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Optic neuritis

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faqar2003

This document discusses different classifications and etiologies of optic neuritis. Optic neuritis can be classified ophthalmoscopically as retrobulbar neuritis, papillitis, or neuroretinitis. It can also be classified etiologically as demyelinating, parainfectious, infective, or autoimmune. Demyelinating optic neuritis is most common and is often associated with multiple sclerosis. Symptoms include monocular visual impairment and eye pain that typically improves over several weeks. Treatment may involve steroids to speed recovery in severe cases.

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Thu-alfaqar Gheni Ophthalmolgy Department / Al-Diwaniyah Teaching Hospital
CLASSIFICATION: Can be classified both ophthalmoscopically and etiologically:  Retrobulbar neuritis  Papillitis  Neuroretinitis  Demyelinating **  Parainfective (rubella,mumps&chicken pox)  Infective (sinusitis.syphilis,cat-scratch fever)  Autoimmune OPHTHALMOSCOPICALLY ETIOLOGICALLY
Ophthalmoscopical Classification Retrobulbar neuritis Papillitis Neuroretinitis  the optic disc appears normal, at least initially, because the optic nerve head is not involved  Optic nerve head affected  papillitis in association with inflammation of the retinal nerve fibre layer and a macular star figure  The optic disc appears normal  The condition may be truly described as ‘ the patient sees nothing and the doctor sees nothing.’  Hyperaemia and oedema of the optic disc  May be a/w peripapillary flame-shaped haemorrhages  Cells may be seen in the posterior vitreous.  Macular star (exudates that form around the macula give the appearance of the star)  is the most common type in adults and is frequently associated with multiple sclerosis  the most common type of optic neuritis in children, but can also affect adults.  It is the least common type  only rarely a manifestation of
Normal optic disc, primary optic atrophy. The condition may be truly described as ‘ the patient sees nothing and the doctor sees nothing.’ Retrobulbar neuritis
swollen disc with blurring and hyperaemia of disc margin; venous dilation and engorgement ; vitreous haziness because of inflammatory exudates and cells that invaded the vitreous( mild vitritis); Flame-shaped hemorrhages and cotton wool spot (soft exudates) on and around the disc; secondary optic atrophy Papillitis
Macular star (Exudates in a star-shaped pattern radiating from the macula) Neuroretinitis
According to aetiology  Demyelinating:- This is by far the most common cause.  Parainfectious:- following a viral infection or immunization.  Infectious:- This may be sinus-related, or associated with conditions such as cat-scratch disease, syphilis, Lyme disease, cryptococcal meningitis and herpes zoster.  Non-infectious :- sarcoidosis systemic autoimmune diseases such as systemic lupus erythematosus, polyarteritis nodosa and other vasculitides.
Demyelinating optic neuritis  Demyelination :- a pathological process in which normally myelinated nerve fibres lose their insulating myelin layer. The myelin is phagocytosed by microglia and macrophages, subsequent to which astrocytes lay down fibrous tissue in plaques. Demyelinating disease disrupts nervous conduction within the white matter tracts of the brain, brainstem and spinal cord.
Demyelinating conditions that may involve the visual system include the following:  • Isolated optic neuritis:- no clinical evidence of generalized demyelination, although in a high proportion of cases this subsequently develops.  • Multiple sclerosis (MS):- by far the most common demyelinating disease .  • Devic disease (neuromyelitis optica) :- a very rare disease that may occur at any age, characterized by bilateral optic neuritis and the subsequent development of transverse myelitis (demyelination of the spinal cord) within days or weeks.  • Schilder disease:- a very rare relentlessly progressive generalized disease with an onset prior to the age of 10 years and death within 1–2 years. Bilateral optic neuritis without subsequent improvement may occur.
Multiple sclerosis  an idiopathic demyelinating disease involving central nervous system white matter.  It is more common in women than men.  typically in the third–fourth decades, generally with relapsing/remitting demyelination that may switch later to an unremitting pattern, and less commonly with progressive disease from the outset.
