This week, part 2 of our 2-part primer on Huntington Disease. Treatment. From tried-and-true therapies to the latest-and-greatest compounds being studied in clinical trials. Enjoy! Produced by James E. Siegler and Stephen Aradi. Music by Mike Durek, Jesse Spillane, Lee Rosevere, Jason Shaw and Dr. Turtle. Sound effects by Mike Koenig, 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  Claassen DO, Carroll B, De Boer LM, Wu E, Ayyagari R, Gandhi S and Stamler D. Indirect tolerability comparison of Deutetrabenazine and Tetrabenazine for Huntington disease. J Clin Mov Disord. 2017;4:3. Paulsen JS, Nehl C, Hoth KF, Kanz JE, Benjamin M, Conybeare R, McDowell B and Turner B. Depression and stages of Huntington's disease. J Neuropsychiatry Clin Neurosci. 2005;17:496-502. Bates GP, Dorsey R, Gusella JF, Hayden MR, Kay C, Leavitt BR, Nance M, Ross CA, Scahill RI, Wetzel R, Wild EJ and Tabrizi SJ. Huntington disease. Nat Rev Dis Primers. 2015;1:15005. 

#127 HD part 2: Current and emerging therapeutics

BrainWaves is an academic audio podcast whose mission is to educate medical providers through clinical cases and topical reviews in neurology and medicine. Follow us on Twitter @brainwavesaudio, or just tune in every Thursday for the latest shows! **NOT FOR CLINICAL DECISION MAKING**

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This week on the program, we bring to you a special episode on the impact of the COVID-19 pandemic on the neurology 2021 match--and potentially MANY subsequent matches. Jim Siegler is joined by the Assistant Program Director of the Cooper Neurology Residency Program, Olga Thon, and the creators of the @NMatch2021 Twitter account (who you should DEFINITELY follow after listening to our episode). If you are an applicant for the 2021 cycle, this show is MANDATORY. (only kidding, this is just a podcast) Produced by James E. Siegler, with assistance by Olga Thon, Adriana Romirez, Dylan Del Papa, Justine Ker, and Alvin Singh. Music courtesy of Akash Gandi, Little Glass Men, Kevin MacLeod, Josh Woodward, Julie Maxwell, and Kai Engel. The opening theme was composed by Jimothy Dalton. Sound effects by Mike Koenig and Daniel Simion. Unless otherwise mentioned in the podcast, no competing financial interests exist in the content of this episode. 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.

#173 Matching during the COVID-19 pandemic

Halloween is one of the most exciting and festive holidays, but this year I imagine many of us are going to spend it indoors. To help pass the time, enjoy this seasonal special about the neurologic manifestations of zombie-ism. Today's program is a re-run from 2017, featuring Dr. Brian Hanrahan, and has been remastered and updated with some recent additions in light of the coronavirus pandemic. Produced by James E. Siegler. Music courtesy of Andrew Sacco, Ars Sonor, Yan Terrien, and Unheard Music Concepts. The opening theme was composed by Jimothy Dalton. Sound effects by Mike Koenig, with some original recordings out of Studio 3. Unless otherwise mentioned in the podcast, no competing financial interests exist in the content of this episode. 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  Smith TC. Zombie infections: Epidemiology, treatment, and prevention. Bmj. 2015;351:h6423 Adams AJ, Banister SD, Irizarry L, Trecki J, Schwartz M, Gerona R. "Zombie" outbreak caused by the synthetic cannabinoid amb-fubinaca in new york. The New England journal of medicine. 2017;376:235-242 Rabinovitz B, Jaywant A, Fridman CB. Neuropsychological functioning in severe acute respiratory disorders caused by the coronavirus: Implications for the current covid-19 pandemic. Clin Neuropsychol. 2020:1-27 Gilmour SJ, Saito E, Yoneoka D. Importance of survival strategies after a zombie pandemic. Bmj. 2016;532:i259 Hughes DP, Andersen SB, Hywel-Jones NL, Himaman W, Billen J, Boomsma JJ. Behavioral mechanisms and morphological symptoms of zombie ants dying from fungal infection. BMC Ecol. 2011;11:13 

#83 Halloween Special: Zombies in neurology

You know the triad for normal pressure hydrocephalus (NPH). Wet, wobbly, and wacky. And you have probably heard of the Evan’s index—the relative proportion of the lateral ventricles in reference to the inner table of the skull. But you might not have heard of high-convexity tight sulci. Now you have. Produced by James E. Siegler. Music courtesy of Jason Shaw, Javolenus, and Lee Rosevere, under a Creative Commons License. The opening theme was composed by Jimothy Dalton. 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  Kitagaki H, Mori E, Ishii K, Yamaji S, Hirono N and Imamura T. CSF spaces in idiopathic normal pressure hydrocephalus: morphology and volumetry. AJNR American journal of neuroradiology. 1998;19:1277-84. Sasaki M, Honda S, Yuasa T, Iwamura A, Shibata E and Ohba H. Narrow CSF space at high convexity and high midline areas in idiopathic normal pressure hydrocephalus detected by axial and coronal MRI. Neuroradiology. 2008;50:117-22. Allali G, Laidet M, Armand S, Momjian S, Marques B, Saj A and Assal F. A combined cognitive and gait quantification to identify normal pressure hydrocephalus from its mimics: The Geneva's protocol. Clinical neurology and neurosurgery. 2017;160:5-11. Graff-Radford J, Gunter JL, Jones DT, Przybelski SA, Schwarz CG, Huston J, 3rd, Lowe V, Elder BD, Machulda MM, Gunter NB, Petersen RC, Kantarci K, Vemuri P, Mielke MM, Knopman DS, Graff-Radford NR and Jack CR, Jr. Cerebrospinal fluid dynamics disorders: Relationship to Alzheimer biomarkers and cognition. Neurology. 2019;93:e2237-e2246. 

