Ep-PAINE-nym



Bell’s Palsy

 

Other Known Aliases – facial nerve palsy, cranial nerve VII palsy

 

Definitionparalysis of cranial nerve VII that can can effect both motor and sensory function

Bellspalsy.JPG

Clinical SignificanceThis condition affects up to 20 patients per 100,000 population with no gender, race, or geographic predilection.  It is the most common cause of unilateral acute peripheral nerve palsies.  Although benign in clinical course, providers must pay close attention to differentiate between Bell’s palsy and a supranuclear lesion (stroke).  The most significant clinical difference between these two condition is the ability to raise the eyebrow and wrinkle the forehead.

https://pamadaydotnet.files.wordpress.com/2018/05/picture15.png?w=440

History – Named after Sir Charles Bell (1774-1842), a Scottish surgeon, anatomist, physiologist, neurologist, and noted philosophical theologian who received his medical doctorate at the University of Edinburgh in 1799.  While still a student, he illustrated and published an extraordinary textbook entitled “A System of Dissection Explaining the Anatomy of the Human Body”  After graduation, he was admitted and enrolled at The Royal College of Surgeons where he proved himself to be as skilled in surgery as in anatomy. He further published two subsequent volumes of “Anatomy of the Human Body”, with his brother John (also a skilled anatomist and surgeon).  He was such a prolific teacher and professor that the faculty at the University of Edinburgh blocked his advancement and he was forced to move to London where he first opened a private school of anatomy and then took over the Great Windmill Street School of Anatomy (founded by William and John Hunter).  In 1811, he published “An Idea of a New Anatomy of the Brain” considered to be the quintessential textbook of neurology.  In 1821, he published a paper entitled ” On The Nerves: Giving an Account of some Experiments on Their Structure and Functions, which lead to a new arrangement of the systems” where he described the trajectory of the facial nerve and the unilateral facial paralysis that could result.  This paper is still considered one of the classics of neurology and led to the disease bearing his name.  In 1824, he became the first professor of anatomy and surgery of the College of Surgeons in London and was knighted by King William IV due to his contributions of the advancement of medicine. 

 

Photograph of Sir Charles Bell


References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Bell C, Shaw A. Reprint of the “Idea of a New Anatomy of the Brain,” with Letters, &c. Journal of anatomy and physiology. 1868; 3(Pt 1):147-82. [pubmed]
  7. http://rstl.royalsocietypublishing.org/content/111/398.full.pdf+html

PAINE #PANCE Pearl – Neurology



Questions

 

  1. What is the popular scoring system for determining a patient’s need for anticoagulation to prevent stroke in atrial fibrillation?
  2. If you do start anticoagulation, what are some scoring systems to determine a patient’s risk for bleeding while on anticoagulation?


Answers

 

  1. CHA2DS2-VASc is the most utilized scoring systems for determining anticoagulant selection to prevent stroke in patients with atrial fibrillation.  The components are as follows
    1. CHF (+1)
    2. HTN (+1)
    3. Age
      1. < 65yr (0)
      2. 65-74 (+1)
      3. ≥ 75 (+2)
    4. DM (+1)
    5. Stroke/TIA (+2)
    6. Vascular disease (+1)
    7. Sex category (+1 for female)
    8. Interpretation:
      1. 0 points (low risk) – consider antiplatelet only
      2. 1 point (low/moderate risk) – antiplatelet or anticoagulation
      3. ≥ 2 points (moderate/high risk) – anticoagulation
  2. If you are deciding on whether to start anticoagulation or not, you should determine the bleeding risk of your patient on anticoagulation.  There are three scoring systems that can help with this:
    1. ATRIA
      1. Anemia (Hgb < 13g/dL in male and < 12g/dL in female)(+3)
      2. Severe renal disease (GFR < 30mL/min or dialysis)(+3)
      3. Age ≥ 75yr (+2)
      4. History of bleeding (+1)
      5. HTN (+1)
      6. Interpretation:
        1. < 4 points – low risk
        2. 4 points – intermediate risk
        3. > 4 points – high risk
    2. HASBLED
      1. HTN (uncontrolled or > 160mmHg)(+1)
      2. Renal disease (+1)
      3. Liver disease (+1)
      4. Stroke history (+1)
      5. Prior bleeding history (+1)
      6. Labile INR (+1)
      7. Age ≥ 65 (+1)
      8. Medications (+1)
      9. Alcohol (+1)
      10. Interpretation:
        1. 0 points – low risk
        2. 1-3 points – moderate risk
        3. ≥ 4 points – high risk
    3. HEMORR2HAGES
      1. Hepatic or renal disease (+1)
      2. Ethanol use (+1)
      3. Malignancy (+1)
      4. Older than 75 (+1)
      5. Reduced platelet count/function (+1)
      6. Rebleeding risk (+2)
      7. HTN (+1)
      8. Anemia (+1)
      9. Genetics (+1)
      10. Excessive fall risk (+1)
      11. Stroke history (+1)
      12. Interpretation:
        1. 0-1 points – low risk
        2. 2-3 points – intermediate risk
        3. ≥ 4 points – high risk


