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Canadian Guidelines for Hereditary Transthyretin Amyloidosis Polyneuropathy Management
- Monica Alcantara, Michelle M. Mezei, Steven K. Baker, Ari Breiner, Priya Dhawan, Amanda Fiander, Nowell M. Fine, Christopher Hahn, Hans D. Katzberg, Shahin Khayambashi, Rami Massie, Genevieve Matte, Brendan Putko, Zaeem Siddiqi, Diego Delgado, Vera Bril
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- Journal:
- Canadian Journal of Neurological Sciences / Volume 49 / Issue 1 / January 2022
- Published online by Cambridge University Press:
- 26 February 2021, pp. 7-18
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Hereditary transthyretin-mediated (hATTR) amyloidosis is a progressive disease caused by mutations in the TTR gene leading to multisystem organ dysfunction. Pathogenic TTR aggregation, misfolding, and fibrillization lead to deposition of amyloid in multiple body organs and frequently involve the peripheral nerve system and the heart. Common neurologic manifestations include: sensorimotor polyneuropathy (PN), autonomic neuropathy, small-fiber PN, and carpal tunnel syndrome. Many patients have significant progression due to diagnostic delays as hATTR PN is not considered within the differential diagnosis. Recently, two effective novel disease-modifying therapies, inotersen and patisiran, were approved by Health Canada for the treatment of hATTR PN. Early diagnosis is crucial for the timely introduction of these disease-modifying treatments that reduce impairments, improve quality of life, and extend survival. In this guideline, we aim to improve awareness and outcomes of hATTR PN by making recommendations directed to the diagnosis, monitoring, and treatment in Canada.
Practice Guidelines for Canadian Neurophysiology Laboratories During the COVID-19 Pandemic
- Canadian Society of Clinical Neurophysiologists (CSCN), Canadian Association of Electroneurophysiology Technologists (CAET), Association of Electromyography Technologists of Canada (AETC), Board of Registration of Electromyography Technologists of Canada (BRETC), Canadian Board of Registration of Electroencephalograph Technologists (CBRET), Juan Pablo Appendino, Steven K. Baker, Kristine M. Chapman, Tamara Dykstra, Tabrez Hussein, Michelle-Lee Jones, Michelle M. Mezei, Seyed M. Mirsattari, Marcus Ng, Joanne Nikkel, Vaso Obradovic, Cecile Phan, Lawrence Robinson, Angela Scott, Jose Tellez-Zenteno, Michelle Van Niekerk, Shannon Venance, Fraser Moore
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- Journal:
- Canadian Journal of Neurological Sciences / Volume 48 / Issue 1 / January 2021
- Published online by Cambridge University Press:
- 19 August 2020, pp. 25-30
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The COVID-19 pandemic has had a major impact on clinical practice. Safe standards of practice are essential to protect health care workers while still allowing them to provide good care. The Canadian Society of Clinical Neurophysiologists, the Canadian Association of Electroneurophysiology Technologists, the Association of Electromyography Technologists of Canada, the Board of Registration of Electromyography Technologists of Canada, and the Canadian Board of Registration of Electroencephalograph Technologists have combined to review current published literature about safe practices for neurophysiology laboratories. Herein, we present the results of our review and provide our expert opinion regarding the safe practice of neurophysiology during the COVID-19 pandemic in Canada.
Chronic Inflammatory Demyelinating Polyneuropathy: Time to Maximal Recovery in Patients Receiving Intravenous Immunoglobulin Therapy
- Adrian R. Opala, Kevin Kennedy, Steven K. Baker
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- Canadian Journal of Neurological Sciences / Volume 47 / Issue 4 / July 2020
- Published online by Cambridge University Press:
- 13 April 2020, pp. 531-537
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Background:
The response of chronic inflammatory demyelinating polyneuropathy (CIDP) to intravenous immunoglobulins (IVIgs) treatment is well established. However, it remains unclear whether patients not responding to two IVIg treatments or those whose condition stabilizes (ICE trial) may benefit from additional doses. We aim to identify the time period required to reach maximal strength gains from IVIg treatment.
Methods:Retrospective chart review of 14 patients with CIDP was performed. Change in handgrip (HG), Knee extension (KE), elbow flexion, and dorsiflexion was analyzed with a dynamometer during IVIg therapy. Strength improvements in Nm or kg, cumulative grams (g) of IVIg, and time in days required for maximal strength recovery were determined per function (± standard error of the mean). Ancillary therapy was recorded for all patients.
