Good Hope, Heartlands, and Solihull Eye Clinics

Optic nerve
(not glaucoma; notes, links)

David Kinshuck, from lectures

Abbreviations

  • APD=afferent pupillary defect
  • pmr = polymyalgia rheumatica
  • GCA = giant cell arteritis
  • NAION = None-arteritic ischaemic optic neuropathy
  • crp /CRP = blood test (thick/sticky blood)
  • ESR = another similar blood test (thick/sticky blood)
  • TAB = temporal artery biopsy
  • wr = a test for syphilis
  • dob = date of birth
  • OSA  = obstructive sleep apnoea

General

  • Optic nerve needs athens key
  • City Hospital...headache nurse Julie Edwards
  • IIH...Mr Mike Burden, QE

Anterior ischaemic optic neuropathy

3 types

  1. None-arteritic
  2. Perioperative
  3. arteritic

features

  • Always APD unless bilateral
  • Often peripapillary flame shaped haemorrhages
  • Smaller discs, less cup...look at other eye = Disc at risk
  • subretinal fluid may be present Eye 14

NAION: None-arteritic ischaemic optic neuropathy

This is described here.  Free Eye 15   photo 1   photo 2   composite   after..optic atrophy

In this condition the arteries to the otic nerve block and the artery infarcts.

  • there is no inflammation as there is in  GCA ION
  • patients are younger
  • some are arteriopaths..that is they smoke
  • it is sometimes associated with a drop in blood pressure
  • review SO16

The optic nerve of such patients tend to be smaller than normal, and the optic canal, the bony canal through which the optic nerve passes, narrower than normal. Such an optic nerve is said to be 'nerve at risk' or a 'crowded' disc'. In a patient when the condition is brought on by a drop in blood pressure, there should be no further episodes. But if there are no 'precipitating' factors the condition may recur later in the other eye.

So a typical patient,

  • probably with a small optic nerve 'at risk',
  • may be an ex-smoker,
  • may have some hip surgery.
  • After the surgery that may develop haematemesis (vomiting blood) and
  • the blood pressure may drop, and then
  • the vision goes (as the optic nerve infarcts due to lack of blood supply).

There is no treatment.

  • Aspirin is prescribed.
  • Smoking needs to stop

Occurs

  • Male= female; age 11-90; peak 55-70;

Risk factors

  • Sudden drop in blood pressure (eg haematemesis)
  • Diabetes, Smoking, hypertension, high cholesterol, obesity,
  • Disk at risk (a congenitally small optic disc: small, crowded, a compartment syndrome develops if there is any damage at all)
  • obstructive sleep apnoea OSA,
    • very common,
    • often not obese
    • 75%. OSA, risk four times higher getting ion
    • But common in control group, may be a marker of systemic diseases
    • ask partner...is snoring worse sleeping on back
  • Low bp..Hypotension
    • ? Due to time of bp drug taking
  • Also erectile dysfunction drugs, Viagra,  vasodilate and cause hypotension
  • anaemia, risk even without hypotension..need to restore haematocrit
  • hyperhomocysteinaemia.. can lead to thrombogenesis
  • Coagulapathies; Interferon alpha; migraine; cataract surgery
  • Amiodarone, helps with heart problems, very variable half-life, 6 x risk, Bilateral simultaneously, resolves weeks. search 16
  • interferon JNO 15

Pathogenesis

  • Blood flow reduced in central retina artery..this causes a little swelling..this then obstructs flow further  (a 'compartment syndrome')
  • Probably a laminar cribrosa capillary problem
  • possibly hypertension, hyperlipidaemia, and sleep apnoea

Findings

  • Swollen disc
  • Pain rare , never severe pain, no pain on movement,
  • Vision very variable, field defect or hm
  • Colour vision mirror acuity
  • Field any defect, typical arcuate, usually inferior
  • Can have incipient ion..disc swelling with haemorrhage, which can go on to get ION with loss of sight ...incipient...swollen disc but good vision

Rate of development/effect

  • Spontaneously
  • wake up with reduced vision
  • After surgery with a sudden drop of blood pressure, e.g. spinal surgery
  • Risk is increased 1 year after cataract surgery
  • Loss of sight is sudden; any part of field, or altitudinal;
  • 90% painless 10% achy from ischaemia
  • loss of colour vision.
  • 42% improve, small % progress
  • other eye 33%..don't get dehydrated

On examination

  • swollen disc if anterior; posterior, is less common: FFA shows leakage
  • Must differentiate from gca....test crp, esr, ...?Biopsy temporal artery
  • always rapd, sometimes with flame haemorrhages, disc hyperaemic and oedematous in acute stage

differential

  • Sudden deterioration, episodes of transient, or step over a few days, age  40,
  • 55..gca
  • AION, none-arteritic: sudden loss, painless, swollen disc, apd, peripapillary, small crowded disc
  • 10% on something else
  • ? How vision lost..slow...? meningioma, breast secondary, alcoholic, industry with toxins,
  • test..vision, ocular systematic, crainials,

Later

  • Disc selling persists 2 months then becomes pale!!!
  • Arteritic leads to a cupped disc
  • 49% improve spontaneously

Other eye

  • 20% 5y
  • No prophylactic therapy other than reducing risk factors Systemic risk of further vascular events, TIA,CVA, ni , etc

Case

  • Painless blurred vision waking up
  • Was inferior, then affected superior field
  • High lipids and bp 6/18, inferior field defect & disc at risk

Optic neuropathy due to SSRI drugs

Eye 15...could this be a coincidence?