#171 High-convexity tight sulci

The locked-in syndrome is a rare clinical consequence following many types of neurologic injury. In general, the locked-in patient is fully paralyzed, with perhaps minimal function of the fingers, the eyes, or the mouth. What's more, the gross motor dysfunction is enormously disproportional to the cognitive function of the individual. The locked-in patient is conscious and completely aware of their surroundings. They can often hear, see, smell, and feel just as any other person would. But because of their profound physical disability, they have a very limited means of communicating even the simplest thoughts. "I feel hot." "My head hurts." "My cheek itches." It may surprise you that the patient's perspective of their condition is wholly different from how the healthcare provider or caregiver imagines it to be. This week on the program, Dr. Lauren Elman (Pennsylvania Medical Center ALS Multi-disiplinary Clinic) reviews this discrepancy. Dr. Elman also shares her experience  managing this inevitable consequence of ALS when all life-sustaining measures are desired. REFERENCES  Patterson JR and Grabois M. Locked-in syndrome: a review of 139 cases. Stroke; a journal of cerebral circulation. 1986;17:758-64. Trail M, Nelson ND, Van JN, Appel SH and Lai EC. A study comparing patients with amyotrophic lateral sclerosis and their caregivers on measures of quality of life, depression, and their attitudes toward treatment options. Journal of the neurological sciences. 2003;209:79-85. Rousseau MC, Baumstarck K, Alessandrini M, Blandin V, Billette de Villemeur T and Auquier P. Quality of life in patients with locked-in syndrome: Evolution over a 6-year period. Orphanet J Rare Dis. 2015;10:88. Kuzma-Kozakiewicz M, Andersen PM, Ciecwierska K, Vazquez C, Helczyk O, Loose M, Uttner I, Ludolph AC and Lule D. An observational study on quality of life and preferences to sustain life in locked-in state. Neurology. 2019;93:e938-e945. Niedermeyer S, Murn M and Choi PJ. Respiratory Failure in Amyotrophic Lateral Sclerosis. Chest. 2019;155:401-408. 

#168 (Mis)understanding the locked-in state

On rounds, I sometimes joke that the two most important organs in the body are the right and left hemisphere. Only one of many, terrible, dad jokes my poor residents and students have to endure. But what happens when one organ fails, or you have to remove it? This week on BrainWaves, we’ll review a unique neurologic condition where such incredible hemispheric dysfunction can only be treated by surgically removing half of the brain. And OMG, a show about pediatrics! ** IF YOU’RE TAKING YOUR NEUROLOGY BOARDS THIS SUMMER, CHECK OUT THE PENN NEUROLOGY BOARD REVIEW COURSE AT https://upenn.cloud-cme.com/default.aspx?P=5&EID;=65373. AND FOR A DISCOUNTED RATE ON THE ONLINE OR STREAMING RESOURCES, USE PROMO CODE ‘WAVES2020’. ** Produced by James E. Siegler. Music courtesy of Ars Sonor, Daniel Birch, Jon Watts, and Lish Grooves. The opening theme was composed by Jimothy Dalton. Sound effects by Mike Koenig and Daniel Simion. Unless otherwise mentioned in the podcast, no competing financial interests exist in the content of this episode. 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  Rasmussen T, Olszewski J and Lloydsmith D. Focal seizures due to chronic localized encephalitis. Neurology. 1958;8:435-45. Hart YM, Cortez M, Andermann F, Hwang P, Fish DR, Dulac O, Silver K, Fejerman N, Cross H, Sherwin A and et al. Medical treatment of Rasmussen's syndrome (chronic encephalitis and epilepsy): effect of high-dose steroids or immunoglobulins in 19 patients. Neurology. 1994;44:1030-6. Andrews PI, Dichter MA, Berkovic SF, Newton MR and McNamara JO. Plasmapheresis in Rasmussen's encephalitis. Neurology. 1996;46:242-6. Leach JP, Chadwick DW, Miles JB and Hart IK. Improvement in adult-onset Rasmussen's encephalitis with long-term immunomodulatory therapy. Neurology. 1999;52:738-42. Bien CG, Granata T, Antozzi C, Cross JH, Dulac O, Kurthen M, Lassmann H, Mantegazza R, Villemure JG, Spreafico R and Elger CE. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. Brain. 2005;128:454-71. Varadkar S, Bien CG, Kruse CA, Jensen FE, Bauer J, Pardo CA, Vincent A, Mathern GW and Cross JH. Rasmussen's encephalitis: clinical features, pathobiology, and treatment advances. The Lancet Neurology. 2014;13:195-205. Tan AP, Wong YLJ, Lin BJ, Yong HRC and Mankad K. Clinico-radiological approach to cerebral hemiatrophy. Childs Nerv Syst. 2018;34:2377-2390. 

#127 HD part 2: Current and emerging therapeutics

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