Results:Improvements in strength of each function were significant (p < 0.05). Earliest improvement was in HG (137.07 ± 21.23) and latest in KE (238.15 ± 38.9). Majority of patients improved by 200 days of therapy. HG required the lowest cumulative grams of IgG (561.71 ± 97.21) and KE the most (798 ± 120.7).
Conclusion:Our study has demonstrated the effectiveness of multiple treatments with IVIg to reach significant improvement in strength. Different muscle groups manifested different time dependency, reflecting the requirement of variable amounts of IVIg. Improvement was identified on an ongoing basis, with therapy lasting between 20.2 and 37.3 weeks, requiring between 562 and 798 g of IVIg.
Chronic Inflammatory Demyelinating Polyneuropathy and Concurrent Membranous Nephropathy
- Samina Nazarali, Emily K. Mathey, Damu Tang, Peter J. Margetts, Steven K. Baker
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- Canadian Journal of Neurological Sciences / Volume 47 / Issue 4 / July 2020
- Published online by Cambridge University Press:
- 04 March 2020, pp. 585-587
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Application Technology and Best Management Practices for Minimizing Herbicide Runoff
- James L. Baker, Steven K. Mickelson
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- Weed Technology / Volume 8 / Issue 4 / December 1994
- Published online by Cambridge University Press:
- 12 June 2017, pp. 862-869
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The fate of field-applied herbicides, including losses in surface runoff with water and sediment, is highly dependent on herbicide properties. The two most important properties are soil adsorption and persistence. Adsorption affects the potential for a herbicide to be lost primarily with sediment, runoff water, or possibly leaching water. Solubility, often though not always inversely correlated with adsorption, is of secondary importance, although low solubility can limit transport with water. Persistence affects the time available to be lost in runoff. Studies have shown that for soil-applied herbicides; extraction into runoff water or movement with sediment takes place from a thin soil layer at the surface. In addition, for herbicides studied, there is little interaction between surface crop residue and applied herbicides, and washoff from the residue readily occurs with small amounts of rainfall. Runoff loss equals the volume of carrier (water or sediment) times the concentration in that carrier; therefore, practices that reduce either, or both, can reduce losses. Rate of application has been directly related to concentration and therefore loss. Reducing rate, such as by banding, soil incorporation, and avoidance of application to crop residue reduce losses. The choice of herbicide and herbicide formulation, in conjunction with application technology, as they affect properties, rate, and placement, play a large role in determining runoff loss. Runoff losses of herbicides that are strongly adsorbed and therefore transported mainly with sediment can be reduced by erosion control; runoff volume reduction can reduce losses with water of moderately to weakly adsorbed herbicides. Conservation tillage has potential to reduce both runoff and erosion. Timing of application relative to expected intense storms, both in the short and long term, can reduce the potential for runoff. If possible to determine thresholds, herbicide use could be avoided if weed infestation is below the economic effect level. Buffer or filter strips have the potential to reduce transport of herbicides lost from fields to surface water resources.