 

Optic neuropathy in inborn errors of metabolism

 

Arteritic ischaemic optic neuropathy (Giant Cell Arteritis) ION

This is a condition of the elderly. It is explained in detail NIH.  The arteries to the eye become inflamed and block. Other arteries can be affected. Without its blood supply, the optic nerve 'infarcts'. It becomes swollen, and later pale. The sight is damaged; often half or nearly all the sight is greatly reduced in that eye. The condition can affect the other eye.

The disease is an 'inflammatory' disease, and will be stopped with steroid treatment (in most cases). The steroid dose has to be very high.

Because the steroids have such serious side effects, it is very important to make the correct diagnosis

  • in 90% of patients the ESR and CRP are very high
  • borderline cases, test bloods, and unless CRP completely normal, consider urgent FFA to check disc
  • in nearly all patients a temporal artery biopsy will show the condition (if enough artery is biopsied, at least 2 cm.)
  • ~50% patients have other GCA symptoms, loss of weight, headache, feeling unwell, anaemia, for the last 3 months
  • 50% will have had polymyalgia...that is shoulder stiffness and muscle aches, jaw aches when eating (claudication)
  • Central retinal artery occlusion without embolus may be gca without embolus; if there is an embolus this is not GCA
  • Cupped disc before or after is not none-arteritic (this always has a disc at risk) but it may be GCA
  • GCA..there may be isolated cotton wool spots
  • Transient diplopia or visual loss in elderly may be GCA
  • If both circulations are affected..retina and nerve..this is GCA not NAION, e.g. retinal and disc swelling
  • definitive diagnosis from biopsy of temporal artery
  • Headache is a late feature of gca
  • If there is a lump over the temporal artery, with pulsation...think GCA
  • Pmr with incomplete response, ?gca
  • High platelets, anaemia also = GCA
  • generally start treatment before all test results, to avoid more damage to the optic nerve
  • Can get GCA age 50y
  • Pain in head localised, pain over artery, scalp tenderness, swelling over artery = 71% GCA
  • biopsies of temporal artery (TAB) 40% sensitivity of tab
  • Ultrasound of artery can help, ask peter good, can do combination
  • severe headache, thinks of subarachnoid haemorrhage or dissection, or recent meningitis
  • thinks of secondary causes of optic neuropathy such as vascular, space occupying lesions, infections, sinuses, etc,
  • Algorithm JNO 15
  • MRI changes Eye 16 enhancement, 4 types:
    1. none-specific orbital
    2. optic neve parenchyma
    3. perineural sheath
    4. chiasm

Ethinicity

  • GCA does occur in African-Americans and in Asian patients, but is probably less common. Eye 17

Specific symptoms in African-Americans (slightly different from caucasians)

  • Eye 17
  • fever 11%;
  • weight loss 40%; malaise ~25%; anorexia 20%;
  • headache 75%;
  • jaw claudication 30%; neck pain 56%; scalp tenderness 24%; myalgia 17%; anaemia 34%
  • mean age 72y, caucasion 76y.

Genes

Cases

  1. first
    • Pain or stiffness in legs, crp very high,
    • given steroids, esr did not drop, rheumatoid factor+ ve,
    • but then later did not get better,
    • biopsy GCA (condition was GCA)
  2. Another patient
    • Started with pmr symptoms and very high esr, steroids, methotrexate Knee effusions, tired,
    • eventually lumps on head and headache,
    • gca biopsy positive (condition was GCA)
  3. another patient
    • Biopsy negative but patient still had headache and inflammatory markers raised
  4. another
    • 72y lady high crp and headache
    • better with steroids, biopsy negative,
    • but steroids reduced, headache returned,
    • eventually..lymphoma etc

Normally in Birmingham treatment is started and the biopsy carried out at Birmingham and Midland Eye Centre. Patients are then transferred to the neuro-ophthalmology team at the QE. The treatment...

  • high dose intravenous methyl-prednisolone for 3 days
  • ESR  CRP, temporal artery biopsy, full examination and other tests are carried out
  • steroid tablets are started at a high dose
  • typically after a month the dose of prednisolone will have been reduced to 20 mg; after that by the second month 10mg ugh t be achieved, and then the dose reduced by 1mg a month for the next 10 months
  • the steroid dose has to be increased if the ESR rises significantly
  • we need to find alternative treatment to steroids so we can reduce side effects
  • Walvick 2011 " The odds of a positive biopsy were 1.5 times greater with an erythrocyte sedimentation rate of 47 to 107 mm/hr, 5.3 times greater with a CRP >2.45 mg/dL, and 4.2 times greater with platelets >400,000/μL."

