Atlas of neuro-ophthalmology

329 Pages·2004·33.683 MB·English

by  Spoor, Thomas C

A
TLAS OF
N -
EURO OPHTHALMOLOGY
Thomas C Spoor
MD FACS
Professor Emeritus Ophthalmology and Neurosurgery
Wayne State University School of Medicine
Oculoplastic, Orbital and Neuro-ophthalmic Surgery
St John Macomb Hospital
Warren
USA
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Contents
Preface vii
List of Abbreviations viii
Afferent visual system
1 Pupils 1
2 Visual loss 11
3 The optic nerve 47
4 Optic neuritis 89
5 The swollen optic disc 105
6 Ischemic optic neuropathies 129
7 Traumatic optic neuropathies 143
8 Neuro-ophthalmic sequelae of closed head injury 157
Efferent visual dysfunction
9 Ptosis: differential diagnosis and treatment 181
10 Horizontal gaze disorders 203
11 Vertical motility dysfunction 239
12 Parasellar syndromes 265
13 Orbital disorders 273
Index 313

Dedication
To my female dominant society: wife Deanne, daughter Kristen and
mother Edna, thank you for your love, help and support.
Thanks also to Mary Tluczek, my secretary for more years and
more books than either of us care to remember and Trina Fennel
medical illustrator par excellence whose illustrations have enhanced
five books and countless lectures, courses and presentations.

