Fall 2014: Week 9: Neuro: Optic Neuropathies Flashcards
Optic Neuritis:
- Define
a. Swollen Optic Disc (2 types)
b. NL Optic Disc (2 types) - If the Pt does not have signs of MS or other systemic Dz, the Optic Neuritis is referred to as what?
- a. Papillitis, Anterior Optic Neuritis
b. Retrobulbar Optic Neuritis; Retrobulbar Neuritis - Monosymptomatic or Idiopathic
Optic Neuritis: Deymelinating
- ALMOST ALWAYS OCCURS how?
- MOST COMMON Type of OPTIC NEURITIS?
- as an Isolated Phenomenon or in Pts who have or will develop MS
- Acute demyelinating Optic Neuritis
Optic Neuritis: Demyelinating
- Symptoms (4)
- Blurred Central Vision
- Loss of VF
- Eye Pain
a. May precede or occur concurrently w/VA Loss and EXACERBATED by EYE MOVEMENT! - Positive Visual Phenomenon
a. Flashing black squares; Photopsia; Showers of SPARKS
Optic Neuritis: Demyelinating
- Uhthoff’s Sign
- Lhermitte’s Sign
- Transient Visual blurring during exercise, hot bath, or emotional stress
- Sudden, Transient, Electric like shocks extending down the spine triggered by flexing the head forward
Optic Neuritis: Demyelinating: Signs
- What are they?
- Reduced VA (Mild reduction to NLP)
- Reduced CS
- Abnormal CV
- VF Loss (Mild to severe; Central/Paracentral Scotoma (90%); Any type of VF Defect)
- RAPD
- Reduced Brightness Sensation
- Optic Disc (Edema (Papillitis); NL (Retrobulbar); Pallor (Old/Chronic, Diffuse, Localized, TEMPORAL)
- Rare vitritis overlying optic disc
- Retinal Vein Sheathing
- Abnormal VEP
Optic Neuritis: Demyelinating: Optic Disc Edema
- Acute Optic Neuritis
- Papilledema
- AION
- Slight or markedly blurred discs; Rare Peripapillary Hemes
- Severe “Choked Disc”
- Multiple Disc/Peripapillary Hemes
Optic Neuritis: Demyelinating
- In cases where cellular vitritis RxN is extensive, etiologies other than MS should be considered…
- Sarcoidosis; TB; Syphilis; Lyme Dz; SLE; Polyarteritis Nodosa; Toxoplasmosis; AIDS (Cryptococcus; CMV; HSV); Aspergillosis; Toxocariasis; Histoplasmosis; Cancer
Optic Neuritis: Demyelinating
- Serologic and CSF: Not needed in ACUTE OPTIC NEURITIS UNLESS what?
- What is important though?
- 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
- MRI
Optic Neuritis: Demyelinating: Tx
- Optic Neuritis Tx Trial
a. DO NOT TREAT with what? - What should be done?
- No treatment group?
- a. DONT TREAT w/ORAL PREDNISOLONE ALONE! (Increased recurrence rate; Did not speed recovery, did not improve VA)
- IV Methylprednisolone followed by Oral Prednisolone (Reduced rate of developing MS during first 2 yrs)…Speeds visual recovery by 2-3 wks.
- Visual function @1 yr = Treatment Group
Optic Neuritis: Demyelinating
- Visual Prognosis?
- Residual Deficits
- GOOD; Natural Hx. (Worsens over several days to 2 wks, then improves).
- Reduced VA; Reduced CV; RAPD
Optic Neuritis: Demyelinating: MS
- Cause?
- Tx?
- AI Dz that affects the Brain and Spinal Cord;
- No cure. (Interferon,among other things)
(Optic Neuritis is the presenting sign of MS in 20% of CASES)!!
Optic Neuritis: Demyelinating: NMO (Neuromyelitis Optica)
- AKA?
- What is it?
- Monophasic?