Contributors
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- By Mitchell Aboulafia, Frederick Adams, Marilyn McCord Adams, Robert M. Adams, Laird Addis, James W. Allard, David Allison, William P. Alston, Karl Ameriks, C. Anthony Anderson, David Leech Anderson, Lanier Anderson, Roger Ariew, David Armstrong, Denis G. Arnold, E. J. Ashworth, Margaret Atherton, Robin Attfield, Bruce Aune, Edward Wilson Averill, Jody Azzouni, Kent Bach, Andrew Bailey, Lynne Rudder Baker, Thomas R. Baldwin, Jon Barwise, George Bealer, William Bechtel, Lawrence C. Becker, Mark A. Bedau, Ernst Behler, José A. Benardete, Ermanno Bencivenga, Jan Berg, Michael Bergmann, Robert L. Bernasconi, Sven Bernecker, Bernard Berofsky, Rod Bertolet, Charles J. Beyer, Christian Beyer, Joseph Bien, Joseph Bien, Peg Birmingham, Ivan Boh, James Bohman, Daniel Bonevac, Laurence BonJour, William J. Bouwsma, Raymond D. Bradley, Myles Brand, Richard B. Brandt, Michael E. Bratman, Stephen E. Braude, Daniel Breazeale, Angela Breitenbach, Jason Bridges, David O. Brink, Gordon G. Brittan, Justin Broackes, Dan W. Brock, Aaron Bronfman, Jeffrey E. Brower, Bartosz Brozek, Anthony Brueckner, Jeffrey Bub, Lara Buchak, Otavio Bueno, Ann E. Bumpus, Robert W. Burch, John Burgess, Arthur W. Burks, Panayot Butchvarov, Robert E. Butts, Marina Bykova, Patrick Byrne, David Carr, Noël Carroll, Edward S. Casey, Victor Caston, Victor Caston, Albert Casullo, Robert L. Causey, Alan K. L. Chan, Ruth Chang, Deen K. Chatterjee, Andrew Chignell, Roderick M. Chisholm, Kelly J. Clark, E. J. Coffman, Robin Collins, Brian P. Copenhaver, John Corcoran, John Cottingham, Roger Crisp, Frederick J. Crosson, Antonio S. Cua, Phillip D. Cummins, Martin Curd, Adam Cureton, Andrew Cutrofello, Stephen Darwall, Paul Sheldon Davies, Wayne A. Davis, Timothy Joseph Day, Claudio de Almeida, Mario De Caro, Mario De Caro, John Deigh, C. F. Delaney, Daniel C. Dennett, Michael R. DePaul, Michael Detlefsen, Daniel Trent Devereux, Philip E. Devine, John M. Dillon, Martin C. Dillon, Robert DiSalle, Mary Domski, Alan Donagan, Paul Draper, Fred Dretske, Mircea Dumitru, Wilhelm Dupré, Gerald Dworkin, John Earman, Ellery Eells, Catherine Z. Elgin, Berent Enç, Ronald P. Endicott, Edward Erwin, John Etchemendy, C. Stephen Evans, Susan L. Feagin, Solomon Feferman, Richard Feldman, Arthur Fine, Maurice A. Finocchiaro, William FitzPatrick, Richard E. Flathman, Gvozden Flego, Richard Foley, Graeme Forbes, Rainer Forst, Malcolm R. Forster, Daniel Fouke, Patrick Francken, Samuel Freeman, Elizabeth Fricker, Miranda Fricker, Michael Friedman, Michael Fuerstein, Richard A. Fumerton, Alan Gabbey, Pieranna Garavaso, Daniel Garber, Jorge L. A. Garcia, Robert K. Garcia, Don Garrett, Philip Gasper, Gerald Gaus, Berys Gaut, Bernard Gert, Roger F. Gibson, Cody Gilmore, Carl Ginet, Alan H. Goldman, Alvin I. Goldman, Alfonso Gömez-Lobo, Lenn E. Goodman, Robert M. Gordon, Stefan Gosepath, Jorge J. E. Gracia, Daniel W. Graham, George A. Graham, Peter J. Graham, Richard E. Grandy, I. Grattan-Guinness, John Greco, Philip T. Grier, Nicholas Griffin, Nicholas Griffin, David A. Griffiths, Paul J. Griffiths, Stephen R. Grimm, Charles L. Griswold, Charles B. Guignon, Pete A. Y. Gunter, Dimitri Gutas, Gary Gutting, Paul Guyer, Kwame Gyekye, Oscar A. Haac, Raul Hakli, Raul Hakli, Michael Hallett, Edward