Fall 2014: Week 9: Neuro: Optic Neuropathies Flashcards

1
Q

Optic Neuritis:

  1. Define
    a. Swollen Optic Disc (2 types)
    b. NL Optic Disc (2 types)
  2. If the Pt does not have signs of MS or other systemic Dz, the Optic Neuritis is referred to as what?
A
  1. a. Papillitis, Anterior Optic Neuritis
    b. Retrobulbar Optic Neuritis; Retrobulbar Neuritis
  2. Monosymptomatic or Idiopathic
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2
Q

Optic Neuritis: Deymelinating

  1. ALMOST ALWAYS OCCURS how?
  2. MOST COMMON Type of OPTIC NEURITIS?
A
  1. as an Isolated Phenomenon or in Pts who have or will develop MS
  2. Acute demyelinating Optic Neuritis
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3
Q

Optic Neuritis: Demyelinating

  1. Symptoms (4)
A
  1. Blurred Central Vision
  2. Loss of VF
  3. Eye Pain
    a. May precede or occur concurrently w/VA Loss and EXACERBATED by EYE MOVEMENT!
  4. Positive Visual Phenomenon
    a. Flashing black squares; Photopsia; Showers of SPARKS
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4
Q

Optic Neuritis: Demyelinating

  1. Uhthoff’s Sign
  2. Lhermitte’s Sign
A
  1. Transient Visual blurring during exercise, hot bath, or emotional stress
  2. Sudden, Transient, Electric like shocks extending down the spine triggered by flexing the head forward
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5
Q

Optic Neuritis: Demyelinating: Signs

  1. What are they?
A
  1. Reduced VA (Mild reduction to NLP)
  2. Reduced CS
  3. Abnormal CV
  4. VF Loss (Mild to severe; Central/Paracentral Scotoma (90%); Any type of VF Defect)
  5. RAPD
  6. Reduced Brightness Sensation
  7. Optic Disc (Edema (Papillitis); NL (Retrobulbar); Pallor (Old/Chronic, Diffuse, Localized, TEMPORAL)
  8. Rare vitritis overlying optic disc
  9. Retinal Vein Sheathing
  10. Abnormal VEP
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6
Q

Optic Neuritis: Demyelinating: Optic Disc Edema

  1. Acute Optic Neuritis
  2. Papilledema
  3. AION
A
  1. Slight or markedly blurred discs; Rare Peripapillary Hemes
  2. Severe “Choked Disc”
  3. Multiple Disc/Peripapillary Hemes
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7
Q

Optic Neuritis: Demyelinating

  1. In cases where cellular vitritis RxN is extensive, etiologies other than MS should be considered…
A
  1. Sarcoidosis; TB; Syphilis; Lyme Dz; SLE; Polyarteritis Nodosa; Toxoplasmosis; AIDS (Cryptococcus; CMV; HSV); Aspergillosis; Toxocariasis; Histoplasmosis; Cancer
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8
Q

Optic Neuritis: Demyelinating

  1. Serologic and CSF: Not needed in ACUTE OPTIC NEURITIS UNLESS what?
  2. What is important though?
A
  1. Hx or exam suggest they have a systemic or local infection or inflammation or pt’s course does not follow that of typical optic neuritis
  2. MRI
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9
Q

Optic Neuritis: Demyelinating: Tx

  1. Optic Neuritis Tx Trial
    a. DO NOT TREAT with what?
  2. What should be done?
  3. No treatment group?
A
  1. a. DONT TREAT w/ORAL PREDNISOLONE ALONE! (Increased recurrence rate; Did not speed recovery, did not improve VA)
  2. IV Methylprednisolone followed by Oral Prednisolone (Reduced rate of developing MS during first 2 yrs)…Speeds visual recovery by 2-3 wks.
  3. Visual function @1 yr = Treatment Group
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10
Q

Optic Neuritis: Demyelinating

  1. Visual Prognosis?
  2. Residual Deficits
A
  1. GOOD; Natural Hx. (Worsens over several days to 2 wks, then improves).
  2. Reduced VA; Reduced CV; RAPD
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11
Q

Optic Neuritis: Demyelinating: MS

  1. Cause?
  2. Tx?
A
  1. AI Dz that affects the Brain and Spinal Cord;
  2. No cure. (Interferon,among other things)

(Optic Neuritis is the presenting sign of MS in 20% of CASES)!!

