Ocular Motility II Kenn Freedman M.D.

Supranuclear Cranial Nerves Extra-ocular Muscles

Older woman with diabetes suffered sudden onset of Right IIIrd nerve palsy, left elevation defect and left sided weakness

Oculomotor Nerve Complex Nucleus in Midbrain Exits interpeduncular space passing several vessels including PCA Cavernous sinus Superior Orbital Fissure Superior and Inferior Divisions Superior: Levator and SR Inferior: MR, IR, IO

Left IIIrd Nerve palsy

Third Nerve Palsy Aneurysm Microvascular – DM, HTN, heart disease Trauma Neoplasm Syphilis Other, Undetermined

Third Nerve Palsy

Third nerve palsy Microvascular

Young woman presented with left sided headache and drooping of her eyelid

Patient could not move her eye up, down or toward her nose, but she could abduct. Her pupil on the left was much larger than the right.

PCA Aneurysm

Bilateral Ptosis with poor movement except abduction

Nuclear IIIrd nerve palsy

Brainstem Syndromes Weber’s - ipsalateral pupil involved IIIrd - contalateral hemiplegia - fasicle of IIIrd Nerve where traverses cerebral peduncle Benedikt’s – ipsalateral pupil involved IIIrd - contralateral limb intention tremor, hypokinesia and ataxia - Fasicle of IIIrd nerve as it traverses the red nucleus

Management of Third Nerve Palsy When to do neuro-imaging and/or arteriogram? Important factors: PAIN, PUPIL, PROGRESSION Other Possible testing: CBC, ESR, BS

In general You get imaging on PUPIL INVOLVED Third nerve palsies

Relative Pupil Sparing 0.5mm <Anisocoria < 2mm (Larger pupil still RTL) Out of 24 patients: 10 - had compressive lesions! 10 - “infarction” 4 - other Neurology 2001; 56: 797

Imaging Options MRI MRA – no contrast Cerebral Arteriogram – some risk

Management Isolated Third Nerve Palsy If patient is diabetic/ hypertensive and the pupil is not involved and they do not have too much pain*, then it would be reasonable to follow them up without imaging studies, depending on your comfort level. You should see some resolution of a microvascular palsy in at least two months.

Aberrant Regeneration One of many possible findings due to misdirection of axon fibers as healing occurs 1.Lid retraction on downgaze 2.Lid elevation or pupil constriction with attempted adduction 3.Globe retraction with attempted upgaze or downgaze 4.Others also possible

Aberrant Regeneration

Lid Lag on Downgaze Congenital Ptosis -Levator Maldevelopment Graves Ophthalmopathy Surgery, Trauma Aberrant Regeneration of 3 rd -pseudo von Graefe’s phenomenum

Primary Aberrant Regeneration? Motility problems like those described above without an acute third nerve palsy preceding them. Suggestive of a cavernous sinus mass

Trochlear Nerve Superior Oblique Long course of nerve from posterior midbrain to orbit Midbrain

Fourth Nerve Palsy Note head tilt

4 th Nerve Palsy Diplopia –usually vertical Sometimes Cyclo-diplopia Head tilt and/or turn Diplopia can worse or better on downgaze Findings can evolve over time

Fourth Nerve Palsy Hypertropia Overaction of Ipsalateral Inferior Oblique Muscle Underaction of SO not often obvious Excyclotorsion Incommitant

Fourth Cranial Nerve Palsy Incommitance Hypertropia Hypertropia worse on contraleral gaze Hypertropia worse on ipsalateral head tilt E.g. “ right – left - right” or “left - right – left”

Right- Left- Right 2 RHT 7 RHT 18 RHT 15 RHT 3 RHT

Three Step Test is only valid for Neurologic and not mechanical muscle problems Assumes only one paretic muscle

Think in terms of a paretic muscle

DX: Left SO palsy

Excylotorsion With red maddox rod over Right and white over Left Shows a right excylcotorsion consistent with a right SO palsy

Fourth Nerve Palsy Congenital* Traumatic Microvascular Neoplasm Aneurysm – not common Other * Congenital – often decompensate later in life with “sudden” onset of diplopia, will have large vertical fusional amplitudes

