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Morning Report

Morning Report . Palak Kachhadia 08/31/2017. Scenario . Patient is a 32 year female who comes into your clinic complaining of generalized weakness and right eyelid droop. Her symptoms started 5 days prior and have gotten progressively worse…. What do you want to know?. History. PMH: HTN

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Morning Report

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  1. Morning Report Palak Kachhadia 08/31/2017

  2. Scenario • Patient is a 32 year female who comes into your clinic complaining of generalized weakness and right eyelid droop. Her symptoms started 5 days prior and have gotten progressively worse…..

  3. What do you want to know?

  4. History • PMH: HTN • SH: smokes 1 pack a day for the past 8 years. Drinks alcohol occasionally. Works as a secretary at a local family practitioner’s office. • FH: Has a history of RA on her mother’s side along with HTN, CAD and CVA • Medications: Hydrochlorothiazide, OCPs • Allergies: none

  5. Physical Exam • General? • HEENT? • CV? • Respiratory? • Abdomen? • Skin? • Extremities? • Neurological? What are your differentials? What labs would you like to order?

  6. MYASTHENIA GRAVIS

  7. Epidemiology • Incidence: 10-20 new cases/million individuals • Prevalence: 150-200/million • Increased recognition and survival • Bimodal: • 2nd and 3rd decades: women • 6th to 8th decades: men

  8. Q1: What is the pathophysiology? • Antibody mediation dysfunction of post-synaptic transmission • Autoimmune antibodies on post-synaptic membrane leads to AChR cross-linking and internalization in the post-synaptic membrane • A) Anti-AChR (70-90%) • B) Anti-MuSK (5-8%) • C) Sero-negative to AChR and MuSK (5-25%)

  9. Q2: What are the most common Signs and Symptoms? • Muscular: fluctuating muscle weakness with repetitive movement, neck flexion and extension weakness, proximal limb weakness • Ocular: Ptosis, diplopia • Bulbar: dysarthria, dysphagia, chewing difficulty

  10. Q3: How do you make the Diagnosis? • History taking + PE + Bedside/EP tests! • Bedside maneuvers: Ice pack test, Tensilon (edrophonium test) • Serologic testing for AChR-Ab and/or MuSK-Ab • AChR-Ab and MuSK-Ab correlate poorly with disease severity • CT: Thymoma? • Electrophysiological tests: • A) RNS (Repetitive Nerve Stimulation) • B) SFEMG (single fiber electromyography)

  11. Single Fiber EMG Simultaneous recording of AP of two muscle fibers innervated by the same motor axon. Records variability in time (jitter)

  12. Ice Pack Testing • https://www.bing.com/videos/search?q=ice+pack+test+video&view=detail&mid=6AC6FEA5367A7B756CA46AC6FEA5367A7B756CA4&FORM=VIRE

  13. Evidence Based Medicine

  14. Evidence Based Medicine

  15. Q4: Name 5 differences between Lambert-Eaton Syndrome and MG

  16. Lambert Eaton Syndrome • Antibodies against P/Q type calcium channels • Impaired release of ACh from nerve terminals

  17. Q5: What Treatments can you offer? • Acetyl cholinesterase inhibitors (pyridostigmine) • Rapid Induction Immunotherapy (IVIG, plasmapheresis): bridge therapy, myasthenic crisis, maintenance treatment in refractory MG • Steroids: low dose vs. high dose? • Azathioprine • MycophenolateMofetil • Cyclosporine and Tacrolimus • Rituximab • Cyclophosphamide • Thymectomy

  18. Steroids • Mild to moderate MG: Oral 20-40 mg of prednisone/day • Myasthenic Crisis: high dose steroids receiving concurrent induction immunotherapy (1mg/kg/day) over 3-4 weeks • Taper: slowly to avoid relapse (5-10 mg/month decrease) • Supplement with Calcium (1500 mg/day) and Vitamin D (400-800 U/day)

  19. Azathioprine • Interferes with DNA synthesis of T & B lymphocytes • Delayed onset: 6-12 months • Start with 50 mg/day with increase of 50 mgevery 2 weeks • Goal: 2-3 mg/kg/day • Labs: check liver function and CBC every week for 2 months and then monthly for a year

  20. MycophenolateMofetil • Inhibits purine synthesis and therefore B and T lymphocytes • Start with 500 mg BID and increase to 1000-1500 mg BID within 3-6 months • Fewer side effects, well tolerated • Labs: cbc, cmp monthly for 6 months and then yearly

  21. Cyclosporine and Tacrolimus • Inhibits calcineurin and blocks synthesis of IL-2 and Interferon • Faster onset (1-2 months) • Maintenance dose of 5mg/kg/day with serum level between 100-150 ng/mL • T: 0.05-0.1mg/kg/day with levels between 2-9 ng/mL • Renal Toxicity: Cyclosporine >> Tacrolimus

  22. Rituximab • Monoclonal antibody against CD-20 on B cells • Used more with refractory MG • Particularly effective for MG-MuSK • Minimal reactions, well tolerated!

