2 - Retinal Disorders II Flashcards

1
Q

Hereditary retinal dystrophies

-describe familial drusen (2)

A

Numerous drusen

Choroidal NV

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2
Q

Photopsia, dyschromotopsia, nyctalopia, dark adaptation difficulties
Fixed, non-prog VF defect
Fundus app normal 50%, disc pallor, vessel attenuation, vitritis

A

Melanoma-assoc retinopathy (MAR)

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3
Q

Paraneoplastic retinopathy

-differentials (2)

A

Abnormal ERG distinguishes from optic neuropathy***

Rapid rate of visual deterioration distinguishes from other retinal degenerations (e.g. retinitis pigmentosa)***

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4
Q

Hereditary retinal dystrophies

-describe Stargardt’s (5)

A

Yellow flecks

Beaten bronze app

RPE disturbance

Dark silent choroid on FA

Stable vision until 40’s

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5
Q

Hereditary retinal dystrophies

-describe juvenile retinoschisis (3)

A

Aka X-linked retinoschisis

Foveal retinoschisis

Stellate spoke-like app with microcysts

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6
Q

Cancer-assoc retinopathy (CAR)

  • antibodies to
  • pathogenesis
  • ERG
  • cells primarily affected
A

23-kDa antigen

Autoimmune pathogenesis***

Diffuse loss of ERG/abnormal scotopic***

Rods primarily affected***

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7
Q

Bilateral progressive vision loss
Disc swelling
Abnormal immunoglobulins CSF
ON demyelination

A

Paraneoplastic ganglion cell neuronopathy (PGCN)

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8
Q

Big blind spot syndrome

  • pathogenesis: inflammation, herpesvirus, (?)
  • acute onset photopsia
  • enlarged blind spot occassionally assoc with mild disc edema
  • peripapillary pigmentary changes
  • mimic temporal VF defects of chiasmal disease
  • focal and multifocal ERGs directed at peripapillary retina***
A

Acute idiopathic blind spot enlargement (AIBSE)

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9
Q

Multiple choroidal nevi is typical retinal finding
Bilateral subacute vision loss
Subretinal proliferation of pigmentary cells and yellow-orange lesions at level of RPE
Uveitis and rapidly progressive cataract

A

Diffuse uveal melanocytic proliferation (DUMP)

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10
Q

Paraneoplastic retinopathy

-describe/define

A

Subacute deterioration of vision and retinal function from non-metastatic, remote effects of a malignancy

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11
Q

Paraneoplastic retinopathy

  • signs/symptoms (3)
  • ERG
A

Acute onset photopsia
Abnormal CV
Paracentral scotoma doesn’t respect horiz meridian
-damage to PRL layer (not NFL like glaucoma)

Diffuse retinal dysfunction

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12
Q

Hereditary retinal dystrophies
-cone or cone-rod dystrophies
—presents with__
—possible first sign

A

Progressive and heritable central vision loss without ophthalmoscopic app, suggesting alternative dystrophy***

Dyschromatopsia***

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13
Q

Melanoma-assoc retinopathy (MAR)

  • antibodies to
  • pathogenesis
  • ERG
A

Retinal bipolar cells

Autoimmune pathogenesis***

Rod dysfunction (scotopic B-wave) and normal cone function***
-similar to CSNB
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14
Q

Cancer-assoc cone dysfunction

  • antibodies to
  • ERG
A

CAR antigen + 50-kDa protein

Abnormal cone ERG***

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15
Q

Paraneoplastic retinopathy

-lab findings (2)

A

ID of serum anti-retinal antibodies***

Periphlebitis on FA

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16
Q

Hereditary retinal dystrophies

-describe Best’s disease (4)

A

Egg yolk app

RPE disturbance - breaks BRB

May develop NV

Usually bilateral

17
Q

Paraneoplastic retinopathy

-main tx

A

Systemic corticosteroids***

18
Q

Paraneoplastic retinopathy vs big blind spot syndrome

A

PR: assoc with cancer (esp malignant/matastatic), more severe

BBS: idiopathic inflamm, peripapillary or multiple areas, more mild

19
Q

Hereditary retinal dystrophies

-describe myopic degeneration (3)

A

Peripapillary atrophy

Staphyloma

Lacquer cracks (breaks in Bruch’s)

20
Q

Subacute photopsia, vision loss, nyctalopia
Progressive VF loss beginning mid-periph scotoma
Mild vitritis, narrow retinal vessels
Relentlessly progressive
Steroids may help

A

Cancer-assoc retinopathy (CAR)

21
Q

Big blind spot syndrome

  • pathogenesis: inflammation, herpes virus, (?)
  • acute onset photopsia, unilateral vision loss and scotomas
  • multiple white lesions at the level of the RPE
  • full-field ERG abnormalities***
  • FA can confirm retinal epith defects
A

multiple evanescent white dot syndrome (MEWDS)

22
Q

Hereditary retinal dystrophies

  • abnormal light sensitivity
  • insidious central veision loss, dyschromotopsia, VF disturbances
  • abnormal ERG (esp cones)
  • OCT thinning of outer retina-choroid complex
  • optic disc pallor
A

Cone or cone-rod dystrophies

23
Q

Paraneoplastic ganglion cell neuronopathy (PGCN)

  • antibodies to
  • pathogenesis
A

Retinal ganglion cells

Autoimmune pathogenesis***

24
Q

Women with reproductive tract cancers, men with retroperitoneal

A

Diffuse uveal melanocytic proliferation (DUMP)

25
Q

Acute onset VF defect around physiological blind spot + photopsia
All likely affect outer retina
More commonly women

A

Big blind spot syndrome