C. Halper, Jean Hampton, R. James Hankinson, K. R. Hanley, Russell Hardin, Robert M. Harnish, William Harper, David Harrah, Kevin Hart, Ali Hasan, William Hasker, John Haugeland, Roger Hausheer, William Heald, Peter Heath, Richard Heck, John F. Heil, Vincent F. Hendricks, Stephen Hetherington, Francis Heylighen, Kathleen Marie Higgins, Risto Hilpinen, Harold T. Hodes, Joshua Hoffman, Alan Holland, Robert L. Holmes, Richard Holton, Brad W. Hooker, Terence E. Horgan, Tamara Horowitz, Paul Horwich, Vittorio Hösle, Paul Hoβfeld, Daniel Howard-Snyder, Frances Howard-Snyder, Anne Hudson, Deal W. Hudson, Carl A. Huffman, David L. Hull, Patricia Huntington, Thomas Hurka, Paul Hurley, Rosalind Hursthouse, Guillermo Hurtado, Ronald E. Hustwit, Sarah Hutton, Jonathan Jenkins Ichikawa, Harry A. Ide, David Ingram, Philip J. Ivanhoe, Alfred L. Ivry, Frank Jackson, Dale Jacquette, Joseph Jedwab, Richard Jeffrey, David Alan Johnson, Edward Johnson, Mark D. Jordan, Richard Joyce, Hwa Yol Jung, Robert Hillary Kane, Tomis Kapitan, Jacquelyn Ann K. Kegley, James A. Keller, Ralph Kennedy, Sergei Khoruzhii, Jaegwon Kim, Yersu Kim, Nathan L. King, Patricia Kitcher, Peter D. Klein, E. D. Klemke, Virginia Klenk, George L. Kline, Christian Klotz, Simo Knuuttila, Joseph J. Kockelmans, Konstantin Kolenda, Sebastian Tomasz Kołodziejczyk, Isaac Kramnick, Richard Kraut, Fred Kroon, Manfred Kuehn, Steven T. Kuhn, Henry E. Kyburg, John Lachs, Jennifer Lackey, Stephen E. Lahey, Andrea Lavazza, Thomas H. Leahey, Joo Heung Lee, Keith Lehrer, Dorothy Leland, Noah M. Lemos, Ernest LePore, Sarah-Jane Leslie, Isaac Levi, Andrew Levine, Alan E. Lewis, Daniel E. Little, Shu-hsien Liu, Shu-hsien Liu, Alan K. L. Chan, Brian Loar, Lawrence B. Lombard, John Longeway, Dominic McIver Lopes, Michael J. Loux, E. J. Lowe, Steven Luper, Eugene C. Luschei, William G. Lycan, David Lyons, David Macarthur, Danielle Macbeth, Scott MacDonald, Jacob L. Mackey, Louis H. Mackey, Penelope Mackie, Edward H. Madden, Penelope Maddy, G. B. Madison, Bernd Magnus, Pekka Mäkelä, Rudolf A. Makkreel, David Manley, William E. Mann (W.E.M.), Vladimir Marchenkov, Peter Markie, Jean-Pierre Marquis, Ausonio Marras, Mike W. Martin, A. P. Martinich, William L. McBride, David McCabe, Storrs McCall, Hugh J. McCann, Robert N. McCauley, John J. McDermott, Sarah McGrath, Ralph McInerny, Daniel J. McKaughan, Thomas McKay, Michael McKinsey, Brian P. McLaughlin, Ernan McMullin, Anthonie Meijers, Jack W. Meiland, William Jason Melanson, Alfred R. Mele, Joseph R. Mendola, Christopher Menzel, Michael J. Meyer, Christian B. Miller, David W. Miller, Peter Millican, Robert N. Minor, Phillip Mitsis, James A. Montmarquet, Michael S. Moore, Tim Moore, Benjamin Morison, Donald R. Morrison, Stephen J. Morse, Paul K. Moser, Alexander P. D. Mourelatos, Ian Mueller, James Bernard Murphy, Mark C. Murphy, Steven Nadler, Jan Narveson, Alan Nelson, Jerome Neu, Samuel Newlands, Kai Nielsen, Ilkka Niiniluoto, Carlos G. Noreña, Calvin G. Normore, David Fate Norton, Nikolaj Nottelmann, Donald Nute, David S. Oderberg, Steve Odin, Michael O’Rourke, Willard G. Oxtoby, Heinz Paetzold, George S. Pappas, Anthony J. Parel, Lydia Patton, R. P. Peerenboom, Francis Jeffry Pelletier, Adriaan T. Peperzak, Derk Pereboom, Jaroslav Peregrin, Glen Pettigrove, Philip Pettit, Edmund L. Pincoffs, Andrew Pinsent, Robert