• Systemic features may include:  ○ Spinal cord e.g. weakness, stiffness, sphincter disturbance, sensory loss.  ○ Brainstem, e.g. diplopia, nystagmus, dysarthria, dysphagia.  ○ Cerebral, e.g. hemiparesis, hemianopia, dysphasia.  ○ Psychological, e.g. intellectual decline, depression, euphoria.  ○ Transient features, e.g. the Lhermitte sign (electrical sensation on neck flexion) the Uhthoff phenomenon (sudden worsening of vision or other symptoms on exercise or increase in body temperature).
Ophthalmic features  ○ Common. Optic neuritis (usually retrobulbar), internuclear ophthalmoplegia, nystagmus.  ○ Uncommon Skew deviation, ocular motor nerve palsies, hemianopia.  ○ Rare. Intermediate uveitis and retinal periphlebitis.
• Investigation  ○ Lumbar puncture :- oligoclonal bands on protein electrophoresis of cerebrospinal fluid in 90–95%.  ○ MRI:- almost always shows characteristic white matter lesions  ○ VEPs :- are abnormal (conduction delay and a reduction in amplitude) in up to 100% of patients with clinically definite MS.
Association between optic neuritis and multiple sclerosis  • The overall 15-year risk of developing MS following an acute episode of optic neuritis is about 50%;  with no lesions on MRI the risk is 25%,  but over 70% in patients with one or more lesions on MRI;  the presence of MRI lesions is therefore a very strong predictive factor.
 • A substantially lower risk of developing MS when there are no MRI lesions is conferred by the following factors, providing critical support in deciding whether to commence immunomodulatory MS-prophylactic treatment following an optic neuritis episode: ○ Male gender. ○ Absence of a viral syndrome preceding the optic neuritis. ○ Absence of periocular pain. ○ Optic disc swelling, disc/peripapillary haemorrhages or macular exudates. ○ Vision reduced to no light perception. • Optic neuritis is the presenting feature of MS in up to 30%. • Optic neuritis occurs at some point in 50% of patients with established MS.
Clinical features of demyelinating optic neuritis  Symptoms :- ○ Subacute monocular visual impairment. ○ Usual age range 20–50 years (mean around 30). ○ Some patients experience tiny white or coloured flashes or sparkles (phosphenes). ○ Discomfort or pain in or around the eye is present in over 90% and typically exacerbated by ocular movement; it may precede or accompany the visual loss and usually lasts a few days. ○ Frontal headache and tenderness of the globe may also be present.
 • Signs  ○ Visual acuity (VA):- usually 6/18–6/60, but may rarely be worse.  ○ Other signs of optic nerve dysfunction :- particularly impaired colour vision and a relative afferent pupillary defect.  ○ The optic disc is normal in the majority of cases (retrobulbar neuritis); the remainder show papillitis.  ○ Temporal disc pallor may be seen in the fellow eye, indicative of previous optic neuritis. papillitis Temporal disc pallor
Visual field defects  ○ Diffuse depression of sensitivity in the entire central 30° is the most common.  ○ Altitudinal/arcuate defects focal central/centrocaecal scotomas are also frequent.  ○ Focal defects are frequently accompanied by an element of superimposed generalized depression.
Course:-  Vision worsens over several days to 3 weeks and then begins to improve. Initial recovery is fairly rapid and then slower over 6–12 months.
Prognosis ○ More than 90% of patients recover visual acuity to 6/9 or better. ○ Subtle parameters of visual function, such as colour vision, may remain abnormal. ○ A mild relative afferent pupillary defect may persist. ○ Temporal optic disc pallor or more marked optic atrophy may ensue. ○ About 10%develop chronic optic neuritis with slowly progressive or stepwise visual loss.
