#158 Teaching through clinical cases: A primer on proximal weakness

03/05/20 • 18 min

Generalized weakness is a common complaint. Much of the time, we as neurologists don’t even see these patients in the hospital or the clinic. But more often than not, “generalized” weakness may be the only way a patient describes difficulty brushing their hair or climbing stairs. In these situations, it’s not exactly generalized weakness. It’s something else. This week on BrainWaves, we review the major patterns of weakness that characterize the most common muscle disorders. With a quick review of EMG findings. Enjoy!

Produced by James E. Siegler. Music courtesy of Jahzzar, Kevin McLeod, Patches, and Montplaisir. Unless otherwise mentioned in the podcast, no competing financial interests exist in the content of this episode. Sound effects by Mike Koenig and Daniel Simion. BrainWaves' podcasts and online content are intended for medical education only and should not be used for clinical decision making. Be sure to follow us on Twitter @brainwavesaudio for the latest updates to the podcast.

REFERENCES

Suresh E and Wimalaratna S. Proximal myopathy: diagnostic approach and initial management. Postgraduate medical journal. 2013;89:470-7.
Walter MC, Reilich P, Thiele S, Schessl J, Schreiber H, Reiners K, Kress W, Muller-Reible C, Vorgerd M, Urban P, Schrank B, Deschauer M, Schlotter-Weigel B, Kohnen R and Lochmuller H. Treatment of dysferlinopathy with deflazacort: a double-blind, placebo-controlled clinical trial. Orphanet J Rare Dis. 2013;8:26.
Fayssoil A, Ogna A, Chaffaut C, Chevret S, Guimaraes-Costa R, Leturcq F, Wahbi K, Prigent H, Lofaso F, Nardi O, Clair B, Behin A, Stojkovic T, Laforet P, Orlikowski D and Annane D. Natural History of Cardiac and Respiratory Involvement, Prognosis and Predictive Factors for Long-Term Survival in Adult Patients with Limb Girdle Muscular Dystrophies Type 2C and 2D. PloS one. 2016;11:e0153095.
Harris E, Bladen CL, Mayhew A, James M, Bettinson K, Moore U, Smith FE, Rufibach L, Cnaan A, Bharucha-Goebel DX, Blamire AM, Bravver E, Carlier PG, Day JW, Diaz-Manera J, Eagle M, Grieben U, Harms M, Jones KJ, Lochmuller H, Mendell JR, Mori-Yoshimura M, Paradas C, Pegoraro E, Pestronk A, Salort-Campana E, Schreiber-Katz O, Semplicini C, Spuler S, Stojkovic T, Straub V, Takeda S, Rocha CT, Walter MC, Bushby K and Jain COSC. The Clinical Outcome Study for dysferlinopathy: An international multicenter study. Neurol Genet. 2016;2:e89.
Clark KEN and Isenberg DA. A review of inflammatory idiopathic myopathy focusing on polymyositis. European journal of neurology : the official journal of the European Federation of Neurological Societies. 2018;25:13-23.
Wicklund MP. The Limb-Girdle Muscular Dystrophies. Continuum (Minneap Minn). 2019;25:1599-1618.
Preston DC and Shapiro BE. Electromyography and neuromuscular disorders: clinical-electrophysiologic correlations. 3rd ed. London; New York: Elsevier Saunders; 2013.
“rAAVrh74.MHCK7.DYSF.DV for Treatment of Dysferlinopathies.” Clinicaltrials.gov. Retrieved from https://clinicaltrials.gov/ct2/show/NCT02710500?cond=dysferlinopathy&draw=2&rank=1 on 22 December 2019.

For great video examples of EMG findings in nerve and muscle disease, check out KImura & Kohara's YouTube recordings: https://www.youtube.com/watch?v=6-3PP_S-Q8I&list=PLCC_NQWOABCY18PEzlGELGOR_-IDNaDQ_

REFERENCES

Suresh E and Wimalaratna S. Proximal myopathy: diagnostic approach and initial management. Postgraduate medical journal. 2013;89:470-7.
Walter MC, Reilich P, Thiele S, Schessl J, Schreiber H, Reiners K, Kress W, Muller-Reible C, Vorgerd M, Urban P, Schrank B, Deschauer M, Schlotter-Weigel B, Kohnen R and Lochmuller H. Treatment of dysferlinopathy with deflazacort: a double-blind, placebo-controlled clinical trial. Orphanet J Rare Dis. 2013;8:26.
Fayssoil A, Ogna A, Chaffaut C, Chevret S, Guimaraes-Costa R, Leturcq F, Wahbi K, Prigent H, Lofaso F, Nardi O, Clair B, Behin A, Stojkovic T, Laforet P, Orlikowski D and Annane D. Natural History of Cardiac and Respiratory Involvement, Prognosis and Predictive Factors for Long-Term Survival in Adult Patients with Limb Girdle Muscular Dystrophies Type 2C and 2D. PloS one. 2016;11:e0153095.
Harris E, Bladen CL, Mayhew A, James M, Bettinson K, Moore U, Smith FE, Rufibach L, Cnaan A, Bharucha-Goebel DX, Blamire AM, Bravver E, Carlier PG, Day JW, Diaz-Manera J, Eagle M, Grieben U, Harms M, Jones KJ, Lochmuller H, Mendell JR, Mori-Yoshimura M, Paradas C, Pegoraro E, Pestronk A, Salort-Campana E, Schreiber-Katz O, Semplicini C, Spuler S, Stojkovic T, Straub V, Takeda S, Rocha CT, Walter MC, Bushby K and Jain COSC. The Clinical Outcome Study for dysferlinopathy: An international multicenter study. Neurol Genet. 2016;2:e89.
Clark KEN and Isenberg DA. A review of inflammatory idiopathic myopathy focusing on polymyositis. European journal of neurology : the official journal of the European Federation of Neurological Societies. 2018;25:13-23.
Wicklund MP. The Limb-Girdle Muscular Dystrophies. Continuum (Minneap Minn). 2019;25:1599-1618.
Preston DC and Shapiro BE. Electromyography and neuromuscular disorders: clinical-electrophysiologic correlations. 3rd ed. London; New York: Elsevier Saunders; 2013.
“rAAVrh74.MHCK7.DYSF.DV for Treatment of Dysferlinopathies.” Clinicaltrials.gov. Retrieved from https://clinicaltrials.gov/ct2/show/NCT02710500?cond=dysferlinopathy&draw=2&rank=1 on 22 December 2019.

