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54. P.046 Real-world survival effectiveness of edaravone in amyotrophic lateral sclerosis: a propensity score weighted, registry-based, Canada-wide cohort study

Author

Abrahao, A, Vyas, MV, Parks, A, Hodgkinson, V, Dyck, A, Benstead, T, Briemberg, H, Genge, A, Grant, I, Jewett, G, Johnston, W, Kalra, S, Marrero, A, Massie, R, Melanson, M, O’Connell, C, Pfeffer, G, Schellenberg, KL, Taylor, S, Shoesmith, C, Matte, G, Zinman, L, and Korngut, L

Abstract

Background: ALS is a progressive neurodegenerative disease without a cure and limited treatment options. Edaravone, a free radical scavenger, was shown to slow disease progression in a select group of patients with ALS over 6 months; however, the effect on survival was not investigated in randomized trials. The objective of this study is to describe real-world survival effectiveness over a longer timeframe. Methods: This retrospective cohort study included patients with ALS across Canada with symptom onset up to three years. Those with a minimum 6-month edaravone exposure between 2017 and 2022 were enrolled in the interventional arm, and those without formed the control arm. The primary outcome of tracheostomy-free survival was compared between the two groups, accounting for age, sex, ALS-disease progression rate, disease duration, pulmonary vital capacity, bulbar ALS-onset, and presence of frontotemporal dementia or C9ORF72 mutation using inverse propensity treatment weights. Results: 182 patients with mean ± SD age 60±11 years were enrolled in the edaravone arm and 860 in the control arm (mean ± SD age 63±12 years). Mean ± SD time from onset to edaravone initiation was 18±10 months. Tracheostomy-free survival will be calculated. Conclusions: This study will provide evidence for edaravone effectiveness on tracheostomy-free survival in patients with ALS.

Published
2023
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59. A.P.1: Proteomic analysis in 72 myofibrillar myopathy (MFM) patients identifies new disease-relevant proteins accumulating in aggregates and reveals subtype-specific proteomic profiles

Author

Maerkens, A., Olivé, M., Tasca, G., Claeys, K., Barresi, R., Sarkozy, A., Pfeffer, G., Evangelista, T., Feldkirchner, S., Reimann, J., Hanisch, F., Stenzel, W., Schessl, J., Schoser, B., Goldfarb, L., Udd, B., Chinnery, P., Lochmüller, H., Schröder, R., Marcus, K., Vorgerd, M., and Kley, R.A.

Published
2014
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67. Adult-onset spinocerebellar ataxia syndromes due to MTATP6 mutations.

Author

Pfeffer G, Blakely EL, Alston CL, Hassani A, Boggild M, Horvath R, Samuels DC, Taylor RW, Chinnery PF, Pfeffer, Gerald, Blakely, Emma L, Alston, Charlotte L, Hassani, Adam, Boggild, Mike, Horvath, Rita, Samuels, David C, Taylor, Robert W, and Chinnery, Patrick F

Abstract

Background: Spinocerebellar ataxia syndromes presenting in adulthood have a broad range of causes, and despite extensive investigation remain undiagnosed in up to ∼50% cases. Mutations in the mitochondrially encoded MTATP6 gene typically cause infantile-onset Leigh syndrome and, occasionally, have onset later in childhood. The authors report two families with onset of ataxia in adulthood (with pyramidal dysfunction and/or peripheral neuropathy variably present), who are clinically indistinguishable from other spinocerebellar ataxia patients.Methods: Genetic screening study of the MTATP6 gene in 64 pedigrees with unexplained ataxia, and case series of two families who had MTATP6 mutations.Results: Three pedigrees had mutations in MTATP6, two of which have not been reported previously and are detailed in this report. These families had the m.9185T>C and m.9035T>C mutations, respectively, which have not previously been associated with adult-onset cerebellar syndromes. Other investigations including muscle biopsy and respiratory chain enzyme activity were non-specific or normal.Conclusions: MTATP6 sequencing should be considered in the workup of undiagnosed ataxia, even if other investigations do not suggest a mitochondrial DNA disorder. [ABSTRACT FROM AUTHOR]

Published
2012
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72. The History of Carpal Tunnel Syndrome.

Author

PFEFFER, G. B., GELBERMAN, R. H., BOYES, J. H., and RYDEVIK, B.

Abstract

Carpal tunnel syndrome is the most frequently diagnosed, best understood and most easily treated entrapment neuropathy. During the first half of the 20th century, however, most patients with carpal tunnel syndrome were diagnosed as having compression of either the brachial plexus or thenar nerve motor branch of the median nerve. As late as 1950, only twelve patients with operative release of the transverse carpal ligament for idiopathic carpal tunnel syndrome had been reported. The delay in accurate anatomical localization of this compressive neuropathy can be attributed both to the confusion caused by the diverse manifestations of median nerve compression in the carpal tunnel, and to some interesting developments that altered early investigations in this area. [ABSTRACT FROM PUBLISHER]

Published
1981
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76. Weather and Overlord: Contemporary Lessons

Author

AIR WAR COLL MAXWELL AFB AL, Pfeffer, G. J., AIR WAR COLL MAXWELL AFB AL, and Pfeffer, G. J.

Abstract

OVERLORD, the Allied invasion of Normandy on 6 June 1944, represents one of the most weather-sensitive military operations ever undertaken. The actual weather constraints for a successful landing were critical and complex. The dramatic story of how weather played in the D-Day decision has been recounted elsewhere. Instead, this report focuses on the process, i.e., how weather support was provided, in order to gain insights applicable to contemporary military operations. The report review the functioning of the supporting weather services including organization, command relationships, scientific state of the art, security, intelligence, and communications. Key conclusions ad lessons applicable to today's operations are presented.

Published
1985

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