References

  1. Lip GY, Nieuwlaat R, Pisters R, Lane DA, Crijns HJ. Refining clinical risk stratification for predicting stroke and thromboembolism in atrial fibrillation using a novel risk factor-based approach: the euro heart survey on atrial fibrillation. Chest. 2010; 137(2):263-72. [pubmed]
  2. Fang MC, Go AS, Chang Y, et al. A new risk scheme to predict warfarin-associated hemorrhage: The ATRIA (Anticoagulation and Risk Factors in Atrial Fibrillation) Study. Journal of the American College of Cardiology. 2011; 58(4):395-401. [pubmed]
  3. Pisters R, Lane DA, Nieuwlaat R, de Vos CB, Crijns HJ, Lip GY. A novel user-friendly score (HAS-BLED) to assess 1-year risk of major bleeding in patients with atrial fibrillation: the Euro Heart Survey. Chest. 2010; 138(5):1093-100. [pubmed]
  4. Gage BF, Yan Y, Milligan PE, et al. Clinical classification schemes for predicting hemorrhage: results from the National Registry of Atrial Fibrillation (NRAF). American heart journal. 2006; 151(3):713-9. [pubmed]
  5. Ruff T.  Which risk score best predicts bleeding with warfarin in atrial fibrillation?.  Online – American College of Cardiology. Sept. 26, 2011 [link]

Ep-PAINE-nym



Huntington’s Disease

 

Other Known AliasesHuntington’s chorea

 

DefinitionAutosomal dominant condition caused by expansion of the cytosine-adenine-guanine (CAG) trinucleotide repeats in the HD gene located on short arm of chromosome 4p16.3 that encodes the protein huntingtin.

Image result for huntington's disease

Clinical SignificanceThis condition affects 4-15 in 100,000 peoples of European descent and is extremely rare in non-European lineage.  The classic manifestations of the disease include chorea, psychiatric illness, and dementia.  These symptoms begin very slow and are often missed for a period of time, but always progress to severe deterioration of neuromuscular function.  It is uncurable and treatment is directed towards support and planning of care.  Average length of survival after symptoms onset is 10-20 years

Image result for huntington's disease

History – Named after George Huntington (1850-1916), an American physician who received his medical doctorate from Columbia University in 1871 at the age of 21.  He came from a long line of physicians dating back to 1797, when his grandfather opened the family practice in East Hampton.  He took meticulous notes on the disease that bears his name from going on house calls with his father early in his childhood, as well as reading and transcribing notes from his father and grandfather.  He only published two papers in his career, the first of which was on this disease.  He read this manuscript before the Meigs and Mason Academy of Medicine in Middleport, Ohio in 1872 (just 1 year after graduating medical school) and received such acclaim that it was published in the Medical and Surgical Reporter of Philadelphia just 2 months later.  This paper was published in the German literature later that year and his name was forever attached to this disease.  Even William Osler read and commented on this paper in 1908 saying ” In the history of medicine there are few instances in which a disease has been more accurately, more graphically, or more briefly described.”