- Relapsing?
- Features?
- Dx CRITERIA?
- Tx?
- Devic’s Dz
- Idiopathic Inflammatory demyelinating Dz of the CNS characterized by attacks of ON and Myelitis
- 10%
- 90%
- Mean Age: 30; (Females); AA, Japanese, Hispanics, Pacific Islanders;
**ACUTE SEVERE RBON (Have to differentiate from MS);
1/3 die of respiratory FAILURE
- 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
Optic Neuritis: Bacterial & Viral
- 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. ~1-3 wks
b. Children
c. B/L > U/L
d. Either
e. none…get good visual recovery after usually.
f. After VACCINATION
Optic Neuritis: Sarcoidosis
- What is it/What does it look like?
- Type of ON? (Anterior or Retrobulbar)?
- Vitritis seen?
- Pain w/ON?
- Extremely sensitive to what drug?
- RAPID RECOVERY W/WHAT?
- Granulomatous inflammation of the ON (White, Lumpy Appearance)
- Either
- Yes
- YES!
- to STEROIDS
- w/Tx, but worsens w/Steroid Taper
Optic Neuritis: Syphilis
- U/L or B/L?
- Ant or RBON?
- Vitritis seen?
- COMMON in PTS with WHAT DZ?
- Dx? (2)
- Tx?
- May cause 2 types of neuritis…?
- Either
- Either
- YES
- HIV
- Serology; CSF Assays
- IV PCN
- Neuroretinitis or Perineuritis
Optic Neuritis: HIV+/AIDS
- Big thing to know?
- Ant or RBON?
a. What are some examples of infectious agents? - What else may also occur?
- Infectious agents that don’t normally cause Optic Neuritis will do so when immunocompromised from Drugs or Dz
- Either
a. Cryptococcus, CMV, HSV, Syphilis, TB, Fungus, Toxoplasmosis - Neuroretinitis/Optic PErineuritis may occur
Optic Neuritis: SLE & Other AI Optic Neuropathies
- Ant or RBON?
- VA loss is quick or slow?
- PAthogenesis?
- Tx?
- AUTOIMMUNE OPTIC NEURITIS
a. Serologic Evidence of what?
- Either
- SLOW, PRogressive
- ISCHEMIA! (Not inflammation)
- IV or Oral Corticosteroids
- a. of VASCULITIS (+ANA) w/No signs of systemic involvement other than Optic Neuropathy
Optic Neuritis: SLE & Other AI Optic Neuropathies
- Sjogren’s Syndrome
a. AI Dz of Mononuclear infiltration…destroys salivary and lacrimal glands leading to what 2 THINGS? - Types?
- What is a KNOWN COMPLICATION?
- Dx Criteria?
- a. Xerostomia and Xerophthalmia
- Primary SS and Secondary SS (CT Dz associated)
- OPTIC NEURITIS
- 4/6 plus serum + or Bx +
a. Xerostomia x 3 mos
b. Schirmer
Optic Neuritis: Lyme Dz
- Ant or RBON?
- Infection of what?
a. Done via what? - Serology?
- Tx? (3)
- Can cause what retinal inflammation?
- Either
- Spirochete Inf
a. Borrelia Burgdoferi and transmitted via tick BITE! - Lyme Titer
- Amoxicilin; Cefuroxime; or Doxycycline
- Neuroretinitis
Optic Neuritis: Lyme Dz
- Stage 1 (Localized)
- Stage 2 (Disseminated)
- Stage 3 (LATE; Chronic)
- Erythema Migrans; Myalgia, Arthralgia; HA; Flulike symptoms.
- 2ndary annular lesions; Brief arthritis attacks; Joint pain; Meningitis; Bell Palsy; Cranial Neuritis; Malaise, Fatigue
- Prolonged arthritis attacks, Chronic arthritis; Encephalopathy, Polyneuropathy, Leukoencephalitis
Optic Neuritis: Sinus Dz
- Spread of INF from PARANASAL SINUSES to what?