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12
Q

Optic Neuritis: Demyelinating: NMO (Neuromyelitis Optica)

  1. AKA?
  2. What is it?
  3. Monophasic?
  4. Relapsing?
  5. Features?
  6. Dx CRITERIA?
  7. Tx?
A
  1. Devic’s Dz
  2. Idiopathic Inflammatory demyelinating Dz of the CNS characterized by attacks of ON and Myelitis
  3. 10%
  4. 90%
  5. Mean Age: 30; (Females); AA, Japanese, Hispanics, Pacific Islanders;

**ACUTE SEVERE RBON (Have to differentiate from MS);

1/3 die of respiratory FAILURE

  1. OPTIC NEURITIS, ACUTE MYELITIS

a. 2 of 3 CHARACTERISTICS
i. Dz-onset brain MRI non-diagnostic x MS

ii. MRI Spinal cord lesions extending over 3+ vertebral segments
iii. NMO-IgG Seropositive
7. Immunosuppressants

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13
Q

Optic Neuritis: Bacterial & Viral

  1. Parainfectious Optic Neuritis:
    a. Occurs after how long after an infection?
    b. Usually seen in whom?
    c. B/L or U/L more often?
    d. NL or Edematous Optic Disc?
    e. Tx?
    f. OPTIC NEURITIS may occur after what?
A
  1. a. ~1-3 wks
    b. Children
    c. B/L > U/L
    d. Either
    e. none…get good visual recovery after usually.
    f. After VACCINATION
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14
Q

Optic Neuritis: Sarcoidosis

  1. What is it/What does it look like?
  2. Type of ON? (Anterior or Retrobulbar)?
  3. Vitritis seen?
  4. Pain w/ON?
  5. Extremely sensitive to what drug?
  6. RAPID RECOVERY W/WHAT?
A
  1. Granulomatous inflammation of the ON (White, Lumpy Appearance)
  2. Either
  3. Yes
  4. YES!
  5. to STEROIDS
  6. w/Tx, but worsens w/Steroid Taper
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15
Q

Optic Neuritis: Syphilis

  1. U/L or B/L?
  2. Ant or RBON?
  3. Vitritis seen?
  4. COMMON in PTS with WHAT DZ?
  5. Dx? (2)
  6. Tx?
  7. May cause 2 types of neuritis…?
A
  1. Either
  2. Either
  3. YES
  4. HIV
  5. Serology; CSF Assays
  6. IV PCN
  7. Neuroretinitis or Perineuritis
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16
Q

Optic Neuritis: HIV+/AIDS

  1. Big thing to know?
  2. Ant or RBON?
    a. What are some examples of infectious agents?
  3. What else may also occur?
A
  1. Infectious agents that don’t normally cause Optic Neuritis will do so when immunocompromised from Drugs or Dz
  2. Either
    a. Cryptococcus, CMV, HSV, Syphilis, TB, Fungus, Toxoplasmosis
  3. Neuroretinitis/Optic PErineuritis may occur
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17
Q

Optic Neuritis: SLE & Other AI Optic Neuropathies

  1. Ant or RBON?
  2. VA loss is quick or slow?
  3. PAthogenesis?
  4. Tx?
  5. AUTOIMMUNE OPTIC NEURITIS
    a. Serologic Evidence of what?
A
  1. Either
  2. SLOW, PRogressive
  3. ISCHEMIA! (Not inflammation)
  4. IV or Oral Corticosteroids
  5. a. of VASCULITIS (+ANA) w/No signs of systemic involvement other than Optic Neuropathy
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18
Q

