Neuro-ophthalmology

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Neuro-ophthalmology

  1. 1. Neuro-ophthalmology Dr Russell Watkins
  2. 2. This Lecture <ul><li>ONH abnormalities </li></ul><ul><li>ONH swelling </li></ul><ul><li>Chiasmal lesions </li></ul><ul><li>Retrochiasmal lesions </li></ul>
  3. 3. ONH Abnormalities <ul><li>Congenital optic pit </li></ul><ul><ul><li>Usually unilateral </li></ul></ul><ul><ul><li>Often inferotemporal </li></ul></ul><ul><ul><li>Associated with serous macular detachment </li></ul></ul><ul><ul><li>Arcuate scotomata & other field defects can occur </li></ul></ul>
  4. 5. ONH Abnormalities <ul><li>Optic disc coloboma </li></ul><ul><ul><li>Deep excavation </li></ul></ul><ul><ul><li>May produce various field defects </li></ul></ul><ul><ul><li>Vision may be normal or reduced </li></ul></ul><ul><li>“ Morning glory” syndrome </li></ul><ul><ul><li>Unilateral coloboma with excavation filled with glial tissue & surrounded by pigment </li></ul></ul><ul><ul><li>“ Spoke-like” origin of vessels </li></ul></ul><ul><ul><li>Poor vision & associated with retinal detachment </li></ul></ul>
  5. 8. ONH Abnormalities <ul><li>Tilted disc </li></ul><ul><ul><li>Usually bilateral </li></ul></ul><ul><ul><li>Associated with high myopia, astigmatism, field defects </li></ul></ul><ul><li>Optic nerve hypoplasia </li></ul><ul><ul><li>Small ONH with pallid halo </li></ul></ul><ul><ul><li>Poor vision & various field defects & an RAPD if unilateral </li></ul></ul><ul><ul><li>Associated with aniridia & microphthalmos </li></ul></ul>
  6. 11. ONH Abnormalities <ul><li>Myelinated nerve fibres </li></ul><ul><ul><li>Congenital </li></ul></ul><ul><ul><li>White flame-shaped patches, usually adjacent to ONH, producing an enlarged blindspot </li></ul></ul>
  7. 14. ONH Abnormalities <ul><li>Optic disc drusen </li></ul><ul><ul><li>Congenital, often familial, usually bilateral </li></ul></ul><ul><ul><li>Optic cup absent </li></ul></ul><ul><ul><li>May be buried or exposed on ONH, which become more prominent with age </li></ul></ul><ul><ul><li>Buried drusen are differential of ONH swelling </li></ul></ul><ul><ul><li>Exposed drusen appear as multiple glistening bodies </li></ul></ul><ul><ul><li>Autofluorescence </li></ul></ul>
  8. 15. ONH Abnormalities <ul><li>ONH drusen (cont.) </li></ul><ul><ul><li>Associated with </li></ul></ul><ul><ul><ul><li>Vitreous haemorrhage </li></ul></ul></ul><ul><ul><ul><li>SRNVM </li></ul></ul></ul><ul><ul><ul><li>Angioid streaks </li></ul></ul></ul><ul><ul><ul><li>Retinitis pigmentosa </li></ul></ul></ul><ul><li>Bergmeister’s papilla </li></ul><ul><ul><li>Benign glial remnant (hyaloid artery) on ONH </li></ul></ul>
  9. 19. ONH Swelling <ul><li>Causes </li></ul><ul><ul><li>Papilloedema </li></ul></ul><ul><ul><li>Papillitis </li></ul></ul><ul><ul><li>Anterior ischaemic optic neuropathy </li></ul></ul><ul><ul><li>Pseudopapilloedema </li></ul></ul><ul><ul><li>Accelerated hypertension </li></ul></ul><ul><ul><li>Intraocular inflammation </li></ul></ul><ul><ul><li>CRVO </li></ul></ul><ul><ul><li>Optic nerve compression </li></ul></ul>
  10. 20. ONH Swelling <ul><li>Causes (cont.) </li></ul><ul><ul><li>ONH infiltration e.g. met, lymphoma </li></ul></ul><ul><ul><li>Ocular hypotony </li></ul></ul><ul><ul><li>Toxic optic neuropathy </li></ul></ul><ul><ul><li>Diabetic papillopathy </li></ul></ul>
