efferent visual dysfunction in neuro-degenerative diseases: clinical pearls...
TRANSCRIPT
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Efferent Visual Dysfunction in Neuro-degenerative Diseases:
Clinical Pearls
MJ Thurtell [email protected]
H. Stanley Thompson Neuro-Ophthalmology Service
Department of Ophthalmology & Visual Sciences University of Iowa
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Disclosures
• Financial disclosure: I do not have any relevant financial interests or relationships to disclose
• Off-label use of product disclosure: I will be discussing off-label use of drugs for nystagmus
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Objectives
• To review symptoms and signs of efferent dysfunction in neurodegenerative disease
• To review the neuro-ophthalmic deficits associated with cerebellar degenerations and progressive supranuclear palsy
• To discuss management strategies to address the neuro-ophthalmic deficits in cerebellar degenerations and progressive supranuclear palsy
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• Often vague and/or non-specific
• May suggest efferent dysfunction: – Diplopia – Oscillopsia – Difficulty with depth perception
• May suggest afferent dysfunction: – Blurred vision – Difficulty reading
• May be asymptomatic
Symptoms of efferent visual dysfunction
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ANTERIOR CORTEX: impaired voluntary
eye movements (e.g., anti-saccade test errors)
POSTERIOR CORTEX: impaired visually-guided eye movements (e.g., oculomotor apraxia)
MIDBRAIN: vertical saccadic
palsy, convergence insufficiency
PONS: horizontal saccadic palsy
CEREBELLUM: nystagmus, impaired smooth pursuit, saccadic dysmetria
BASAL GANGLIA: fixation instability,
increased saccadic latency, saccadic
hypometria
Signs of efferent visual dysfunction in neurodegenerative disease
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Clinical pearl: ocular motor apraxia
Q. How can you differentiate a supranuclear motility deficit from a nuclear-infranuclear motility deficit?
A. The deficit from a supranuclear lesion can
be overcome with the doll’s eyes maneuver (vestibulo-ocular reflex) whereas the deficit from a nuclear-infranuclear lesion cannot
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Extraocular muscle
Neuromuscular junction
Orbital apex
Cavernous sinus Subarachnoid space
Superior orbital fissure
Cranial nerve nucleus and fascicle
Cranial nerve
Supranuclear and internuclear pathways
Eye
Inner ear
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Clinical pearl: ocular motor apraxia
Chen JJ & Thurtell MJ. J Neurol Neurosurg Psychiatry 2012; 83: 1117-1118
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Clinical pearl: saccadic slowing
Q. How can you differentiate slow saccades due to a nuclear-infranuclear (e.g., sixth nerve nucleus) lesion from those due to a supranuclear (e.g., PPRF) lesion?
A. The slow saccades due to a nuclear-
infranuclear lesion are associated with limited ductions, whereas those due to a supranuclear lesion are often not
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Courtesy of RJ Leigh & DS Zee
Slow saccades from bilateral PPRF lesions
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Neuro-ophthalmic features of cerebellar degenerations
• Fixation abnormalities: – Downbeat nystagmus – Gaze-evoked and rebound nystagmus – Periodic alternating nystagmus – Macrosaccadic oscillations
• Other motility abnormalities: – Comitant esotropia – Alternating skew deviation – Saccadic dysmetria – Impaired smooth pursuit – Impaired VOR suppression
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Clinical pearl: downbeat nystagmus
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Gaze-evoked nystagmus
Rebound nystagmus
Clinical pearl: gaze-evoked and rebound nystagmus
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Clinical pearl: saccadic dysmetria
• Saccadic hypermetria: – Lesion: cerebellar fastigial nuclei – Saccades are too big, may have associated
macrosaccadic oscillations – Pearl: elicit using close visual targets
• Saccadic hypometria: – Lesion: dorsal cerebellar vermis – Saccades are too small, may be “staircase” – Pearl: elicit using more distant visual targets
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• Prism for primary position misalignment
• Single-vision distance and reading glasses
• Medications for downbeat nystagmus: – 4-aminopyridine (5 mg qid) – Clonazepam (0.5-1 mg bid)
• Medications for periodic alternating nystagmus: – Baclofen (5-10 mg tid) – Memantine (5-10 mg qid)
• Medications for macrosaccadic oscillations: – Memantine (5-10 mg qid)
Clinical pearl: managing motility deficits of cerebellar degenerations
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• Eyelid abnormalities: – Lid retraction – Decreased blink rate – Blepharospasm
• Fixation abnormalities: – Square-wave jerks
Neuro-ophthalmic features of progressive supranuclear palsy
Dry eye, blepharitis
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Courtesy of RJ Leigh & DS Zee
Clinical pearl: square-wave jerks
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• Eyelid abnormalities: – Lid retraction – Decreased blink rate – Blepharospasm
• Fixation abnormalities: – Square-wave jerks
• Other motility abnormalities: – Convergence insufficiency – Vertical>>horizontal saccadic palsy
• Saccades are slow • Saccades are hypometric
Neuro-ophthalmic features of progressive supranuclear palsy
Dry eye, blepharitis
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Chen et al. Front Neurol 2010; 1:147 [doi: 10.3389/fneur.2010.00147]
Nor
mal
P
SP
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Clinical pearl: demonstrating directional saccadic palsy
Courtesy of RJ Leigh & DS Zee
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Courtesy of S Wray
Clinical pearl: oscillations in CNS Whipple’s disease Oculomasticatory myorhythmia
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• Eyelid hygiene: – Lid scrubs and warm compresses
• Eye lubrication: – Artificial tears QID – Lubricating ointment QHS
• Single-vision distance and reading glasses
• Prism in reading glasses for convergence insufficiency; consider monocular occlusion
• Prism to assist with downward saccadic palsy
Clinical pearl: managing neuro-ophthalmic deficits of PSP
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