BrainWaves Podcast

Although you should not confuse this with amaurosis fugax, a transient loss of vision in one eye usually screams vascular event. But not always... Keep an eye out for how the plot thickens in this week's episode of BrainWaves. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. The content in this episode was vetted and approved by Imran Jivraj. REFERENCES 1. Biousse V and Trobe JD. Transient monocular visual loss. Am J Ophthalmol. 2005;140:717-21. 2. Kattah JC, Wang DZ and Reddy C. Intravenous recombinant tissue-type plasminogen activator thrombolysis in treatment of central retinal artery occlusion. Arch Ophthalmol. 2002;120:1234-6. 3. Schumacher M, Schmidt D, Jurklies B, Gall C, Wanke I, Schmoor C, Maier-Lenz H, Solymosi L, Brueckmann H, Neubauer AS, Wolf A, Feltgen N and Group EA-S. Central retinal artery occlusion: local intra-arterial fibrinolysis versus conservative treatment, a multicenter randomized trial. Ophthalmology. 2010;117:1367-75 e1. 4. Ravits J and Seybold ME. Transient monocular visual loss from narrow-angle glaucoma. Archives of neurology. 1984;41:991-3.

Trauma and hypertension account for the overwhelming majority of cases of intracerebral hemorrhage. Today, we address the minority. In this week's episode, Dr. Steven Messe discusses the atypical causes of ICH and how they are managed. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. REFERENCES 1. Biffi A and Greenberg SM. Cerebral amyloid angiopathy: a systematic review. J Clin Neurol. 2011;7:1-9. 2. Gilden D, Cohrs RJ, Mahalingam R and Nagel MA. Varicella zoster virus vasculopathies: diverse clinical manifestations, laboratory features, pathogenesis, and treatment. The Lancet Neurology. 2009;8:731-40. 3. Mast H, Young WL, Koennecke HC, Sciacca RR, Osipov A, Pile-Spellman J, Hacein-Bey L, Duong H, Stein BM and Mohr JP. Risk of spontaneous haemorrhage after diagnosis of cerebral arteriovenous malformation. Lancet. 1997;350:1065-8. 4. Ruiz-Sandoval JL, Cantu C and Barinagarrementeria F. Intracerebral hemorrhage in young people: analysis of risk factors, location, causes, and prognosis. Stroke; a journal of cerebral circulation. 1999;30:537-41. 5. Siegler JE and Ichord RN. Teaching NeuroImages: Multicompartmental intracranial hemorrhage in a pediatric patient. Neurology. 2016;87:e284.

Yeah, we talk about Bell's Palsy here. A lot actually. But there's also hemifacial spasm, blepharospasm, facial myokimia, and pathologic sialorrhea as it pertains to neurodegenerative disease. Not to mention the facial nerve anatomy! Sit down and buckle up, you may want to take notes on this one. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. Any cases discussed in this episode are fictional and do not contain any patient health identifying information. REFERENCES 1, Gilden DH. Clinical practice. Bell's Palsy. The New England journal of medicine. 2004;351:1323-31. 2. Sweeney CJ and Gilden DH. Ramsay Hunt syndrome. Journal of neurology, neurosurgery, and psychiatry. 2001;71:149-54. 3. Gaio E, Marioni G, de Filippis C, Tregnaghi A, Caltran S and Staffieri A. Facial nerve paralysis secondary to acute otitis media in infants and children. J Paediatr Child Health. 2004;40:483-6.

