Intern Report 2/23 – Wernickes

Teaching Points:

  • Wernicke’s Syndrome presents as a clinical triad of:
    • Encephalopathy
    • Ataxia
    • Ophthalmoplegia
  • The presence of all three characteristics is not needed in order to make the clinical diagnosis.
    • An under-recognized disease
  • Due to the lack of vitamin B1 (thiamine).
  • Most common presentation is encephalopathy.
    • Can manifest as indifference, confusion, poor attention span.
    • Can rarely present as agitation in the setting of alcohol withdrawal.
    • Severe cases can progress to stupor and coma
  • Second most common sign is ataxia
    • Ataxia can present itself before encephalopathy
  • Ophthalmoplegia
    • Presence of lateral right and left nystagmus, lack of smooth pursuit
    • Severe cases can cause pupillary defects
  • Patients do not have to be alcoholics in order to get Wernicke’s disease.
    • Can be seen in those with poor oral intake, malnutrition, high catabolic states (sepsis), and those with poor utilization of thiamine (poor overall body substrate).
  • Initiation of glucose prior to thiamine administration may actually precipitate Wernicke’s syndrome.
  • Treatment involves high dose thiamine administration, with 500mg IV q8 hours x 2 days, then daily for 5-7 days.
  • Response can be seen within hours to days.
  • Untreated and prolonged Wernicke’s may lead to Korsakoff psychosis.
    • Presents with anterograde and retrograde amnesia (long term memory retained), and confabulation.
    • A more irreversible disease course.

Resident Report 2/22 -TTP

Teaching Points:

  • TTP is a hematologic emergency. It is manifested by the clinical pentad of:
    • Thrombocytopenia
    • Microangiopathic hemolytic anemia (schistocytes on smear)
    • Acute renal insufficiency
    • Fevers
    • Encephalopathy
  • Only need thrombocytopenia and MAHA in order to make the diagnosis
  • Treatment involves initiation of plasmapheresis
  • ADAMTS 13 Testing
    • ADAMTS 13 functions as a protease that cleaves the von Willebrand multimers
    • Deficiency of ADAMTS 13 leads to accumulating amounts of vWF, causing platelet aggregation (leading to thrombocytopenia) and disruption/breaking of RBCs (MAHA)
    • Activity influenced by plasma products and transfusions.
    • Levels of Activity
      • >50% activity argues against TTP.
      • <10% activity suggests TTP
      • 10-50% activity is an ambiguous area
    • Presence of an ADAMTS13 inhibitor
      • Presence suggested acquired TTP
      • Lack of inhibitor suggests hereditary cause of TTP.

Intern Report 2/16 – Pleural Effusions

Teaching Points

  • Parapneumonic effusions are effusions associated with pneumonia, which can occur in about 20-25%
  • If <10mm on lateral film and/or decreasing with antibiotic treatment, may continue to monitor and just treat with antibiotics.
  • Lights Criteria¬† – differentiates transudative and exudative fluid.
    • Criteria
      • Pleural TP/Serum TP >0.5
      • Pleural LD/Serum LD >0.6
      • Pleural LD > 2/3 ULN plasma LD
    • Need at least one positive result to categorize pleural fluid as exudative.
      • Setup of Light’s criteria leads to increased sensitivity and decreased specificity.
      • Purpose is due to the fact that you don’t want to miss a process causing an exudative fluid so you want your rate of false negatives to be low. Hence an increased sensitivity.
  • Cell count
    • Predominant neutrophlic – bacterial
    • Predominant lymphocytic – think TB or malignancy
    • Predominant eosinophil – think irritation due to blood, air, etc
  • Complicated parapneumonic effusion associated with worse outcomes, necessitates need for drainage.
    • pH<7.2 (normal pleural fluid pH 7.6)
    • Pleural glucose <60

Resident Report 2/10 – Paraproteinemias

Teaching Points:

  • Multiple Myeloma
    • Plasma cell dyscrasia causing monoclonal antibody development complicated with end organ damage
    • Criteria For Diagnosis
      • M-spike (suggest overproduction of a specific antibody)
      • Bone Marrow Biopsy with >10% plasma cells
      • Evidence of end-organ damage (CRAB)
        • Hypercalcemia – 25%
        • Renal insufficiency – 50%
          • Prone to worsening renal disease as a result of diuresis and NSAID therapy
        • Anemia – 75%
        • Bone involvement – 50%
          • Patients present with bony pain worsened with movement.
    • Differential Diagnosis
      • Smoldering Multiple Myeloma
        • M-spike protein >3g/dl
        • Bone marrow biopsy with plasma cells >10%
        • No evidence of end organ damage (CRAB criteria)
      • MGUS
        • M-spike <3g/dL
        • Bone marrow biopsy with plasma cells <10%
        • No evidence of end organ damage (CRAB criteria)
    • Waldenstrom’s macroglobulinemia differs from multiple myeloma in that the monoclonal spike is due specifically to IgM.
      • IgM is a tetramer and instead manifests as hyperviscosity syndrome.
      • Patients may also have lymphadenopathy, splenomegaly, etc.

