Lessons from 35 Years of Neuro-Ophthalmic Practice

10/29/2012

The Thrill of Victory, The Agony of Defeat: Lessons from 35 Years of Neuro-Ophthalmic Practice

William T. Shults, MD Portland, Oregon

“Experience is just the name we give our mistakes” Oscar Wilde

“Some people make the same mistake over and over again and call it experience” Herb Fred, MD

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“Listen to the patient, he’s trying to tell you what’s wrong with him.” Eugene Stead, MD

“The final diagnosis is often as dependent on an accurate history as on a clinical examination” Sir Gordon Holmes

Iatrogenic Papilledema

MT, 43 yr-old Latino, 10 yr hx of peptic ulcer  9/2/71: Surgery for obstruction → stormy post- op course requiring hyperalimentation via catheter in R subclavian vein  Developed headaches, diplopia and blurred vision soon afterwards → cerebral edema 2° water retention!  “This is all due to that thing they stuck in my chest”

Iatrogenic Papilledema

1/27/72: Readmitted 4 mos later with TVO’s and bilateral papilledema 1/27/72 Ophthalmology Consult:  Acuity: OD 20/30, OS 20/20  Color: Normal OU  EOMs: Full OU  Fields and Fundi: see next slides

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Iatrogenic Papilledema

2/1/72: 4 vessel arteriogram: NI 2/8/72: Pneumoencephalogram: NI 2/9/72: Discharged 4/3/72: Readmitted to Neurology with persistent papilledema, LP→ 220 OP, 2nd angio nl  Recommended repeat pneumo → Patient “Adios”

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Iatrogenic Papilledema

10/9/72: Over 1 year after illness began → readmitted with increasing blur  Repeat LP → OP now 375

 Repeat angio: R transverse and sigmoid sinus occlusion (next slides)

Iatrogenic Papilledema

10/27/72: LP shunt 12/28/72: Acuity OD 20/30 OS 20/40-1  Fields and Fundi: see next slides

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Iatrogenic Papilledema

Measles and a Broken Leg

The Neuro-Ophthalmic Patient with Multiple Diagnosis

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Data Overload

KM, 31 year-old secretary  8/77: Developed transient visual obscurations, OS with headaches  Headaches cleared after chiropractic manipulation, TVOs persisted

Data Overload

No history of exposure to steroids, nalidixic acid, lithium, tetracycline or excess vitamin A Normal weight Neurologically healthy Long-standing right exotropia and amblyopia Iris colobomas (see next slides)

Data Overload

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Data Overload

Examination (8/78):  Acuity: OD HM; OS 20/30  Color: OD nil; OS 10/10 correct HRR  Pupils: Right RAPD  Fields and Fundi: see next slides

Data Overload

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Data Overload

What’s going on here?

What would you do next?

Data Overload

“Disease will sometimes peer up over the hedge of health with only its eyes showing”

John Stone, MD

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Post Traumatic Vision Loss?

55 year old waitress  8/15/90 Rear-ended in MVA  Struck forehead but no LOC  Lost all vision in OD immediately after accident  VA gradually recovered over several days but inferior nasal field defect persisted

Post Traumatic Vision Loss?

Examination (8/23/90): +2 -2  Acuity: OD 20/25 , OS 20/20  Color: Normal  Pupils: No RAPD!  Fields and Fundi: see next slides

Post Traumatic Vision Loss?

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Post Traumatic Vision Loss?

MRI SCANS

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Post-traumatic Diplopia

DR, 58 year old housewife  2/8/89: Involved in MVA with severe facial trauma → right zygomatic fracture, bilateral orbital ecchymoses

 Blepharoptosis noted OS sometime thereafter  3/89: Noted diplopia → Ill-defined impaired ocular motility

 Tensilon test neg → referred

Post-traumatic Diplopia

Examination  External: Narrowed palpebral aperture OS, 2mm enophthalmos on left  Afferent package: Intact  EOMs: Limited adduction, abduction, elevation  Forced ductions: Restricted

Post-traumatic Diplopia

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Post-traumatic Diplopia

Original CT scan review:  Poor scan quality, showed altered tissue behind left globe compatible with post- traumatic scarring  MRI Scan: Next slides

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Post-traumatic Diplopia

Post-traumatic diplopia

8/89: Orbital biopsy by card carrying orbital surgeon.  Biopsy negative Now what?

Post-traumatic diplopia

Patient followed with stable motility findings 3/90 developed “woody” firmness beneath left eye MRI repeated

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Post-traumatic diplopia

Rebiopsy Dx: Metastatic Carcinoma of breast

Post-traumatic diplopia

References  Mottow-Lippa L, Jakoblec FA, Iwamoto T: Pseudoinflammatory metastatic breast carcinoma of the orbit and lids, Ophthalmology 88:575-580, 1981.  Manor RS, Enophthalmos caused by orbital metastatic breast carcinoma; ACTA Opthalmologica 52:881-884,1974.  Cline RA, Rootman J: Enophthalmos: a clinical review. Ophthalmology 91:229-237, 1984.