- Common?
- Main Infection that causes it?
- Tx?
- EVEN when SINUS Dz is PRESENT in the SETTING of OPTIC NEURITIS, ONE MUST BE WARY of ATTRIBUTING the OPTIC NEURITIS to THIS CAUSE!!!!
- to Optic Nerve
- RARE!!!
- Aspergillosis, other Fungal INF
- eradicate Sinus INF
Optic Neuritis: Children
- Most often Ant or RBON?
- U/L or B/L
- Usually occurs after how long after VIRAL INF?
- Less often ASSOCIATED w/what?
- Evaluate all PTs UNDER 15 YRS with what tests?
- Tx?
- ANTERIOR
- B/L usually
- 1-2 after
- MS development
- MRI and LP
- IV Methylprednisolone (1-2 mg/kg/day x 3-5 days)…NO CORTICOSTEROID TAPER NEEDED!
Neuroretinitis
- Acute/Chronic U/L or B/L Visual LOSS associated with what?
- Seen in whom most often?
- ACUTE; U/L. Exudative MACULOPATHY that has HARD EXUDATES ARRANGED in a STAR FIGURE AROUND THE FOVEA!
- Children/Young Adults (antecedent Viral INF ~50%).
* Age 20-30’s. but any age.
Neuroretinitis: Signs and Symptoms
- Pain?
- VA?
- AC?
- Disc edema resolved in how many weeks?
- Macular scar resolves in how long?
- Px?
- Usually PAINLESS (Aching sensation behind affected eye; Mild discomfort w/Eye movement)
- 20/20 to NLP..so anything really.
* CV affected; CECOCENTRAL SCOTOMA; RELATIVE APD; Disc Edema (Mild to Severe); Vitritis - CELLS and FLARE
- 6-8 wks
- 6-12 mos.
- GOOD. (may have some blured vision and Metamorphopsia)
Neuroretinitis: Leber’s Idiopathic Stellate Neuroretinitis
- Tx?
- Pts w/Viral or presumed idiopathic neuroretinitis may or may not need Tx.
Neuroretinitis: Infectious Dz
- MAIN one?
- Tx?
- CAT SCRATCH DZ (Bartonella HENSELAE)
or Syphilis, Lyme, Toxoplasmosis, Toxocariasis, Histoplasmosis
- Treat underlying cause. ABs x INF. Corticosteroids.
Optic Perineuritis
- Only what of the Optic Nerve is INFLAMED?
a. Pathologically? - Sx? (3 things)
- Etiology (3)
- MRI Shows what?
- Tx?
- only the PERIPHERY
a. Pia and Arachnoid are infiltrated w/PMN Leukocytes surrounding the ON and Subarachnoid Space - a. B/L Disc Edema
b. Decreased VA
c. Eye Pain - a. Syphilis
b. Sarcoidosis
c. Viral Encephalitides - Circumferential enhancement around Optic Nerve
- Corticosteroids.
* Recurrence can occur after stopping Tx
Traumatic Optic Neuropathy
- DIRECT INJURY
a. What is it? - INDIRECT INJURY
a. Injuries that occur when what happens?
b. Most COMMON SITE of indirect injury is what?
- a. External Object Penetrates the tissues to impact the ON
- 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)
Traumatic Optic Neuropathy
- Causes?
- Open injury creates a RISK for what?
- PALPATE ORBITAL RIM for what?
- MVA and Bike Accidents; FALLS
- for TETANUS
- CREPITUS
Traumatic Optic Neuropathy
- Orbital Heme can create what kind of Optic Neuropathy?
a. SCH 360: MUST GET WHAT STAT? - Tx?
- a Compressive Optic Neuropathy
a. NEUROIMAGING STAT - CANTHOTOMY and Cantholysis to permit expansion of the orbital contents; Orbital Decompression
Nutritional Optic Neuropathy
- Common?