This week's BrainWaves episode features a case of a middle-aged woman with progressive headache and cranial neuropathies. The diagnosis of aseptic meningitis is made and a differential diagnosis is illustrated. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. Any cases discussed in this episode are fictional and do not contain any patient health identifying information. REFERENCES 1. Nigrovic LE. Aseptic meningitis. Handbook of clinical neurology. 2013;112:1153-6. 2. van de Beek D, de Gans J, Spanjaard L, Weisfelt M, Reitsma JB and Vermeulen M. Clinical features and prognostic factors in adults with bacterial meningitis. The New England journal of medicine. 2004;351:1849-59. 3. Hasbun R, Abrahams J, Jekel J and Quagliarello VJ. Computed tomography of the head before lumbar puncture in adults with suspected meningitis. The New England journal of medicine. 2001;345:1727-33. 4. Lee BE, Chawla R, Langley JM, Forgie SE, Al-Hosni M, Baerg K, Husain E, Strong J, Robinson JL, Allen U, Law BJ, Dobson S and Davies HD. Paediatric Investigators Collaborative Network on Infections in Canada (PICNIC) study of aseptic meningitis. BMC Infect Dis. 2006;6:68. 5. Negrini B, Kelleher KJ and Wald ER. Cerebrospinal fluid findings in aseptic versus bacterial meningitis. Pediatrics. 2000;105:316-9. 6. Logan SA and MacMahon E. Viral meningitis. Bmj. 2008;336:36-40. 7. Ginsberg L and Kidd D. Chronic and recurrent meningitis. Pract Neurol. 2008;8:348-61. 8. Jolles S, Sewell WA and Leighton C. Drug-induced aseptic meningitis: diagnosis and management. Drug Saf. 2000;22:215-26. 9. Chamberlain MC and Glantz M. Myelomatous meningitis. Cancer. 2008;112:1562-7. 10. Nieuwenhuizen L and Biesma DH. Central nervous system myelomatosis: review of the literature. Eur J Haematol. 2008;80:1-9. 11. Sobol U and Stiff P. Neurologic aspects of plasma cell disorders. Handbook of clinical neurology. 2014;120:1083-99.

This week, I'm handing the mic over to Dr. Laura Mainardi so she can give us some pointers on the evaluation of hand weakness. We're thumbing through a few tips on working up wrist drop in particular. Hoping you give us two thumbs up on this one! ;-) BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. REFERENCES 1. Preston DC & Shapiro BE. Electromyography and neuromuscular disorders, 3rd ed. Saunders (2013).

Prions are perhaps some of the most terrifying infectious particles known to man. But did you know that patients with some prion diseases actually shed these contagious proteins in their urine? These and other facts about prion diseases are discussed in this week's episode of BrainWaves. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. REFERENCES 1. Zabel MD and Reid C. A brief history of prions. Pathog Dis. 2015;73:ftv087. 2. Geschwind MD. Prion Diseases. Continuum (Minneap Minn). 2015;21:1612-38. 3. Rutala WA and Weber DJ. Creutzfeldt-Jakob disease: recommendations for disinfection and sterilization. Clin Infect Dis. 2001;32:1348-56. 4. Glatzel M, Abela E, Maissen M and Aguzzi A. Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease. The New England journal of medicine. 2003;349:1812-20. 5. Moda F, Gambetti P, Notari S, Concha-Marambio L, Catania M, Park KW, Maderna E, Suardi S, Haik S, Brandel JP, Ironside J, Knight R, Tagliavini F and Soto C. Prions in the urine of patients with variant Creutzfeldt-Jakob disease. The New England journal of medicine. 2014;371:530-9. 6. Wieser HG, Schindler K and Zumsteg D. EEG in Creutzfeldt-Jakob disease. Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology. 2006;117:935-51. 7. Lapergue B, Demeret S, Denys V, Laplanche JL, Galanaud D, Verny M, Sazdovitch V, Baulac M, Haik S, Hauw JJ, Bolgert F, Brandel JP and Navarro V. Sporadic Creutzfeldt-Jakob disease mimicking nonconvulsive status epilepticus. Neurology. 2010;74:1995-9. 8. Siegler JE, Jacobs DA, Amado D, Adams JL and Berger JR. Rapidly progressive dementia with hypoglycorrhachia. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia. 2015;22:1685-7.

