PSEUDOTUMOR CEREBRI
 
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PSEUDOTUMOR CEREBRI

 

DEFINITION:

A syndrome characterized by an elevated intracranial pressure (ICP) but normal cerebral spinal fluid (CSF) and ventricles.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • any
  • risk factors:
    • see below

PATHOGENESIS:

1. Causes of Pseudotumor Cerebri

1. Metabolic

  • Galactosemia
  • hypophosphatemia
  • hypo/hypervitaminosis A

2. Endocrine

  • Addison Disease
  • hypoparathyroidism
  • menarche
  • obesity
  • oral contraceptives
  • prolonged or withdrawal
  • from corticosteroids

3. Infections

  • OM (Otitic Hydrocephalus)
  • Guillain-Barre Syndrome
  • Lyme Disease
  • roseola infantum

4. Drugs

  • chlortetracycline
  • nalidixic acid
  • tetracycline

5. Hematologic

  • anemia (hemolytic and iron-deficiency)
  • polycythemia
  • Wiskott-Aldrich Syndrome

6. Obstruction of Intracranial Venous Drainage

  • head injury
  • lateral sinus
  • posterior sagittal sinus
  • superior vena cava

7. Others

  • chronic lung disease (CO2 retention)
  • disseminated lupus erythromatosus

2. Pathogenesis

  • etiology unknown
  • the elevated ICP may be due to alterations in CSF absorption and production, cerebral edema, abnormalities in vasomotor control and cerebral blood flow, and/or venous obstruction
  • compression of the sixth cranial nerve may produce diplopia

CLINICAL FEATURES:

1. Features of elevated ICP

  • blurred vison
  • diplopia
  • intermittent headaches
  • nausea and vomiting
  • papilledema
    • choked optic discs
    • elevated disc margins
    • enlarged blind spot
    • tortuous & distended retinal veins
  • Macewen ("crack-pot") Sign
    • in infants, percussion of the skull produces a resonant sound due to separation of the cranial sutures

2. Complications

  • optic atrophy and blindness most common complications

INVESTIGATIONS:

1. Lumber Pucture

  • may be diagnostic and therapeutic
  • usually normal but may be a mild pleocytosis

2. Imaging Studies

1. CT/MRI

  • ventricles - normal and midline (may also be slightly reduced in size)

2. Angiography

  • obstruction (thrombosis) of sinuses (i.e., lateral)

MANAGEMENT:

1. Supportive

  • treat underlying cause

2. Medical

1. Reduce ICP

  • serial lumbar punctures
    • may decrease opening pressures by 50%
    • can do as often as bid
  • medications
    • acetazolamines, corticosteroids

3. Surgery

  • indicated for medical failure or evidence of optic atrophy
    • lumboperitoneal shunt
    • subtemporal decompression

4. Prognosis

  • usually resolves spontaneously after 3 months
  • about a 10% recurrence rate
  • about 10% of visual defects last up to 1 year or are permanent

 

 

 

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