- Dx of what?
- What is the rule about deficiencies?
- Seen when and in whom?
- W/the Exception of what VITAMIN, no specific nutrient deficiency has been conclusively proved to cause Optic Neuropathy in humans…
- RARE
- of Exclusion
- Multiple deficiencies
- War and Famine and in Economically Disadvantaged
- VITAMIN B 12
Nutritional Optic Neuropathy
- Characteristics
- Vitamin B12 Deficiency
a. Serology?
b. Tx?
- Painless; B/L but asymmetric;
Central/Centrocecal Scotoma; ON NL, Slightly Hyperemic, or edematous –> Optic Atrophy - Meat and Dairy Products; HIGH RISK (Vegans, impaired intestinal absorption; GI Sx)
a. VIT B12, Folate, Homocysteine, and Methylmalonic Acid
b. IV HYDROXOCOBALAMIN
Toxic Optic Neuropathy: METHANOL
- Found in what?
- VICTIM USUALLY MISTAKES or SUBSTITUTES IT FOR WHAT?
- VISION?
- Signs/Sx?
- SERUM METHANOL LEVELS?
- Tx?
- Antifreeze, Solvents, Fuels
- ETOH (smell and taste is similar)
- TOTAL BLINDNESS!
- N/V; Respiratory Distress –> Coma and Death; HA, VA Loss; Abdominal Pain, Confusion, Weakness, Drowsiness
- Metabolic ACIDOSIS; >20 mg/dL
* MRI: HIGH T2 SIGNAL in BASAL GANGLIA and Parieto-Occipital White Matter - ETOH to interfere w/Metabolism; Hemodialysis; Bicarbonate x Metabolic Acidosis
Toxic Optic Neuropathy: ETHYLENE Glycol
- Found in WHAT?
- When is it taken?
a. Survivors have PERMANENT deficits to what? - Sx?
- Signs?
- Tx?
- AUTOMOBILE ANTIFREEZE
- Accidentally or Suicide Attempt
a. Residual Neurologic and Ophthalmologic Deficits - N/V; Abdominal Pain; Stupor/Coma; Cardiac Failure; Renal Failure
- Papilledema; Optic Disc: NL to Optic Atrophy; Nystagmus; Ophthalmoplegia; Metabolic Acidosis; Large Anion Gap
- ETOH; Hemodialysis; Bicarbonate x Acidosis
Toxic Optic Neuropathy: ETHAMBUTOL
- 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? - HIGH RISK PTs?
- Signs?
- Tx?
- 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) - Old peeps, Low body wt; Renal impairment; DM; Liver Issues; Poor nutrition; Tobacco use; ETOH Use
- B/L, SYMMETRIC VA LOSS; Dyschromatopsia; VF Loss: (Central, Peripheral constriction, Bitemporal), Optic Atrophy
- D/c ETHAMBUTOL; Vision may improve slowly over several months (not necessarily reversible)
Toxic Optic Neuropathy: Sildenafil Citrate
- Type of Inhibitor?
a. Main DRUG? - OCULAR issues?
- Can cause WHAT?
- Can also occur with what other drugs?
- Phosphodiesterase Type 5 inhibitor x ED
a. VIAGRA - Transient alteration of color perception (SEEING BLUE) (Weak effect on Type 6)
- NAION!
- Cialis and Levitra
Toxic Optic Neuropathy: AMIODARONE
- Use?
- Ocular issues?
- Signs?
- Tx?
- Atrial and Ventricular Tachyarrhythmias unresponsive to other agents
- CORNEAL VERTICILLATA (dose and duration dependent; Resolve w/Tx cessation in 3-4 mos). Also, OPTIC NEUROPATHY!
- Insidious, B/L VA LOSS; B/L Disc EDEMA (Resolves several mos after drug discontinuation)
- D/C it in all Pts w/Optic Neuropathy
Toxic Optic Neuropathy: Tobacco-Alcohol AMBLYOPIA (TAA)
- Mechanism?
