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Neuro-Ophthalmology Phase 2 lecture
Dr Simon Hickman Consultant Neurologist Royal Hallamshire Hospital Sheffield
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Neuro-Ophthalmology Basic anatomy of the visual system and a clinical approach to Neuro-Ophthalmology. Common problems: Acute visual loss Visual field defects Papilloedema Abnormal eye movements Eyelid abnormalities Pupils
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Neuro-Ophthalmology There is not a clear ophthalmological cause of visual problem: Loss of vision Double vision
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History Presenting complaint History of presenting illness
Age, sex, occupation, major complaint History of presenting illness Details Temporal profile of symptoms Associated symptoms Past Neurological and Ophthalmological history Past Medical History Family history Social history
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History – loss of vision
Right, left or both eyes Nasal, temporal, superior or inferior field of vision Degree of visual deficit Black, grey, white or distorted Colour perception Red desaturation Higher cortical visual dysfunction Vague complaints Visuo-spatial or visuo-perceptual loss
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History – Temporal profile
Acute or chronic Rapidity of onset Sudden – vascular Acute – inflammatory/infectious Subacute – inflammatory/infectious/neoplastic Chronic – neoplastic/degenerative Recovering / Progressing Episodic Migraine NB – the date of onset of perceiving monocular visual loss may not be date of onset of disease
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History – associated symptoms
Headaches Migraine, tumours, raised intracranial pressure Malaise, fever, myalgia, headache, jaw claudication Giant cell arteritis Painful monocular visual loss Optic neuritis
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Past history Neurological Ophthalmological Medical/Surgical Medication
Migraine, stroke, TIA’s, MS, epilepsy etc Ophthalmological Refractive error, cataracts, glaucoma, strabismus, amblyopia, eye patching or surgery Medical/Surgical Hypertension, diabetes, IHD, arrhythmias, cancer, rheumatological disease, infectious disease Medication Ethambutol optic neuropathy Vigabatrin retinopathy
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Family history Inherited disorders
Leber’s hereditary optic neuropathy Autosomal dominant/recessive optic neuropathy Diseases with increased genetic risk Multiple sclerosis
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Social history Smoking/alcohol Diet Occupation Carcinoma
Tobacco-alcohol amblyopia Diet Nutritional optic neuropathy Occupation Specific diseases How current illness will affect patient and their families lives
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Examination of vision Visual acuity Colour vision Visual field
Higher cortical visual function Pupils Fundi Eyelids Eye movements Directed neurological and medical examination
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Visual acuity 6m retro-illuminated departmental visual acuity for each eye Best refraction Pinhole Near vision if appropriate
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Other modalities of vision testing
Pelli Robson contrast sensitivity, Ishihara plates, Farnsworth Munsell 100 Hue Test, perimetry
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Visual pathway Hickman SJ. Neurological visual field defects. Neuro-Ophthalmology 2011;35:
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Papillomacular bundle
>90% of retinal nerve fibres in the optic nerve Projects images from the macula: High metabolic activity Diseases: Optic neuritis Leber’s hereditary optic neuropathy Toxic and nutritional optic neuropathy Central or centro-caecal scotoma Colour vision loss Parvocellular ganglion cell loss
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Acute optic nerve disorders
Inflammatory Optic neuritis Sarcoidosis SLE/Vasculitic Neuromyelitis optica Compressive Meningioma Glioma Pituitary adenoma Metastases Tuberculoma Grave’s disease Arterial aneurysms Sinus mucocoeles Vascular Non-arteritic anterior ischaemic optic neuropathy Giant cell arteritis - arteritic AION Infectious Syphilis Tuberculosis Lyme disease Viral Toxic and Metabolic Vitamin B12 deficiency Tobacco-alcohol amblyopia Tropical amblyopia Methanol Ethambutol Genetic Leber’s hereditary optic neuropathy
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Optic chiasm All the nasal retinal fibres decussate in the optic chiasm
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Bitemporal hemianopia
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Coronal MRI
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Post-contrast sagittal MRI
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Complete blindness
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Optic tract lesions Text Text Incongruous visual field defects
ie. do not exactly match up between the two eyes Text Text
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Retrogeniculate visual field defects
Almost always congruous (homonymous) Nasal and temporal fibres from corresponding points in visual field are closely opposed May demarcate as upper or lower defects defined by both vertical and horizontal meridia
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Optic radiations and striate cortex
Lesions of Meyer's loop and inferior striate cortex below the calcarine fissure produce superior homonymous quadrantanopias Parietal lobe lesions and superior striate cortex lesions produce inferior homonymous quadrantanopias Text Text Text
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Striate (primary visual) cortex
Medial and posterolateral surfaces of the occipital lobe, around the calcarine fissure The central zone of each hemifield is subserved by retinal axons that eventually terminate at the most posterior pole of the visual cortex. Homonymous hemianopia "with macular sparing" occurs with occipital lesions that spare the posterolateral striate cortex, due to collateral blood supply to occipital pole from posterior temporal artery or branches from the middle cerebral artery Text Text Horton JC, Hoyt WF. Arch Ophthalmol 1991;109:
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Left homonymous hemianopia with macular sparing
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The use of the printed page to reveal the uniformity of migraine scotomas
Jolly (1902) Gowers (1904) Wilkinson + Robinson (1985) Ekbom (1975) noted that his scotoma was “no larger than the fraction of a letter” when reading a newspaper – may have been missed in other circumstances Scotoma often grey and indistinct rather than black – may also have colours, especially at the edge Schott, G. D. Brain : ; doi: /brain/awl348
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Fortification spectra
1st drawings from 19th century Schott, G. D. Brain : ; doi: /brain/awl348
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Higher cortical visual function
Describing pictures Line/letter cancellation tasks Drawing a clock Perceiving fragmented objects/letters Face recognition
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Global visual deficits
Visuospatial and/or visuoperceptual abnormalities can occur with: Degenerative diseases Posterior cortical atrophy Creutzfeldt-Jakob disease Tumours Infections Progressive multi-focal leucoencephalopathy Hypoxic–ischaemic brain injury
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Fundi - papilloedema Swelling, Capillary dilatation, Haemorrhage, Cotton wool spots, Choroidal folds
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Papilloedema - associated diseases
Idiopathic intracranial hypertension Young obese women Malignant hypertension Retinal changes will usually be present Addison’s disease Hypoparathyroidism Hypercapnea Chronic obstructive pulmonary disease Sleep apnoea Right heart failure with pulmonary hypertension Renal failure Severe iron deficiency anaemia
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Papilloedema Stasis of axoplasmic flow at the optic nerve head caused by raised intracranial pressure Visual loss due to intra-neuronal ischaemic damage peripherally-placed fibres sub- serving peripheral vision being most vulnerable Perimetry required to assess for visual impairment as central visual acuity and colour vision are generally preserved early on Wall M. Semin Neurol 2000;20: Rowe FJ, Sarkies NJ. Eye 1998;12:
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Double vision
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History – efferent dysfunction
Double vision Monocular or binocular Monocular may be functional or ocular (eg cataract) Polyopsia may be cortical (eg migraine) Horizontal or vertical Worse in right-, left-, up-or downgaze Worse in near or distance Oscillopsia Nystagmus is often asymptomatic Fluctuating/fatiguing Myasthenia gravis
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History – associated symptoms
Headaches Tumours, raised intracranial pressure Systemic weakness, dysarthria, dysphagia Myasthenia gravis Dysarthria and ataxia Posterior fossa lesion
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Eye movements
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III, IV and VI nerve anatomy
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Cranial nerve paralysis as a cause of binocular diplopia
Number of cases for each affected cranial nerve III IV VI IV and VI Hypertension 8 15 26 — Diabetes 4 10 12 Trauma 6 1 Congenital Herpes zoster 2 Myasthenia gravis Migraine Sinusitis Demyelination Miller Fisher syndrome Neurosarcoid Malignancy Blocked VP shunt Unknown Comer RM, et al. Causes and outcomes for patients presenting with diplopia to an eye casualty department. Eye 2007;21:
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III nerve Innervates the extra-ocular muscles:
Levator palpebrae superioris, superior rectus, inferior rectus, medial rectus, and inferior oblique Parasympathetic presynaptic outflow from the Edinger-Westphal nucleus: Pupillary sphincter and ciliary body Controls Eyelid elevation Elevation, depression and adduction of the eye Pupillary constriction Lens accommodation
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Trochlear nerve palsy Innervates the superior oblique muscle
Often congenital palsy Head tilt com/pi/emed/ckb/ clinical_procedures/ jpg
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Abducens nerve Innervates the lateral rectus muscle
May be false localising sign due to ↑CSF pressure or ↓CSF volume
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Eyelids Partial ptosis Complete ptosis Varying / fatiguing ptosis
Horner’s syndrome involvement of Müller’s muscle Complete ptosis Third nerve palsy Varying / fatiguing ptosis Myasthenia gravis
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Myasthenia gravis Autoimmune disorder of neuromuscular junction
Acetylcholine receptors destroyed at motor end plate Characterized by weakness and fatigability Ocular muscles - double vision and ptosis Bulbar muscles Skeletal muscles Variability in symptoms Diurnal variation Change over weeks to months
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Nystagmus Definition: Prevalence - 24 in 10,000 Impact on vision:
rhythmic to-and-fro oscillations of the eyes Prevalence - 24 in 10,000 (Sarvananthan et al. 2007) Impact on vision: Worse than age related macular degeneration (Pilling at al. 2005)
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Nystagmus Forms in Infancy
latent / manifest latent Present in Infancy idiopathic infantile Spasmus nutans Albinism low vision / retinal disease neurological syndromes
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Acquired Nystagmus Forms
multiple sclerosis Acquired internuclear ophthalmoplegia downbeat nystagmus (Arnold-Chiari Malformation) Wernicke’s encephalopathy stroke / tumour
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Pupillary light reflex
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Pupil Efferent Horner’s Tonic III nerve palsy
Efferent disturbances of the pupil are usually unilateral and will therefore cause anisocoria. I
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Swinging flashlight test - Relative afferent pupillary defect
common spot.aao. org/ aaoesite/ promo/techniques_ cfm
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