Arteritis, usually giant cell

  • mostly giant cell arteritis, but there are other types
  • high % Caucasian
  • sudden loss, episodes of amaurosis;
  • confused

On examination

  • swollen disc if anterior; posterior, is less common: FFA shows leakage
  • pale infarcted disc; cannot improve infarcted nerve
  • cotton wool spots in retina

Manage

  • 5% progress even with steroids
  • admit, talk to relative, talk early on,
  • 3 days of intravenous methyl prednisolone
  • biopsy temporal artery with a week, decent length of artery needed
  • taper off steroids reduce from the high does quickly, slower rate of reduction at lower doses
  • no effective steroid sparing agents
  • initially, clinical diagnosis; if the biopsy negative another diagnosis
  • Bloods to decide gca or not, naaion rarely worse, see 3 visits over 9 months, some get a little better

PMR

  • polymyalgia...dont trust symptoms when on steroids, use ESR/CRP

Headache and high ESR.

  • patient should be under care of physician not ophthalmologist
  • ophthalmologist role is just to help with biopsy

Link

 

 

Occult giant cell arteritis

As GCA above, but note:

  • 20% GCA is occult, that is there are no disc signs or headache or PMR or other symptoms
  • may present as vague loss of sight and double vision
  • any older person with loss of sight that is not specifically amd etc, consider
  • test bloods, and unless CRP completely normal, consider urgent FFA to check disc
  • FFA should demonstrate the choroidal ischaemia
  • Hayreh 98   case  Mitra    Lancet14

Atypical GCA

  • typical..Headache, visual, PMR, temporal artery firm
  • Atypical
    • Tender supraorbital nodule
    • Diplopia, vertical, headache, firm temporal artery
    • Anterior segment ischaemia
    • third n palsy
    • CVA. Hemianopia with headache and jaw tender
    • Facial nerve palsy
    • Youngest age 56y  Most of the patients. had a headache, 1 jaw claudication, none fever

Ocular ischaemia

surgery helps Eye 2012

 

Disc swelling/papilloedema

Papilloedema

  • = disc swelling and raised intracranial pressure combined

Investigate

  • history
  • at an Eye Centre..B scan
    • fluid around optic nerve indicates disc swelling.. can rule out disc drusen quickly
    • highly sensitive Eye14
  • MRI
  • HVF (visual fields)
  • lumbar puncture
  • investigate all patients if overweight
  • monitor fields if waiting for neurosurgery
  • Recognize: Refer: Monitor
  • drug history, eg Minocycline   tetracycline JNO 16

Papilloedema patients

  • get transient obscuration, when changing head position
  • pulsatile tinnitus

Papilloedema unusual if

  • asymmetric, unilateral, choroidal folds alone
  • Pseudopapilloedema
  • drusen with blurred disc margin
  • headaches (these are common)
  • can still get obscuration
  • small crowded disc

Note

  • spontaneous venous pulsation suggests papilloedema
  • ultrasound will show distended optic nerve sheath (papilloedema)
  • OCT shows thickened nerve fibre layer at disc
  • differential diagnosis: SLE & disc swelling; diabetic papillitis; pale (meningioma)

Definite papilloedema

  • refer, lumbar puncture, neuro-ophthalmologist
  • possible: neuro-ophthalmologist

papilloedema due to IIH?

papilloedema, probable IIH  enlarge

 

Cerebral venous thrombosis

Presentation

  • headache, papilloedema, proptosis, many other presentations
  • ?B scan of optic nerve to confirm papilloedema
  • must use contrast ...ct venogram is fine, takes 5 minutes, not MRV
  • 33% have thrombophilia; local trauma, infections
  • anticoagulants help
  • shunt quickly if sinus thrombosis and papa ill oedema confirmed

 

IIH: idiopathic intracranial hypertension

IIH occurs 90% in women, and 95% are <50y age, and is linked to obesity. Patients present with headaches and papilloedema (a type of swollen optic nerve). There may be blurred vision, or double vision. There may be visual obscurations (the sight reduces for a few seconds and returns by itself) and pulsatile tinnitis (noises in the ear linked to heart beating). Brain scans are needed and the intracranial pressure (measured by a lumbar puncture) is raised. The scan will need to exclude other conditions. IIH was formally termed benign intracranial hypertension. Eye news 17:

Diagnostic criteria

  1. Symptoms and signs of raised intracranial pressure including papilloedema.
  2. Elevated CSF pressure >25 (left lateral decubitus position). This is raised intracranial pressure (ICP). Maximum healthy ICP are 25 adult, 28 children. 
  3. Normal csf protein etc.
  4. No venous sinus thrombosis on CT/MRI angiography.
  5. No secondary causes such as hydrocephalus, space occupying lesion, etc.
  6. No cns signs except cranial nerve palsy.