- Who is affected?
- Sx?
- Tx?
- Unknown…belief it has to do w/Malnutrition and Vit B12 deficiency may play a role.
- Middle-aged/Old peeps; Pipe/Cigar Smokers/ High Alcohol Consumption
- Painless; Slowly progressive; VA Loss; Dyschromatopsia; CENTROCECAL SCOTOMA; Optic Disc NL –> PALLOR
- stop smoking/ETOH
Healthy diet. MVI (?)
IM Hydroxocobalamin
Hereditary Optic Neuropathy: LEBER’S (LHON)
- Affects whom more?
- Age of Onset?
- Sx?
- MALES >Females
- 15-25 yrs
- 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)
Hereditary Optic Neuropathy: (LHON) MITOCHONDRIAL DNA
- Inheritance Pattern?
- Screening for what?
- Tx?
- MATERNAL (all offspring of mother carrier will inherit the trait and ONLY MOM can pass on the trait)
- LHON (Primary mutations…90% of cases)
- AVOID Tobacco, Excessive ETOH, Environmental Toxins
* Vision Rehab; Genetic Counseling; ECGx Cardiac Conduction Abnormalities
Hereditary Optic Neuropathy: DOMINANT OPTIC ATROPHY
- AKA?
- MOST COMMON WHAT?
- ONSET?
- VA?
- Sx?
- Signs?
- Etiology?
- Tx?
- Kjer’s Optic Atrophy (AD)
- HEREDITARY OPTIC NEUROPATHY
- 1st Decade
- 20/20-20/60; (<20/70 less common)
- Mild, Slow, Insidious Progressive VA loss; Dyschromatopsia (Tritanopia) VF LOSS (Central, Paracentral or Cecocentral)
- Optic Atrophy; Nefative FLR; Mild Macular Pigmentary Changes; Arterial Attenuation
- 60 different mutations, deletions and Insertions of OPA1 GENE
- Vision REHAB
Hereditary Optic Neuropathy: INHERITED ATAXIA
- These represent a group of chronic progressive neurodegenerative conditions that involve what?
- WHAT IS NOT UNCOMMON AMONG PTs w/HEREDITARY ATAXIAS!?
- Onset?
- Sx?
- involving the CEREBELLUM and its CONNECTIONS
- OPTIC ATROPHY
- 8-15 yrs but
Hereditary Optic Neuropathy: SPINOCEREBELLAR ATAXIA (SCA)
- AUTOSOMAL WHAT?
- MOST COMMON WHAT?
- Vision loss?
- VF?
- Optic Atrophy?
- AD (SCA1 or SCA3)
- HEREDITARY ATAXIA!!
- Mild
- Constricted
- Diffuse Optic Atrophy
Hereditary Optic Neuropathy: Charcot-Marie-Tooth (CMT)
- Heredofamilial d/o w/PROGRESSIVE WHAT?
- Onset?
- Signs/Sx?
- MAIN THINGS TO KNOW? (VISION and OPTIC NERVE)
- Weakness and Atrophy
- 2-15 yrs
- Pes cavus; Foot deformities; Scoliosis; Slowly progressive weakness and wasting of the feet and legs, THEN HANDS
- PROGRESSIVE, B/L BLINDNESS and OPTIC ATROPHY
Hereditary Optic Neuropathy: MYOTONIC DYSTROPHY
- Autosomal what?
- CHARACTERISTICS?
- Other FEATURES?
- AD
- Progressive Myopathy; CHRISTMAS TREE CATARACTS!; DM; Cardiomyopathy w/conduction defects
- Frontal baldness, bifacial weakness, pigmentary retinopathy
PTOSIS, OPTIC ATROPHY, OCULAR HYPOTONIA, EXTERNAL OPHTHALMOPLEGIA