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Clinical Problem Solving 12/16/08

Clinical Problem Solving 12/16/08. Moderator: Stuart Cohen MD Discussant: Carlos Estrada MD. Case #1. 21 y/o Caucasian Male presents to clinic with 8 day h/o fever and malaise 2 days PTA developed sore throat Denies myalgias, cough, shortness of breath No nausea, vomiting or diarrhea

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Clinical Problem Solving 12/16/08

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  1. Clinical Problem Solving12/16/08 Moderator: Stuart Cohen MD Discussant: Carlos Estrada MD

  2. Case #1 • 21 y/o Caucasian Male presents to clinic with 8 day h/o fever and malaise • 2 days PTA developed sore throat • Denies myalgias, cough, shortness of breath • No nausea, vomiting or diarrhea • Denies nasal congestion, rhinorrhea • Denies headache, stiff neck, visual changes

  3. Case #1 • No rash, joint pain • No dysuria, frequency or urgency • No sick contacts • No recent travel outside of country or hikes in the woods

  4. Case #1 • PMH: None • MEDS: None • Fam Hx: non-contributory • Soc Hx: • Senior at University of Alabama Tuscaloosa • No tobacco or drug use • Social alcohol consumption • Not sexually active

  5. Case #1 • PE: 120/60, 70, 12, 100.2 • HEENT • Erythematous posterior pharynx with cobblestoning, large tonsils with exudate • Bilateral anterior cervical and posterior cervical adenopathy (tender) • CV: RRR with no M/R/G • Lungs: Clear bilaterally, ABD: soft, non-tender, non-distended, No hepatosplenomegaly • EXT: No C/C/E • Neuro: Non-focal

  6. Case #1 • Data • Rapid Strep: Positive • WBC 13.3, HCT 39, Plts 244 • 18% segs, 50% lymphs, 18% Monos, • 8% Atypical lymphs • Monospot: Positive • EBV VCA IgM: Positive • Throat Culture: No growth

  7. Mono/Strep both? • True co-infection with GAS and EBV is rare but reported in the literature • GAS colonization in patients with EBV is more likely (some reports suggest up to 30%)

  8. Centor Criteria • Tonsillar exudates • Tender anterior cervical adenopathy • Fever by history • Absence of cough • 0 or 1 positive  NPV of 80% • 3 or 4 positive  PPV of 50-60%

  9. Diagnostic testing for GAS • Rapid Streptococcal Antigen Test (RAST) • Sensitivity 80-90% • Specificity 90- 95% • Throat Culture • Sensitivity 90% • Specificity 95%

  10. Rationale for antibiotics • To prevent Rheumatic Fever • To prevent peritonsillar abscess • To prevent transmission • To relieve symptoms • For patients with severe pharyngitis, symptoms resolve 2.5 days earlier with antibiotics

  11. Antibiotics in patients with EBV • Macular-papular pruritic rash which is frequently prolonged • Offending Antibiotics • Amoxicillin • Penacillin • Azithromycin • Levofloxacin • Cephalexin • Not reported with Clindamycin • Mechanism of rash unknown • Rash does not presage true drug allergy

  12. Case #2 • 44 y/o white male presents with 3 month h/o left upper extremity weakness • Developed abruptly with no associated pain • limited to distal hand muscles with numbness and paralysis of all finger movements

  13. Case #2 • 1 yr PTA, developed abrupt onset right shoulder pain which lasted for 4 weeks • 1 month after being struck in the neck with water heater • Followed by slow progression of weakness and wasting of shoulder girdle muscles (unable to abduct his arm) • Partial return of function of right arm weakness over past 9 months

  14. Case #2 • Evaluation at University of Michigan 1 yr ago: • MRI brain and c-spine – Normal study • Shoulder films- Normal • EMG: right brachial plexopathy

  15. Review of Systems • Denies recent trauma, vaccines or surgical procedures • No recent fevers, chills, night sweats • Denies chest pain, SOB, cough or dysphagia • Denies abdominal pain, nausea or vomiting • Reports 40# weigh loss over past 9 months • New onset left lower extremity weakness