B. Pippin, Alvin Plantinga, Louis P. Pojman, Richard H. Popkin, John F. Post, Carl J. Posy, William J. Prior, Richard Purtill, Michael Quante, Philip L. Quinn, Philip L. Quinn, Elizabeth S. Radcliffe, Diana Raffman, Gerard Raulet, Stephen L. Read, Andrews Reath, Andrew Reisner, Nicholas Rescher, Henry S. Richardson, Robert C. Richardson, Thomas Ricketts, Wayne D. Riggs, Mark Roberts, Robert C. Roberts, Luke Robinson, Alexander Rosenberg, Gary Rosenkranz, Bernice Glatzer Rosenthal, Adina L. Roskies, William L. Rowe, T. M. Rudavsky, Michael Ruse, Bruce Russell, Lilly-Marlene Russow, Dan Ryder, R. M. Sainsbury, Joseph Salerno, Nathan Salmon, Wesley C. Salmon, Constantine Sandis, David H. Sanford, Marco Santambrogio, David Sapire, Ruth A. Saunders, Geoffrey Sayre-McCord, Charles Sayward, James P. Scanlan, Richard Schacht, Tamar Schapiro, Frederick F. Schmitt, Jerome B. Schneewind, Calvin O. Schrag, Alan D. Schrift, George F. Schumm, Jean-Loup Seban, David N. Sedley, Kenneth Seeskin, Krister Segerberg, Charlene Haddock Seigfried, Dennis M. Senchuk, James F. Sennett, William Lad Sessions, Stewart Shapiro, Tommie Shelby, Donald W. Sherburne, Christopher Shields, Roger A. Shiner, Sydney Shoemaker, Robert K. Shope, Kwong-loi Shun, Wilfried Sieg, A. John Simmons, Robert L. Simon, Marcus G. Singer, Georgette Sinkler, Walter Sinnott-Armstrong, Matti T. Sintonen, Lawrence Sklar, Brian Skyrms, Robert C. Sleigh, Michael Anthony Slote, Hans Sluga, Barry Smith, Michael Smith, Robin Smith, Robert Sokolowski, Robert C. Solomon, Marta Soniewicka, Philip Soper, Ernest Sosa, Nicholas Southwood, Paul Vincent Spade, T. L. S. Sprigge, Eric O. Springsted, George J. Stack, Rebecca Stangl, Jason Stanley, Florian Steinberger, Sören Stenlund, Christopher Stephens, James P. Sterba, Josef Stern, Matthias Steup, M. A. Stewart, Leopold Stubenberg, Edith Dudley Sulla, Frederick Suppe, Jere Paul Surber, David George Sussman, Sigrún Svavarsdóttir, Zeno G. Swijtink, Richard Swinburne, Charles C. Taliaferro, Robert B. Talisse, John Tasioulas, Paul Teller, Larry S. Temkin, Mark Textor, H. S. Thayer, Peter Thielke, Alan Thomas, Amie L. Thomasson, Katherine Thomson-Jones, Joshua C. Thurow, Vzalerie Tiberius, Terrence N. Tice, Paul Tidman, Mark C. Timmons, William Tolhurst, James E. Tomberlin, Rosemarie Tong, Lawrence Torcello, Kelly Trogdon, J. D. Trout, Robert E. Tully, Raimo Tuomela, John Turri, Martin M. Tweedale, Thomas Uebel, Jennifer Uleman, James Van Cleve, Harry van der Linden, Peter van Inwagen, Bryan W. Van Norden, René van Woudenberg, Donald Phillip Verene, Samantha Vice, Thomas Vinci, Donald Wayne Viney, Barbara Von Eckardt, Peter B. M. Vranas, Steven J. Wagner, William J. Wainwright, Paul E. Walker, Robert E. Wall, Craig Walton, Douglas Walton, Eric Watkins, Richard A. Watson, Michael V. Wedin, Rudolph H. Weingartner, Paul Weirich, Paul J. Weithman, Carl Wellman, Howard Wettstein, Samuel C. Wheeler, Stephen A. White, Jennifer Whiting, Edward R. Wierenga, Michael Williams, Fred Wilson, W. Kent Wilson, Kenneth P. Winkler, John F. Wippel, Jan Woleński, Allan B. Wolter, Nicholas P. Wolterstorff, Rega Wood, W. Jay Wood, Paul Woodruff, Alison Wylie, Gideon Yaffe, Takashi Yagisawa, Yutaka Yamamoto, Keith E. Yandell, Xiaomei Yang, Dean Zimmerman, Günter Zoller, Catherine Zuckert, Michael Zuckert, Jack A. Zupko (J.A.Z.)
