Fall 2014: Week 9: Neuro: Optic Neuropathies

Question 1

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?

Answer 1

1. a. Papillitis, Anterior Optic Neuritis
   b. Retrobulbar Optic Neuritis; Retrobulbar Neuritis
2. Monosymptomatic or Idiopathic

Question 2

Optic Neuritis: Deymelinating

1. ALMOST ALWAYS OCCURS how?
2. MOST COMMON Type of OPTIC NEURITIS?

Answer 2

1. as an Isolated Phenomenon or in Pts who have or will develop MS
2. Acute demyelinating Optic Neuritis

Question 3

Optic Neuritis: Demyelinating

1. Symptoms (4)

Answer 3

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

Question 4

Optic Neuritis: Demyelinating

1. Uhthoff’s Sign
2. Lhermitte’s Sign

Answer 4

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

Question 5

Optic Neuritis: Demyelinating: Signs

1. What are they?

Answer 5

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

Question 6

Optic Neuritis: Demyelinating: Optic Disc Edema

1. Acute Optic Neuritis
2. Papilledema
3. AION

Answer 6

1. Slight or markedly blurred discs; Rare Peripapillary Hemes
2. Severe “Choked Disc”
3. Multiple Disc/Peripapillary Hemes

Question 7

Optic Neuritis: Demyelinating

1. In cases where cellular vitritis RxN is extensive, etiologies other than MS should be considered…

Answer 7

1. Sarcoidosis; TB; Syphilis; Lyme Dz; SLE; Polyarteritis Nodosa; Toxoplasmosis; AIDS (Cryptococcus; CMV; HSV); Aspergillosis; Toxocariasis; Histoplasmosis; Cancer

Question 8

Optic Neuritis: Demyelinating

1. Serologic and CSF: Not needed in ACUTE OPTIC NEURITIS UNLESS what?
2. What is important though?

Answer 8

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

Question 9

Optic Neuritis: Demyelinating: Tx

1. Optic Neuritis Tx Trial
   a. DO NOT TREAT with what?
2. What should be done?
3. No treatment group?

Answer 9

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

Question 10

Optic Neuritis: Demyelinating

1. Visual Prognosis?
2. Residual Deficits

Answer 10

1. GOOD; Natural Hx. (Worsens over several days to 2 wks, then improves).
2. Reduced VA; Reduced CV; RAPD

Question 11

Optic Neuritis: Demyelinating: MS

1. Cause?
2. Tx?

Answer 11

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)!!

Question 12

Optic Neuritis: Demyelinating: NMO (Neuromyelitis Optica)

1. AKA?
2. What is it?
3. Monophasic?
4. Relapsing?
5. Features?
6. Dx CRITERIA?
7. Tx?

Answer 12

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

6. 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

Question 13

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?

Answer 13

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

Question 14

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?

Answer 14

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

Question 15

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…?

Answer 15

1. Either
2. Either
3. YES
4. HIV
5. Serology; CSF Assays
6. IV PCN
7. Neuroretinitis or Perineuritis

Question 16

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?

Answer 16

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

Question 17

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?

Answer 17

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

Question 18

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?

Answer 18

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

Question 19

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?

Answer 19

1. Either
2. Spirochete Inf
   a. Borrelia Burgdoferi and transmitted via tick BITE!
3. Lyme Titer
4. Amoxicilin; Cefuroxime; or Doxycycline
5. Neuroretinitis

Question 20

Optic Neuritis: Lyme Dz

1. Stage 1 (Localized)
2. Stage 2 (Disseminated)
3. Stage 3 (LATE; Chronic)

Answer 20

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

Question 21

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!!!!

Answer 21

1. to Optic Nerve
2. RARE!!!
3. Aspergillosis, other Fungal INF
4. eradicate Sinus INF

Question 22

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?

Answer 22

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!

Question 23

Neuroretinitis

1. Acute/Chronic U/L or B/L Visual LOSS associated with what?
2. Seen in whom most often?

Answer 23

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.

Question 24

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?

Answer 24

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)

Question 25

Neuroretinitis: Leber's Idiopathic Stellate Neuroretinitis 1. Tx?

Answer 25

1. Pts w/Viral or presumed idiopathic neuroretinitis may or may not need Tx.

Question 26

Neuroretinitis: Infectious Dz 1. MAIN one? 2. Tx?

Answer 26

1. CAT SCRATCH DZ (Bartonella HENSELAE) or Syphilis, Lyme, Toxoplasmosis, Toxocariasis, Histoplasmosis 2. Treat underlying cause. ABs x INF. Corticosteroids.

Question 27

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?

Answer 27

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

Question 28

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?

Answer 28

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)

Question 29

Traumatic Optic Neuropathy 1. Causes? 2. Open injury creates a RISK for what? 3. PALPATE ORBITAL RIM for what?

Answer 29

1. MVA and Bike Accidents; FALLS 2. for TETANUS 3. CREPITUS

Question 30

Traumatic Optic Neuropathy 1. Orbital Heme can create what kind of Optic Neuropathy? a. SCH 360: MUST GET WHAT STAT? 2. Tx?

Answer 30

1. a Compressive Optic Neuropathy a. NEUROIMAGING STAT 2. CANTHOTOMY and Cantholysis to permit expansion of the orbital contents; Orbital Decompression

Question 31

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...

Answer 31

1. RARE 2. of Exclusion 3. Multiple deficiencies 4. War and Famine and in Economically Disadvantaged 5. VITAMIN B 12

Question 32

Nutritional Optic Neuropathy 1. Characteristics 2. Vitamin B12 Deficiency a. Serology? b. Tx?

Answer 32

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

Question 33

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?

Answer 33

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

Question 34

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?

Answer 34

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

Question 35

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?

Answer 35

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)

Question 36

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?

Answer 36

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

Question 37

Toxic Optic Neuropathy: AMIODARONE 1. Use? 2. Ocular issues? 3. Signs? 4. Tx?

Answer 37

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

Question 38

Toxic Optic Neuropathy: Tobacco-Alcohol AMBLYOPIA (TAA) 1. Mechanism? 2. Who is affected? 3. Sx? 4. Tx?

Answer 38

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

Question 39

Hereditary Optic Neuropathy: LEBER'S (LHON) 1. Affects whom more? 2. Age of Onset? 3. Sx?

Answer 39

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)

Question 40

Hereditary Optic Neuropathy: (LHON) MITOCHONDRIAL DNA 1. Inheritance Pattern? 2. Screening for what? 3. Tx?

Answer 40

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

Question 41

Hereditary Optic Neuropathy: DOMINANT OPTIC ATROPHY 1. AKA? 2. MOST COMMON WHAT? 3. ONSET? 4. VA? 5. Sx? 6. Signs? 7. Etiology? 8. Tx?

Answer 41

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

Question 42

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?

Answer 42

1. involving the CEREBELLUM and its CONNECTIONS 2. OPTIC ATROPHY 3. 8-15 yrs but

Question 43

Hereditary Optic Neuropathy: SPINOCEREBELLAR ATAXIA (SCA) 1. AUTOSOMAL WHAT? 2. MOST COMMON WHAT? 3. Vision loss? 4. VF? 5. Optic Atrophy?

Answer 43

1. AD (SCA1 or SCA3) 2. HEREDITARY ATAXIA!! 3. Mild 4. Constricted 5. Diffuse Optic Atrophy

Question 44

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)

Answer 44

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

Question 45

Hereditary Optic Neuropathy: MYOTONIC DYSTROPHY 1. Autosomal what? 2. CHARACTERISTICS? 3. Other FEATURES?

Answer 45

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