penyakit saraf optik

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    Yunita Sp.M

    (Neuro-ophthalmology division)

    Penyakit saraf optik

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    Saraf optik intraokular, intraorbital,

    intrakanalikular dan intrakranial

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    Penyakit pada saraf optik

    Neuropati optik

    Papiledema

    Atrofi papil

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    Neuropati optik

    Tipikal Idiopatik

    Berhubungan dengan MS

    Atipikal

    Infeksius Imun

    Sarcoidosis

    Neuropati optik non-inflamasi

    ION Kompresif

    Infiltratif, dsb

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    Neuritis optik tipikal

    Idiopatik atau berhubungan dengan MS

    visus monokuler akut disertai nyeri periokular (92%)

    Umur 15-45 tahun, F > M

    VA 20/20NLP RAPD (+)

    Diskus optik tampak normal, edema pada 1/3 kasus.

    Visus < dalam 1 Minggu, membaik di minggu 2 hingga 1

    tahun.

    MRI ~ multifokal demielinisasi ~ MS

    VEP bila terdapat tanda klinis (RAPD)

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    Neuritis optik tipikal

    Penanganan neuritis optik tipikal

    Metil prednisolon IV 1 g/hari ~3 hari, dilanjutkan prednison

    oral 1 mg/kgBB selama 11 hari

    MRI

    Interferon beta 1a

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    Neuritis optik atipikal

    Neuritis optik yang berhubungan dengan uveitis maupuninflamasi atau infeksi sitemik

    Diagnosis :

    Tanda klinis yang berbeda dengan neuritis optik tipikal atau

    hasil hasil neuroimaging yang berhubungan dengan penyakit

    selain MS.

    Terbagi atas :

    Infeksius

    Imunologis

    Sarcoidal

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    a | Optic nerve head in demyelinating

    optic neuritis, showing nonspecific optic

    disc swelling without hemorrhages or

    exudates. b | A case of infective neuroretinitis,

    showing disc swelling with hemorrhages

    and macular exudates, also known as a

    macular star (arrow).

    c | Viral optic neuritis with globalnonspecific optic disc swelling.

    d | Syphilitic opticneuritis with optic

    nerve head swelling, hyperemia and

    hemorrhages.

    e | Optic nerve head in HIV-associatedoptic neuritis, showing massive swelling

    with extensive exudates and hemorrhages.

    f | Toxocara-associated optic neuropathy,

    with infiltration, swelling and massive

    distortion of normal optic nerve head

    anatomy.

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    Neuropati non inflamasi

    Ischemic optic neuropathy Anterior (AION)

    Arteritik (AAION)

    Non-arteritik (NAAION)

    Posterior (PION)

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    Characteristic AION NAION

    Age Mean, 70 Years Mean, 60 Years

    Sex F > M F = M

    Associated Symptoms

    Headache, scalp tenderness,

    jaw claudication, transient

    visual lost

    Usually none

    Visual acuityUsually severe (20/200

    in > 60% cases)

    DiscPale swelling common cup

    normalPale or hyperemic, cup small

    ESR Mean, 70 mm/hr Mean 20-40 mm/hrFFA Disc delay and choroid delay Disk delay

    Natural historyRarely improve, fellow eye,

    54%-95%

    16%-42,7% improve, fellow

    eye, 12%-19%

    Treatment Systemic steroids None proven

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    Arteritik- AION

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    Gambaran fundus NAION

    Gambaran FFA NAION

    Non-Arteritik- AION

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    Neuropati non inflamasi

    Compressive opticneuropathy

    Monokular/binokular

    Kronik

    Papil normalpucat (jarangudem)

    MRI ~ massa

    Defek lap pandang : nerve

    fiber bundle; kadanghemianopia (massaintrakranial pada khiasma)

    Massa intraorbita ~ proptosis

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    Neuropati non inflamasi

    Infiltrative optic neuropathy Berhubungan dengan limfoma, leukemia, plasmasitoma, atau

    karsinoma

    Akut-subakut

    Nyeri (-)

    Monokular

    Papil normal-udem

    MRI ~ nervus optik dan menings yang membesar.

