Neurology Book

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Pseudotumor Cerebri

Aka: Pseudotumor Cerebri, Idiopathic Intracranial Hypertension, Benign Intracranial Hypertension
  1. Epidemiology
    1. Incidence
      1. General Population: 0.9 per 100,000
      2. Women aged 15-44 years: 3.5 per 100,000
      3. Obese women aged 20-44 years: 19.3 per 100,000
      4. Pseudotumor occurs in children and must be kept in differential in refractory new Headache
        1. (2015) Pediatr Emer Care 31:6-9 [PubMed]
    2. Rare: similar Incidence as
      1. Amyotrophic Lateral Sclerosis
      2. Guillain Barre Syndrome
      3. Muscular Dystrophy
  2. Pathophysiology
    1. Intracranial Hypertension
    2. Optic disc edema
      1. Causes transient Optic Nerve ischemia
  3. Causes
    1. See Increased Intracranial Pressure Causes
  4. Symptoms
    1. Headache
      1. Location: retro-orbital Headache
      2. Provocative: eye movement worsens Headache
      3. Timing: Chronic Daily Headache, worse on awakening
      4. Characteristics: Throbbing Headache
      5. Associated Symptoms: Nausea and Vomiting
    2. Transient Decreased Visual Acuity (75%)
      1. Monocular or Binocular Acute Vision Loss
      2. Lasts for only a few seconds
      3. Permanent visual changes occur in a few patients
        1. Increased blind spot
          1. Blurred Vision or Tunnel Vision
          2. Dark spot in temporal Visual Field
        2. Profound Vision Loss or blindness (severe cases)
      4. Other visual changes
        1. Photophobia
        2. Diplopia
    3. Pulsatile Tinnitus (60%)
      1. Unilateral or bilateral "whooshing" sound
      2. Palliative:
        1. Lumbar Puncture
        2. Jugular venous compression
    4. Musculoskeletal symptoms
      1. Neck Pain or neck stiffness
      2. Back pain
      3. Arthralgias (Shoulder, wrist, knee)
    5. Neurologic Symptoms and Psychiatric Symptoms
      1. Paresthesias
      2. Radicular pain
      3. Facial palsy
      4. Impaired concentration or memory
      5. Major Depression
  5. Signs
    1. Ophthalmoscopy (Fundoscopy)
      1. Decreased venous pulsations
      2. Papilledema
        1. Not predictive of visual outcome
    2. Visual Field defects
      1. Best detected by perimetry (Visual Field testing)
      2. Blind spot enlargement
      3. Inferonasal visual loss
      4. Visual Field constriction (tunnel Vision)
    3. Decreased Ocular Motility
  6. Diagnostics: Lumbar Puncture
    1. Opening Pressure consistent with Pseudotumor Cerebri
      1. Obese Patient > 250 mm of water (25 cm water)
      2. Non-obese Patient > 200 mm of water (20 cm water)
    2. Opening Pressure falsely elevated by:
      1. Sitting position
      2. Prone position (fluoroscopy)
      3. Painful tap
      4. Anxiety
        1. Consider pretreating LP with Valium dose
    3. Send CSF for spinal fluid analysis
      1. CSF Glucose
      2. CSF Protein
      3. CSF Cell Count
      4. CSF Cultures (Bacteria, fungi, Tuberculosis)
      5. CSF Cytology
  7. Imaging
    1. Head MRI (preferred over CT Head)
      1. Negative MRI does not exclude Idiopathic Intracranial Hypertension (but does evaluate for other causes)
      2. Ventricles may be decreased in size (slit-like) or may be normal
      3. Empty Sella Sign
        1. Test Sensitivity: 48 to 74%
        2. Test Specificity: 84 to 94%
      4. Posterior Globe Flattening
        1. Test Sensitivity: 46 to 65%
        2. Test Specificity: 85 to 98%