- Edited by Robert Audi, University of Notre Dame, Indiana
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- The Cambridge Dictionary of Philosophy
- Published online:
- 05 August 2015
- Print publication:
- 27 April 2015, pp ix-xxx
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Metabolic Myopathies Discovered During Investigations of Statin Myopathy
- Steven K. Baker, Georgirene D. Vladutiu, Wendy L. Peltier, Paul J. Isackson, Mark A. Tarnopolsky
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- Canadian Journal of Neurological Sciences / Volume 35 / Issue 1 / March 2008
- Published online by Cambridge University Press:
- 02 December 2014, pp. 94-97
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The statins have emerged as the dominant class of drug for the treatment of hypercholesterolemia. These medications are generally well tolerated. However, myalgias, the most frequent side-effect, occur in up to 7% of patients. Transaminitis and skeletal myotoxicity, with elevated serum creatine kinase (CK) levels (i.e., >10 times the upper limit of normal), occur with reported frequencies of 1% and 0.1%, respectively. Various hypotheses have been proposed to explain the relationship between statin therapy and the spectrum of muscle dysfunction manifested by myalgia, myopathy, and rhabdomyolysis.
Statin-mediatd inhibition of mevalonate metabolism impairs the synthesis of isoprenylated products–the most notable of which is ubiquinone. However, isoprenylation is responsible for the post-translational modification of up to 2% of cellular proteins. Therefore, numerous metabolic pathways are potentially modified by statin-mediated hypoprenylation. Subclinical defects in one or more energy-deriving pathways may be unmasked upon exposure to the pleotropic effects of statins. Such pharmacogenomic synergism may underlie the development of “statin myopathy” in a subset of patients. In this regard, we describe four patients with mutations in the myophosphorylase (PYGM; MIM 232600), myoadenylate deaminase (AMPD1; MIM 102770), and carnitine palmitoyltransferase (CPT2; MIM 600650) genes whose diagnoses became apparent during the course of investigations for statin-induced myalgias and hyperCKemia.
Pseudoephedrine-induced Hemorrhage Associated with a Cerebral Vascular Malformation
- Steven K. Baker, Jamie E. Silva, Ken K.S. Lam, Steven K. Baker
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- Canadian Journal of Neurological Sciences / Volume 32 / Issue 2 / May 2005
- Published online by Cambridge University Press:
- 02 December 2014, pp. 248-252
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Background:
Sympathomimetic-related intracerebral hemorrhage is well-documented. Most cases are associated with phenylpropanolamine use.
Case Report:We report a case of intracerebral hemorrhage occurring in a middle-aged man who suffered from chronic sinusitis and had been ingesting pseudoephedrine daily for one year. This patient was previously well with no known cardiovascular risk factors. Clinical examination revealed no evidence of vasculitis nor coagulopathy and initial neuroimaging (i.e., computed tomography, angiography, magnetic resonance imaging) demonstrated no features consistent with aneurysm, arteriovenous malformation (AVM), cavernoma, nor cerebral metastases. A follow-up cerebral angiogram demonstrated a small AVM arising off a branch of the pericallosal artery and a small arteriovenous fistula arising off the costal marginal branch. The AVM was embolized without incident, however, the AVF was not accessible.
Conclusions:Sympathomimetics have long been associated with intracerebral hemorrhage. Since 1979, over 30 published case reports have documented the relationship between phenylpropanolamine and stroke. Only one report links phenylpropanolamine consumption to an intracerebral hemorrhage in a patient with an AVM. There is a paucity of literature etiologically inculpating other ephedra alkaloids in the causation of intracerebral hemorrhage. This is a case of pseudoephedrine-induced intracerebral hemorrhage in a patient with an underlying vascular malformation.
A Neuromuscular Approach to Statin-Related Myotoxicity
- Steven K. Baker, Imtiaz A. Samjoo
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- Canadian Journal of Neurological Sciences / Volume 35 / Issue 1 / March 2008
- Published online by Cambridge University Press:
- 02 December 2014, pp. 8-21
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Approximately 95% of statin-treated patients tolerate this form of cholesterol management without any adverse effects. However, given their efficacy in reducing low density lipoproteins and cardiovascular events large numbers of patients are selected for statin therapy. Therefore muscle complications are, in fact, quite common. Limited understanding of the underlying pathophysiology has hampered physicians' ability to identify patients at risk for developing statin myotoxicity. A growing number of published case reports/series have implicated statins in the exacerbation of both acquired and genetic myopathies. A clinical management algorithm is presented which outlines a variety of co-morbidities which can potentiate the adverse effects of statins on muscle. In addition, a rational approach to the selection of those patients most likely to benefit from skeletal muscle biopsy is discussed. Ongoing work will define the extent to which statin-intolerant patients represent carriers of recessive metabolic myopathies or pre-symptomatic acquired myopathies. The expanding importance of pharmacogenomics will undoubtedly be realized in the field of statin myopathy research within the next few years. Such critical information is needed to establish more definitive management and diagnostic strategies.
