1/18/2015
The Efferent Visual System: Disorders of Cranial Nerves III, IV & Course Goals VI • To Become Familiar with Presentations of CN III, IV and VI Palsies
• To Understand the Relevant Neuro Anatomy
James L. Fanelli, OD, FAAO • To Understand the Neuro Imaging and Clinical Leonard V. Messner, OD, FAAO Management Lorraine Lombardi, PhD • To Obtain In Clinic Assessment Pearls
Course Format
• Case Presentation – Dr. Fanelli • Relevant Neuro Anatomy – Dr. Lombardi • Neuro Imaging and Management – Messner • Clinical Take Home – Drs Fanelli, Lombardi and Messner
“THE FIRST 4 QUESTIONS” The Diplopic Patient
• Evaluation boils down to knowing • 1. WHO IS THE NEURO-OP ON CALL? the fields of action of the 6 EOM’s • 2. WHAT IS THEIR NUMBER? • 3. HOW SOON CAN THE PATIENT BE SEEN? • 4. WHAT IS THE DIAGNOSIS? • You know the actions, you can figure out the Palsy – Cerca Trova
1 1/18/2015
The Physiological “H” EOM ACTIONS
• You are face to face with the patient
• Their EOM movements, as you view them, SR IO render the following “H” pattern:
LR MR
IR SO
4 Questions We Should Ask • 1-Is Double Vision Present with one eye covered? – “Yes” eliminates neurologic etiologies – Usually a ‘windows’ problem • Media opacities
EOM ACTIONS 4 Questions We Should Ask • 2-Does the Diplopia have a vertical component or a horizontal component SR IO
LR MR
IR SO
2 1/18/2015
EOM ACTIONS 4 Questions We Should Ask • 3-In which direction (R or L) does the diplopia worsen? SR IO
LR MR
IR SO
EOM ACTIONS 4 Questions We Should Ask • 4-Is the diplopia greater at distance or near?
SR IO
LR MR
IR SO
“LANGUAGE OF THE LIGHT” Clinical Assessment of Diplopia • Begins with dissociating the presenting images before each eye EXO R HYPER • Maddox Rod
ESO L HYPER (PATIENT’S VIEW )
3 1/18/2015
Third Nerve Palsies So What is Presentation • CN III Innervates: • Go back to the Physiological H – SR – IR • Assuming a RIGHT CN III Palsy: – MR – IO – Levator – Parasympathetic Iris (constrictor)
EOM ACTIONS EOM ACTIONS
SR IO SR IO
LR MR LR MR
IR SO IR SO
“The Signature” of CN III Paresis
• Hyper deviation which increases in upgaze and reverses in downgaze Oculomotor nerve... • Exo which increases across from the vertically- limited eye Its course and relationships
In the midbrain
4 1/18/2015
Cerebral Hemisphere
Midbrain Midbrain
Oculomotor CN III nucleus
III
Then CN III Subarachnoid space can run into trouble III Posterior Communicating artery Midbrain
III Posterior Aneurysms Communicating pcom can form aneurysms can rupture = Artery pressure
Compresses III pcom Pupillary BERRY Fibers of III… ANEURYSM Dilated pupil
5 1/18/2015
Posterior Posterior communicating communicating artery artery
PUPILLARY PUPILLARY FIBERS of III FIBERS of III Extraocular Extraocular Muscle Muscle Fibers of III Fibers of III
X-section X-section III III
Subarachnoid Aneurysms can hemorrhage rupture
Meningeal Compression ( ie aneurysm) Irritation…pain III vs dura Vasculopathic (ie diabetes) Sub- Lesion of CN III meninges Arachnoid space
BRAIN
Posterior communicating CN III is artery peripheral nervous system and it will regenerate PUPILLARY FIBERS Sometimes to the wrong target organ… Extraocular Muscle Aberrant regeneration Fibers