      5. Optic Nerve Tortuosity
        1. Test Sensitivity: 26 to 48%
        2. Test Specificity: 82 to 92%
      6. Transverse Sinus Stenosis
        1. Test Sensitivity: 65 to 94%
        2. Test Specificity: 92 to 97%
      7. References
        1. Kwee (2019) Eur J Radiol 116: 106-15 +PMID: 31153551 [PubMed]
  8. Differential Diagnosis
    1. Migraine Headache
    2. Chronic Daily Headache
    3. Rebound Headache (Analgesic overuse)
  9. Management: Medical
    1. Neurology Consultation
    2. Weight loss
    3. Dietary changes
      1. Low salt diet
      2. Low tyramine diet
    4. Avoid sulfa conjugated medications
    5. Diuretic
      1. Acetazolamide (Diamox)
        1. Dose: 1 to 4 grams daily divided bid-tid
      2. Furosemide (Lasix)
    6. Systemic Corticosteroids
      1. Reserved for urgent management of Vision Loss
    7. Headache Management
      1. Acute Treatment: NSAIDs
      2. Prophylaxis: Tricyclic Antidepressants
    8. Therapeutic large volume Lumbar Puncture
      1. Removal of 20 ml of spinal fluid
  10. Management: Surgical
    1. Optic Nerve Sheath Decompression
      1. Indicated for associated Decreased Visual Acuity
      2. Window or fenestration cut in Optic Nerve sheath
      3. Results in increased Blood Flow to the Optic Nerve
    2. Cerebrospinal fluid Shunt
      1. Lumboperitoneal shunt (preferred over ventricular)
      2. Short term: Very effective
      3. Long term: Multiple revisions often required
  11. Management: Pregnancy
    1. Careful follow-up
      1. Frequent Neurology evaluation
      2. Frequent Ophthalmology evaluation
      3. Repeated Lumbar Puncture monitoring
    2. Intervention
      1. Acetazolamide (Diamox) after 20 weeks gestation
      2. Systemic Corticosteroids for Vision deterioration
      3. Optic Nerve Sheath Decompression
      4. Ventriculoperitoneal Shunt
    3. Contraindicated Agents
      1. Avoid Tricyclic Antidepressants
      2. Avoid Thiazide Diuretics
  12. Complications
    1. Blindness
  13. References
    1. Friedman (1999) Neurosurg Clin N Am 10(4):609-21 [PubMed]

Pseudotumor Cerebri (C0033845)

Definition (NCI) An idiopathic disorder characterized by chronic increase in the intracranial pressure. It occurs predominantly in obese females of childbearing age. It is associated with papilledema.
Definition (MSH) A condition marked by raised intracranial pressure and characterized clinically by HEADACHES; NAUSEA; PAPILLEDEMA, peripheral constriction of the visual fields, transient visual obscurations, and pulsatile TINNITUS. OBESITY is frequently associated with this condition, which primarily affects women between 20 and 44 years of age. Chronic PAPILLEDEMA may lead to optic nerve injury (see OPTIC NERVE DISEASES) and visual loss (see BLINDNESS).
Concepts Disease or Syndrome (T047)
MSH D011559
ICD9 348.2
ICD10 G93.2
SnomedCT 155052007, 267701004, 68267002
English Benign Intracranial Hypertension, Hypertension, Benign Intracranial, Intracranial Hypertension, Benign, Idiopathic Intracranial Hypertension, Hypertension, Idiopathic Intracranial, BIH - Benign intracr hypertens, Benign intracran hypertension, IIH - Idiopth intracr hyperten, Idiopathic intracr hypertens, PSEUDOTUMOR CEREBRI <BENIGN INTRACRANIAL HYPERTENSION>, PSEUDOTUMOR CEREBRI, INTRACRANIAL HYPERTENSION, IDIOPATHIC, INTRACRANIAL PRESSURE INC. BENIGN, Pseudotumor Cerebri, idiopathic intracranial hypertension, idiopathic intracranial hypertension (diagnosis), pseudotumor cerebri, PSEUDOTUMOR CEREBRI BENIGN