A 33YearOld Woman With a Sudden Loss of Vision

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A 33-Year-Old Woman With a Sudden Loss of Vision
Eugene G. Martin, Ph.D. Associate Professor of
Pathology Laboratory Medicine

• Based upon LABORATORY MEDICINE CASEBOOK. An
  introduction to clinical reasoning
• Jana Raskova, MD Professor of Pathology
  Laboratory MedicineStephen Shea, MD
  Professor of Pathology Laboratory
  MedicineFrederick Skvara, MD Associate
  Professor of Pathology Laboratory MedicineNagy
  Mikhail, MD Assistant Professor of Pathology
  Laboratory MedicineUMDNJ-Robert Wood Johnson
  Medical SchoolPiscataway, NJ

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History - Physical Examination

• 33-Year-Old woman admitted to the hospital for
  sudden bilateral loss of vision.
• History
• A year ago, patient noticed difficulty walking
  which improved spontaneously two weeks later
• Occasional tingling and an electrical sensation
  down her back upon flexing neck
• Occasional difficulty in expressing herself
  verbally
• Physical exam
• Alert and oriented woman in mild distress
• Speech normal
• No light perception in either eye ocular
  movements intact pupils sluggishly reactive to
  light no nystagmus
• Paraparesis with spasticity and dissociated
  sensory loss in the lower extremities. Senory
  level T7,
• Brisk deep tendon reflexes in the knees
• Bilateral, positive Babinski sign

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MRI - Brain

• Abnormal
• Areas of increased signal density are present in
  the deep white matter of the brain (Arrows)
• Arrows represent demyelinated plaques of MS

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HEMATOLOGY
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CHEMISTRY
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Other Tests
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CSF
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CSF Electrophoresis
One broad band in Gamma region

• Several oligoclonal bands are apparent in the
  gamma region of the patients CSF
• Manufacturers control demonstrates oligoclonal
  bands (arrow)

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Labs

• Brain biopsy (Luxol fast bluePAS(x78))
• Evolving plaque of MS
• Note several macrophages containing blue
  fragments of myelin.
• PAS-positive material represents breakdown
  product of myelin
• Brain biopsy (Luxol fast bluePAS(x31))
• Edge of an old plaque of MS
• The plaque in the upper portion of the image
  shows almost complete demyelination
• Bottom half of image demonstrates normal
  myelinated white matter

Nearly complete demyelination
Normal White Matter
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Brain Acute onset of MS

• Brain biopsy (Oil red O stain Polarized light.
  x50)
• Evolving plaque of MS
• Frozen section of a portion of an active plaque.
• Intact myelin stains red and is not birefringent.
  Under polarized light appear yellow and blue
  crystals within the macrophages.

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Myelin Basic Protein in CSF

• Elevated in 90 of MS patients during acute
  exacerbation phase of disease
• MBP is NOT specific for MS
• MBP elevated in
• Neurosyphilis (3o syphilis)
• Head Trauma
• Leukodystrophies
• Leukemia
• Hypoxia
• CVAs
• Intrathecal chemotherapy
• Post irradiation states
• Giuilian-Barre?
• Leukoencephalopathy

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CSF Protein

• Increased CSF Protein
• Traumatic tap
• ? Permeability of blood-CSF barrier
• ? removal of protein (meningitis)
• Obstruction to CSF circulation tumor,
  herniation or adhesions
• ? Synthesis of immunoglobulin CNS lymphoma

• Decreased CSF Protein
• CSF leakage (trauma)
• Removal of large amounts of CSF during a tap
• Hypothyroidism