III Artery to the IIIrd nerve
6 1/18/2015
Aberrant Regeneration •Two divisions Ciliary ganglion of III “look down” Lid up •Superior Levator SR CN III IR IO Constrictor; •Inferior Ciliary muscle IR Degeneration and MR regeneration Lev of the axon
The Clinical Picture CRANIAL NERVE PALSY STRATEGY • CN III Palsy • IS THIS REALLY WHAT I THINK IT IS? (imposters) • DOES IT COME WITH ANYTHING ELSE? (anatomically guided exam) • IF IT IS ISOLATED, WHAT DO I DO? (management)
7 1/18/2015
CN III palsy – pupil involved 52 y/o man with sudden onset/painful diplopia @ distance and near
8 1/18/2015
CN III – pupil spared 65 y/o diabetic man with recent onset diplopia @ distance and near
“Rule of the Pupil” Vasonervorum Pupil Pupil Spared EOM Fibers Involved Pupil Fibers Aneurysm 86% 14%
PCA Ischemic / 23% 77% Vascular
Aneurysm CN III Kissel JT, et al. Ann Neurol 1983 Goldstein JE, et al. Arch Ophthalmol 1960
9 1/18/2015
CN III aberrant regeneration Aberrant Regeneration of CN III 32 y/o woman with traumatic CN III palsy
1. Pseudo-Graefe sign 2. Eyelid synkinesis 3. Light-gaze dissociated pupils
Neuroimaging for CNIII Palsy Clinical Kernels: CN III
• MRI • EOM pattern of hyper deviation that switches on up • MR Angiography and down gaze and increases on gaze away from • Intra-arterial paretic eye DSA • CT Angiography • Aberrant regeneration does NOT occur in cases of microvascular (diabetic) CN III
• Pupil sparing is NOT always indicative of microvascular etiology
4th N Innervation & Motility
• Innervation is easy: Fourth Nerve Palsies – Superior Oblique
• Motility is more complex – Both a horizontal AND vertical component – AND……a TORSIONAL component
10 1/18/2015
EOM ACTIONS EOM ACTIONS
SR IO SR IO
LR MR LR MR
IR SO IR SO
4th N Palsy Torsional Obliques
• The paretic eye is hyper in primary • Remember this: gaze • SUPERIOR muscles INTORT
• The diplopia decreases on same gaze; • INFERIOR muscles EXTORT increases on opposite gaze
• But……..
4th N and SO Muscle
• The SO is primarily an INTORTER Trochlear Nerve...
– Compensating for a faulty intorter, one would TILT your head in the opposite direction Its course and relationships
11 1/18/2015
“Reality Neuro”— Subarachnoid space Most Dangerous areas for CN’s
IV IV
Trochlear III Nucleus Ambient cistern Subarachnoid space
III
AMBIENT CISTERN IV
Trauma pons
The Clinical Picture Ambient Cistern • CN IV Palsy IV
Trauma
12 1/18/2015
“The Signature” of CN IV Evaluation of CN IV Palsy Paresis • Which eye is higher in primary • A hypertropia that increases across from gaze? the vertically-limited eye and on ipsilateral • Hyper worse in right or left head tilt gaze?
• Which eye is higher on head tilt? • Is there excyclotorsion?
Evaluation of CN IV Palsy
• Which eye is higher in primary gaze? • Hyper worse in right or left gaze? • Which eye is higher on head tilt? • Is there excyclotorsion?
Evaluation of CN IV Palsy
• Which eye is higher in primary gaze? • Hyper worse in right or left gaze? • Which eye is higher on head tilt? • Is there excyclotorsion?