Contributors
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- By Dor Abrahamson, Jerry Andriessen, Roger Azevedo, Michael Baker, Ryan Baker, Sasha Barab, Carl Bereiter, Susan Bridges, Mario Carretero, Carol K. K. Chan, Clark A. Chinn, Paul Cobb, Allan Collins, Kevin Crowley, Elizabeth A. Davis, Chris Dede, Sharon J. Derry, Andrea A. diSessa, Michael Eisenberg, Yrjö Engeström, Noel Enyedy, Barry J. Fishman, Ricki Goldman, James G. Greeno, Erica Rosenfeld Halverson, Cindy E. Hmelo-Silver, Michael J. Jacobson, Sanna Järvelä, Yasmin B. Kafai, Yael Kali, Manu Kapur, Paul A. Kirschner, Karen Knutson, Timothy Koschmann, Joseph S. Krajcik, Carol D. Lee, Peter Lee, Robb Lindgren, Jingyan Lu, Richard E. Mayer, Naomi Miyake, Na’ilah Suad Nasir, Mitchell J. Nathan, Narcis Pares, Roy Pea, James W. Pellegrino, William R. Penuel, Palmyre Pierroux, Brian J. Reiser, K. Ann Renninger, Ann S. Rosebery, R. Keith Sawyer, Marlene Scardamalia, Anna Sfard, Mike Sharples, Kimberly M. Sheridan, Bruce L. Sherin, Namsoo Shin, George Siemens, Peter Smagorinsky, Nancy Butler Songer, James P. Spillane, Kurt Squire, Gerry Stahl, Constance Steinkuehler, Reed Stevens, Daniel Suthers, Iris Tabak, Beth Warren, Uri Wilensky, Philip H. Winne, Carmen Zahn
- Edited by R. Keith Sawyer, University of North Carolina, Chapel Hill
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- The Cambridge Handbook of the Learning Sciences
- Published online:
- 05 November 2014
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- 17 November 2014, pp xv-xviii
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Statin-associated Autoimmune Myopathies: A Pathophysiologic Spectrum
- Yufan Wu, Boleslaw Lach, John P. Provias, Mark A. Tarnopolsky, Steven K. Baker
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- Journal:
- Canadian Journal of Neurological Sciences / Volume 41 / Issue 5 / September 2014
- Published online by Cambridge University Press:
- 30 October 2014, pp. 638-647
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Background
Statins have recently been reported to cause a rare autoimmune inflammatory and/or necrotic myopathy that begins or persists after drug cessation.
MethodsWe report on 26 patients seen at a neuromuscular centre between 2005 and 2011 who demonstrated muscle weakness/myalgias and creatine kinase elevations during or after statin treatment with continuation of signs and symptoms despite statin withdrawal.
ResultsAll patients were treated with immunosuppressive therapy with good response; all improved biochemically and 86% improved clinically. Sixty-five percent of patients who attempted to taper off immunosuppressive therapy relapsed. We report on a novel finding whereby five of the seven patients who underwent multiple biopsies throughout their disease demonstrated a transformation of their histological diagnosis, with four progressing from having myofibre necrosis with minimal or no inflammation to a diagnosis of polymyositis.
ConclusionsThis study offers preliminary evidence that statin-associated necrotizing myopathy and statin-associated polymyositis may not be separate entities but are part of the same pathophysiological spectrum. Both entities respond well to immunosuppression.