  23. Cyclophosphamide • Used sparingly • Lots of side effects • Myelosuppression • Hemorrhagic Cystitis • Skin/bladder/blood malignancies

  24. Thymectomy for Tumors • 75% prevalence in those with Ach-R antibody positive MG • 85% Thymic Hyperplasia • 15% Primarily Thymoma

  25. Q6: What autoimmune diseases are associated with MG? • Autoimmune thyroid disease (3-8%) • Rheumatoid Arthritis • Systemic Lupus Erythematosus

  26. Q7: What medications should you avoid if you have MG?

  27. Q7: What medications should you avoid if you have MG?

  28. Q8: What parameters are used for intubation? • Vital Capacity < 20mL/kg • NIF (negative inspiratory force) < 30 cm H2O • Physical exam – how does the patient look? What are the respiratory rates? Is the patient tiring out? • DO NOT USE ABGs OR VBGs

  29. MKSAP • A 24-year-old woman comes to the office for a follow-up evaluation of myasthenia gravis that was diagnosed 1 year ago after frequent episodes of double vision and muscle weakness occurring at the end of the day or after exercise. Symptoms have become less frequent with the use of prednisone and pyridostigmine. The patient has had no shortness of breath or other medical issues. Her mother has rheumatoid arthritis. • On physical examination, temperature is 37.4 °C (99.3 °F); other vital signs are normal. Diplopia is noted in all directions with disconjugate gaze and is exacerbated after sustained upgaze for 1 minute. Muscle strength is 4/5 in the deltoids, biceps, triceps, quadriceps, and hamstrings bilaterally. Weakness is more pronounced after she keeps the arms upright while sitting and standing five times; she needs to use her arms to stand from a seated position on her last attempt. No shortness of breath, dysarthria, or dysphagia is noted. Sensory examination and reflex testing are normal. • Results of laboratory studies show high titers of antiacetylcholine receptor antibodies. • No evidence of myopathy or neuropathy is found on an electromyogram or during nerve conduction studies; repetitive stimulation of the motor nerves showed a decrement in amplitude. A CT scan of the chest shows no thymoma.

  30. According to a recently completed randomized clinical trial, which of the following is the most appropriate treatment? • A) Azathioprine • B) IVIG • C) MycophenolateMofetil • D) Plasmapheresis • E) Thymectomy

  31. Alternate day prednisone vs. prednisone + thymectomy Primary outcomes: Quantitative MG score over 3 years Average dosage of prednisone used over 3 years

  32. MKSAP • A 62-year-old man is admitted to the hospital for a 6-week history of progressive cough, hemoptysis, and shortness of breath. Prior to the onset of symptoms, he reports feeling well except for some difficulty rising out of a chair and walking up a flight of stairs because of lower extremity weakness. He has a 50-pack-year history of cigarette smoking. He has no other medical problems and takes no medications. • On physical examination, temperature is 37.6 °C (99.7 °F), blood pressure is 140/84 mm Hg, pulse rate is 102/min, respiration rate is 14/min, and oxygen saturation is 90% breathing ambient air; BMI is 30. Cardiovascular and pulmonary examination findings are unremarkable. On neurologic examination, he has symmetric proximal muscle weakness in both his upper and lower extremities. There is no palpable peripheral lymphadenopathy. • Chest radiograph shows a right hilar mass. Chest CT confirms an 8-cm right hilar mass adjacent to the mediastinum with bulky bilateral mediastinal lymphadenopathy.

  33. Which of the following is the most likely diagnosis? • A) Adenocarcinoma of the lung • B) Atypical bronchial carcinoid tumor • C) Small cell lung cancer • D) Squamous cell carcinoma of the lung

  34. References • JAMA evidence – The Rational Clinical Examination • Uptodate • Dynamed • MKSAP 17 • Evidence Based Physical Diagnosis, McGee

  35. Questions?

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