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Flashcards in Paraneoplastic Syndromes Deck (12):
1

What is the definition of paraneoplastic syndrome?

  • —A disease or symptom that is an in-direct effect of cancer in the body.
    • —Mediated by humoral factors (by hormones or cytokines) excreted by tumor cells or by an immune response against the tumor.
  • —Typical among middle aged to older patients, and they most commonly present with cancers of the lung, breast, ovaries or lymphoma.
    • —Symptoms of paraneoplastic syndromes can present before the diagnosis of a malignancy, during or after.
    • —NB: Can have non cancer associated paraneoplastic syndromes

2

What are definite paraneoplastic syndromes?

  • —A "classical" syndrome and cancer that develops within five years of diagnosis of the neurologic disorder
  • —A nonclassical syndrome that resolves or significantly improves after cancer treatment without concomitant immunotherapy, provided that the syndrome is not susceptible to spontaneous remission
  • —A nonclassical syndrome with paraneoplastic antibodies and cancer that develops within five years of the diagnosis of the neurologic disorder.
  • —A neurologic syndrome (classical or not) with "well-characterized" paraneoplastic antibodies and no cancer. These well-characterized antibodies include anti-Hu, CV2, Ri, Yo, Tr, Ma2, and amphiphysin.

3

What are possible paraneoplastic syndromes?

  • —A classical syndrome as defined above, no paraneoplastic antibodies, no cancer, but at high risk to have an underlying tumor
  • —A neurologic syndrome (classical or not) with partially characterized paraneoplastic antibodies (eg, not the well-characterized antibodies described above) and no cancer
  • —A nonclassical syndrome, no paraneoplastic antibodies, and cancer present within two years of diagnosis

4

What are the neurologic paraneoplastic syndromes?

  • —CNS
    • —Brain
      • —Seizures
      • —Cerebral dysfunction
      • —Cerebellar dysfunction
      • —Brainstem dysfunction
    • —Eyes
    • —Spinal Cord
  • —PNS
    • —Spinal roots/nerves
    • —Muscle
       

5

What is the pathophysiology and clinical findings of paraneoplastic cerebellar degeneration?

  • —Clinical Symptoms-Acute to sub-acute
    • —Dysarthria
    • —Truncal, limb and gait ataxia
    • —Vertigo
    • —Diplopia and nystagmus
  • —Pathophysiology: Anti-pukinje cell anti-bodies
    • —Anti-Yo = Ovarian Cancer
    • —Anti-Hu = SCLC
    • —Others: Anti-Tr, -VGCC,-Zinc
  • —MRI-Cerebellar atrophy
  • —CSF: pleocytosis, increased protein

6

What is the pathophysiology and clinical findings of paraneoplastic limb encephalitis?

  • —Symptoms
    • —Psychiatric
    • —MS changes/Memory Loss
    • —Seizures
  • —Pathophysiology:
    • —Onco-neural anti-bodies
      • —Anti-Hu = SCLC
      • —Anti-Ma = Testicular cancer
    • —Cell surface or synaptic protein Anti-bodies
      • —VGKC = SCLC/Thymoma
      • —AMPA receptor = SCLC, Breast and Thymus
      • —GABA-b receptor = SCLC
      • —NMDA = Ovarian Cancer/Teratoma

7

What is the pathophysiology and clinical findings of LEMS?

  • —Symptoms
    • —Muscle Weakness (mostly below the neck), LE > UE
    • —Dry mouth
  • —Pathophysiology
    • —Reduced numbers of P/Q Ca++ channels on presynaptic terminals
    • —VGCC anti-body = SCLC, lymphoproliferative disorders, thymoma, NSCLC
  • —EMG-Incremental response
    • The more you use the muscle, the more likely to fire
    • Patients get stronger with activity

8

What is the pathophysiology and clinical findings of paraneoplastic sensory neuronopathies?

  • —Symptoms:
    • —Painful paresthesias & dysesthesias
    • —Asymmetric; Distal or Proximal
    • —Sensory loss (95%)
      • —All modalities involved
      • —Ataxia: Sensory
      • —Pseudoathetosis
    • —Proximal & Distal
      • —Asymmetric or Symmetric
      • —Discomfort: Pain; Paresthesias
  • —Pathophysiology: Anti-Hu = SCLC
  • CSF: pleocytosis, increased protein

9

What is paraneoplastic acanthosis nigricans?

  • —Thickened brown to black, poorly defined, velvety hyperpigmentation of the skin
  • —Found in body folds such as armpits, groin, navel, neck. Mucous membranes.
  • —GI malignancies and GU
    • —Gastric and Adenocarcinoma
  • —Older age approx 40
  • —EGF/TGF-alpha, IGF-1 and FGF

10

What is paraneoplastic dermatomyositis?

  • —Connective tissue disease with inflammation of the muscles and skin
    • —Heliotrope and Gotton’s sign
    • —Malar erythema
    • —Piokiloderma
  • —Lab: increased CPK and ESR
    • —EMG, muscle bx
  • —Adenocarcinoma
    • —Breast, Lung, ovarian
       

11

What are the findings of paraneoplastic Cushing syndrome?

  • —Symptoms
    • —Muscle weakness, peripheral edema, HTN, increased weight, cushingoid features
  • —Lab: hypokalemia, increased cortisol, nl to elevated ACTH
  • —Ectopic ACTH or ACTH-like substance
    • —SCLC, Pancreatic, thymoma, Neuro-endocrine tumors—

12

What are the findings of paraneoplastic SIADH?

  • —Symptoms
    • —Unsteady gait/falls, HA, Nausea, fatigue/lethargy, confusion, seizures
  • —Lab: low sodium, increase urine osmolality
  • —SCLC, some others
  • —Tumor produces ADH (increase free water resorption) and atrial natiuretic peptide (natiuretic and anti-diuretc properties)