Treatment following demyelinating optic neuritis  • Indications for steroid treatment:. When visual acuity within the first week of onset is worse than 6/12, treatment may speed up recovery by 2–3 weeks and may delay the onset of clinical MS over the short term. This may be relevant in the patients with poor vision in the fellow eye or those with occupational requirements, but the limited benefit must be balanced against the risks of high- dose steroids. Therapy does not influence the eventual visual outcome and the great majority of patients do not require treatment. Intravenous methylprednisolone sodium succinate daily for 3 days, followed by oral prednisolone for 11 days Oral prednisolone may increase the risk of recurrence of optic neuritis if used without prior intravenous steroid.
Immunomodulatory treatment (IMT)  reduces the risk of progression to clinical MS in some patients, but the risk versus benefit ratio has not yet been fully defined with the options available, which include interferon beta, teriflunomide and glatiramer.  based on risk profile – particularly the presence of brain lesions – and patient preference;  most do not commence IMT until a second episode of clinical demyelination has occurred,
Parainfectious optic neuritis  Ass. With viral infections such as measles, mumps, chickenpox, rubella, whooping cough and glandular fever, and may also occur following immunization.  Children are affected much more frequently than adults.  usually 1–3 weeks after a viral infection, with acute severe visual loss generally involving both eyes.  Bilateral papillitis is the rule; (occasionally: neuroretinitis or the discs may be normal).  The prognosis for spontaneous visual recovery is very good, and treatment is not required in the majority of patients. However, when visual loss is severe and bilateral or involves an only seeing eye, intravenous steroids should be considered, with antiviral cover where appropriate.
Infectious optic neuritis  Sinus-related optic neuritis  Cat-scratch fever (benign lymphoreticulosis) neuroretinitis.  Syphilis may cause acute papillitis or neuroretinitis during the primary or secondary stages.  Lyme disease (borreliosis)  Cryptococcal meningitis.  •Varicella zoster virus may cause papillitis by spread from contiguous retinitis (i.e. acute retinal necrosis, progressive retinal necrosis) or associated with herpes zoster ophthalmicus. Primary optic neuritis is uncommon but may occur in immunocompromised patients, some of whom may subsequently develop viral retinitis.
Non-infectious optic neuritis  Sarcoidosis  Optic neuritis affects 1–5%.  The response to steroid therapy is often rapid, though vision may decline if treatment is tapered or stopped prematurely, and some patients require long-term low- dose therapy.  Methotrexate may also be used as an adjunct to steroids or as monotherapy in steroid- intolerant patients.
The optic nerve head may exhibit a lumpy appearance suggestive of granulomatous infiltration and there may be associated vitritis
Autoimmune  Autoimmune optic nerve involvement may take the form of retrobulbar neuritis or anterior ischaemic optic neuropathy (NEXT LECTURE).  Some patients may also experience slowly progressive visual loss suggestive of compression.  Treatment is with systemic steroids and other immunosuppressants.
Neuroretinitis  Neuroretinitis refers to the combination of optic neuritis and signs of retinal, usually macular, inflammation. Cat-scratch fever is responsible for 60%of cases. About 25% of cases are idiopathic (Leber idiopathic stellate neuroretinitis).  Other notable causes include syphilis, Lyme disease, mumps and leptospirosis.
  Diagnosis  • Symptoms. Painless unilateral visual impairment, usually gradually worsening over about a week.  • Signs :-  ○ VA is impaired to a variable degree.  ○ Signs of optic nerve dysfunction are usually mild or absent, as visual loss is largely due to macular involvement.  ○ Venous engorgement and splinter haemorrhages may be present in severe case.  ○ Fellow eye involvement occasionally develops.
Papillitis associated with peripapillary and macular oedema A macular star typically appears as disc swelling settles; the macular star resolves with a return to normal or near-normal visual acuity over 6–12 months
 (OCT) demonstrates sub- and intraretinal fluid to a variable extent.  (FA) shows diffuse leakage from superficial disc vessels.  Blood tests may include serology for Bartonella and other causes according to clinical suspicion .  Treatment This is specific to the cause, and often consists of antibiotics. Recurrent idiopathic cases may require treatment with steroids and/or other immunosuppressants.