    Steroid responsif

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    Neuropati non inflamasi

    Radiation optic neuropathy Toksik pada saraf optik maupun retina akibat pemberian terapi

    radiasi (mis pada KNF)

    Radiasi > 6000 cGy

    Akut

    Papil normal

    Monokular kadang binokular

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    Neuropati non inflamasi

    Hereditary optic neuropathy Defek di DNA nuklear

    Dominan / resesif

    Binokular

    Kronik Tritan axis color blindness

    Papil optik :wedge shaped temporalpallor

    RAPD (-) Defek lap pandang sentral-

    secosentral (dominan)

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    Neuropati non inflamasi

    Leber Hereditary opticneuropathy

    Kelainan DNA mitokondrial

    Monikularbinokolar

    (minggu-bulan)

    Akut - subakut

    RAPD (+)

    Defek lap pandang sentral-

    sekosentral

    Diagnosis ~ pem. Darah

    mutasi

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    Neuropati non inflamasi

    Toxic optic neuropathy Methanol

    Ethambutol

    Isoniazid

    Tobacco & alkohol

    Pada dua mata

    Akut-subakut-kronik

    Defek lap pandang sentral-sekosentral

    Diskromatopsia

    Th/ menghilangkan sumber toksik

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    Neuropati non inflamasi

    Nutritional deficiency optic neuropathy Defisiensi vit B, khususnya B12

    Binokular

    Diskromatopsia

    Defek lap pandang sentral-sekosentral

    Diagnosis : eliminasi penyebab penurunan visus bilateral dan def

    lap. pandang sentral-sekosentral

    Th/ thiamin 50 mg/hari, multivitamin, sianokobalamin. Stop

    merokok dan konsumsi alkohol

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    Neuropati non inflamasi

    Traumatic optic neuropathy 0,5-5% pada trauma kepala Prekanalikular

    Avulsi/evulsi

    Visus hilang pada saat trauma

    Papil ~ lubang abu2 dikelilingihematom

    Trauma 1 cm di belakang bola mata ~CRAO

    Trauma > 1 cm ~ papil normal

    Kanalikular

    Kontusi melalui frontal Nekrosis iskemikkompresi kanal

    Th/steroid dosis tinggi, dekompresikanal

    O ti di h

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    Optic disc changes

    Retrobulbar neuritisEarly compression

    Normal

    Papilloedema

    Papillitis and neuroretinitis

    Swelling

    Optic nerve sheath meningioma

    Occasionally optic nerve glioma

    Optico-ciliaryshunts

    Postneuritic Compression

    Atrophy AION

    Hereditary optic atrophies

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    Papilloedema

    It is a passive, non inflammatory edema of the optic disc due mostly

    to raised intracranial pressure. Most of cases are bilateral.

    Etiology :

    a. Rise of intra cranial pressure due to any space-occupying lesion

    of the cranium, such as brain tumor, cerebral abscess, brain haematoma

    b. Local orbital causes .. Unilateral papilloedema

    c. Certain general diseases, such as malignant hipertention, nephritis,

    leukemia.

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    Clinical features :

    Symptoms, are often negligible

    - Transient blurring of sight lasting from 5-20 seconds- Normal visual acuity for several months, but failure of sight with associated

    with secondary optic atrophy.

    Sign,

    -Hyperemic optic disc with retinal vein engorgement at initial stage

    -At progressive conditions, blurred of the disc margin appear.-In the later stage, swelling of optic disc, 2-8i dioptres

    -Flamed shape haemorrhages and soft exudate around the disc.

    -Exudate of the macula ----- macular star.

    -Field changes : blind spot (earlier), central scotoma (macular involvement),

    concentric visual field (optic atrophy)

    Course

    A short period of oedema ---- little or no permanent change.

    Several weeks or month durations ------ secondary optic atrophy ---- blindness

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    Differential diagnosis: papillitis

    Papillitis

    -Disc swellings 2 D

    -Venous engorgmenet and

    retinal haemorrhages +++

    -Loss of sight sudden and profound

    -Transient and recovery 1-2 weeks

    -Field defect central scotoma for redand green color

    Papilloedema

    -Disc swelling 6D-Venous engorgmenet and

    retinal haemorrhages+

    -Visual loss negligible and gradual

    - Blindness in untreated patients

    -Field defect concentric contractrion

    of the peripheral field

    Treatment

    Remove the underlying causes (intracranial)

    -Tumorremove with surgical intervention

    -Abcess- drainage

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    Primary Papil Atrophy

    Causes:

    -Following retrobulbar neuritis

    -Compression by tumours or

    aneurysm

    -Hereditary optic neuropathy

    -Toxic and nutritional opticneuropathy

    -Visual pathway lesion

    (retrolaminar ON to LGB)

    Flat, clear delienated

    margin

    No antecedent ONHswelling

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    Secondary Papil Atrophy

    Preceded by ONH swelling

    White/dirty grey

    Slightly raised

    Poorly delienated margin

    Causes:

    -Chronic papilloedema

    -AION-Papilitis

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