PAINE #PANCE Pearl – Neurology



Question

There are two tests that you can perform at bedside in patients with suspected myasthenia gravis. One is an easy adjunct to the neurologic exam and the other is only included for historical purposes. Name these tests.



Answer

The most reliable way to diagnose myasthenia gravis is through serologic laboratory studies assessing acetylcholine receptor and muscle-specific tyrosine kinase antibodies. But……………there are two bedside tests that can help prior to expensive labs.

  • Ice Pack Test
    • Used as part of the neurologic examination, it is based on the physiologic principle that neuromuscular transmission improves at lower muscle temperature. In patients with myasthenia gravis, placing an ice pack over a closed eyelid for 2 minutes can improve ptosis in 80% of patients.
https://www.nejm.org/doi/full/10.1056/NEJMicm1509523
  • Edrophonium Test
    • Taught more for historical purposes, edrophonium is an acetylcholinesterase inhibitor with a rapid onset and short duration of action. The main effect is prolonging acetylcholine in the neuromuscular junction to improve muscular strength.
    • It is not available in the US, nor used in the diagnosis

Ep-PAINE-nym



Adie’s Pupil

Other Known Aliases – Holmes-Adie pupil

Definition – pupil with parasympathetic denervation that constricts poorly to light, but reacts better to accommodation.

Clinical SignificanceThe tonic pupil is the result of damage to the parasympathetic ciliary ganglion and the exact pathological cause is still unknown, but infectious inflammation to the ciliary ganglion is the most commonly accepted etiology. Adie’s pupils are hypersensitive to very low dose acetylcholine agonists, such as pilocarpine, and is used to diagnose this condition.

HistoryNamed after William John Adie (1886-1935), who was a British physician and neurologist and received his medical doctorate from the University of Edinburgh in 1911. Upon graduating, he served in the British military during World War I as a medical officer. Following the war, he worked in various hospitals practicing neurology and making a name for himself, culminating in Fellowship in the Royal College of Physicians in 1926. He was also one of the founders of the Association of British Neurologists in 1932. The history of the eponym is interesting because there were numerous publications prior to Adie’s work describing this clinical syndrome and Adie referenced them in his 1931 article. The eponymonic term was given to him by French neurologist Jean-Alexandre Barré in 1934. Also, Gordon Morgan Holmes contemporaneously published the same findings in the same year. This led to the common eponym Holmes-Adie pupils.


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. Adie WJ. Pseudo-Argyll Robertson pupils with absent tendon reflexes. A benign disorder simulating tabes dorsalis. British Medical Journal, London, 1931, I: 928-930. [article]
  7. Holmes GM. Partial iridoplegia associated with symptoms of other disease of the nervous system. Transactions of the Ophthalmological Societies of the United Kingdom, 1931, 51: 209-228.

Ep-PAINE-nym



Ménière’s Disease

Other Known Aliasesendolymphatic hydrops

Definitionabnormal fluid and ion homeostasis of the inner that leads to distortion and distention of the membranous, endolymph-containing portions of the labyrnthine system. It is currently unclear why this occurs and several etiologies have been proposed.

Clinical SignificanceMénière’s disease classically has the triad of tinnitus, sensorineural hearing loss, and episodic vertigo lasting from 20 minutes to 24 hours. The course and severity are variable and the frequency may actually decline over time. Treatment is geared towards diet and lifestyle modifications, vestibular suppressants, diuretics, and interventional procedures in severe or refractory cases.

HistoryNamed after Prosper Ménière (1799-1862), who was a French physician and recieved his medical doctorate from the Hôtel-Dieu de Paris in 1828. He studied and assisted Guillaume Dupuytren at this famed hospital in France. During a particularly bad outbreak of cholera, he was sent by the king to Aude and Haute-Garonne to oversee this medical campaign and was so successful that he was made a knight of the Legion of Honour. Later, he became chief of medicine at the Imperial Institution for Deaf Mutes in Paris and published his findings on his eponymous disease in 1861.


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. Ménière P. Sur une forme particulière de surdité grave dépendant d’une lésion de l’oreille interne. Gazette médicale de Paris. 1861;S3(16):29.

PAINE #PANCE Pearl – Pediatrics



Question

A 3-week old baby girl is sent to your emergency department after being seen by their pediatrician for irritability, poor feeding, and a seizure just prior to arrival at the pediatrician’s office. Vital signs are BP-103/73, HR-137, RR-25, O2-100% on room air, and Temp-39.2oC (102.5oF). Physical examination reveals a lethargic infant with decreased motor tone and a full, bulging frontal fontanelle. What is the most important diagnostic study to obtain and what is the empiric treatment of choice while awaiting results?



Answer

  1. A full or bulging fontenelle is suggestive of meningeal edema and swelling are concerning for meningitis. Couple this with the lethargy and poor motor tone and this infant bought herself a lumbar puncture.
  2. Now….because of her age (<30 days old), you have to cover for a specific set of pathogens due to a developing immune system. Classically, neonatal sepsis bugs include group B streptococcus (GBS), Escherichia coli, and Listeria monocytogenes. Empiric antibiotic coverage (until gram stain results) is:
    1. Ampicilin (GBS)
    2. Gentamycin (gram negative coverage)
    3. Cefotaxime (wider gram negative coverage)
2004 – IDSA Guidelines

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

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]