 

https://upload.wikimedia.org/wikipedia/commons/c/c7/First_page_of_the_article_%22On_Chorea%22_by_G._Huntington_Wellcome_L0005209.jpg


References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Huntington’s Disease.  https://ghr.nlm.nih.gov/condition/huntington-disease
  7. Huntington G.  On Chorea.  Medical and Surgical Reporter of Philadelphia.  1972;26(15):317-321 [article]

#41 – Bell’s Palsy



***LISTEN TO THE PODCAST HERE***

 



Anatomy and Physiology of Cranial Nerve VII

Cranial Nerve VII (the facial nerve) is a mixed nerve (having both sensory and motor function) with:

  • Somatic afferents from the external auditory canal
  • Afferent fibers from taste receptors from the anterior 2/3rds of the tongue
  • Parasympathetic fibers innervating lacrimal, submandibular, and sublingual salivary glands
  • Motor fibers innervating the facial muscles

There are six (6) segments of the nerve after it emerges from the pons of the brainstem:

  1. Intracranial (cisternal)
  2. Meatal (from brainstem to internal auditory canal)
  3. Labyrinthine (internal auditory canal to geniculate ganglion)
    1. Lacrimal gland, nasal/palate, pterygopalatine ganglion
  4. Tympanic (geniculate ganglion to pyramidal eminence)
    1. Stapedius nerve
  5. Mastoid (pyramidal eminence to stylomastoid foramen)
    1. Chorda tympani à tongue, salivary glands, lingual nerve
  6. Extratemporal (stylomastoid foramen to post parotid branches)
    1. 5 major facial branches
      1. Temporal
      2. Zygomatic
      3. Buccal
      4. Mandibular
      5. Cervical


Definition and Epidemiology

Bell’s Palsy is an acute peripheral nerve palsy of unknown etiology and makes up almost half of such cases.  It is estimated that the annual incidence is around 20 patient per 100,000 population.  There is no race, geographic, or gender correlation., though there is some old data on increased risk during 3rd trimester of pregnancy.


Pathogenesis

Had been hotly debated for many years as to the cause of this condition and is generally considered to be caused by Herpes Simplex virus due to associated serologic evidence in effected patients.  Newer data has been published, but is not entirely conclusive.  Most agree that is due to some viral pathogen with herpes being the most common, but also implicating CMV, EBV, adenovirus, and coxsackievirus. Specifically, the signs and symptoms are a result of viral mediated inflammatory demyelination of the nerve.


Signs and Symptoms

Most common presentation is acute (over several hours) unilateral facial paralysis with:

  • Motor
    • Inability to close the eye
    • Eyebrow sagging with inability to wrinkle the forehead
    • Obliteration of the nasolabial fold
    • Dropping of the affected corner of the mouth
  • Sensory
    • Hyperacusis
    • Loss of taste on the anterior 2/3rd of tongue

Symptoms are progressive and usually reach peak involvement within 3 weeks of onset.  Patients should begin to have return of function within 2-4 months.


Diagnostic Studies

Bell’s palsy is a clinical diagnosis and diagnostic studies are generally not indicated unless the presentation is atypical, still progressing at 3 weeks, or there is no return of function by 4 months.  History of facial twitching or spasms preceding the paralysis raises suspicion of compressive neuropathy from tumor or mass.