Optic neuritis

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Optic neuritis

  • 1. Thu-alfaqar Gheni Ophthalmolgy Department / Al-Diwaniyah Teaching Hospital
  • 2. CLASSIFICATION: Can be classified both ophthalmoscopically and etiologically:  Retrobulbar neuritis  Papillitis  Neuroretinitis  Demyelinating **  Parainfective (rubella,mumps&chicken pox)  Infective (sinusitis.syphilis,cat-scratch fever)  Autoimmune OPHTHALMOSCOPICALLY ETIOLOGICALLY
  • 3. Ophthalmoscopical Classification Retrobulbar neuritis Papillitis Neuroretinitis  the optic disc appears normal, at least initially, because the optic nerve head is not involved  Optic nerve head affected  papillitis in association with inflammation of the retinal nerve fibre layer and a macular star figure  The optic disc appears normal  The condition may be truly described as ‘ the patient sees nothing and the doctor sees nothing.’  Hyperaemia and oedema of the optic disc  May be a/w peripapillary flame-shaped haemorrhages  Cells may be seen in the posterior vitreous.  Macular star (exudates that form around the macula give the appearance of the star)  is the most common type in adults and is frequently associated with multiple sclerosis  the most common type of optic neuritis in children, but can also affect adults.  It is the least common type  only rarely a manifestation of
  • 4. Normal optic disc, primary optic atrophy. The condition may be truly described as ‘ the patient sees nothing and the doctor sees nothing.’ Retrobulbar neuritis
  • 5. swollen disc with blurring and hyperaemia of disc margin; venous dilation and engorgement ; vitreous haziness because of inflammatory exudates and cells that invaded the vitreous( mild vitritis); Flame-shaped hemorrhages and cotton wool spot (soft exudates) on and around the disc; secondary optic atrophy Papillitis
  • 6. Macular star (Exudates in a star-shaped pattern radiating from the macula) Neuroretinitis
  • 7. According to aetiology  Demyelinating:- This is by far the most common cause.  Parainfectious:- following a viral infection or immunization.  Infectious:- This may be sinus-related, or associated with conditions such as cat-scratch disease, syphilis, Lyme disease, cryptococcal meningitis and herpes zoster.  Non-infectious :- sarcoidosis systemic autoimmune diseases such as systemic lupus erythematosus, polyarteritis nodosa and other vasculitides.
  • 8. Demyelinating optic neuritis  Demyelination :- a pathological process in which normally myelinated nerve fibres lose their insulating myelin layer. The myelin is phagocytosed by microglia and macrophages, subsequent to which astrocytes lay down fibrous tissue in plaques. Demyelinating disease disrupts nervous conduction within the white matter tracts of the brain, brainstem and spinal cord.
  • 9. Demyelinating conditions that may involve the visual system include the following:  • Isolated optic neuritis:- no clinical evidence of generalized demyelination, although in a high proportion of cases this subsequently develops.  • Multiple sclerosis (MS):- by far the most common demyelinating disease .  • Devic disease (neuromyelitis optica) :- a very rare disease that may occur at any age, characterized by bilateral optic neuritis and the subsequent development of transverse myelitis (demyelination of the spinal cord) within days or weeks.  • Schilder disease:- a very rare relentlessly progressive generalized disease with an onset prior to the age of 10 years and death within 1–2 years. Bilateral optic neuritis without subsequent improvement may occur.
  • 10. Multiple sclerosis  an idiopathic demyelinating disease involving central nervous system white matter.  It is more common in women than men.  typically in the third–fourth decades, generally with relapsing/remitting demyelination that may switch later to an unremitting pattern, and less commonly with progressive disease from the outset.
  • 11. • Systemic features may include:  ○ Spinal cord e.g. weakness, stiffness, sphincter disturbance, sensory loss.  ○ Brainstem, e.g. diplopia, nystagmus, dysarthria, dysphagia.  ○ Cerebral, e.g. hemiparesis, hemianopia, dysphasia.  ○ Psychological, e.g. intellectual decline, depression, euphoria.  ○ Transient features, e.g. the Lhermitte sign (electrical sensation on neck flexion) the Uhthoff phenomenon (sudden worsening of vision or other symptoms on exercise or increase in body temperature).