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“Hindsight is an exact science.”

Fagan’s Rule on Past Prediction

Papilledema: True or False?

FD, 57 year old tool and dye maker  Saw cornea consultant on 3/23/93 for RK pre- op assessment Noted to have asymptomatic bilateral disc edema →referred for neuro-ophthalmic consultation

 No history of visual complaints of any kind

Papilledema: True or False?

No history of headache, obesity, intracranial bruits or exposure to pseudotumorigenic drugs Past history: Hypertensive for 10 years Habits:  Smokes 2 packs of cigarettes/day

 Recovering alcoholic

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Papilledema: True or False?

Examination:  Acuity: OD 20/20, OS 20/20 with moderate myopic Rx  Color: OD 9/10, OS 9/10 correct with AOHRR  Contrast: OD 1.50, OS 1.65, Pelli-Robson  Pupils: no RAPD  Fields and fundi: see next slides

Papilledema: True or False?

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Papilledema: True or False?

MRI scan: Normal Neurology consult: No localizing abnormalities

Lumbar puncture: OP 140mm H2O Now what?

Papilledema: True or False? RK performed mid-April with 20/20 result OU About 1 month later noted abrupt loss of central vision OS Examination on 6/2/1993: -1 -3  Acuity: OD 20/20 , OS 20/50  Color: OD 10/10, OS 9/10 correct, AOHHR  Contrast: OD 1.65, OS 1.35  Pupils: 0.3 log unit RAPD, OS  Fields and fundi: see next slides

Papilledema: True or False?

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Papilledema: True or False?

ANTERIOR ISCHEMIC OPTIC NEUROPATHY WITH PRESYMPTOMATIC DISC SWELLING Hayreh SS: Anterior ischemic optic neuropathy: V. Optic disc edema an early sign, Arch Ophthalmology 99: 1030-1040, 1981. “Symptomless optic disc edema may precede the vision loss in AION and could constitute the earliest sign of the disease.”

“The Second Cranial Nerve is Ours” Henry J. L. Van Dyk, MD

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Meningiomas: Importance of Proper Neuro-Imaging

BC, 35 year old woman  3/97: “Smudged” area superonasal field, reduced light brightness and color, OD Visual acuity: OD 20/20-1, OS 20/20 Disc pallor noted OD, no RAPD  6/97: Acuity now OD 20/25, OS 20/20 Visual fields: see next slide

Meningiomas: Importance of Proper Neuro-Imaging

MRI Scan:  Done with standard angulation (rather than reverse angulation in plane of the optic nerve)  Thick slices

 Without Gadolinium

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Meningiomas: Importance of Proper Neuro-Imaging BRAIN MAGNETIC RESONANCE IMAGING

Scans were done with 2 second rep time transversely, 1.5 second coronally and sagittal images done near midline with 0.5 second rep time for maximal differentiation of CSF and neural tissue. Additional fat suppressed T, weighted axial and T, weighted coronal images are obtained through the orbits using thin sections obtained with the 1.5 tesla Siemens Magnetom.

Ventricle size and position are normal. The signal intensity of the brain parenchyma is unremarkable. Visualized cranial nerves and vascular structures are within normal limits. No calvarial abnormalities are identified. No sinus mucosal disease is seen.

Mild mucosal thickening is seen in the ethmoid sinuses. The globe, optic nerves and muscle cones are normal. No abnormal fat is seen in the orbits.

IMPRESSION No significant abnormality.

Meningiomas: Importance of Proper Neuro-Imaging

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Meningiomas: Importance of Proper Neuro-Imaging

9/97: Referred for neuro-ophth consult  Acuity: 20/15, OU  Color: OD 4/10, OS 10/10, HRR  Contrast: OD 1.35, OS 1.65  Pupils: 1.8 log unit RAPD, OD  Fundus: Atrophic OD, Normal OS  Repeat MRI: see next slides

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Meningiomas: Importance of Proper Neuro-Imaging

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Meningiomas: Importance of Proper Neuro-Imaging

The Definition of Neuro- Ophthalmology

RR, 78 year old man  Three months of diplopia and one month of dim vision in the left eye  CT scan with contrast “normal”

 Referred for neuro-ophthalmology evaluation  Visual fields, CT, MRI: see next slides

The Definition of Neuro- Ophthalmology

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The Definition of Neuro- Ophthalmology

CT SCANS

The Definition of Neuro- Ophthalmology

MRI SCANS

The Definition of Neuro- Ophthalmology

MRI SCANS

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“He who ignores the ancient German literature will discover many new things.” Simmons Lessell, MD

“Nihil Novum Sub Sole” (There is nothing new under the sun)