  • Electromyography (EMG)
    • The most simplest of the electrodiagnostic tests that can be used to show action potentials on active volition.  Some degree of potential infers that the nerve is still intact and therefor, can improve.
  • Nerve Conduction Studies (NCS)
    • Supramaximal stimulation near the parotid gland with measured evoked potentials over the orbicularis oculi, nasalis, and lower facial muscles  can measure the degree of axonal loss.  Studies have shown >75% is the critical cutoff for low likelihood of full recovery.  Ideally, this should be performed within 2 weeks of symptom onset for a more accurate prognosis.
  • Facial Nerve Stimulation
    • Should be considered with 2 weeks of symptoms onset if surgical decompression is considered due to potential for reversibility.
  • Needle EMG
    • Utilized after 3 weeks to assess the degree of axonal damage and evidence of subclinical reinnervation for recovery
  • Imaging
    • High-resolution, contrast-enhanced CT (for bony pathology) or gadolinium-enhanced MRI (for soft tissue structures) of the brain, temporal bone, and parotid gland.
  • Lyme serology should be entertained in patients from endemic regions.

Severity Grading

The House-Brackman scale is the most common grading scale that uses objective criteria to score the severity, mark progression, and track return of function.


Treatment

Mainstay of therapy for Bell’s palsy is corticosteroids (for the inflammation) and antivirals (for the viral pathogens).  Current recommendations are:

  • Prednisone 60-80mg daily for 7 days
  • Valacyclovir 1000mg TID for 7 days
  • Within 72 hours of symptoms onset

 

Eye care should include artificial tears and an eye patch for sleeping.


Prognosis

Favorable prognosis if any recovery is seen within 21 days of symptom onset with data showing 71% with complete resolution, 13% with slight residual sequelae, and 16% with residual weakness, synkinesis, or contracture.  94% of patients with incomplete involvement have full resolution, while only 60% of complete involvement returned to normal function.



References

  1. Monkhouse WS. The anatomy of the facial nerve. Ear, nose, & throat journal. 1990; 69(10):677-83, 686-7. [pubmed]
  2. May M, Klein SR. Differential diagnosis of facial nerve palsy. Otolaryngologic clinics of North America. 1991; 24(3):613-45. [pubmed]
  3. Hilsinger RL, Adour KK, Doty HE. Idiopathic facial paralysis, pregnancy, and the menstrual cycle. The Annals of otology, rhinology, and laryngology. ; 84(4 Pt 1):433-42. [pubmed]
  4. Peitersen E. Bell’s palsy: the spontaneous course of 2,500 peripheral facial nerve palsies of different etiologies. Acta oto-laryngologica. Supplementum. 2002; [pubmed]
  5. Schirm J, Mulkens PS. Bell’s palsy and herpes simplex virus. APMIS : acta pathologica, microbiologica, et immunologica Scandinavica. 1997; 105(11):815-23. [pubmed]
  6. Kennedy PG. Herpes simplex virus type 1 and Bell’s palsy-a current assessment of the controversy. Journal of neurovirology. 2010; 16(1):1-5. [pubmed]
  7. Morgan M, Nathwani D. Facial palsy and infection: the unfolding story. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America. 1992; 14(1):263-71. [pubmed]
  8. Liston SL, Kleid MS. Histopathology of Bell’s palsy. The Laryngoscope. 1989; 99(1):23-6. [pubmed]
  9. Valls-Solé J. Electrodiagnostic studies of the facial nerve in peripheral facial palsy and hemifacial spasm. Muscle & nerve. 2007; 36(1):14-20. [pubmed]
  10. May M, Klein SR, Taylor FH. Indications for surgery for Bell’s palsy. The American journal of otology. 1984; 5(6):503-12. [pubmed]
  11. Gilden DH. Clinical practice. Bell’s Palsy. The New England journal of medicine. 2004; 351(13):1323-31. [pubmed]
  12. House JW, Brackmann DE. Facial nerve grading system. Otolaryngology–head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery. 1985; 93(2):146-7. [pubmed]
  13. Gronseth GS, Paduga R, . Evidence-based guideline update: steroids and antivirals for Bell palsy: report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2012; 79(22):2209-13. [pubmed]
  14. Baugh RF, Basura GJ, Ishii LE, et al. Clinical practice guideline: Bell’s palsy. Otolaryngology–head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery. 2013; 149(3 Suppl):S1-27. [pubmed]
  15. Schwartz SR, Jones SL, Getchius TS, Gronseth GS. Reconciling the clinical practice guidelines on Bell’s palsy from the AAO-HNSF and the AAN. Otolaryngology–head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery. 2014; 150(5):709-11. [pubmed]
  16. de Almeida JR, Guyatt GH, Sud S, et al. Management of Bell palsy: clinical practice guideline. CMAJ : Canadian Medical Association journal = journal de l’Association medicale canadienne. 2014; 186(12):917-22. [pubmed]
  17. Peitersen E. The natural history of Bell’s palsy. The American journal of otology. 1982; 4(2):107-11. [pubmed]