Previous Episode

#80 The meningitis that keeps coming back: 2020 update

This week on BrainWaves, we're revisiting the 2017 episode on recurrent meningitis featuring Dr. Jon Rosenberg (New York Presbyterian-Columbia). While we may not have made much headway in the treatment of these patients since the original show came out, we have made some progress in the neurodiagnostics.

Produced by James E. Siegler and Jon Rosenberg. Music courtesy of Coldnoise, Three Chain Links, and Uncanny. Unless otherwise mentioned in the podcast, no competing financial interests exist in the content of this episode. Sound effects by Mike Koenig and Daniel Simion. BrainWaves' podcasts and online content are intended for medical education only and should not be used for clinical decision making. Be sure to follow us on Twitter @brainwavesaudio for the latest updates to the podcast.

REFERENCES

Zunt JR and Baldwin KJ. Chronic and subacute meningitis. Continuum (Minneap Minn). 2012;18:1290-318.
Ginsberg L and Kidd D. Chronic and recurrent meningitis. Pract Neurol. 2008;8:348-61.
Rosenberg J and Galen BT. Recurrent Meningitis. Curr Pain Headache Rep. 2017;21:33.
Baldwin KJ and Avila JD. Diagnostic Approach to Chronic Meningitis. Neurol Clin. 2018;36:831-849.
Wilson MR, O'Donovan BD, Gelfand JM, Sample HA, Chow FC, Betjemann JP, Shah MP, Richie MB, Gorman MP, Hajj-Ali RA, Calabrese LH, Zorn KC, Chow ED, Greenlee JE, Blum JH, Green G, Khan LM, Banerji D, Langelier C, Bryson-Cahn C, Harrington W, Lingappa JR, Shanbhag NM, Green AJ, Brew BJ, Soldatos A, Strnad L, Doernberg SB, Jay CA, Douglas V, Josephson SA and DeRisi JL. Chronic Meningitis Investigated via Metagenomic Next-Generation Sequencing. JAMA Neurol. 2018;75:947-955.
Wilson MR, Sample HA, Zorn KC, Arevalo S, Yu G, Neuhaus J, Federman S, Stryke D, Briggs B, Langelier C, Berger A, Douglas V, Josephson SA, Chow FC, Fulton BD, DeRisi JL, Gelfand JM, Naccache SN, Bender J, Dien Bard J, Murkey J, Carlson M, Vespa PM, Vijayan T, Allyn PR, Campeau S, Humphries RM, Klausner JD, Ganzon CD, Memar F, Ocampo NA, Zimmermann LL, Cohen SH, Polage CR, DeBiasi RL, Haller B, Dallas R, Maron G, Hayden R, Messacar K, Dominguez SR, Miller S and Chiu CY. Clinical Metagenomic Sequencing for Diagnosis of Meningitis and Encephalitis. The New England journal of medicine. 2019;380:2327-2340.

Next Episode

#159 The pupil can be a great teacher

They say the eyes are the window to the soul. Well, maybe. They also happen to be the first organ we (perhaps, unknowingly) examine whenever we encounter a patient. This week on the program, we build on the prior episode about anisocoria (featuring Dr. Ali Hamedani, episode 74) and discuss the examination of the pupil, and all that these 3-5 millimeters of tissue can teach us.

Plus, some Dad jokes at the end...

Produced by James E. Siegler. Music courtesy of Chris Haugen, Daniel Birch, and Lee Roosevere. Unless otherwise mentioned in the podcast, no competing financial interests exist in the content of this episode. Sound effects by Mike Koenig and Daniel Simion. BrainWaves' podcasts and online content are intended for medical education only and should not be used for clinical decision making. Be sure to follow us on Twitter @brainwavesaudio for the latest updates to the podcast.

REFERENCES

Bouffard MA. The Pupil. Continuum (Minneap Minn). 2019;25:1194-1214.
Liu G, Volpe NJ and Galetta S. Neuro-Ophthalmology: Diagnosis and Management: Elsevier; 2018.

https://www.amazon.com/Liu-Volpe-Galettas-Neuro-Ophthalmology-Management/dp/032334044X