I am not sure there are many more things terrifying than watching your child experience what looks like, just a cold, and then over the course of a few hours becomes paralyzed. In this episode, Drs. Ana Cristancho and Sarah Hopkins describe what we know about this recent polio-like outbreak in the US. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. This episode was vetted and approved by Dr. Sarah Hopkins. REFERENCES 1. Aliabadi N, Messacar K, Pastula DM, Robinson CC, Leshem E, Sejvar JJ, Nix WA, Oberste MS, Feikin DR and Dominguez SR. Enterovirus D68 Infection in Children with Acute Flaccid Myelitis, Colorado, USA, 2014. Emerg Infect Dis. 2016;22:1387-94. 2. Greninger AL, Naccache SN, Messacar K, Clayton A, Yu G, Somasekar S, Federman S, Stryke D, Anderson C, Yagi S, Messenger S, Wadford D, Xia D, Watt JP, Van Haren K, Dominguez SR, Glaser C, Aldrovandi G and Chiu CY. A novel outbreak enterovirus D68 strain associated with acute flaccid myelitis cases in the USA (2012-14): a retrospective cohort study. Lancet Infect Dis. 2015;15:671-82. 3. Messacar K, Schreiner TL, Van Haren K, Yang M, Glaser CA, Tyler KL and Dominguez SR. Acute flaccid myelitis: A clinical review of US cases 2012-2015. Annals of neurology. 2016;80:326-38.

The acute vestibular syndrome is easy enough to identify, but it can be a challenge to diagnose. In this week's episode of BrainWaves, Dr. Ali Hamedani tries to simplify the approach to acute, intermittent, and chronic complaints of dizziness. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. Any cases discussed in this episode are fictional and do not contain any patient health identifying information. REFERENCES Kerber KA. Acute constant dizziness. Continuum (Minneap Minn). 2012;18:1041-59. Lempert T. Vestibular migraine. Semin Neurol. 2013;33:212-8. Kim JS and Zee DS. Clinical practice. Benign paroxysmal positional vertigo. The New England journal of medicine. 2014;370:1138-47. Kattah JC, Talkad AV, Wang DZ, Hsieh YH and Newman-Toker DE. HINTS to diagnose stroke in the acute vestibular syndrome: three-step bedside oculomotor examination more sensitive than early MRI diffusion-weighted imaging. Stroke; a journal of cerebral circulation. 2009;40:3504-10.

For those of you who've witnessed it, giving levodopa to a patient with idiopathic Parkinson Disease is almost a miracle to watch. Unlike practically every other condition afflicting the nervous system, the unmistakable unrest of PD essentially surrenders to this tiny tablet. But what did we use before levodopa? In this week's episode of BrainWaves, we recount the history (and serendipity) behind the development of this miracle drug. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. REFERENCES 1. Parkinson J. An essay on the shaking palsy. 1817. J Neuropsychiatry Clin Neurosci. 2002;14:223-36; discussion 222. 2. Glatstein M, Alabdulrazzaq F and Scolnik D. Belladonna Alkaloid Intoxication: The 10-Year Experience of a Large Tertiary Care Pediatric Hospital. Am J Ther. 2016;23:e74-7. 3. Koranyi EK. A preamble on parkinsonism. J Psychiatry Neurosci. 1999;24:296-9. 4. Bucy P and Case T. Tremor: Physiologic mechanism and abolition by surgical means. Arch Neurol Psych. 1939;41:721-746. 5. Walter BL, Abosch A and Vitek JL. From Neuroscience to Neurology: Molecular Medicine, and the Therapeutic Transformation of Neurology 2004. 6. Hornykiewicz O. A brief history of levodopa. Journal of neurology. 2010;257:S249-52. 7. Tolosa E, Marti MJ, Valldeoriola F and Molinuevo JL. History of levodopa and dopamine agonists in Parkinson's disease treatment. Neurology. 1998;50:S2-10; discussion S44-8. 8. Birkmayer W and Hornykiewicz O. [The L-3,4-dioxyphenylalanine (DOPA)-effect in Parkinson-akinesia]. Wien Klin Wochenschr. 1961;73:787-8. 9. Cotzias GC, Van Woert MH and Schiffer LM. Aromatic amino acids and modification of parkinsonism. The New England journal of medicine. 1967;276:374-9. 10. Willis AW, Schootman M, Kung N, Wang XY, Perlmutter JS and Racette BA. Disparities in deep brain stimulation surgery among insured elders with Parkinson disease. Neurology. 2014;82:163-71.