PAINE #PANCE Pearl – Neurology



Questions

 

  1. What is the popular scoring system for determining a patient’s need for anticoagulation to prevent stroke in atrial fibrillation?
  2. If you do start anticoagulation, what are some scoring systems to determine a patient’s risk for bleeding while on anticoagulation?

 

Image result for atrial fibrillation stroke

Image result for atrial fibrillation stroke

Ep-PAINE-nym



Klinefelter Syndrome

 

Other Known Aliases47XXY, Klinefelter-Reifenstein-Albright Syndrome

 

DefinitionGenetic condition resulting from two (or more) X chromosomes in a male patient.  It is the most common sex chromosome abnormality causing hypogonadism.

Human chromosomesXXY01.png

Clinical SignificanceThis condition affects 1 in 500-1000 newborn males in the United States.  Symptoms can range from subtle (sometimes not even noticed) to severe learning, developmental, and cognitive deficiencies.  The most prominent features are sterility, small testes, taller stature, less androgenic body hair, and gynecomastia.  Due to these developmental abnormalities, it is often not diagnosed until after puberty

Image result for klinefelter syndrome

History – Named after Harry Fitch Klinefelter, Jr. (1912-1990), an American rheumatologist and endocrinologist, who earned is medical doctorate from Johns Hopkins University.  He worked in the prestigious clinic of Dr. Fuller Albright at Massachusetts General Hospital where he studied a group of nine boys who all had similar features of gynecomastia, aspermatogenesis, and increased follicle-stimulating hormone.  He credits Dr. Albright with this discovery, as it was his clinic and he first noticed the pattern, but Albright wanted Klinefelter to do the research work on it as a new fellow attending.  The group (with Dr. Edward Reifenstein) published this cases series in 1942 and Dr. Albright insisted that Klinefelter take lead authorship.

 

Image result


References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Klinefelter Syndrome.  https://ghr.nlm.nih.gov/condition/klinefelter-syndrome
  7. Klinefelter HF, Reifenstein EC, Albright F. Syndrome Characterized by Gynecomastia, Aspermatogenesis without A-Leydigism, and Increased Excretion of Follicle-Stimulating Hormone.  JCEM.  1942;2(11):615-627 [article]
  8. Loriau DL.  Chapter 89: Harry F. Klinefelter (1912-1990).  A Biographical History of Endocrinology. 2016.  [article]

PAINE #PANCE Pearl – Pediatrics



Question

 

A 3yo girl has been diagnosed with acute bacterial otitis media in your clinic and requires amoxicillin for treatment.  The parents say she doesn’t take medicine every well and would appreciate the lowest VOLUME per dose.  She weighs 32 lbs.  What are the different dosing strategies using the current formulation of liquid amoxicillin?

 



Answer

 

Amoxicillin comes in 125mg/5mL, 200mg/5mL, 250mg/5mL, and 400mg/5mL and the recommended daily dose for bacterial acute otitis media is 90mg/kg/day twice daily for 7 days. She weighs 32lbs, which is 14.5kg, so she would need 1305mg per day, or 650mg per dose. For the different concentrations it could be:

  • For 125mg/5mL – 26mL per dose
  • For 200mg/5mL – 16mL per dose
  • For 250mg/5mL – 13mL per dose

 

If you want to have the LOWEST volume, then using the 400mg/5mL concentration would be 8.1mL per dose and you would need to dispense 115mL for a 7 day prescription.