SLIDE 1 10/29/2012 1
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
Oscar Wilde
“Some people make the same mistake
- ver and over again and call it
experience” Herb Fred, MD
SLIDE 2 10/29/2012 2
“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
- n 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-
- p 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
SLIDE 3 10/29/2012 3
Iatrogenic Papilledema Iatrogenic Papilledema 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”
SLIDE 4 10/29/2012 4
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
Iatrogenic Papilledema Iatrogenic Papilledema
10/27/72: LP shunt 12/28/72: Acuity OD 20/30 OS 20/40-1
Fields and Fundi: see next slides
SLIDE 5
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Iatrogenic Papilledema 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
SLIDE 7 10/29/2012 7
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 Data Overload
SLIDE 8 10/29/2012 8
Data Overload
What’s going on here? What would you do next?
Data Overload
“Disease will sometimes peer up
- ver the hedge of health with
- nly its eyes showing”
John Stone, MD
SLIDE 9 10/29/2012 9
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):
Acuity: OD 20/25+2, OS 20/20-2 Color: Normal Pupils: No RAPD! Fields and Fundi: see next slides
Post Traumatic Vision Loss?
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Post Traumatic Vision Loss? Post Traumatic Vision Loss? 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
Blepharoptosis noted OS sometime thereafter 3/89: Noted diplopia → Ill-defined impaired
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 Post-traumatic Diplopia 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
Biopsy negative
Now what?
Post-traumatic diplopia
Patient followed with stable motility findings 3/90 developed “woody” firmness beneath left eye MRI repeated
SLIDE 14 10/29/2012 14
Post-traumatic diplopia 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.
SLIDE 15 10/29/2012 15 “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-
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
SLIDE 16 10/29/2012 16
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? 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:
Acuity: OD 20/20-1, OS 20/50-3 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? 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
SLIDE 19 10/29/2012 19 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 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
SLIDE 20 10/29/2012 20 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
- rbits 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 Meningiomas: Importance of Proper Neuro-Imaging
SLIDE 21 10/29/2012 21 Meningiomas: Importance of Proper Neuro-Imaging 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
SLIDE 22
10/29/2012 22 Meningiomas: Importance of Proper Neuro-Imaging Meningiomas: Importance of Proper Neuro-Imaging Meningiomas: Importance of Proper Neuro-Imaging
SLIDE 23 10/29/2012 23 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
SLIDE 24
10/29/2012 24 The Definition of Neuro- Ophthalmology
CT SCANS
The Definition of Neuro- Ophthalmology
MRI SCANS
The Definition of Neuro- Ophthalmology
MRI SCANS
SLIDE 25 10/29/2012 25
“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
1955: right 6th palsy, panhypopit → massive
sella → 3500 rads → hormone Rx
1955-1978: 6th 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
SLIDE 26 10/29/2012 26
Wild & Crazy EOMs Wild & Crazy EOMs
1/79: Intermittent diplopia 3/79: Misdiagnosed “convergence spasm”
Churchill’s commentary on man, “Man will
- ccasionally 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
SLIDE 27
10/29/2012 27
Oculomotor Neuromyotonia Oculomotor Neuromyotonia Oculomotor Neuromyotonia
SLIDE 28 10/29/2012 28
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
SLIDE 29 10/29/2012 29
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
SLIDE 30 10/29/2012 30
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
SLIDE 31 10/29/2012 31
? 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
SLIDE 32 10/29/2012 32
? 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 ? Hysterical Visual Loss
MRI SCANS
SLIDE 33
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? Hysterical Visual Loss ? Hysterical Visual Loss ? Hysterical Visual Loss
SLIDE 34 10/29/2012 34
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
SLIDE 35 10/29/2012 35
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 The Unlucky Resident
Fundoscopy:
Mild temporal pallor OU Depigmentation and pigment clumping below
left disc
See next slide
SLIDE 36
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The Unlucky Resident 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.”
SLIDE 37
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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)
SLIDE 38
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The Unlucky Resident The Unlucky Resident
MRI Scans 6/2/86 & 6/18/86
The Unlucky Resident
SLIDE 39
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The Unlucky Resident The Unlucky Resident The Unlucky Resident
SLIDE 40
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The Unlucky Resident The Unlucky Resident
OD 25 days after accident OD 139 days after accident
The Unlucky Resident
OS 25 days after accident OS 139 days after accident
SLIDE 41
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“Education never ends Watson. It is a series of lessons with the greatest for the last.” Sherlock Holmes
