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Pseudotumor Cerebri
Aka: Pseudotumor Cerebri, Idiopathic Intracranial Hypertension, Benign Intracranial Hypertension- Epidemiology
- Pathophysiology
- Intracranial Hypertension
- Optic disc edema
- Causes transient Optic Nerve ischemia
- Causes
- Symptoms
- Headache
- Transient Decreased Visual Acuity (75%)
- Monocular or Binocular Acute Vision Loss
- Lasts for only a few seconds
- Permanent visual changes occur in a few patients
- Increased blind spot
- Blurred Vision or Tunnel Vision
- Dark spot in temporal Visual Field
- Profound Vision Loss or blindness (severe cases)
- Increased blind spot
- Other visual changes
- Photophobia
- Diplopia
- Pulsatile Tinnitus (60%)
- Unilateral or bilateral "whooshing" sound
- Palliative:
- Lumbar Puncture
- Jugular venous compression
- Musculoskeletal symptoms
- Neck Pain or neck stiffness
- Back pain
- Arthralgias (Shoulder, wrist, knee)
- Neurologic Symptoms and Psychiatric Symptoms
- Paresthesias
- Radicular pain
- Facial palsy
- Impaired concentration or memory
- Major Depression
- Signs
- Ophthalmoscopy (Fundoscopy)
- Decreased venous pulsations
- Papilledema
- Not predictive of visual outcome
- Visual Field defects
- Best detected by perimetry (Visual Field testing)
- Blind spot enlargement
- Inferonasal visual loss
- Visual Field constriction (tunnel vision)
- Decreased Ocular Motility
- Ophthalmoscopy (Fundoscopy)
- Diagnostics: Lumbar Puncture
- Opening Pressure consistent with Pseudotumor Cerebri
- Obese Patient > 250 mm of water
- Non-obese Patient > 200 mm of water
- Opening Pressure falsely elevated by:
- Sitting position
- Prone position (fluoroscopy)
- Painful tap
- Anxiety
- Consider pretreating LP with Valium dose
- Send CSF for spinal fluid analysis
- CSF Glucose
- CSF Protein
- CSF Cell Count
- CSF Cultures (Bacteria, fungi, Tuberculosis)
- CSF Cytology
- Opening Pressure consistent with Pseudotumor Cerebri
- Radiology
- Differential Diagnosis
- Management: Medical
- Neurology Consultation
- Weight loss
- Dietary changes
- Low salt diet
- Low tyramine diet
- Avoid sulfa conjugated medications
- Diuretic
- Acetazolamide (Diamox)
- Dose: 1 to 4 grams daily divided bid-tid
- Furosemide (Lasix)
- Acetazolamide (Diamox)
- Systemic Corticosteroids
- Reserved for urgent management of Vision Loss
- Headache Management
- Acute Treatment: NSAIDs
- Prophylaxis: Tricyclic Antidepressants
- Therapeutic large volume Lumbar Puncture
- Removal of 20 ml of spinal fluid
- Management: Surgical
- Optic Nerve Sheath Decompression
- Indicated for associated Decreased Visual Acuity
- Window or fenestration cut in Optic Nerve sheath
- Results in increased Blood Flow to the Optic Nerve
- Cerebrospinal fluid Shunt
- Lumboperitoneal shunt (preferred over ventricular)
- Short term: Very effective
- Long term: Multiple revisions often required
- Optic Nerve Sheath Decompression
- Management: Pregnancy
- Careful follow-up
- Frequent Neurology evaluation
- Frequent Ophthalmology evaluation
- Repeated Lumbar Puncture monitoring
- Intervention
- Acetazolamide (Diamox) after 20 weeks gestation
- Systemic Corticosteroids for vision deterioration
- Optic Nerve Sheath Decompression
- Ventriculoperitoneal Shunt
- Contraindicated Agents
- Avoid Tricyclic Antidepressants
- Avoid Thiazide Diuretics
- Careful follow-up
- Complications
- Blindness
- References