Weight loss

  • there are many studies indicating that losing weight helps in the condition. 2017
  • Other treatments merely help headaches or lower the pressure temporally (acetazolamide) or more long term such as CSF shunting operations
  • bariatric surgery may turn out to be the best treatment for the condition
  • Exenatide (an anti-diabetic drug) may be very helpful treating idiopathic intracranial hypertension. This is a glucagon-like peptide-1 (GLP-1) receptor agonist 2017.

Headaches

  • The headaches after the papilloedema may still be unpleasant, and are due to a combination of raised intracranial pressure, medication overuse, stress or migraine.
  • An exacerbation of headache may respond to ibuprofen (but need to check for papilloedema).
  • Topiramate 2007

New research: a sequence of events

  • New studies identify that there may be anatomical abnormalities causing stenosis of the dural sinus (congenital). But in addtion some of this stenosis may be from brain swelling linked to a certain type of biochemistry, and it is the combination that causes the condition. The biochemical problem is linked to a congenital difference in the patients from normal and the combination with obesity.
  • Thus IIH is due to a combination of obesity, which due to genetic biochemical differences, causes extra brain swelling around the dural sinuses in the brain, and the narrow dural sinuses, which then obstructs the flow of CSF, and this ncreases the intracranial pressure.

Symptoms

  • 92% headache
  • 72% obscuration
  • 60% noises/tinnitus
  • diagnosis of exclusion
  • Headache commonest sign, but 10% no headache

Examination

  • if IIH is suspected, a lumbar puncture is needed
  • a lumbar puncture is needed to exclude other diseases : pressure/protein/cytology/glucose ned examining
  • MRVenography to exclude sinus thrombosis
  • fields
    • full at onset, later field loss
    • large blind spot, generalised constriction, reversible
    • arcuate/altitudinal, (infarcts, not reversible)

Children

  • IIH affects children, more girls, but is commoner after puberty
  • if may follow recent weight gain in older children
  • Younger children half overweight, older most overweight,
  • Downs, turners, sickle cell, anaemia, transretinoic acid, growth hormone, thyroxine etc all may contribute
  • may need sedation for lp, deep sedation, have slightly higher opening pressure.
  • VIn palsy and papilloedema children.

Treat papilloedema itself

  • azetazolamide (diamox) helps JAMA 14   JNO 16    JNO 16
    • older patient 4 g day maximum
  • Frusemide acute to buy time, steroids, surgery
  • Shunt, for fulminant, progressing, or severe headache
  • if the IIH is severe with a really swollen disc a shunt is often needed

Treat underlying problem, obesity

  • treating obesity is the main treatment
  • Bariatric Surgery JNO 16...  Bariatric surgery helps..20. Minutes for gastric band
  • Weight loss helps, androgen pathway improves 5 alpha reductse...this is linked to papilloedema.
  • Psychological treatment is the main treatment to help patients lose weight, as they put weight on tinnitus and headaches and papilloedema returned.
  • 11beta hsd1 elevated in obesity in Choroidal plexus...this level reduces with weight loss and is related to improvements in papilloedema
  • Glp1 glucagon like peptide 1, which reduces appetite, liraglutide, may be helpful 2017

Monitoring

  • retest fields if stable JNO 16 

Problems

  • How do you persuade patients to lose weight, weight loss is the treatment, and can discharge, work out which patients can lose weight and which can't.
  • Patients don't like term obesity, overweight not the first visit, discuss later in outpatients
  • Headache is the main determinant of quality of life
  • increasing in incidence due to increasing obesity
  • empty sella (effect of high ICP)
  • disc haemorrhages increase risk BJO 17
  • stop minocycline (can cause IIH)

 

Support

 

Case1

  • 16y girl, 6 week headache,good vision, lp 25
  • Spontaneous venous pulsation present os not papilloedema
  • drusen b scan optic nerve and needed to treat tension headache

Case2

  • Elevated nerves and field defect..hyperaemic disc swollen= iih
  • 24 h lp monitoring, pressure 

 

Diabetic papillopathy

Ophthalmology 2012.

'Diabetic papillopathy was associated markedly with a drastic recent reduction in glycemia and a small cup-to-disc diameter ratio. This supports the hypothesis that diabetic papillopathy may be an early worsening phenomenon occurring in anatomically predisposed patients in response to a recent rapid decrease in glycemia'.

Photos

Diabetic ischaemic optic neuropathy

A devastating complication that is common in heavy smokers. Essentially the optic nerves infarct..the condition is usually bilateral, but may not occur in both eyes a he same time.