Optic Neuritis: SLE & Other AI Optic Neuropathies

  1. Sjogren’s Syndrome
    a. AI Dz of Mononuclear infiltration…destroys salivary and lacrimal glands leading to what 2 THINGS?
  2. Types?
  3. What is a KNOWN COMPLICATION?
  4. Dx Criteria?
A
  1. a. Xerostomia and Xerophthalmia
  2. Primary SS and Secondary SS (CT Dz associated)
  3. OPTIC NEURITIS
  4. 4/6 plus serum + or Bx +
    a. Xerostomia x 3 mos

b. Schirmer

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19
Q

Optic Neuritis: Lyme Dz

  1. Ant or RBON?
  2. Infection of what?
    a. Done via what?
  3. Serology?
  4. Tx? (3)
  5. Can cause what retinal inflammation?
A
  1. Either
  2. Spirochete Inf
    a. Borrelia Burgdoferi and transmitted via tick BITE!
  3. Lyme Titer
  4. Amoxicilin; Cefuroxime; or Doxycycline
  5. Neuroretinitis
20
Q

Optic Neuritis: Lyme Dz

  1. Stage 1 (Localized)
  2. Stage 2 (Disseminated)
  3. Stage 3 (LATE; Chronic)
A
  1. Erythema Migrans; Myalgia, Arthralgia; HA; Flulike symptoms.
  2. 2ndary annular lesions; Brief arthritis attacks; Joint pain; Meningitis; Bell Palsy; Cranial Neuritis; Malaise, Fatigue
  3. Prolonged arthritis attacks, Chronic arthritis; Encephalopathy, Polyneuropathy, Leukoencephalitis
21
Q

Optic Neuritis: Sinus Dz

  1. Spread of INF from PARANASAL SINUSES to what?
  2. Common?
  3. Main Infection that causes it?
  4. Tx?
  5. EVEN when SINUS Dz is PRESENT in the SETTING of OPTIC NEURITIS, ONE MUST BE WARY of ATTRIBUTING the OPTIC NEURITIS to THIS CAUSE!!!!
A
  1. to Optic Nerve
  2. RARE!!!
  3. Aspergillosis, other Fungal INF
  4. eradicate Sinus INF
22
Q

Optic Neuritis: Children

  1. Most often Ant or RBON?
  2. U/L or B/L
  3. Usually occurs after how long after VIRAL INF?
  4. Less often ASSOCIATED w/what?
  5. Evaluate all PTs UNDER 15 YRS with what tests?
  6. Tx?
A
  1. ANTERIOR
  2. B/L usually
  3. 1-2 after
  4. MS development
  5. MRI and LP
  6. IV Methylprednisolone (1-2 mg/kg/day x 3-5 days)…NO CORTICOSTEROID TAPER NEEDED!
23
Q

Neuroretinitis

  1. Acute/Chronic U/L or B/L Visual LOSS associated with what?
  2. Seen in whom most often?
A
  1. ACUTE; U/L. Exudative MACULOPATHY that has HARD EXUDATES ARRANGED in a STAR FIGURE AROUND THE FOVEA!
  2. Children/Young Adults (antecedent Viral INF ~50%).
    * Age 20-30’s. but any age.
24
Q

Neuroretinitis: Signs and Symptoms

  1. Pain?
  2. VA?
  3. AC?
  4. Disc edema resolved in how many weeks?
  5. Macular scar resolves in how long?
  6. Px?
A
  1. Usually PAINLESS (Aching sensation behind affected eye; Mild discomfort w/Eye movement)
  2. 20/20 to NLP..so anything really.
    * CV affected; CECOCENTRAL SCOTOMA; RELATIVE APD; Disc Edema (Mild to Severe); Vitritis
  3. CELLS and FLARE
  4. 6-8 wks
  5. 6-12 mos.
  6. GOOD. (may have some blured vision and Metamorphopsia)
25
Q