Contributors
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- By Rose Teteki Abbey, K. C. Abraham, David Tuesday Adamo, LeRoy H. Aden, Efrain Agosto, Victor Aguilan, Gillian T. W. Ahlgren, Charanjit Kaur AjitSingh, Dorothy B E A Akoto, Giuseppe Alberigo, Daniel E. Albrecht, Ruth Albrecht, Daniel O. Aleshire, Urs Altermatt, Anand Amaladass, Michael Amaladoss, James N. Amanze, Lesley G. Anderson, Thomas C. Anderson, Victor Anderson, Hope S. Antone, María Pilar Aquino, Paula Arai, Victorio Araya Guillén, S. Wesley Ariarajah, Ellen T. Armour, Brett Gregory Armstrong, Atsuhiro Asano, Naim Stifan Ateek, Mahmoud Ayoub, John Alembillah Azumah, Mercedes L. García Bachmann, Irena Backus, J. Wayne Baker, Mieke Bal, Lewis V. Baldwin, William Barbieri, António Barbosa da Silva, David Basinger, Bolaji Olukemi Bateye, Oswald Bayer, Daniel H. Bays, Rosalie Beck, Nancy Elizabeth Bedford, Guy-Thomas Bedouelle, Chorbishop Seely Beggiani, Wolfgang Behringer, Christopher M. Bellitto, Byard Bennett, Harold V. Bennett, Teresa Berger, Miguel A. Bernad, Henley Bernard, Alan E. Bernstein, Jon L. Berquist, Johannes Beutler, Ana María Bidegain, Matthew P. Binkewicz, Jennifer Bird, Joseph Blenkinsopp, Dmytro Bondarenko, Paulo Bonfatti, Riet en Pim Bons-Storm, Jessica A. Boon, Marcus J. Borg, Mark Bosco, Peter C. Bouteneff, François Bovon, William D. Bowman, Paul S. Boyer, David Brakke, Richard E. Brantley, Marcus Braybrooke, Ian Breward, Ênio José da Costa Brito, Jewel Spears Brooker, Johannes Brosseder, Nicholas Canfield Read Brown, Robert F. Brown, Pamela K. Brubaker, Walter Brueggemann, Bishop Colin O. Buchanan, Stanley M. Burgess, Amy Nelson Burnett, J. Patout Burns, David B. Burrell, David Buttrick, James P. Byrd, Lavinia Byrne, Gerado Caetano, Marcos Caldas, Alkiviadis Calivas, William J. Callahan, Salvatore Calomino, Euan K. Cameron, William S. Campbell, Marcelo Ayres Camurça, Daniel F. Caner, Paul E. Capetz, Carlos F. Cardoza-Orlandi, Patrick W. Carey, Barbara Carvill, Hal Cauthron, Subhadra Mitra Channa, Mark D. 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Yee, Viktor Yelensky, Yeo Khiok-Khng, Gustav K. K. Yeung, Angela Yiu, Amos Yong, Yong Ting Jin, You Bin, Youhanna Nessim Youssef, Eliana Yunes, Robert Michael Zaller, Valarie H. Ziegler, Barbara Brown Zikmund, Joyce Ann Zimmerman, Aurora Zlotnik, Zhuo Xinping
- Edited by Daniel Patte, Vanderbilt University, Tennessee
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- Book:
- The Cambridge Dictionary of Christianity
- Published online:
- 05 August 2012
- Print publication:
- 20 September 2010, pp xi-xliv
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On The Unique Structural Organization of the Saccharomyces Cerevisiae Pyruvate Dehydrogenase Complex
- James K. Stoops, Z. Hong Zhou, John P. Schroeter, Steven J. Kolodziej, R. Holland Cheng, Timothy S. Baker, Diane L. B. McCarthy, Mohammed A. Yazdi, Cheol-Young Maeng, Lester J. Reed
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- Journal:
- Microscopy and Microanalysis / Volume 4 / Issue S2 / July 1998
- Published online by Cambridge University Press:
- 02 July 2020, pp. 954-955
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- July 1998
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Dihydrohpoamide acetyl transferase (E2), a catalytic and structural component of a multienzyme complex that catalyzes the oxidative decarboxylation of pyruvate, forms the central core to which the other components are bound. We have utilized protein engineering and 3-D electron microscopy to study the structural organization of the largest multienzyme complex known (Mr ∼ 107). The structures of the truncated 60-mer core (tE2) and complexes of the tE2 associated with a binding protein (BP), and the BP associated with its dihydrohpoamide dehydrogenase (BP'E3) and the intact E2 associated with BP and the pyruvate dehydrogenase (E1) were determined (Figs. 1 and 2). The tE2 core is a pentagonal dodecahedron consisting of 20 cone-shaped trimers interconnected by 30 bridges.
Previous studies have given rise to the generally accepted belief that BP and BP'E3 components are bound on the outside of the E2 scaffold and that E1 is similarly bound to the core in variable positions by flexible tethers.