 

 

disc drusen page optic disc drusen large
papilloedema (sinus thrombosis) papilloedema from sinus thrombosis large
papilloedema (IIH) papilloedema due to idiopathic intracrainial hypertension papilloedema due to IIH largeOCT    large

 

Low B12

NEJM 2013

 

Alcohol, nutritional nutritional deficiency optic neuropathy

  • Slowly progressive binocular visual loss from optic neuropathy caused by systemic chronic nutritional deficiency, particularly of B vitamin
  • Chronic alcoholism and severe malnourishment are principal settings
  • Adequate nutrition and cessation of alcohol intake only effective remedies
  • Visual recovery unpredictable

Clinical Findings

  • Slowly progressive painless binocular visual loss, symmetric visual acuity loss rarely worse than 20/200, pupil reactions normal, optic discs normal or show temporal pallor, central or caecocentral scotomas, profound acquired color vision loss, peripheral neuropathy common

Examine

  • Delayed latency visual evoked potentials provide objective evidence of optic neuropathy
  • Nerve conduction velocity testing helps diagnose associated peripheral neuropathy
  • Blood count to rule out anemia of vitamin B 12 deficiency

Differential Diagnosis

  • Toxic optic neuropathy Hereditary optic neuropathy (dominant or Leber type) Optic neuritis Compressive optic neuropathy Paraneoplastic optic neuropathy Psychogenic visual loss

Treatment

  • Improve diet, stop alcohol intake,
  • single-dose intramuscular thiamine 100 mg followed by oral thiamine 50 mg/day indefinitely (we recommenced Vitamin b compound strong 3 times/day)
  • Folic acid 5 mg once day
  • intramuscular vitamin B 12 if appropriate
  • alcohol services

Prognosis

  • Visual recovery depends on extent of damage

Retinal artery occlusion

  • excellent review
  • risk: BP, cholesterol, diabetes
  • investigate: carotid and echocardiogram (ECG)
  • rigors..important AE 2015
  • young patients <50:
    • hypercoagulable screen (protein C/S, factor 5 Leiden, anti-phosopholipid antibody)
    • vasculitic screen (ANA, ENA, ANCA, ACE)
    • myeloproliferative disease /sickle cell
  • treat
    • acute
      • sublingual isosorbide, hyperbaric oxygen
      • ocular massage to dislodge embolus
      • intravenous diamox, + anterior chamber paracentesis, withdraw small amount of aqueous (increase perfusion pressure) if within 6 hours
      • streptokinase (thrombolysis, or standard thrombolysis treatment) did produce a little improvement in results, but far more adverse events, some serious ...not recommended

Brain tumours in children

see www.bmj.com

brain stem

  • 10%: gait, coordination, cranial nerve, pyramidal, headache,squint,facial palsy, papilloedema
  • 5-10%: ICP features, abnormal eye movement, behavioural change/school problem

cerebellar

  • >10%: nausea, vomit, nausea,vomit, headache, gait, coordination, papilloedema, abnormal eye movement
  • 5-10% ICP features, weight loss, facial motor weakness, reduced consciousness, vertigo, auditory, squint, stiff neck,head tilt, accidental head injury

cerebral hemisphere

  • >10%, as others, seizures
  • 5-10%, as others diabetes insipidus, hemiplegia, reduced visual fields, behavioural change, developmental and school problems, facial motor, short stature, weight loss, vertigo, auditory, vision and other eye problems

Case (notes from Mike Burden talk 2013)

patient, 50y, Could not do ischiara..could see the colour of the dots, but, could not interpret make into number: posterior cortical atrophy

 

Optic atrophy

  • test colour, field, acuity,
  • APD reduced, disc any appearance,
  • normal disc can still be optic neuropathy
  • Large differential diagnosis

Optic neuritis

  • Optic neuritis treatment trial
  • acuity,field, any field defect
  • 33% disc swollen, probably no haemorrhage,
  • most patients had mild pain around eye worse worse movement,
  • certain age likely diagnosis 18-45 y.
  • Investigate if not confident
  • Progressive hours days, then gets better
  • Normal disc mild swelling
  • 2-5% on trial did not get good recovery, but not typical, so there could be another cause so investigate Diagnosis at the beginning is provisional, see fours weeks later to confirm getting better and must investigate
  • No specific test
  • Smoking, infection, vitamin d, all important and need addressing
  • Clinical isolated syndrome: CIS, IO14
  • Spontaneous near total recovery of visual function within 6 months in >85%
  • Differential diagnosis: Atypical optic neuritis: Ischemic optic neuropathy, compressive optic neuropathy , infiltrative (neoplastic) optic neuropathy, Leber hereditary optic neuropathy, Paraneoplastic optic neuropathy

Exclude

  • Cancer, vasculitis, autoimmune disease
  • disc pale means long standing not typical, cells not typical
  • not typical investigate 

Explain

  • cannot biopsy, no point biopsying another area of the body or testing...if it is just a tiny area of inflammation around the nerve, explain better Monitor visual function Cannot diagnosis everything, but need proper history exam, think of alternatives, eg think GCA, must use imagination to think what else might be going on, image with contrast, No mri typical, do do bloods, routine and autoimmune, wr for syphylis
  • a small number of patients may have a steroid response, and these may need to continue.