Neuroretinitis: Leber’s Idiopathic Stellate Neuroretinitis

  1. Tx?
A
  1. Pts w/Viral or presumed idiopathic neuroretinitis may or may not need Tx.
26
Q

Neuroretinitis: Infectious Dz

  1. MAIN one?
  2. Tx?
A
  1. CAT SCRATCH DZ (Bartonella HENSELAE)

or Syphilis, Lyme, Toxoplasmosis, Toxocariasis, Histoplasmosis

  1. Treat underlying cause. ABs x INF. Corticosteroids.
27
Q

Optic Perineuritis

  1. Only what of the Optic Nerve is INFLAMED?
    a. Pathologically?
  2. Sx? (3 things)
  3. Etiology (3)
  4. MRI Shows what?
  5. Tx?
A
  1. only the PERIPHERY
    a. Pia and Arachnoid are infiltrated w/PMN Leukocytes surrounding the ON and Subarachnoid Space
  2. a. B/L Disc Edema
    b. Decreased VA
    c. Eye Pain
  3. a. Syphilis
    b. Sarcoidosis
    c. Viral Encephalitides
  4. Circumferential enhancement around Optic Nerve
  5. Corticosteroids.
    * Recurrence can occur after stopping Tx
28
Q

Traumatic Optic Neuropathy

  1. DIRECT INJURY
    a. What is it?
  2. INDIRECT INJURY
    a. Injuries that occur when what happens?
    b. Most COMMON SITE of indirect injury is what?
A
  1. a. External Object Penetrates the tissues to impact the ON
  2. a. force of collision is imparted into the skull and this energy is absorbed by the ON
    b. is the Bony Optic Canal (INTRACANALICULAR INJURY)
29
Q

Traumatic Optic Neuropathy

  1. Causes?
  2. Open injury creates a RISK for what?
  3. PALPATE ORBITAL RIM for what?
A
  1. MVA and Bike Accidents; FALLS
  2. for TETANUS
  3. CREPITUS
30
Q

Traumatic Optic Neuropathy

  1. Orbital Heme can create what kind of Optic Neuropathy?
    a. SCH 360: MUST GET WHAT STAT?
  2. Tx?
A
  1. a Compressive Optic Neuropathy
    a. NEUROIMAGING STAT
  2. CANTHOTOMY and Cantholysis to permit expansion of the orbital contents; Orbital Decompression
31
Q

Nutritional Optic Neuropathy

  1. Common?
  2. Dx of what?
  3. What is the rule about deficiencies?
  4. Seen when and in whom?
  5. W/the Exception of what VITAMIN, no specific nutrient deficiency has been conclusively proved to cause Optic Neuropathy in humans…
A
  1. RARE
  2. of Exclusion
  3. Multiple deficiencies
  4. War and Famine and in Economically Disadvantaged
  5. VITAMIN B 12
32
Q

Nutritional Optic Neuropathy

  1. Characteristics
  2. Vitamin B12 Deficiency
    a. Serology?
    b. Tx?
A
  1. Painless; B/L but asymmetric;
    Central/Centrocecal Scotoma; ON NL, Slightly Hyperemic, or edematous –> Optic Atrophy
  2. Meat and Dairy Products; HIGH RISK (Vegans, impaired intestinal absorption; GI Sx)
    a. VIT B12, Folate, Homocysteine, and Methylmalonic Acid
    b. IV HYDROXOCOBALAMIN
33
Q

Toxic Optic Neuropathy: METHANOL

  1. Found in what?
  2. VICTIM USUALLY MISTAKES or SUBSTITUTES IT FOR WHAT?
  3. VISION?
  4. Signs/Sx?
  5. SERUM METHANOL LEVELS?
  6. Tx?
A
  1. Antifreeze, Solvents, Fuels
  2. ETOH (smell and taste is similar)
  3. TOTAL BLINDNESS!
  4. N/V; Respiratory Distress –> Coma and Death; HA, VA Loss; Abdominal Pain, Confusion, Weakness, Drowsiness
  5. Metabolic ACIDOSIS; >20 mg/dL
    * MRI: HIGH T2 SIGNAL in BASAL GANGLIA and Parieto-Occipital White Matter
  6. ETOH to interfere w/Metabolism; Hemodialysis; Bicarbonate x Metabolic Acidosis
34
Q