13 1/18/2015
Evaluation of CN IV Palsy SUPERIOR OBLIQUE • Which eye is higher in primary SUPERIOR RECTUS gaze? • Hyper worse in right or left gaze? • Which eye is higher on head tilt? INFERIOR RECTUS • Is there excyclotorsion? INFERIOR OBLIQUE
reverse reverse
14 1/18/2015
Traumatic CN IV palsy 28 y/o woman s/p closed head trauma Right hyper greater in left gaze and right head tilt Measuring Excyclotorsion • Subjective –Maddox rod –Bagolini striated lenses • Objective –Fundus photos
15 1/18/2015
Objective vs. Subjective Excyclotorsion
• Objective = Subjective Recent onset
• Objective > Subjective Long-standing
OBJECTIVE • Objective without subjective Infantile ANGLE of EXCYCLOTORSION
Etiology of Adult Superior Oblique Palsies (Mollan SP, et al. Eye 2009) • N = 150 • 7 unilateral – • 133 unilateral- complicated isolated: – 71% trauma – 38% congenital – 14% tumor – 29% trauma – 14% undetermined – 23% vasculopathic – 7% undetermined • 10 bilateral: – 50% trauma – 20% tumor – 20% undetermined
Isolate CN IV Palsy Management • Observation (improvement within several months for ischemic vascular) • Prism (base-down over paretic eye/split between both eyes) – Rx vertical prism as single vision/NVO • Surgery – Wait for spontaneous improvement (at lest 6 months) • Check for V-pattern eso in kids (indicator for bilateral CN IV palsy)
16 1/18/2015
Clinical Kernels: CN IV Clinical Kernels: CN IV
• ALL cranial nerves travel through the sub • Assessment of excyclotortion is helpful arachnoid space, and as such they are in determining onset of problem: susceptible to compression – Long standing, patients adapt, and objective – Trauma and bleeds measure > subjective complaints – CN 4: Long intracranial/subarachnoid course – Recent onset, patients haven’t ‘adapted’, noticeable findings, noticeable complaints • SO is primarily an intorter, therefore HEAD TILT will be an integral finding
6th N Innervation and Motility
• Innervation is easy: – Lateral Rectus Sixth Nerve Palsies • Motility is easy: – No vertical component – Only horizontal component
EOM ACTIONS EOM ACTIONS
SR IO SR IO
LR LR MR MR
IR SO IR SO
17 1/18/2015
Motility Pattern Compensation for CN VI Palsy • Inability to Abduct, therefore paretic eye has eso posture IN PRIMARY GAZE • Since the paretic eye cannot Abduct and is eso, the patient will TURN THEIR HEAD to • Eso increases on gaze TOWARD paretic the SAME side eye
Abducens Nerve...
Its course and relationships PONS
VI
Subarachnoid space prepontine cistern
Prepontine cistern Small posterior pons Petroclinoid cranial fossa ligament cerebellum with Chiari Clivus…occipital bone
18 1/18/2015
Subarachnoid space petroclinoid ligament Increased intracranial pressure Orbital cavity Papilledema and Abduction deficit Cavernous Sinus with pons III, IV… The brain and its coverings and Subarachnoid space The brain and Petroclinoid its coverings (meninges) Ligament clivus ..and optic nerve
The Clinical Picture “The Signature” of CN VI Paresis
• CN VI Palsy
• Eso which increases in the action of the paretic eye
CNVI Palsy Motility Evaluation CN VI Motility Evaluation
• Duction > version • “Glissades” • Asymmetric OKN • Negative forced duction
19 1/18/2015
27 y/o AA Woman
• c/o horizontal diplopia (right gaze > left) • h/o recurrent headaches (am > pm) • BVA: – 20/20 OD – 20/20 OS
20 1/18/2015
Etiology of CN VI Palsy S/P Surgical Decompression Mayo Clinic Study of Olmstead Co. MN USA from 1978-1992 (n = 137) • Undetermined: 26% • Hypertension: 19% • HTN & diabetes: 12% • Trauma: 12% • MS: 7% • Neoplasm: 5% (complicated) • Diabetes (alone): 4% • CVA: 4% • s/p neurosurgery: 3% • Aneurysm: 2% (complicated) • Other: 8% Patel SV, et al. Ophthalmology 2004
21 1/18/2015
40 y/o woman Acute horizontal diplopia greater at distance and on left gaze Recent onset paresthesias R > L FLAIR
FLAIR T1 post
T1 post
Clinical Kernels: CN VI Poly Cranial Neuropathies • Sudden onset unilateral, think small vessel • Involvement of CN III, IV and/or VI can be occlusive disease in vasculopathic population found simultaneously – But trauma is trauma • Acquired bilateral: look at the optic nerves and • Investigation centers on locations in the think about increased ICP head where III, IV and VI travel together – Orbital apex – Long climb up the clivus in sub arachnoid space • Any associated proptosis??? • BO prism can optically correct – Cavernous sinus • Can’t have a complete Neuro-op lecture without mentioning the Cavernous Sinus
22 1/18/2015
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