Retinal Disorders II Flashcards
Paraneoplastic retinopathy
Cancer associated retinopathy
Melanoma assocaited retinopathy
Diffuse uveal melanocytic proliferation
Paraneoplastic cell neuronopathy
Big blind spot syndrome
- acute idiopathic blind spot enlargement
- multiple evanescence’s white dot syndrome (MEWDS)
Paraneoplastic retinopathy
Refers to a syndrome of subacute deterioration of vision and retinal function resulting from a non metastatic, remote effect of a malignancy
Signs and symptoms of paraneoplastic retinopathy
- acute onset of photopsia
- abnormal color vision
- scotomas usually begins in the midperiphery (paracentral scotoma)
- ERG shows evidence of diffuse retinal dysfunction, suggesting the diagnosis of PR
Why does paraneoplastic retinopathy scotoma not respect the horizontal meridian
Because the damage is in the outer retina, not the NFL like glaucoma is
Cancer related retinopathy
Autoimmune (recoverin)
Diffuse loss of ERG (abnormal scotopic ERG)
Rods are primarily affected
Melanoma associated retinopathy (MAR)
Autoimmune pathogensis
ERG shows rod dysfunction (scotopic b wave) and normal cone function
Paraneoplastic ganglion cell neuronopathy
Autoimmune pathogenesis
Progressive loss of vision
May have optic disc swelling
Bilateral diffuse uveal melanocytic proliferation
Develops in women with reproductive tract cancers and men with cancers in retroperitoneal area
Retinal findings are very typical of multiple choroidal nevi and yellowish orange lesions of RPE
Cancer associated cone dysfunction
Autoimmune
Abnormal cone ERG
Flash of light and light srnzsitrivity
Color vision deficiency
Lab findings for paraneoplastic retinopathy
Identification of serum antiretinal antibodies is an important diagnostic aid (commercial)
Differential diagnosis for paraneoplastic retinopathy
An abnormal ERG will distinguish it from optic neruopathy
Paraneoplastic retinopathy treatment and management
Systemic corticosteroids is the main therapy
RP vs PR
RP is peripheral and gradual
PR is very rapid progression
Big blind spot syndrome
Acute onset of VF defect around the physiological blind spot accompanied by photopsia
- all likely affect the outer retina
- more common in women
Acute idiopathic blind spot enlargement (AIBSE)
A big blind spot syndrome
- not sure of pathophysiology
- photopsia
- VF defect
- ERG: focal and multifocal directed at the peripapillary retina
- peripapillary pgientary changes
- mild ONH swelling
MEWDS
Big blind spot syndrome
- Multiple evanescent white lesions at the level of the RPE
- full field ERG abnormalities
Differences between AIBSE and MEWDS
AIDBSE will have a normal full field ERG and problems on focal ERG
MEWDS will have a problem on full field ERG
Differences between paraneoplastic retinopathy and big blind spot syndromes
PR • assocaited with cancer (Malignant) • More severe Big blind spot syndrome • idiopathic inflamamtion of the peripapillary area or on the whole retina
Cone or cone-rod dystrophies
- hereditary retinal dystrophy
- presents with progressive and heritable central vision loss who do not have an ophthlamocscopic appearance suggesting an alternative dystrophy
- dyschromatopsia (May be first sign)
Best diseases DiffDx from cone or cone rod dystrophies
Egg yolk appearance RPE disturbance May develop NVC DXed when younger 20/80 to 20/200
Stargardts diffdx from cone or cone rod dystrophy
- yellow flecks
- beaten bronze
- RPE disturbance
- dark silent choroid on FA
- Dx when younger
- stable after teen years
- when 40, they have second deterioration from this disease
- bilateral
- 20/80-2/200
Familial drusen as dffdx from con dystrophy
Numerous drusen Choroidal neovascularization Vision can depend -macula involvement affects vision -increases chance to develop AMD
Myopic degeneration as diffdx from cone dystrophy
- peripapillary atrophy
- staphyloma
- lacquer cracks (break of bruchs membrane)