Toxic Optic Neuropathy: ETHYLENE Glycol

  1. Found in WHAT?
  2. When is it taken?
    a. Survivors have PERMANENT deficits to what?
  3. Sx?
  4. Signs?
  5. Tx?
A
  1. AUTOMOBILE ANTIFREEZE
  2. Accidentally or Suicide Attempt
    a. Residual Neurologic and Ophthalmologic Deficits
  3. N/V; Abdominal Pain; Stupor/Coma; Cardiac Failure; Renal Failure
  4. Papilledema; Optic Disc: NL to Optic Atrophy; Nystagmus; Ophthalmoplegia; Metabolic Acidosis; Large Anion Gap
  5. ETOH; Hemodialysis; Bicarbonate x Acidosis
35
Q

Toxic Optic Neuropathy: ETHAMBUTOL

  1. DRUG for WHAT Dz?
    a. Dose related Toxicity?
    b. SHOULD BE TAKING LESS THAN WHAT?
    c. D/C when?
    d. Toxicity usually develops after how long of Tx?
  2. HIGH RISK PTs?
  3. Signs?
  4. Tx?
A
  1. TB
    a. 25 mg/kg/d loading dose 1MO (KNOW)!
    c. At 8 wks if TB susceptible to INH or at 6 mos.
    d. After 2 mos. of Tx. (usually b/w 3-6 mos or as late as 1 yr)
  2. Old peeps, Low body wt; Renal impairment; DM; Liver Issues; Poor nutrition; Tobacco use; ETOH Use
  3. B/L, SYMMETRIC VA LOSS; Dyschromatopsia; VF Loss: (Central, Peripheral constriction, Bitemporal), Optic Atrophy
  4. D/c ETHAMBUTOL; Vision may improve slowly over several months (not necessarily reversible)
36
Q

Toxic Optic Neuropathy: Sildenafil Citrate

  1. Type of Inhibitor?
    a. Main DRUG?
  2. OCULAR issues?
  3. Can cause WHAT?
  4. Can also occur with what other drugs?
A
  1. Phosphodiesterase Type 5 inhibitor x ED
    a. VIAGRA
  2. Transient alteration of color perception (SEEING BLUE) (Weak effect on Type 6)
  3. NAION!
  4. Cialis and Levitra
37
Q

Toxic Optic Neuropathy: AMIODARONE

  1. Use?
  2. Ocular issues?
  3. Signs?
  4. Tx?
A
  1. Atrial and Ventricular Tachyarrhythmias unresponsive to other agents
  2. CORNEAL VERTICILLATA (dose and duration dependent; Resolve w/Tx cessation in 3-4 mos). Also, OPTIC NEUROPATHY!
  3. Insidious, B/L VA LOSS; B/L Disc EDEMA (Resolves several mos after drug discontinuation)
  4. D/C it in all Pts w/Optic Neuropathy
38
Q

Toxic Optic Neuropathy: Tobacco-Alcohol AMBLYOPIA (TAA)

  1. Mechanism?
  2. Who is affected?
  3. Sx?
  4. Tx?
A
  1. Unknown…belief it has to do w/Malnutrition and Vit B12 deficiency may play a role.
  2. Middle-aged/Old peeps; Pipe/Cigar Smokers/ High Alcohol Consumption
  3. Painless; Slowly progressive; VA Loss; Dyschromatopsia; CENTROCECAL SCOTOMA; Optic Disc NL –> PALLOR
  4. stop smoking/ETOH
    Healthy diet. MVI (?)