  16. Case #2 • PMH: • Severe depression with recent psychiatric hospitalization • Right upper extremity weakness (brachial plexopathy) • Meds: Risperdal, lexapro • FamHx: non-contributory • SocHx: • Separated from wife- living at his sister’s house • Past truck driver • Past tobacco use, no alcohol, no drugs

  17. Case #2 • Physical Exam: • VS: AF, 94/60, 60, 12 (weight 115#) • Appearance: Cachectic but NAD • HEENT: OP clear, fundi-no papilledema, no lymphadenoapthy or thyromegaly • CV: RRR with no M/R/G • Lungs: Clear bilaterally • ABD: S/NT/ND/ pos bowel sounds, No hepatosplenomegaly • Ext: NO C/C/E

  18. Case #2 • Neurological exam: • Mental status: normal memory, comprehension and cognition. • Cranial nerves II – XII: visual fields full; extraocular movements intact; facial/jaw strength normal; hearing normal; gag normal; tongue midline without atrophy; normal shrug. • Motor strength testing: • RUE- 3/5 grip, 4/5 wrist flexion and extension, 4+/5 biceps/triceps • LUE- 0/5 grip, 0/5 wrist flexion, 4/5 wrist extension, 4+/5 bic/triceps • RLE- 5/5 strength • LLE- 4/5 strength with foot drop

  19. Case #2 • Neurological exam (cont’d): • Deep tendon reflexes: 2+ everywhere except absent left knee and ankle; toes –downgoing bilaterally. • Sensory examination: Proprioception intact. Vibration diminished in feet and hands. Pinprick diminished in C5/C6 dist on right upper extremity and C8/T1 distribution on left upper extremity. • Coordination: normal finger-to-nose and heel-to-shin testing. • Gait: normal station; normal heel, toe and tandem walking.

  20. Case #2 • WBC 11, HCT 34, Plt 362 • 138 109 22 89 43 29 1.3 • AST 24, ALT 34, AlkPhos 84, Bili 1.0 • Tot protein 7.6, albumin 2.8 • B12, Folate- normal • TFT’s- normal • HIV- negative, HCV- negative, HbsAg- negative • UA: 3+ blood, 2+ protein, >25 RBC’s • Urine Protein/Creatine: 730 mg/24hr

  21. Case #2 • ESR 10, ANA < 1:80, RF (-) • C3,C4,CH50 - normal • SPEP, UPEP, IFE: Negative • P-ANCA (-), C-ANCA 1:320 • Anti-MPO <13, Anti-PR3: 129

  22. Case #2 • CXR- No acute disease • EMG/NCS • Left brachial plexopathy involving lower trunk (C8/T1) • Selective motor denervation in left femoral and posterior tibial innervated muscles (compatible with plexopathy) • Kidney biopsy • Focal necrotizing glomerulonephritis with crescents, pauci-immune • (consistent with ANCA associated vasculitis)

  23. Approach to Weakness

  24. Vasculitic neuropathies • Systemic Vasculitic Neuropathy • Nonsystemic Vasculitic Neuropathy • No associated systemic disease

  25. Systemic Vasculitic Neuroapthy • Pathogenesis • Immune complex deposition • Mixed cryoglobulinemia, Polyarteritis nodosa • Cell-mediated immunity • ANCA associated vasculitides • Patterns of nerve involvement • Ischemic lesions occur randomly along course of peripheral nerve  asymmetric, patchy involvement of both sensory and motor nerves • Longest nerves are affected first • typically lower extremity (sciatic nerve)- often present with foot drop

  26. Systemic Vasculitic Neuroapthy • Clinically present with pain, paresthesias, numbness and weakness • Mononeuritis multiplex • Polyneuropathy • radiculopathy and/or plexopathy • Non-neurologic symptoms of fever, malaise, weight loss and specific organ involvement

