on findings from the PATH OLE. In addition, the review highlights key elements of the second revision of the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guideline for the diagnosis and treatment of CIDP. Finally, this review discusses the characteristics of patients with CIDP who may benefit from SCIg treatment.
{"title":"Optimizing chronic inflammatory demyelinating polyneuropathy care with subcutaneous immunoglobulin: The Polyneuropathy and Treatment with Hizentra Open-Label Extension (PATH OLE) study and beyond","authors":"Mazen M. Dimachkie","doi":"10.17161/rrnmf.v3i2.16304","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i2.16304","url":null,"abstract":"Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous, acquired autoimmune neurological disorder affecting peripheral nerves. CIDP is characterized by progressive weakness, reduced or absent tendon reflexes and impaired sensory function in the lower and upper limbs. CIDP diagnosis is mainly based on clinical, laboratory and electrophysiologic criteria and there are currently no diagnostic or prognostic biomarkers. First-line treatment options include corticosteroids, intravenous immunoglobulin (IVIg) and plasma exchange (PLEX). While IVIg and corticosteroids are the most common therapies administered for CIDP, there are challenges associated with their use, including systemic adverse events (AEs), some of which can be serious. Studies have shown that subcutaneous immunoglobulin (SCIg) may be associated with improved quality of life, which is attributed partially to the patients’ freedom to administer SCIg at home and at their convenience. While AEs with SCIg mostly consist of local site reactions, SCIg is associated with fewer systemic AEs compared with IVIg, and these are commonly mild, though severe reactions may rarely occur. A number of studies in the last decade have assessed SCIg in CIDP. One of these studies, the Polyneuropathy and Treatment with Hizentra® (PATH) study, was a global phase 3, double-blind, randomized, placebo-controlled trial that assessed the efficacy, safety, and tolerability of SCIg treatment in patients with CIDP. Based on the results of the PATH study, the US Food and Drug Administration (FDA) approved SCIg as a maintenance treatment for CIDP in 2018. This review summarizes and discusses the results of the PATH study and its open-label extension (OLE) study and provides an overview of the April 2021 update to the Hizentra® FDA-approved U.S. package insert based on findings from the PATH OLE. In addition, the review highlights key elements of the second revision of the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guideline for the diagnosis and treatment of CIDP. Finally, this review discusses the characteristics of patients with CIDP who may benefit from SCIg treatment.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116642969","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-06-22DOI: 10.17161/rrnmf.v3i2.17954
Donald Frey
{"title":"AN OPEN MESSAGE TO THE PEOPLE OF THE UNITED STATES OF AMERICA","authors":"Donald Frey","doi":"10.17161/rrnmf.v3i2.17954","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i2.17954","url":null,"abstract":"","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127944953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-06-22DOI: 10.17161/rrnmf.v3i2.15903
A. Bhashyam, Salman F Bhai
Introduction Patients with transient symptoms pose diagnostic dilemmas and may even be misdiagnosed with Functional Neurologic Disorder or somatization. We present a case of a patient with transient neuromuscular symptoms while cycling under a powerline. While this phenomenon is not reported in the clinical neurological or musculoskeletal literature, several online forums related to cycling describe bicyclists experiencing a similar sensation. To further investigate, we searched for all instances of microshocks occurring in cyclists using the following search term in Google: “electrical” AND (“bicycle” or “cycle”) AND (“microshock” or “shock”). Between 2006-2021, we identified 51 unique reports across 18 blog forums of cyclists describing microshocks in their groin or upper extremity while traversing under high voltage power lines (Table 1). In 11 of 18 forums, at least one of the posts described friends or family members characterizing the experiences as “impossible” or “hallucinations.” After an initial posting, additional shared experiences of microshock by other cyclists were offered in 12 forums. Seven forums speculated about shortand long-term health effects, while 13 forums specifically inquired about the etiology of the symptoms. Based on this review, electrical microshocks while cycling under high voltage powerlines may be more common than suspected a priori and are likely to be dismissed by close contacts or healthcare workers. These events commonly generate questions about health effects and potential causes for symptoms. Such questions are likely to be better addressed by healthcare providers or peerreviewed literature as compared to unverified blog postings by anonymous authors.1 This is the primary reason why we present this case.
{"title":"BIKE Syndrome: Biking Induced Kinetic Electroshock Syndrome","authors":"A. Bhashyam, Salman F Bhai","doi":"10.17161/rrnmf.v3i2.15903","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i2.15903","url":null,"abstract":"Introduction Patients with transient symptoms pose diagnostic dilemmas and may even be misdiagnosed with Functional Neurologic Disorder or somatization. We present a case of a patient with transient neuromuscular symptoms while cycling under a powerline. While this phenomenon is not reported in the clinical neurological or musculoskeletal literature, several online forums related to cycling describe bicyclists experiencing a similar sensation. To further investigate, we searched for all instances of microshocks occurring in cyclists using the following search term in Google: “electrical” AND (“bicycle” or “cycle”) AND (“microshock” or “shock”). Between 2006-2021, we identified 51 unique reports across 18 blog forums of cyclists describing microshocks in their groin or upper extremity while traversing under high voltage power lines (Table 1). In 11 of 18 forums, at least one of the posts described friends or family members characterizing the experiences as “impossible” or “hallucinations.” After an initial posting, additional shared experiences of microshock by other cyclists were offered in 12 forums. Seven forums speculated about shortand long-term health effects, while 13 forums specifically inquired about the etiology of the symptoms. Based on this review, electrical microshocks while cycling under high voltage powerlines may be more common than suspected a priori and are likely to be dismissed by close contacts or healthcare workers. These events commonly generate questions about health effects and potential causes for symptoms. Such questions are likely to be better addressed by healthcare providers or peerreviewed literature as compared to unverified blog postings by anonymous authors.1 This is the primary reason why we present this case.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114529398","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-06-22DOI: 10.17161/rrnmf.v3i2.16253
Joshua Freeman
{"title":"Physician Specialists: What's in a Name?","authors":"Joshua Freeman","doi":"10.17161/rrnmf.v3i2.16253","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i2.16253","url":null,"abstract":"","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121561689","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-06-22DOI: 10.17161/rrnmf.v3i2.15879
Mai Yamakawa, M. Pasnoor
N/A
N/A
{"title":"Case of Vasculitic Neuropathy and Myelopathy","authors":"Mai Yamakawa, M. Pasnoor","doi":"10.17161/rrnmf.v3i2.15879","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i2.15879","url":null,"abstract":"<jats:p>N/A</jats:p>","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114862400","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-06-22DOI: 10.17161/rrnmf.v3i2.17011
R. Barohn
{"title":"Jane Cooke Wright, MD: A pioneering cancer physician and researcher","authors":"R. Barohn","doi":"10.17161/rrnmf.v3i2.17011","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i2.17011","url":null,"abstract":"","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117317223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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