IM Hydroxocobalamin

39
Q

Hereditary Optic Neuropathy: LEBER’S (LHON)

  1. Affects whom more?
  2. Age of Onset?
  3. Sx?
A
  1. MALES >Females
  2. 15-25 yrs
  3. Acute or subacute; Painless, Central VA loss in ONE EYE. Second eye involved wks to MONTHS LATER. (VA: s Sx.

Optic Disc (Hyperemia); Dilated/Tortuous vasculature

Retinal and Disc HEMES

MAcular EDEMA (EXUDATES)

40
Q

Hereditary Optic Neuropathy: (LHON) MITOCHONDRIAL DNA

  1. Inheritance Pattern?
  2. Screening for what?
  3. Tx?
A
  1. MATERNAL (all offspring of mother carrier will inherit the trait and ONLY MOM can pass on the trait)
  2. LHON (Primary mutations…90% of cases)
  3. AVOID Tobacco, Excessive ETOH, Environmental Toxins
    * Vision Rehab; Genetic Counseling; ECGx Cardiac Conduction Abnormalities
41
Q

Hereditary Optic Neuropathy: DOMINANT OPTIC ATROPHY

  1. AKA?
  2. MOST COMMON WHAT?
  3. ONSET?
  4. VA?
  5. Sx?
  6. Signs?
  7. Etiology?
  8. Tx?
A
  1. Kjer’s Optic Atrophy (AD)
  2. HEREDITARY OPTIC NEUROPATHY
  3. 1st Decade
  4. 20/20-20/60; (<20/70 less common)
  5. Mild, Slow, Insidious Progressive VA loss; Dyschromatopsia (Tritanopia) VF LOSS (Central, Paracentral or Cecocentral)
  6. Optic Atrophy; Nefative FLR; Mild Macular Pigmentary Changes; Arterial Attenuation
  7. 60 different mutations, deletions and Insertions of OPA1 GENE
  8. Vision REHAB
42
Q

Hereditary Optic Neuropathy: INHERITED ATAXIA

  1. These represent a group of chronic progressive neurodegenerative conditions that involve what?
  2. WHAT IS NOT UNCOMMON AMONG PTs w/HEREDITARY ATAXIAS!?
  3. Onset?
  4. Sx?
A
  1. involving the CEREBELLUM and its CONNECTIONS
  2. OPTIC ATROPHY
  3. 8-15 yrs but
43
Q

Hereditary Optic Neuropathy: SPINOCEREBELLAR ATAXIA (SCA)

  1. AUTOSOMAL WHAT?
  2. MOST COMMON WHAT?
  3. Vision loss?
  4. VF?
  5. Optic Atrophy?
A
  1. AD (SCA1 or SCA3)
  2. HEREDITARY ATAXIA!!
  3. Mild
  4. Constricted
  5. Diffuse Optic Atrophy
44
Q

Hereditary Optic Neuropathy: Charcot-Marie-Tooth (CMT)

  1. Heredofamilial d/o w/PROGRESSIVE WHAT?
  2. Onset?
  3. Signs/Sx?
  4. MAIN THINGS TO KNOW? (VISION and OPTIC NERVE)
A
  1. Weakness and Atrophy
  2. 2-15 yrs
  3. Pes cavus; Foot deformities; Scoliosis; Slowly progressive weakness and wasting of the feet and legs, THEN HANDS
  4. PROGRESSIVE, B/L BLINDNESS and OPTIC ATROPHY
45
Q

Hereditary Optic Neuropathy: MYOTONIC DYSTROPHY

  1. Autosomal what?
  2. CHARACTERISTICS?
  3. Other FEATURES?
A
  1. AD
  2. Progressive Myopathy; CHRISTMAS TREE CATARACTS!; DM; Cardiomyopathy w/conduction defects
  3. Frontal baldness, bifacial weakness, pigmentary retinopathy

PTOSIS, OPTIC ATROPHY, OCULAR HYPOTONIA, EXTERNAL OPHTHALMOPLEGIA