  11. 21. ONH Swelling <ul><li>Papilloedema </li></ul><ul><ul><li>Disc swelling in the presence of  ICP </li></ul></ul><ul><ul><li>VA usually unaffected until late </li></ul></ul><ul><ul><li>Visual obscurations may occur </li></ul></ul><ul><ul><li>Staged as </li></ul></ul><ul><ul><ul><li>Early </li></ul></ul></ul><ul><ul><ul><li>Acute decompensated </li></ul></ul></ul><ul><ul><ul><li>Chronic </li></ul></ul></ul><ul><ul><ul><li>Terminal </li></ul></ul></ul>
  12. 22. ONH Swelling <ul><li>Early papilloedema </li></ul><ul><ul><li>Absent spontaneous venous pulsation </li></ul></ul><ul><ul><li>Nerve fibre swelling at ONH </li></ul></ul><ul><ul><li>ONH capillary dilatation ±peripapillary haemorrhage </li></ul></ul><ul><ul><li>Retinal folds </li></ul></ul>
  13. 23. ONH Swelling <ul><li>Acute decompensated papilloedema </li></ul><ul><ul><li>Grossly swollen hyperaemic ONH </li></ul></ul><ul><ul><li>BV masking </li></ul></ul><ul><ul><li>Loss of cup </li></ul></ul><ul><ul><li>Haemorrhages </li></ul></ul><ul><ul><li>CWS </li></ul></ul>
  14. 24. ONH Swelling <ul><li>Chronic papilloedema </li></ul><ul><ul><li>“ Champagne cork” appearance </li></ul></ul><ul><ul><li>Fewer haemorrhages </li></ul></ul><ul><ul><li>Macular star </li></ul></ul><ul><ul><li>Later, arcuate scotomata </li></ul></ul><ul><li>Terminal papilloedema </li></ul><ul><ul><li>Flat, pale, atrophied ONH </li></ul></ul><ul><ul><li>Arteriolar attenuation </li></ul></ul><ul><ul><li>Poor VA </li></ul></ul>
  15. 29. ONH Swelling <ul><li>Optic neuritis </li></ul><ul><ul><li>Acute  VA </li></ul></ul><ul><ul><li>Paracentral or central scotoma </li></ul></ul><ul><ul><li>Pain with ocular movement </li></ul></ul><ul><ul><li>Colour desaturation </li></ul></ul><ul><ul><li>RAPD </li></ul></ul><ul><ul><li>Uthoff’s phenomenon </li></ul></ul><ul><ul><li>Pulfrich’s phenomenon </li></ul></ul><ul><ul><li>VER abnormalities in inflammatory disease only </li></ul></ul>
  16. 30. ONH Swelling <ul><li>Types of optic neuritis </li></ul><ul><ul><li>Papillitis </li></ul></ul><ul><ul><ul><li>Swollen hyperaemic ONH with haemorrhages </li></ul></ul></ul><ul><ul><li>Retrobulbar neuritis </li></ul></ul><ul><ul><ul><li>Normal ONH </li></ul></ul></ul><ul><ul><li>Neuroretinitis </li></ul></ul><ul><ul><ul><li>Papillitis with macular star </li></ul></ul></ul>
  17. 31. ONH Swelling <ul><li>Causes of optic neuritis </li></ul><ul><ul><li>Multiple sclerosis (most commonly) </li></ul></ul><ul><ul><li>Devic’s disease (neuromyelitis optica) </li></ul></ul><ul><ul><ul><li>Young adults </li></ul></ul></ul><ul><ul><ul><li>Rapid, severe, bilateral visual loss </li></ul></ul></ul><ul><ul><ul><li>Paraplegia </li></ul></ul></ul><ul><ul><li>Viral encephalitis </li></ul></ul><ul><ul><li>Infectious mononucleosis </li></ul></ul><ul><ul><li>HZO </li></ul></ul>
  18. 32. ONH Swelling <ul><li>Causes of optic neuritis (cont.) </li></ul><ul><ul><li>Inflammation of adjacent structures (orbit, meninges, sinuses) </li></ul></ul><ul><ul><li>Granulomatous ONH inflammation e.g. TB, sarcoidosis, syphilis </li></ul></ul><ul><ul><li>Intraocular inflammation </li></ul></ul>
  19. 38. ONH Swelling <ul><li>Anterior ischaemic optic neuropathy (AION) </li></ul><ul><ul><li>GCA </li></ul></ul><ul><ul><li>Other vasculitides </li></ul></ul><ul><ul><li>Arteriosclerosis </li></ul></ul><ul><ul><li>Systemic hypertension </li></ul></ul><ul><ul><li>Systemic hypotension </li></ul></ul><ul><ul><li>Carotid artery disease </li></ul></ul><ul><ul><li>Diabetes mellitus </li></ul></ul>