Typical

  • Eye News 16
  • age 20-50y, Caucasian,
  • if related to pregnancy...within 3-6m post partum,
  • rarely related to other inflammatory diseases
  • monophasic,
  • unilateral,
  • pain around eyes for 2-3 days before symptoms,
  • progressive visual loss 2d-2w,
  • colour and contrast significantly reduced
  • spontaneous recovery to  better/= 6/12 in 80%
  • any field defect
  • steroids hasten recovery
  • disc normal or swollen but not severely swollen. Rarely haemorrhages or exudates
  • ac cells,retinal vasculitis (in MS)
  • 85% optic neuritis enhanced optic nerve with MRI
  • All sorts of things present at optic neuritis..atypical optic neuritis..full investigations
  • Studying unexplained optic neuritis, 20% compressive
  • Managing expectations...diagnosis, treatment, improve...not often can do these
  • Review everything 

neuromyelitis optic neuropathy

Case 1

  • Age 54 y lady
  • 3d slight ache left eye diabetic, periocular pain worse with movement
  • Swollen left nerve..
  • Mri nerve enhanced, diagnosis optic neuritis 

Case 2

  • Lost left eye..diagnosed ischaemic optic neuropathy
  • But then over a year. Lost field right eye.
  • Mri slight enhancement...sarcoid diagnosed
  • This patient...sarcoid..\z the prednisolone dose was reduced thw condition returned,
  • condition termed Crion=
  • Chronic relapsing (steroid dependant) optic neuropathy

Case 3

  • Blurred vision recent, hyperphagia, enhanced hypothalamus, devic, aquaporin 4 enhancement
  • nmo..neuromyelitis optic neuropathy ....retuximab needed
    Bilateral adults, Hypothalamic symptoms,  S
  • devics disease
  • aquaporin 4 enhancement 70% sensitive 

Case 4

  • Probable left ion, count fingers
  • Patient says getting gradually worse ..
  • review mri..meningioma seen

Case 5

  • Afrocarribean
  • Lost vision after 3 d pain, but no recovery
  • Needs mri contrast, ca ace, ace raised 112
  • Aquaporin negative, cxr normal 
  • Will monitor, no treatment yet, no other signs of sarcoid 

Paediatric optic neuritis 

Case 6

  • Age 6
  • Blurred vision left apd, reduced field
  • Swollen left disc  
  • Drusen with anterior ischaemic optic neuropathy, after 4 weeks drusen became evident 

Acute optic neuritis children 

All trans retinoic acid...papilloedema with optic neuropathy. Ethambutol,

Case age 7

  • Bilateral papilloedema,mild, secondary to viral illness

 

Children and headaches

Most children with headaches do not have ophthalmic abnormalities JPOS 14

 

Transient Monocular Visual Loss (Amaurosis Fugax)

  • from Jonathan D. Trobe MD Rapid Diagnosis in Ophthalmology: Neuro-Ophthalmology
  • Amaurosis fugax is an old term that is out of favor because it does not specify whether transient visual loss is monocular or binocular Defined as a sudden visual loss affecting one eye that lasts <60 min
  • Sometimes associated with scintillations (photopsias, positive visual phenomena)
  • Caused by reduced perfusion of eye (ocular transient ischemic attack, TIA)
  • Common causes:
  • cervical carotid stenosis
  • systemic hypotension
  • idiopathic (possible retinal artery vasospasm)
  • impending retinal or optic nerve infarction
  • papilloedema (DK: more like trainsient obscurrations, lasting seconds)
  • Evidence that carotid endarterectomy benefits patients suffering only ocular TIA is weak

Clinical Findings

  • Eye examination is usually normal but may show intra-arterial retinal platelet–fibrin–cholesterol (Hollenhorst) plaque, optic disc edema, or venous stasis retinopathy
  • Examination
    • Carotid ultrasound, CT angiography, or magnetic resonance angiography to rule out stenosis, dissection, and dysplasia
    • Blood pressure (including orthostatic) testing to rule out hypertension or hypotension
    • Electrocardiography to rule out atrial fibrillation
    • Cardiac echography to rule out cardioembolic source
    • Blood tests to rule out hypercoagulable state:
      • complete blood count
      • lipids
      • HbA1c
      • serum protein electrophoresis
      • prothrombin and partial thromboplastin times
      • antiphospholipid antibodies antithrombin-3
      • factor V Leiden
      • prothrombin gene mutation
      • homocysteine
      • sickle hemoglobin
      • serum viscosity

Differential Diagnosis

  • Embolism from cervical carotid artery, aortic arch, or cardiac valve or wall
  • Systemic hypertension or hypotension
  • Hypercoagulable state
  • Impending retinal vascular occlusion
  • ocular ischaemia
  • Retinal vasospasm (see Retinal migraine)
  • Papilledema

Treatment

Direct at underlying condition: Endarterectomy often advocated for >70% ipsilateral cervical carotid stenosis, but evidence of benefit for purely ocular TIA is weak Reduce modifiable arteriosclerotic risk factors (diabetes, hypertension, dyslipidemia, lack of exercise, obesity, cigarette smoking) . Correct very high blood pressure but avoid excessive blood pressure lowering (may lead to perfusion failure and stroke of eyes or brain) . Aspirin 81 mg/day for underlying arteriosclerosis.