Fourth Nerve Palsy (Traumatic) Upshoot in adduction characteristic of Overaction of left inferior oblique

Upshoot in Adduction Most Commonly IOOA DVD Duane’s Syndrome

Right Fourth Nerve Palsy

Bilateral Fourth Nerve Palsy Alternating Hypertropia e.g. LHT in right gaze RHT in left gaze Large Excyclotorsion >10-15 degrees V pattern

Vertical Misalignment Fourth Nerve Palsy Graves Disease Post-operative muscle problem Skew Deviation Third Nerve Palsy – inferior or superior division Brown’s Syndrome Other Orbital Disease Plus More

Management of Isolated Fourth Nerve Palsy Usually no work up necessary as most cases are traumatic or congenital. If no history of trauma or signs of congenital palsy then : Does patient have vasculopathic risk factors? Yes: Observe No: Medical evaluation, maybe image

Abduction Deficit New onset diplopia Patient asked to look To the left

Abduction Deficit Sixth Cranial Nerve Palsy Graves Ophthalmopathy Myasthenia Gravis Orbital – tumor, inflammatory Duane’s Syndrome Type I Medial Wall Fracture Past LR recession More!

What’s this abduction deficit due to?

Patient had R+R OS for Exotropia, why does she have decreased abduction?

Agenesis of sixth nerve nucleus and, with abberent innervation of the Lateral Rectus muscle by branches third cranial nerve, hence multiple motility problems can be seen Duane’s Type I Type II Type III Duane’s Syndrome

For Example Duane’s Type I loss of abduction, often esotropic (no diplopia) variable loss of adduction narrowing of fissure on attempted abduction upshoot or downshoot in attempted adduction possible

Sixth Nerve Palsy Microvascular Neoplastic (Posterior Fossa, Orbit, Cavernous sinus, etc) Trauma Increased Intracranial Pressure Aneurysm Post-viral and post-immunization Other – MS, Syphilis, PML Undetermined

Sixth Nerve Palsies in Children*: 1. Tumors 45% 2. Increased ICP (15%) non-tumor 3. Traumatic 12% 4. Congenital 11% 5. Inflammatory 7% 6. Miscellaneous 5% ( post-immunization, post-viral) 7. Idiopathic 5% * JPOS; 1999; 36: 305

Brainstem Syndromes with Sixth Cranial Nerve Palsy Foville’s Syndrome* - - lesion in region of sixth nerve nucleus - ipsalateral gaze palsy, facial palsy, loss of taste, Horner’s Syndrome, facial anesthesia, deafness Millard-Gubler Syndrome – Sixth and contralateral hemiparesis

Primary Closing Lids Looking Left What’s Wrong? Where is at least one lesion? Pontine CVA?

Pontine CVA

Insert MRI scan of Eutenaurer

Total Ophthalmoplegia, loss of vision and ptosis OD Cavernous sinus tumor probable meningioma

Multiple Cranial Nerve Palsies (3,4,6, etc) Superior Orbital Fissure Syndrome Suspect Orbital Inflammatory Process –pseudotumor, and cellulitis (think fungal) Cavernous SinusThrombosis Orbital or Cavernous sinus tumor Vascular: AV fistulas or aneurysms Invasive Periorbital Skin Cancers with perinerual spread GCA Diabetic Other: HZO, Mucocele, Wernicke’s encephalopathy, Guillain-Barre or Miller Fisher Syndromes

Cranial Nerve Palsy History DM, HTN, CV disease Neurologic disease Shunting procedures Pain Age

Cranial Nerve Palsy Exam Standard Eye Exam, but also include: Exophthalmetry Checking other cranial nerve function (5,7,8) – COMPANY THEY KEEP

Cranial Nerve Palsy Major Considerations Microvascular Trauma Neoplastic Aneurysm Congenital Other: GCA, Sarcoid Consider: MS and Myasthenia

General Approach to CN Palsies Other Localizing signs Pupils Pain Progression FOLLOW-UP, microvascular palsies resolve usually in about 2 months

Matching Millard-Gubler Weber’s Miller Fisher Duanes Benedikt’s III IV VI Multiple CN