  20. 39. ONH Swelling <ul><li>General features of AION </li></ul><ul><ul><li> VA (variable) </li></ul></ul><ul><ul><li>RAPD </li></ul></ul><ul><ul><li>Visual field defect </li></ul></ul><ul><ul><li>Colour vision abnormalities </li></ul></ul><ul><ul><li>Variable ONH swelling </li></ul></ul><ul><ul><li>Other signs dependent on aetiology </li></ul></ul>
  21. 40. ONH Swelling <ul><li>Features of non-arteritic AION </li></ul><ul><ul><li>Infarction of prelaminar ONH due to vascular insufficiency of PCA </li></ul></ul><ul><ul><li>Age of onset 45-80 yrs </li></ul></ul><ul><ul><li>Painless VA loss maximal at onset, with little recovery (seen in ~5% of cases) </li></ul></ul><ul><ul><li>Recurrences in same eye are rare </li></ul></ul><ul><ul><li>Fellow eye involved subsequently in 30 to 40% </li></ul></ul>
  22. 41. ONH Swelling <ul><li>Features of arteritic AION </li></ul><ul><ul><li>Infarction of prelaminar ONH due to vascular insufficiency of PCA 2° to vasculitis </li></ul></ul><ul><ul><li>Age of onset older than 60yrs; Peak 70-80 </li></ul></ul><ul><ul><li>F:M = 3:1; more common in Caucasians </li></ul></ul><ul><ul><li>Painless VA loss; can be preceding transient obscurations or profound VA  (HM-NLP); VA loss is maximal at onset </li></ul></ul><ul><ul><li>Recurrences in same eye can occur </li></ul></ul><ul><ul><li>Fellow eye involved subsequently within 10 days in 70% of cases if not treated </li></ul></ul>
  23. 44. ONH Swelling <ul><li>Other causes of ONH swelling……. </li></ul>
  24. 48. Chiasmal Lesions <ul><li>Clinical features of chiasmal lesions </li></ul><ul><ul><li>Headache </li></ul></ul><ul><ul><li>±Visual loss </li></ul></ul><ul><ul><li>RAPD </li></ul></ul><ul><ul><li>Colour vision abnormalities </li></ul></ul><ul><ul><li>Visual field defects </li></ul></ul><ul><ul><li>Diplopia </li></ul></ul><ul><ul><li>Endocrine dysfunction </li></ul></ul><ul><ul><li>ONH may appear normal or pale </li></ul></ul>
  25. 49. Chiasmal Lesions <ul><li>Visual field defects </li></ul><ul><ul><li>Bitemporal hemianopia </li></ul></ul><ul><ul><li>Junctional scotomata </li></ul></ul><ul><ul><ul><li>Central scotoma accompanied by contralateral superotemporal VF defect </li></ul></ul></ul>
  26. 51. Chiasmal Lesions <ul><li>Pituitary tumours </li></ul><ul><ul><li>Middle aged adults (M=F) present with visual problems or hormonal imbalance </li></ul></ul><ul><ul><li>30% of tumours non-functioning </li></ul></ul><ul><ul><li>Chromophobe adenoma (often prolactinoma) most common </li></ul></ul><ul><ul><li>Bitemporal hemianopia & optic atrophy </li></ul></ul>
  27. 55. Chiasmal Lesions <ul><li>Meningioma </li></ul><ul><ul><li>Adults (esp. middle aged women) </li></ul></ul><ul><ul><li>Present with visual loss </li></ul></ul><ul><ul><li>In region of chiasm, situated at sphenoidal ridge, tuberculum sellae, olfactory groove </li></ul></ul><ul><ul><li>Often asymmetrical field loss e.g. junctional syndrome </li></ul></ul><ul><ul><li>Optic atrophy </li></ul></ul><ul><ul><li>Hyperostosis on SXR </li></ul></ul>
  28. 59. Chiasmal Lesions <ul><li>Craniopharyngioma </li></ul><ul><ul><li>Children & young adults (M=F) </li></ul></ul><ul><ul><li>Slow-growing, often cystic </li></ul></ul><ul><ul><li>Present with features of  ICP, hormonal imbalance or visual loss </li></ul></ul><ul><ul><li>Various patterns of VF defect </li></ul></ul><ul><ul><li>Papilloedema or optic atrophy </li></ul></ul>
  29. 62. Chiasmal Lesions <ul><li>Aneurysms </li></ul><ul><ul><li>Middle aged adults (M=F) </li></ul></ul><ul><ul><li>Present with visual loss or ophthalmoplegia </li></ul></ul><ul><ul><li>Internal carotid, anterior communicating or ophthalmic arteries </li></ul></ul><ul><ul><li>VF defect depends on site of lesion </li></ul></ul><ul><ul><li>Variable prognosis </li></ul></ul>