 

Transient Binocular Visual Loss

Abrupt visual loss affecting both eyes and lasting <60 min, caused by dysfunction of visual cortex or optic nerves in both eyes

Common causes

  • migraine
  • visual cortex transient ischemic attack (TIA)
  • visual cortex seizure
  • papilloedema

History

  • Migraine: scintillations (often with zigzag border) that march across one hemifield and last 20–30 min; headache may follow
  • TIA: homonymous or total visual loss lasting minutes that may have scintillations; headache is rare
  • Focal seizure: stationary, often colored visual scintillations lasting seconds to minutes; persistent homonymous hemianopia sometimes
  • Papilloedema: blackouts of vision lasting seconds, often precipitated by upright posture

Examination

  • ?TIA: echocardiography or vascular imaging may show source of emboli
  • cardiac telemetry may show atrial fibrillation blood pressure testing may show hypertension or hypotension.
  • ?focal seizure:
  • brain imaging may show mass, encephalomalacia, enhancing meninges electroencephalography may show epileptic focus.

Papillitis

  • from Jonathan D. Trobe MD Rapid Diagnosis in Ophthalmology: Neuro-Ophthalmology
  • also termed neuroretinitis: a term used to signify that the optic disc and retina are both inflamed, but the process originates in the optic nerve, so papillitis is a better term
  • A form of optic neuritis in which the optic disc is swollen and sometimes there are disc surface hemorrhages and perifoveal hard exudates (macular star figure)

Common causes:

  • idiopathic, cat scratch disease, syphilis, Lyme disease, toxoplasmosis

Clinical Findings

  • Acute uniocular visual loss
  • sometimes accompanied by ipsilateral periocular pain exacerbated by eye movement
  • Reduced visual acuity and/or nerve fiber bundle visual field defect
  • Afferent pupil defect
  • Optic disc swelling, often florid, sometimes with disc surface hemorrhages
  • Perifoveal hard exudates (macular star figure) typically become prominent only as disc swelling is disappearing Sometimes accompanied by vitritis or retinal perivascular cuffing (vasculitis)
  • Usually an isolated clinical manifestation

Examine

blood count, electrolyteshepatic, renal, or coagulation profile, aESR/CRP, chest x-rays,yphilis, Bartonella, Lyme, toxoplasmosis serologies

Differential Diagnosis

Ischemic optic neuropathy, Leber hereditary optic neuropathy, Infiltrative optic neuropathy, Hypertensive optic neuropathy, Diabetic papillopathy, Papilledema, Central retinal vein occlusion, Paraneoplastic optic neuropathy

Treatment

Corticosteroids (dose depends on diagnosis and severity of visual loss): Corticosteroid-sparing agents (mycophenolate, cyclosporine, methotrexate, azathioprine, tumor necrosis factor inhibitors) may be used if patient is corticosteroid-intolerant or requires chronic intensive therapy: Antibiotics for infection, if present

 

Posterior Ischaemic Optic Neuropathy

  • Acute visual loss (usually monocular) with features of optic nerve dysfunction
  • No optic disc edema
  • Common causes: previous cranial radiation intracranial surgeryacute systemic hypotension, herpes zoster or sino-orbital fungal infection systemic vasculitis (Wegener granulomatosis, giant cell arteritis, lupus erythematosus, relapsing polychondritis, polyarteritis nodosa)

Hypotensive Ischaemic Optic Neuropathy

  • Acute monocular or binocular visual loss caused by infarction of optic nerve as the result of systemic hypotension
  • Most patients have arteriosclerotic risk factors: cardiac bypass or prolonged lumbar spine surgery most common setting
  • Systemic hypotension often documented in anesthesia record
  • Visual loss often not apparent until days after surgery Optic disc(s) may be swollen or normal at outset. If normal, may swell days later
  • Affected discs become pale weeks later
  • Visual recovery is variable, no effective treatment

Clinical Findings

  • Visual loss reported within days of surger
  • yVisual acuity and/or visual field loss (nerve fiber bundle defects) in affected eye(s)
  • No afferent pupil defect if both eyes affected equally
  • Fundi are normal or show swollen optic disc(s)

Examine

  • Haemoglobin often very low (from blood loss)
  • Orbit and brain imaging is usually normal