This Thanksgiving, amidst all the turkey, the stuffing, the cranberry sauce, the ham, and the cornucopia of dessert options, you may be inclined to let the food coma sink in. But for those interested in the science behind this fanciful feast, listen to what Dr. Jason Maley has to say about the neurologic complications of some of the common dishes served at your holiday spread. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. REFERENCES 1. Williams ES. Chronic wasting disease. Veterinary Pathology Online. 2005; 42(5):530-49. 2. Clauss HE, Lorber B. Central nervous system infection with Listeria monocytogenes. Current infectious disease reports. 2008; 10(4):300-6. 3. Garcia HH, Del Brutto OH, Cysticercosis Working Group in Peru. Neurocysticercosis: updated concepts about an old disease. The Lancet Neurology. 2005; 4(10):653-61. 4. Richard DM, Dawes MA, Mathias CW, Acheson A, Hill-Kapturczak N, Dougherty DM. L-tryptophan: basic metabolic functions, behavioral research and therapeutic indications. International journal of tryptophan research: IJTR. 2009; 23(2):45. 5. Sobel J. Botulism. Clinical Infectious Diseases. 2005; 41(8):1167-73. 6. Caruana M, Cauchi R, Vassallo N. Putative role of red wine polyphenols against brain pathology in Alzheimer’s and Parkinson’s disease. Frontiers in Nutrition. 2016; 3. 7. Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA. Diagnosis and management of Marchiafava–Bignami disease: a review of CT/MRI confirmed cases. Journal of Neurology, Neurosurgery & Psychiatry. 2013.

Like many other diseases of the nervous system, amyotrophic lateral sclerosis is not a diagnosis you want to receive in the neurology clinic. But once the diagnosis is made, quality of life supersedes quantity of life. Many academic and private hospitals provide a multi-disciplinary ALS clinic to meet the needs of their patients and their loved ones. In this BrainWaves episode, Dr. Lauren Elman discusses her experience with the multidisciplinary ALS clinic at Pennsylvania Hospital. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. Any cases discussed in this episode are fictional and do not contain any patient health identifying information. This episode was vetted and approved by Lauren Elman. REFERENCES 1. Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J, Shoesmith C, Strong MJ, Woolley SC and Quality Standards Subcommittee of the American Academy of N. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73:1227-33. 2. Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J, Shoesmith C, Strong MJ, Woolley SC and Quality Standards Subcommittee of the American Academy of N. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73:1218-26.

Even before the release of the 2015 film, Concussion, there has been a rising concern about mild traumatic brain injury in athletes in recent years. In this week's episode of BrainWaves, we discuss the history of mild TBI and the clinicopathologic concept of chronic traumatic encephalopathy as it pertains to football players. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. REFERENCES 1. Montenigro PH, Corp DT, Stein TD, Cantu RC and Stern RA. Chronic traumatic encephalopathy: historical origins and current perspective. Annu Rev Clin Psychol. 2015;11:309-30. 2. Giza CC, Kutcher JS, Ashwal S, Barth J, Getchius TS, Gioia GA, Gronseth GS, Guskiewicz K, Mandel S, Manley G, McKeag DB, Thurman DJ and Zafonte R. Summary of evidence-based guideline update: evaluation and management of concussion in sports: report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2013;80:2250-7. 3. Guskiewicz KM, Marshall SW, Bailes J, McCrea M, Cantu RC, Randolph C and Jordan BD. Association between recurrent concussion and late-life cognitive impairment in retired professional football players. Neurosurgery. 2005;57:719-26; discussion 719-26. 4. Stamm JM, Bourlas AP, Baugh CM, Fritts NG, Daneshvar DH, Martin BM, McClean MD, Tripodis Y and Stern RA. Age of first exposure to football and later-life cognitive impairment in former NFL players. Neurology. 2015;84:1114-20.

