Neuro-Ophthalmology Phase 2 lecture Dr Simon Hickman Consultant Neurologist Royal Hallamshire Hospital Sheffield 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 Neuro-Ophthalmology There is not a clear ophthalmological cause of visual problem: Loss of vision Double vision History Presenting complaint 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 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 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 History associated symptoms Headaches Migraine, tumours, raised intracranial pressure Malaise, fever, myalgia, headache, jaw claudication

Giant cell arteritis Painful monocular visual loss Optic neuritis Past history Neurological Migraine, stroke, TIAs, 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 Family history Inherited disorders Lebers hereditary optic neuropathy Autosomal dominant/recessive optic neuropathy Diseases with increased genetic risk Multiple sclerosis

Social history Smoking/alcohol Carcinoma Tobacco-alcohol amblyopia Diet Nutritional optic neuropathy Occupation Specific diseases How current illness will affect patient and their families lives Examination of vision Visual acuity

Colour vision Visual field Higher cortical visual function Pupils Fundi Eyelids Eye movements Directed neurological and medical examination Visual acuity 6m retro-illuminated departmental visual acuity for each eye

Best refraction Pinhole Near vision if appropriate Other modalities of vision testing Pelli Robson contrast sensitivity, Ishihara plates, Farnsworth Munsell 100 Hue Test, perimetry Visual pathway Hickman SJ. Neurological visual field defects. Neuro-Ophthalmology 2011;35:242-250. Papillomacular bundle

>90% of retinal nerve fibres in the optic nerve Projects images from the macula: High metabolic activity Diseases: Optic neuritis Lebers hereditary optic neuropathy Toxic and nutritional optic neuropathy Central or centro-caecal scotoma Colour vision loss Parvocellular ganglion cell loss Acute optic nerve disorders


Optic neuritis Sarcoidosis SLE/Vasculitic Neuromyelitis optica Compressive Meningioma Glioma Pituitary adenoma Metastases Tuberculoma Graves 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

Lebers hereditary optic neuropathy Optic chiasm All the nasal retinal fibres decussate in the optic chiasm Bitemporal hemianopia Coronal MRI Post-contrast sagittal MRI

Complete blindness Optic tract lesions Incongruous visual field defects ie. do not exactly match up between the two eyes Tex t Text 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 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cm&part=A3543 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 T e x t http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=neurosci&part=A832

Tex t Tex t Striate (primary visual) cortex Medial and posterolateral surfaces of the occipital lobe, around the calcarine fissure 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 The central zone of each hemifield is subserved by retinal axons that eventually terminate at the most posterior pole of the visual cortex. Te xt Horton JC, Hoyt WF. Arch Ophthalmol 1991;109:816-824. t x e

T Left homonymous hemianopia with macular sparing 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 2007 130:1690-1703; doi:10.1093/brain/awl348 Fortification spectra 1st drawings from 19th century Schott, G. D. Brain 2007 130:1690-1703; doi:10.1093/brain/awl348 Higher cortical visual function

Describing pictures Line/letter cancellation Drawing a clock Perceiving fragmented objects/letters Face recognition tasks 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 Hypoxicischaemic brain injury Fundi - papilloedema Swelling, Capillary dilatation, Haemorrhage, Cotton wool spots, Choroidal folds Papilloedema associated diseases Idiopathic intracranial hypertension Young obese women

Malignant hypertension Retinal changes will usually be present Addisons disease Hypoparathyroidism Hypercapnea Chronic obstructive pulmonary disease Sleep apnoea Right heart failure with pulmonary hypertension Renal failure Severe iron deficiency anaemia 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

Wall M. preserved early on Semin Neurol 2000;20:89-95 Rowe FJ, Sarkies NJ. Eye Double vision 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 History associated symptoms Headaches Tumours, raised intracranial pressure

Systemic weakness, dysarthria, dysphagia Myasthenia gravis Dysarthria and ataxia Posterior fossa lesion Eye movements www.med.mun.ca/anatomyts/head/eye.htm III, IV and VI nerve anatomy www.imaios.com

Cranial nerve paralysis as a cause of binocular diplopia Cause 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

6 Herpes zoster 1 1

2 1 Myasthenia gravis 1 1 Migraine

1 2 1 Sinusitis 1

Demyelination 2 Miller Fisher

syndrome 1 Neurosarcoid

1 Malignancy 1

Blocked VP shunt 1 Unknown 2 6

6 Comer RM, et al. Causes and outcomes for patients presenting with diplopia to an eye casualty department. Eye 2007;21:413-418. 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 adduction of the eye Pupillary constriction Lens accommodation and

Trochlear nerve palsy Innervates the superior oblique muscle Often congenital palsy Head tilt http:// img.medscape. com/pi/emed/ckb/ clinical_procedures / 79926-1412901118 9799- Abducens nerve Innervates the lateral rectus muscle

May be false localising sign due to CSF pressure or CSF volume Eyelids Partial ptosis Horners syndrome involvement of Mllers muscle Complete ptosis Third nerve palsy Varying / fatiguing ptosis Myasthenia gravis

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 Nystagmus

Definition: 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) Nystagmus Forms in Infancy Present in

Infancy latent / manifest latent idiopathi c infantile Spasmus nutans Albinis m low vision / retinal disease

neurologica l syndromes Acquired Nystagmus Forms multiple sclerosis internuclear ophthalmoplegia Acquire

d downbeat nystagmus (Arnold-Chiari Malformation) Wernickes encephalopath y stroke / tumour Pupillary light reflex

http://www.neurology.arizona.edu/Training/newUNIT%2010_files/image026.jpg Pupil Efferent Horners Tonic III nerve palsy Swinging flashlight test Relative afferent

pupillary defect http:// common spot.aao. org/ aaoesite/ promo/techniques_20 0604. cfm

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