Wild & Crazy EOMs

JG, 60 year old farm machine shop operator th  1955: right 6 palsy, panhypopit → massive sella → 3500 rads → hormone Rx th  1955-1978: 6 palsy cleared, patient did well  5/78: MVA → Closed head trauma → decreased acuity OD, no diplopia or motility deficits CT Scan: large pituitary tumor Craniotomy: Incomplete removal → 3700 rads

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Wild & Crazy EOMs

1/79: Intermittent diplopia 3/79: Misdiagnosed “convergence spasm”  Churchill’s commentary on man, “Man will occasionally stumble over the truth but most of the time he will pick himself up and continue on.” 10/79: Episodes of diplopia more frequent  Eye movement pattern suggested oculomotor neuromyotonia

Oculomotor Neuromyotonia

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Oculomotor Neuromyotonia

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Oculomotor Neuromyotonia

History  1970 – Ricker and Mertens described a single patient with brief periods of sustained involuntary contraction of ocular muscles innervated by the third nerve which they termed oculomotor neuromyotonia  1972 – Pabst described a similar case Ocular EMG in both → neurogenic origin

Oculomotor Neuromyotonia

History  1986 – Shults et al described 6 patients, 4 with III nerve neuromyotonia and one each with IV and VI nerve neuromyotonia  1986 – Lessell et al added four cases emphasizing the association with radiation therapy of skullbase neoplasms

“Sometimes it’s better to just stand there, not do something.” Joel Glaser, MD

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Traumatic Abducens Palsy

27 year old woman sustained a right abducens palsy in an auto accident

Traumatic Abducens Palsy

January 19, 1977  21 days post injury

Traumatic Abducens Palsy

March 17, 1977  78 days post injury

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Traumatic Abducens Palsy

April 20, 1977  112 days post injury

Traumatic Abducens Palsy

August 3, 1977  217 days post injury

“Crocks Ain’t Immortal” Neil Miller, MD

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? Hysterical Visual Loss

PR, 59 year old housewife with a history of severe anxiety attacks  1987: Optometric exam showed acuity of 20/20 OU  1/91: Transient decrease in color vision OS  12/91: Further alteration of color perception (patchy indistinctness)  4/92: Stopped driving

? Hysterical Visual Loss

5/92: Saw ophthalmologist because of blurred vision  Acuity: OD 20/200, OS CF @ 3ft  IOP: OD 16, OS 17  GCF: Inferior loss OS  Fundus: Temporal pallor, OS  Referred to internist (no PE in 15 years)

 Internist referred to psychiatrist

? Hysterical Visual Loss

Psychiatrist diagnosed conversion hysteria and placed on Xanax 10/15/1992: Returned to ophthalmologist because of continuing visual failure  Referred to neuro-ophthalmology

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? Hysterical Visual Loss

Neuro-ophthalmology exam:  Acuity: OD CF @ 5ft, OS CF @ 7ft  Color: 0/10 OU  Contrast: unable  Pupils: no RAPD  Fundi: bilateral optic atrophy  Visual fields: see next slide

? Hysterical Visual Loss

MRI SCANS

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? Hysterical Visual Loss

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The Unlucky Resident

26 year old female medical resident  5/4/86: Fell from bike at 35mph landing on left malar eminence Unconscious, left hemiparesis Taken to ER in central Oregon Fractured left zygoma and mandible Initial CT normal

The Unlucky Resident

5/4/86 (continued):  Left pupil dilated during repair of facial fractures

 Repeat CT normal  ICP monitoring line placed into right lateral ventricle 5/7/86:  ICP line removed

The Unlucky Resident

5/8/86:  Transferred to Portland  Upon regaining consciousness noted complete left homonymous hemianopsia 5/14/86:  Repeat CT normal 5/29/86:  Referred for neuro-ophthalmology consult

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The Unlucky Resident

5/29/86 Examination:  Acuity: 20/20 OU  Color: normal  Pupils: Left efferent defect Left afferent defect (0.6 log units)  Visual fields: see next slide Total left homonymous defect

The Unlucky Resident

Fundoscopy:  Mild temporal pallor OU

 Depigmentation and pigment clumping below left disc

 See next slide

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The Unlucky Resident

How would you explain this woman’s field loss in the face of negative CT scans?

“The Sign of the Four”

Sherlock Holmes: “When you have eliminated the impossible, whatever remains, however improbable, must be the truth.”

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The Unlucky Resident

CT Scans: 5/4/86 (Before ICP line)

The Unlucky Resident

CT Scans: 5/7/86 (After ICP line)

The Unlucky Resident

CT Scans: 5/14/86 (ICP line out)

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The Unlucky Resident

MRI Scans 6/2/86 & 6/18/86

The Unlucky Resident

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The Unlucky Resident

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The Unlucky Resident

OD OD 25 days after accident 139 days after accident

The Unlucky Resident

OS OS 25 days after accident 139 days after accident

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“Education never ends Watson. It is a series of lessons with the greatest for the last.”

Sherlock Holmes