Idiopathic Parkinson Disease (PD) is the second most common cause of neurodegenerative disease following Alzheimer's. The risk of PD increases to nearly 1 in 100 in the elderly, and although we have been pharmacologically treating this disorder since the early nineteenth century, we have no cure and no definitely therapy to delay disease progression. In this episode of BrainWaves, Dr. David Coughlin illustrates how some of these therapies have evolved and which classes of medications to choose when managing a patient's symptoms--from tremor to dystonia. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. Any cases discussed in this episode are fictional and do not contain any patient health identifying information. The content in this episode was vetted and approved by Andres Deik. REFERENCES 1. Elias, W. Jeffrey, et al. "A randomized trial of focused ultrasound thalamotomy for essential tremor." New England Journal of Medicine 375.8 (2016): 730-739. 2. Hoehn, Margaret M., and Melvin D. Yahr. "Parkinsonism: onset, progression, and mortality." Neurology 50.2 (1998): 318-318. Hely, Mariese A., et al. "The Sydney multicenter study of Parkinson's disease: the inevitability of dementia at 20 years." Movement Disorders 23.6 (2008): 837-844. 3. McIntyre, Cameron C., et al. "Uncovering the mechanism (s) of action of deep brain stimulation: activation, inhibition, or both." Clinical neurophysiology 115.6 (2004): 1239-1248. 4. Olanow, C. Warren. "Levodopa: effect on cell death and the natural history of Parkinson's disease." Movement Disorders 30.1 (2015): 37-44. 5. Parkinson Study Group. "Levodopa and the progression of Parkinson's disease." N Engl J Med 2004.351 (2004): 2498-2508. 6. Parkinson Study Group. "A randomized controlled trial comparing pramipexole with levodopa in early Parkinson's disease: design and methods of the CALM-PD Study." Clinical neuropharmacology 23.1 (2000): 34-44. 7. PD Med Collaborative Group. "Long-term effectiveness of dopamine agonists and monoamine oxidase B inhibitors compared with levodopa as initial treatment for Parkinson's disease (PD MED): a large, open-label, pragmatic randomised trial." The Lancet 384.9949 (2014): 1196-1205. 8. Weaver, Frances M., et al. "Bilateral deep brain stimulation vs best medical therapy for patients with advanced Parkinson disease: a randomized controlled trial." Jama 301.1 (2009): 63-73.

This week, we are taking a break from educational neurology and will be stressing the importance of collaboration between patients and physicians. Physicians are critical to healthcare because of their expertise and experience, but medical management is a team sport. It is equally important to incorporate the patient's needs and opinions into the equation. But this leaves us with the concept of "therapeutic privilege," specifically how much does the physician need to tell the patient about his or her own health? In this episode, Jim Siegler steps into the shoes of one of his good friends who has faced a recent cancer diagnosis. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. Patrick Green, the patient in this episode, has consented to the discussion of his medical care for the purpose of this broadcast and we are sincerely grateful for his contribution. REFERENCES 1. Epstein RM, Korones DN and Quill TE. Withholding information from patients--when less is more. The New England journal of medicine. 2010;362:380-1.

The Landry-Guillain-Barre-Strohl Syndrome, aka Guillain-Barre Syndrome, is classically thought of as a painless ascending weakness with areflexia that typically follows an infectious prodrome. But there are a dozen variants with unusual clinical presentations, from back pain to pandysautonomia. And sometimes GBS follows a vaccination rather than viral infection. In this BrainWaves episode, we discuss these and other unique facts regarding the history, clinical manifestations, and treatment of this polyneuropathy. BrainWaves podcasts and online content are intended for medical education only and should not be used to guide medical decision making in routine clinical practice. Any cases discussed in this episode are fictional and do not contain any patient health identifying information. REFERENCES 1. Dimachkie MM and Saperstein DS. Acquired immune demyelinating neuropathies. Continuum (Minneap Minn). 2014;20:1241-60. 2. Asbury AK. Guillain-Barre syndrome: historical aspects. Annals of neurology. 1990;27 Suppl:S2-6. 3. Afifi AK. The landry-guillain-barre strohl syndrome 1859 to 1992 a historical perspective. J Family Community Med. 1994;1:30-4. 4. Bril V and Katzberg HD. Acquired immune axonal neuropathies. Continuum (Minneap Minn). 2014;20:1261-73. 5. Iodice V and Sandroni P. Autonomic neuropathies. Continuum (Minneap Minn). 2014;20:1373-97. 6. Lehmann HC, Hartung HP, Kieseier BC and Hughes RA. Guillain-Barre syndrome after exposure to influenza virus. Lancet Infect Dis. 2010;10:643-51. 7. Tam CC, O'Brien SJ, Petersen I, Islam A, Hayward A and Rodrigues LC. Guillain-Barre syndrome and preceding infection with campylobacter, influenza and Epstein-Barr virus in the general practice research database. PloS one. 2007;2:e344.