  27. Systemic Vasculitides • Primary systemic vasculitides • Microscopic polyangiitis • Polyarteritis Nodosa • Churg-Strauss syndrome • Wegener’s Vasculitis • Secondary systemic vasculitides • Rheumatoid Vasculitis • SLE, Sjogren’s • Hepatitis C and mixed cryoglobulinemia • HIV and CMV • Paraneoplastic vasculitic neuropathy

  28. Wegener’s Vasculitis • Wegener’s • c-ANCA (PR3) 75-90% • p-ANCA (MPO) 5-20% • Pulmonary disease in 70-85% • Glomerulonephritis in 70-80% • Arthralgias/Arthritis in 60-70% • Peripheral nerve involvement reported in up to 40% of cases and often occurs early in the disease course • Nerve involvement can be the first and sole symptom of a beginning systemic vasculitis c-ANCA p-ANCA

  29. Case #3 • 54 y/o WM • seen in ED 2 weeks prior after falling - Left wrist fx • In ED, found to be anemic (HCT 24, MCV 74) • Presents to you for follow-up • ROS: • Diarrhea x many months, watery, yellow, 2-12 BM/day, frequent abd distention • Denies abdominal pain, BRBPR, melena • Arthritis- mostly large joints • Frequent Headaches (No photophobia, blurry vision) • ? Unsteady gait

  30. Case #3 • Pmhx: arthritis • Meds: Naprosyn, Flexeril • Fhx: Non- contributory • Sochx: no tobacco, occ alcohol, No IVDA

  31. Case #3 • PE: AF,135/69, 94, 20, 100% RA • Pale conjunctiva, OP- clear • CV: RRR no m/r/g • Lungs: clear • ABD: soft, mild distention, BS present, No hepatosplenomegaly or bruits • Ext: No C/C/E • Skin: No rashes • Rectal: heme negative • Neuro: normal except for mild decreased vibratory sense in bilateral lower extremities

  32. Case #3 • Data: • Hct 24, MCV 74, WBC 4.5 (nl diff), plts 154 • Chem 7: Normal • Retic 2.1%, LDH 168, ferritin <1 • Fe 8, TIBC 318, B12 175 • AST 54, ALT 48, Bili 0.9, AP 132, Alb 3.2, • Amylase 55, TSH 2.4, FT4 1.0 • 25-OH Vit D 12

  33. Case #3 • Work-up: • Head CT in ED: Normal • Colonoscopy: 2 tubular adenomas o/w Normal • EGD :gastropathy without acute bleed (CLO -) • Scalloped mucosa in duodenum • Biopsy: mucosal inflammation, crypt hypoplasia, villous atrophy • Anti-endomysial Ab: (+) 1: 160 • DEXA Osteoporosis T score: – 3.3 spine

  34. Celiac disease • Epidemiology • Occurs primarily in whites of northern European ancestry • Based on classic symptoms of malabsorption • Prevalence 1:4000- 1:8000 • subclinical or oligosymptomatic celiac diseae • Prevalence 1:250- 1:500

  35. Celiac disease • Pathogenesis • Immune disorder that is triggered by an a environmental agent (gliadin component of gluten) in genetically predisposed individuals • IgA antibodies to gliadin • Ig A antibodies to endomysium • (target antigen: tissue transglutaminase)

  36. Celiac disease • Clinical Manifestations • Diarrhea with steatorrhea • Consequences of malabsorption • Weight loss • Anemia (iron deficiency and b12) • Neurologic disorders from b12 deficiency • Osteopenia from vit D and calcium deficiency

  37. Celiac disease • Associated Conditions • Liver disease • Diabetes Mellitus • Thyroid disease • Myocarditis and cardiomyopathy • GI malignancies & non-Hodgkin's lymphoma • Dermatitis herpetiformis

  38. Celiac disease • Treatment • Gluten free diet • Calcium and Vitamin D replacement • IV Iron • Bisphosphonate

  39. The End

  40. Brachial Plexus

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