Clinical Problem Solving 121608

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Clinical Problem Solving12/16/08

• Moderator Stuart Cohen MD
• Discussant Carlos Estrada MD

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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

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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

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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

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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

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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

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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)

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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

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Diagnostic testing for GAS

• Rapid Streptococcal Antigen Test (RAST)
• Sensitivity 80-90
• Specificity 90- 95
• Throat Culture
• Sensitivity 90
• Specificity 95

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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

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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

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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

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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

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Case 2

• Evaluation at University of Michigan 1 yr ago
• MRI brain and c-spine Normal study
• Shoulder films- Normal
• EMG right brachial plexopathy

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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

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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 sisters house
• Past truck driver
• Past tobacco use, no alcohol, no drugs

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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

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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

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Case 2

• Neurological exam (contd)
• 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.

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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
• TFTs- normal
• HIV- negative, HCV- negative, HbsAg- negative
• UA 3 blood, 2 protein, gt25 RBCs
• Urine Protein/Creatine 730 mg/24hr

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Case 2

• ESR 10, ANA lt 180, RF (-)
• C3,C4,CH50 - normal
• SPEP, UPEP, IFE Negative
• P-ANCA (-), C-ANCA 1320
• Anti-MPO lt13, Anti-PR3 129

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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)

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Approach to Weakness
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Vasculitic neuropathies

• Systemic Vasculitic Neuropathy
• Nonsystemic Vasculitic Neuropathy
• No associated systemic disease

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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

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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

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Systemic Vasculitides

• Primary systemic vasculitides
• Microscopic polyangiitis
• Polyarteritis Nodosa
• Churg-Strauss syndrome
• Wegeners Vasculitis
• Secondary systemic vasculitides
• Rheumatoid Vasculitis
• SLE, Sjogrens
• Hepatitis C and mixed cryoglobulinemia
• HIV and CMV
• Paraneoplastic vasculitic neuropathy

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Wegeners Vasculitis

• Wegeners
• 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
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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

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Case 3

• Pmhx arthritis
• Meds Naprosyn, Flexeril
• Fhx Non- contributory
• Sochx no tobacco, occ alcohol, No IVDA

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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

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Case 3

• Data
• Hct 24, MCV 74, WBC 4.5 (nl diff), plts 154
• Chem 7 Normal
• Retic 2.1, LDH 168, ferritin lt1
• 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

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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

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Celiac disease

• Epidemiology
• Occurs primarily in whites of northern European
  ancestry
• Based on classic symptoms of malabsorption
• Prevalence 14000- 18000
• subclinical or oligosymptomatic celiac diseae
• Prevalence 1250- 1500

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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)

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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

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Celiac disease

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

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Celiac disease

• Treatment
• Gluten free diet
• Calcium and Vitamin D replacement
• IV Iron
• Bisphosphonate

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The End
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Brachial Plexus