INTRACRANIAL HYPERTENSION, Pseudotumor Cerebri [Disease/Finding], benign intracranial hypertension, benign hypertension intracranial, nonne's syndrome, cerebri pseudotumor, pseudotumour cerebri, syndrome pseudotumor cerebri, Intracranial Hypertension, Idiopathic, Benign intracranial hyperten., Benign intracran. hypt., Benign intracranial hypertensn, Benign intracranial hypertension, Pseudotumor cerebri, Noninfective serous meningitis, Nonne's syndrome, Otitic hydrocephalus syndrome, BIH - Benign intracranial hypertension, IIH - Idiopathic intracranial hypertension, Idiopathic intracranial hypertension, Pseudotumour cerebri, Benign intracranial hypertension (disorder), cerebri; pseudotumor, increased; pressure, intracranial (benign), intracranial; increased pressure (benign), pseudotumor; cerebri
German PSEUDOTUMOR CEREBRI, Gutartige intrakranielle Drucksteigerung, benigne intrakranielle Hypertonie, Benigne intrakranielle Drucksteigerung, Hypertension, benigne intrakranielle, Idiopathische intrakranielle Drucksteigerung, Intrakranielle Drucksteigerung, benigne, Pseudotumor cerebri
Italian Ipertensione endocranica benigna, Pseudotumor cerebri, Ipertensione intracranica idiopatica, Ipertensione intracranica benigna, Pseudo tumore cerebrale
Dutch pseudotumor cerebri, cerebri; pseudotumor, intracraniaal; drukverhoging (benigne), pseudotumor; cerebri, verhoging; druk, intracraniaal (benigne), benigne intracraniale hypertensie, Benigne intracraniale hypertensie, Hypertensie, benigne intracraniale, Idiopathische intracraniale hypertensie, Pseudo-tumor cerebri, Tumor cerebri, pseudo-
French Pseudotumeur cérébrale, HYPERTENSION INTRACRANIENNE BENIG, PSEUDO-TUMEUR CEREBRALE, Hypertension intracrânienne bénigne, Pseudotumeur cerebri, Syndrome d'hypertension intracrânienne bénigne
Portuguese Pseudotumor do cérebro, HIPERTENSAO INTRACRAN BENI, PSEUDO TUMOR CEREBRAL, Hipertensão intracraniana benigna, Hipertensão Intracraniana Benigna, Hipertensão Intracraniana Idiopática, Pseudotumor Cerebral
Spanish Pseudotumor cerebral, PSEUDOTUMOR CEREBRAL, Pseudotumor Cerebral, hipertensión intracraneal benigna (trastorno), hipertensión intracraneal benigna, meningitis serosa no infecciosa, pseudotumor cerebral, síndrome de Nonne, síndrome de hidrocefalia ótica, Hipertensión intracraneal benigna, Hipertensión Intracraneal Benigna, Hipertensión Intracraneal Idiopática, Seudotumor Cerebral
Russian ПСЕВДОТУМОРАЛЬНЫЙ ЦЕРЕБРАЛЬНЫЙ СИНДРОМ, DOBROKACHESTVENNAIA INTRAKRANIAL'NAIA GIPERTENZIIA, PSEVDOOPUKHOL' GOLOVNOGO MOZGA, INTRAKRANIAL'NAIA GIPERTENZIIA DOBROKACHESTVENNAIA, PSEVDOTUMORAL'NYI TSEREBRAL'NYI SINDROM, GIPERTENZIIA VNUTRICHEREPNAIA DOBROKACHESTVENNAIA, ГИПЕРТЕНЗИЯ ВНУТРИЧЕРЕПНАЯ ДОБРОКАЧЕСТВЕННАЯ, ДОБРОКАЧЕСТВЕННАЯ ИНТРАКРАНИАЛЬНАЯ ГИПЕРТЕНЗИЯ, ИНТРАКРАНИАЛЬНАЯ ГИПЕРТЕНЗИЯ ДОБРОКАЧЕСТВЕННАЯ, ПСЕВДООПУХОЛЬ ГОЛОВНОГО МОЗГА
Swedish Benign intrakraniell tryckökning
Czech pseudotumor cerebri, Benigní intrakraniální hypertenze, Pseudotumor cerebri
Finnish Aivojen valekasvain
Korean 양성 두개강내 고혈압
Polish Guz mózgu rzekomy, Zapalenie opon surowicze, Nadciśnienie śródczaszkowe łagodne, Obrzęk rzekomoguzowy mózgu
Hungarian Jóindulatú koponyaűri nyomásfokozódás, Pseudotumor cerebri
Japanese 仮性脳腫瘍, 偽脳腫瘍, ﾘｮｳｾｲﾄｳｶﾞｲﾅｲｱﾂｺｳｼﾝ, ｷﾞｾｲﾉｳｼｭﾖｳ, 偽性脳腫瘍, 脳腫瘍-仮性, 特発性頭蓋内圧亢進, 脳仮性腫瘍, 脳偽腫瘍, 良性頭蓋内圧亢進, 頭蓋内圧亢進-特発性, 頭蓋内圧亢進-良性, 頭蓋内高血圧症-良性
Norwegian Pseudotumor cerebri, Benign intrakranial hypertensjon
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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