  • 12. Ophthalmic features  ○ Common. Optic neuritis (usually retrobulbar), internuclear ophthalmoplegia, nystagmus.  ○ Uncommon Skew deviation, ocular motor nerve palsies, hemianopia.  ○ Rare. Intermediate uveitis and retinal periphlebitis.
  • 13. • Investigation  ○ Lumbar puncture :- oligoclonal bands on protein electrophoresis of cerebrospinal fluid in 90–95%.  ○ MRI:- almost always shows characteristic white matter lesions  ○ VEPs :- are abnormal (conduction delay and a reduction in amplitude) in up to 100% of patients with clinically definite MS.
  • 14. Association between optic neuritis and multiple sclerosis  • The overall 15-year risk of developing MS following an acute episode of optic neuritis is about 50%;  with no lesions on MRI the risk is 25%,  but over 70% in patients with one or more lesions on MRI;  the presence of MRI lesions is therefore a very strong predictive factor.
  • 15.  • A substantially lower risk of developing MS when there are no MRI lesions is conferred by the following factors, providing critical support in deciding whether to commence immunomodulatory MS-prophylactic treatment following an optic neuritis episode: ○ Male gender. ○ Absence of a viral syndrome preceding the optic neuritis. ○ Absence of periocular pain. ○ Optic disc swelling, disc/peripapillary haemorrhages or macular exudates. ○ Vision reduced to no light perception. • Optic neuritis is the presenting feature of MS in up to 30%. • Optic neuritis occurs at some point in 50% of patients with established MS.
  • 16. Clinical features of demyelinating optic neuritis  Symptoms :- ○ Subacute monocular visual impairment. ○ Usual age range 20–50 years (mean around 30). ○ Some patients experience tiny white or coloured flashes or sparkles (phosphenes). ○ Discomfort or pain in or around the eye is present in over 90% and typically exacerbated by ocular movement; it may precede or accompany the visual loss and usually lasts a few days. ○ Frontal headache and tenderness of the globe may also be present.
  • 17.  • Signs  ○ Visual acuity (VA):- usually 6/18–6/60, but may rarely be worse.  ○ Other signs of optic nerve dysfunction :- particularly impaired colour vision and a relative afferent pupillary defect.  ○ The optic disc is normal in the majority of cases (retrobulbar neuritis); the remainder show papillitis.  ○ Temporal disc pallor may be seen in the fellow eye, indicative of previous optic neuritis. papillitis Temporal disc pallor
  • 18. Visual field defects  ○ Diffuse depression of sensitivity in the entire central 30° is the most common.  ○ Altitudinal/arcuate defects focal central/centrocaecal scotomas are also frequent.  ○ Focal defects are frequently accompanied by an element of superimposed generalized depression.
  • 19. Course:-  Vision worsens over several days to 3 weeks and then begins to improve. Initial recovery is fairly rapid and then slower over 6–12 months.
  • 20. Prognosis ○ More than 90% of patients recover visual acuity to 6/9 or better. ○ Subtle parameters of visual function, such as colour vision, may remain abnormal. ○ A mild relative afferent pupillary defect may persist. ○ Temporal optic disc pallor or more marked optic atrophy may ensue. ○ About 10%develop chronic optic neuritis with slowly progressive or stepwise visual loss.
  • 21. Treatment following demyelinating optic neuritis  • Indications for steroid treatment:. When visual acuity within the first week of onset is worse than 6/12, treatment may speed up recovery by 2–3 weeks and may delay the onset of clinical MS over the short term. This may be relevant in the patients with poor vision in the fellow eye or those with occupational requirements, but the limited benefit must be balanced against the risks of high- dose steroids. Therapy does not influence the eventual visual outcome and the great majority of patients do not require treatment. Intravenous methylprednisolone sodium succinate daily for 3 days, followed by oral prednisolone for 11 days Oral prednisolone may increase the risk of recurrence of optic neuritis if used without prior intravenous steroid.