Differential Diagnosis

  • Bilateral occipital lobe infarction, pituitary apoplexy, optic neuritis

Treatment

  • Correct systemic hypotension: Correct anemiaHyperbaric oxygen is ineffective

Prognosis

  • Recovery of vision may occur within weeks but not later

Toxic Optic Neuropathy

  • Slowly progressive binocular visual loss from optic neuropathy caused by systemic medications or toxins
  • Ethambutol is the most common medication,
  • methanol the most common toxin
  • Alcohol may also be an optic nerve toxin but is still listed under nutritional deficiency optic neuropathy (see Nutritional deficiency optic neuropathy )
  • Withdrawal of toxic agents is the only effective treatment Visual recovery is more likely if offending agent is removed early

Clinical Findings

  • Slowly progressive painless binocular visual loss
  • Symmetric visual acuity loss in the two eyes rarely worse than 20/200
  • Pupil reactions normal Optic discs normal or show temporal pallor
  • Peripapillary nerve fiber layer edema present in methanol toxicity
  • Central or cecocentral scotomas
  • Acquired color vision loss
  • At-risk patients: those taking >15 mg/kg per day of ethambutol or those who have ingested methanol as a substitute for ethanol

Examine

  • Blood chemistries show acidosis in acute methanol toxicity
  • Methanol toxicity: brain imaging may show high T 2 signal in basal ganglia and parieto-occipital white matter

Differential Diagnosis

  • Nutritional deficiency optic neuropath, hereditary optic neuropathy (dominant or Leber type) , optic neuritis, compressive optic neuropathy, araneoplastic optic neuropathy, psychogenic visual loss

Treatment

  • Discontinue ethambutol , treat methanol acidosis

Prognosis

  • Visual recovery depends on extent of damage

 

Dominantly Inherited Optic Neuropathy

  • Slowly progressive binocular visual loss during first decade
  • Binocularly symmetric visual acuity loss
  • Dominant inheritance
  • Mapped to chromosome 3q No treatment

Clinical Findings

  • Slowly progressive binocular visual loss during first decade
  • Visual loss ranges between 20/20 and 20/200 and is usually symmetric
  • Bilateral central or cecocentral scotomas
  • Wedge-shaped temporal optic disc pallor
  • Some patients have sensorineural hearing loss

Ancillary Testing

  • Brain imaging to rule out compressive optic neuropathy unless all findings are classic, including clear family history
  • Genetic studies valuable for documentation

Differential Diagnosis

  • Compressive optic neuropathy, psychogenic visual loss

Treatment

  • None

Prognosis

  • Visual loss stabilizes by end of first decade but does not recover

Leber Hereditary Optic Neuropathy

  • Subacute visual loss affecting one eye then, after weeks to months, the fellow eye
  • Males aged <40 years most at risk
  • Slightly swollen, telangiectatic peripapillary nerve fiber layer in affected eye and sometimes in unaffected fellow eye
  • Diagnosis based on finding mutation at mitochondrial DNA position 11778, 3460, 14484, or 14459
  • Visual loss is generally irreversible No known treatment

Clinical Findings

  • Subacute onset of painless visual loss in one eye
  • Same event weeks to months later in fellow eye
  • Visual acuity of 20/100 to finger counting and central or cecocentral scotomas
  • May be no afferent pupil defect even when only one eye has suffered visual loss
  • Telangiectatic swelling of peripapillary nerve fiber layer in affected eye and sometimes in unaffected fellow eye (pre-eruptive stage)

Ancillary Testing

  • Blood test for mitochondrial DNA at position 11778, 3460, 14484, or 14459 will be positive in nearly all cases

Differential Diagnosis

Optic neuritis, compressive optic neuropathy, posterior ischemic optic neuropathy, anterior ischemic optic neuropathy, paraneoplastic optic neuropathy, psychogenic visual loss

Treatment

  • Patients often placed on mitochondrial cocktail, which includes coenzyme Q10, vitamin E, and B vitamins but no evidence of benefit
  • Patients often advised to stop smoking to avoid free radical generation

Genetic counseling may involve testing of clinically unaffected family members

Prognosis

  • Visual loss usually irreversible: partial recovery may occur with DNA mutations at positions 14484 and 3460

Inherited optic neuropathy

see an  excellent article, also    CML14

Case a (Congress 2014)

  • 1970 dob, male
  • 2006 labarynthitis..tinnitis and vertigo, and deafness, cochlear implants and hearing aids
  • 2007 CLL leukaemia
  • 2010 thickened optic nerves
  • later, uveitis episcleritis, bilateral
  • diagnosed as cogans syndrome, treated with steroids

Case b (Congress 2014)

  • 1990 dob, male
  • 1 week left sided headache, left orbit pain, right facial pain, treated as ear infection
  • confusion later, diplopia, got worse, ct scan normal;
  • more ataxic, unsteady, areflexic, bilateral 6th and left 3rd, then lost vision in left eye
  • = lymphoma

Case c (Congress 2014)

  • 2000 dob,
  • 3rd nerve palsy
  • epsodes of headache, migraine like
  • next day complete ptosis, lasts days,
  • episodes revover then recurrs
  • repeated identical headahces+ painful ophthalmolpleigic myopathy

Medically unexplained visual loss in children and young people