Intern Report 2/9 – Idiopathic Intracranial Hypertension (IIH) aka Pseudotumor Cerebri

Teaching Points:

  • Disorder defined by clinical criteria that include:
    • symptoms and signs isolated to those produced by increased intracranial pressure (headache, papilledema, vision loss)
    • elevated intracranial pressure with normal CSF composition
    • no other causes of intracranial hypertension evident on neuroimaging
  • Clinical Manifestations
    • Young obese female of childbearing age complaining of headaches found to have papilledema on fundoscopic exam.
    • Symptoms include headache, transient visual obscurations, intracranial noises (pulsatile tinnitus), photopsia, back pain, retrobulbar pain, diplopia, sustained vision loss.
  • Exam Findings
    • Most common findings are papilledema, visual field loss, sixth nerve palsy.
  • Evaluation
    • Urgent neuroimaging is required to exclude secondary causes of intracranial hypertension.
      • MRI brain with MR venography
    • If imaging is negative, LP is performed to check opening pressure.
  • Differential Diagnosis (Headache and papilledema)
    • Intracranial mass lesions
    • Obstruction of venous outflow
      • Venous sinus thrombosis
      • Jugular vein compression
      • Neck surgery
    • Obstructive hydrocephalus
    • Decreased CSF absorption
      • bacterial meningitis causing arachnoid granulation adhesions
    • Increased CSF production
      • Choroid plexus papilloma
    • Malignant systemic hypertension
  • Treatment
    • Weight loss, treat OSA or anemia if present, Avoid exacerbating drugs (tetracycline derivatives, Vit A analogs, etc)
    • Acetazolamide. May consider Lasix if symptoms persist
    • Surgical shunting

Resident Report 2/8 – Ischemic Cardiomyopathy

Teaching Pearls:

    • Ischemic cardiomyopathy is most common cause for development of LV thrombus.
    • Cardiogenic shock
      • MAP = CO x SVR
        • MAP decreased due to CO decrease. SVR increases to compensate.
      • Most commonly caused by ischemic cardiomyopathy
      • Presence of transaminitis, AKI, elevated lactic acid suggestive of hypoperfusion (cardiogenic shock)
    • Murmurs
      • Left sided heart disease
        • Murmurs decrease with inspiration
      • Right sided heart disease
        • Murmurs increase with inspiration
      • Squatting Test
        • Increases preload back to the heart
        • Causes increased intensity in all murmurs except HOCM and MVP
      • Valvsalva
        • Decreases preload back to the heart
        • Causes decreased intensity in all murmurs except HOCM and MVP
      • Fixed split S2
        • Due to ASD
        • Inspiration causes increased preload, leading to delayed P2 sound (causing the split)
        • Expiration causes increased blood return to left side of heart, which flows through ASD to right side of heart, also causing delayed P2 sound (causing the split).
        • Inspiratory and expiratory causes same effect towards P2, leading to fixed split S2.

Resident Report 2/3 -Palpable Purpura

Teaching Pearls:

  • Common causes of palpable purpura (leukocytoclastic vasculitis)
    • Infections
      • HBV, HCV, HIV, etc
    • Medications
      • Commonly occurs 7-10 days after the initiation of a medications
      • Common culprit medications include penicillin, cephalosporin, NSAIDs, etc
    • Autoimmune disease (see below)
    • Malignancy
      • Paraneoplastic syndrome
  • Autoimmune Disease with palpable purpura
    • Granulomatosis Polyangiitis (Wegeners)
      • Hepatorenal syndrome with sinusitis
      • c-ANCA/anti-Pr-3 positive
    • Churg Strauss Syndrome
      • Associated with eosinophilia
      • p-ANCA positive
    • Microscopic polyangittis
      • p-ANCA positive
    • Cyroglobulinemic vasculitis
      • HCV positive
      • cryoglobin positive
    • HSP
      • palpable purpura with abdominal pain, arthralgia, renal involvement
      • Skin biopsy positive for LCV with IgA deposits