  • 22. Immunomodulatory treatment (IMT)  reduces the risk of progression to clinical MS in some patients, but the risk versus benefit ratio has not yet been fully defined with the options available, which include interferon beta, teriflunomide and glatiramer.  based on risk profile – particularly the presence of brain lesions – and patient preference;  most do not commence IMT until a second episode of clinical demyelination has occurred,
  • 23. Parainfectious optic neuritis  Ass. With viral infections such as measles, mumps, chickenpox, rubella, whooping cough and glandular fever, and may also occur following immunization.  Children are affected much more frequently than adults.  usually 1–3 weeks after a viral infection, with acute severe visual loss generally involving both eyes.  Bilateral papillitis is the rule; (occasionally: neuroretinitis or the discs may be normal).  The prognosis for spontaneous visual recovery is very good, and treatment is not required in the majority of patients. However, when visual loss is severe and bilateral or involves an only seeing eye, intravenous steroids should be considered, with antiviral cover where appropriate.
  • 24. Infectious optic neuritis  Sinus-related optic neuritis  Cat-scratch fever (benign lymphoreticulosis) neuroretinitis.  Syphilis may cause acute papillitis or neuroretinitis during the primary or secondary stages.  Lyme disease (borreliosis)  Cryptococcal meningitis.  •Varicella zoster virus may cause papillitis by spread from contiguous retinitis (i.e. acute retinal necrosis, progressive retinal necrosis) or associated with herpes zoster ophthalmicus. Primary optic neuritis is uncommon but may occur in immunocompromised patients, some of whom may subsequently develop viral retinitis.
  • 25. Non-infectious optic neuritis  Sarcoidosis  Optic neuritis affects 1–5%.  The response to steroid therapy is often rapid, though vision may decline if treatment is tapered or stopped prematurely, and some patients require long-term low- dose therapy.  Methotrexate may also be used as an adjunct to steroids or as monotherapy in steroid- intolerant patients.
  • 26. The optic nerve head may exhibit a lumpy appearance suggestive of granulomatous infiltration and there may be associated vitritis
  • 27. Autoimmune  Autoimmune optic nerve involvement may take the form of retrobulbar neuritis or anterior ischaemic optic neuropathy (NEXT LECTURE).  Some patients may also experience slowly progressive visual loss suggestive of compression.  Treatment is with systemic steroids and other immunosuppressants.
  • 28. Neuroretinitis  Neuroretinitis refers to the combination of optic neuritis and signs of retinal, usually macular, inflammation. Cat-scratch fever is responsible for 60%of cases. About 25% of cases are idiopathic (Leber idiopathic stellate neuroretinitis).  Other notable causes include syphilis, Lyme disease, mumps and leptospirosis.
  • 29.   Diagnosis  • Symptoms. Painless unilateral visual impairment, usually gradually worsening over about a week.  • Signs :-  ○ VA is impaired to a variable degree.  ○ Signs of optic nerve dysfunction are usually mild or absent, as visual loss is largely due to macular involvement.  ○ Venous engorgement and splinter haemorrhages may be present in severe case.  ○ Fellow eye involvement occasionally develops.
  • 30. Papillitis associated with peripapillary and macular oedema A macular star typically appears as disc swelling settles; the macular star resolves with a return to normal or near-normal visual acuity over 6–12 months
  • 31.  (OCT) demonstrates sub- and intraretinal fluid to a variable extent.  (FA) shows diffuse leakage from superficial disc vessels.  Blood tests may include serology for Bartonella and other causes according to clinical suspicion .  Treatment This is specific to the cause, and often consists of antibiotics. Recurrent idiopathic cases may require treatment with steroids and/or other immunosuppressants.

Editor's Notes

  • #4: The papilla (the blind spot) is the spot there the optic nerve leaves the retina A swollen disc with blurring and hyperaemia of disc margin