  30. 65. Chiasmal Lesions <ul><li>Glioma of optic nerve or chiasma </li></ul><ul><ul><li>Children (75% are under 10); 60% have NF1 </li></ul></ul><ul><ul><li>Very slow-growing tumour </li></ul></ul><ul><ul><li>Present with visual loss </li></ul></ul><ul><ul><li>VF defect depends on site of lesion </li></ul></ul><ul><ul><li>Optic atrophy (occasional papilloedema) </li></ul></ul>
  31. 66. Chiasmal Lesions <ul><li>Other chaismal lesions </li></ul><ul><ul><li>Multiple sclerosis </li></ul></ul><ul><ul><li>Trauma </li></ul></ul><ul><ul><li>Basal meningitis </li></ul></ul><ul><ul><li>Sphenoidal sinus mucocele </li></ul></ul><ul><ul><li>Sphenoidal sinus carcinoma </li></ul></ul>
  32. 67. Retrochiasmal Lesions <ul><li>General features </li></ul><ul><ul><li>All have homonymous VF defects </li></ul></ul><ul><ul><li>The further posterior the lesion, the more congruent the defect </li></ul></ul><ul><ul><li>Visual acuity is usually unaffected </li></ul></ul>
  33. 68. Retrochiasmal Lesions <ul><li>Optic tract lesions </li></ul><ul><ul><li>Rare </li></ul></ul><ul><ul><li>Vague visual complaints </li></ul></ul><ul><ul><li>APD on contralateral side </li></ul></ul><ul><ul><li>Normal ONH; possibly temporal pallor of ipsilateral ONH & bowtie pallor of contralateral disc </li></ul></ul><ul><ul><li>Incongruous hemianopia </li></ul></ul><ul><ul><li>Caused by posteriorly extending chiasmal lesions or vascular lesions </li></ul></ul>
  34. 70. Retrochiasmal Lesions <ul><li>LGN lesions </li></ul><ul><ul><li>Normal visual acuity </li></ul></ul><ul><ul><li>No APD </li></ul></ul><ul><ul><li>Normal ONH; possibly temporal pallor of ipsilateral ONH & bowtie pallor of contralateral ONH </li></ul></ul><ul><ul><li>Incongruous homonymous hemianopia </li></ul></ul><ul><ul><li>Relatively congruous homonymous quadrantanopia </li></ul></ul>
  35. 71. Retrochiasmal Lesions <ul><li>Temporal lobe lesions </li></ul><ul><ul><li>Normal visual acuity, no APD, normal ONH </li></ul></ul><ul><ul><li>Affect Meyer’s loop  upper homonymous quadrantanopia; only large lesions cause inferior extension of defects </li></ul></ul><ul><ul><li>Causes </li></ul></ul><ul><ul><ul><li>Gliomas </li></ul></ul></ul><ul><ul><ul><li>Vascular lesions </li></ul></ul></ul><ul><ul><ul><li>Surgical trauma </li></ul></ul></ul><ul><ul><li>Neuropsychiatric symptoms incl. epilepsy </li></ul></ul>
  36. 72. Retrochiasmal Lesions <ul><li>Parietal lobe lesions </li></ul><ul><ul><li>Normal VA, no APD, normal ONH </li></ul></ul><ul><ul><li>Complete homonymous hemianopia; occasionally lower quadrantanopia </li></ul></ul><ul><ul><li>Decreased OKN towards side of lesion </li></ul></ul><ul><ul><li>Associated with apraxia </li></ul></ul><ul><ul><li>Causes </li></ul></ul><ul><ul><ul><li>Metastases </li></ul></ul></ul><ul><ul><ul><li>Glioma </li></ul></ul></ul><ul><ul><ul><li>Meningioma </li></ul></ul></ul><ul><ul><ul><li>MCA thrombosis (the artery of stroke) </li></ul></ul></ul>
  37. 75. Retrochiasmal Lesions <ul><li>Occipital lobe lesions </li></ul><ul><ul><li>Congruous homonymous hemianopia </li></ul></ul><ul><ul><li>May have macular sparing </li></ul></ul><ul><ul><li>Vascular aetiology in 90% of cases </li></ul></ul><ul><ul><li>Remainder due to </li></ul></ul><ul><ul><ul><li>Trauma (contre-coup) </li></ul></ul></ul><ul><ul><ul><li>Tumours </li></ul></ul></ul>

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