Preface
There have been several generations of great neuro- clinical photographs for use in teaching residents and
ophthalmology texts ranging from major treatises like fellows. Here I hope to pass these photos to posterity so
the multiple Walsh and Hoyt’s (later Miller/Newman’s/ that others may benefit from my triumphs and mistakes.
Walsh and Hoyt’s). Lesser texts, no offense, that are not It has been tremendous, I have no regrets and I hope the
as detailed or compulsive have been equally excellent but next generation enjoys practicing neuro-ophthalmology
not as encyclopedic. Some texts have been unreadable as much as I have.
and totally impractical, while others have been excellent Thanks to my mentor and former preceptor, Dr Jack
office references. Beauty is in the eye of the beholder. Kennerdell. I am still using some of his slides salvaged
Neuro-ophthalmology is like extraocular motility, you from my fellowship year at the University of Pittsburgh,
get it or you do not. If you do not get it and do not some of which appear in this book, sometimes anno-
understand the big picture, you should not be practicing tated and sometimes not, but remembered and appreci-
this subspecialty. If you are trying to be a good general- ated just the same. Thanks also to my entourage of
ist and practice some neuro-op that’s great, but get the fellows and international fellows from 1984 to 2003.
picture. In the world of great neuro-imaging it is hard to Their hard work, and excellent, compulsive patient care
miss a brain tumor, but it is easy to be a bit obtuse beat- was responsible for much of my success and for a few of
ing around the bush looking for an appropriate diagno- my failures. It was however great fun, and made the prac-
sis. I will show you examples of visual loss caused by tice of both academic and clinical neuro-ophthalmology
pituitary tumors and attributed to macular degenera- much more rewarding.
tion, and visual loss secondary to huge tumors attributed There are many compulsively referenced neuro-
to psychosis, diagnosed by well-respected university pro- ophthalmology texts but I have referenced few in this
fessors. Stop learning and the best imaging in the world atlas. However, I have made a point to reference topics
is no substitute for clinical acumen and judgement. The that might be controversial, to direct the reader to pri-
best teacher in neuro-ophthalmology is time, which mary sources so that they can make their own decisions.
unfortunately does not help the undiagnosed and In today’s world of computerized information retrieval it
untreated patient. is very easy to research a subject, so I feel no need or obli-
This book is the result and completion of my career in gation to reference every statement I make. If you dis-
neuro-ophthalmology. It has been a lot of fun, I have agree, research the issue and draw your own conclusions.
helped many people and have hurt a few, but it has been All I wish to do is expose you to a great subspecialty and
a great ride. Over the years I have taken thousands of lure you into a deeper thought process.
Thomas C Spoor
Abbreviations
AAION arteritic anterior ischemic optic neuropathy IOP intraocular pressure
ACE angiotensin-converting enzyme IR inferior rectus (muscle)
AIBSE acute idiopathic blind-spot enlargement LHON Leber’s hereditary optic neuropathy
AIDS acquired immune deficiency syndrome LTG low-tension glaucoma
AION arteritic ischemic optic neuropathy MAR melanoma-associated retinopathy
AMPE acquired monocular elevator palsy MEWDS multiple evanescent white-dot syndrome
APMPPE acute posterior multifocal placoid MG myasthenia gravis
pigment epitheliopathy MLF median longitudinal fasciculus
AR Argyll Robertson (pupils) MR medial rectus (muscle)
AZOOR acute zonal occult outer retinopathy MRI magnetic resonance imaging
CACD cancer-associated cone dysfunction MS multiple sclerosis
CAR cancer-associated retinopathy NAION non-arteritic anterior ischemic optic
CME cystoid macular edema neuropathy
CMV cytomegalic inclusion virus NLP no light perception
CPEO chronic progressive external OD right eye
ophthalmoplegia ONSD optic nerve sheath decompression
CRAO central retinal artery occlusions ONTT Optic Neuritis Treatment Trial
CRVO central retinal vein occlusions OS left eye
CSF cerebrospinal fluid OU both eyes
CT computerized tomography PCA posterior communicating artery
DON dysthyroid optic neuropathy PION posterior ischemic optic neuropathy
ERG electroretinogram PPRF parapontine reticular formation
ERM epiretinal membranes PR paraeoplastic retinopathies
ESR erythrocyte sedimentation rate PTC pseudotumor cerebri
FTA-ABS fluorescent treponemal antibody RAPD relative afferent pupillary defect
absorption riMLF rostral interstitial nucleus of the medial
GCA giant cell arteritis longitudinal fasciculus (muscle)
HIV human immunodeficiency virus RPE retinal pigment epithelium
HMO health maintenance organization SLE systemic lupus erythmatosus
ICA internal carotid artery SR superior rectus (muscle)
ICP intracranial pressure STA superficial temporal artery
INH isoniazid TAO thyroid-associated orbitopathy
INO internuclear ophthalmoplegia VDRL venereal disease research lab
IO inferior oblique (muscle) WBC white blood count
IOI idiopathic orbital inflammation WEBINO wall-eyed bilateral internuclear
IONDT Ischemic Optic Neuropathy ophthalmoplegia
Decompression Trial
Chapter 1 Pupils
INTRODUCTION
The appearance of a patient with a dilated pupil in the
ophthalmologist’s or neurologist’s office raises the possi-
bility of an acute neurologic emergency (Fig. 1.1); how-
ever, this is very rarely the case. An isolated pupillary
abnormality is more often than not a benign event, with
much more ophthalmologic than neurologic significance.
On the other hand, pupillary involvement accompanied
by other neurologic findings (Figs 1.2 and 1.3), obvious
or subtle, may portend a very serious neurologic prob-
lem. A practical, intelligent approach to evaluating a
pupillary abnormalities will save the patient unnecessary
procedures and anxiety. Examination of the pupil also
helps to differentiate the various causes of visual dys-
function. A relative afferent pupillary defect (RAPD) is
the sine qua non of optic nerve dysfunction and its pres-
ence or absence helps differentiate visual loss due to
optic neuropathies from maculopathies and occipital
lobe pathology.
Examination of the pupil with magnification, pre-
ferably with a slit lamp, may avoid unnecessary testing,
anxiety and expense. Obvious synechiae (Fig. 1.4a),
vermilliform motions, sphincter rupture or dysfunction
b
c
Figure 1.2 A patient with an obvious pupil involving third-
Figure 1.1 An isolated, dilated pupil is rarely a neurologic nerve palsy (a and b) due to a posterior-communicating internal
emergency (a). carotid artery aneurysm evidenced on cerebral angiogram (c).
2 PUPILS
a
Figure 1.3 Intracranial aneurysms may also present as a
much more subtle third-nerve palsy. Note the pupil is involved
to the same degree as the extraocular motility.
from trauma or intraocular surgery are obvious
ophthalmologic causes of abnormal pupils and require
no further neurologic evaluation.
FUNCTION
The pupil functions like the f-number ofa camera. It reg-
ulates the amount of light reaching the retina (analogous
b
to the film in a camera), increases the depth offocus, and
diminishes chromatic and spherical aberrations. This is
accomplished by the antagonistic actions of the iris
sphincter and dilator. The iris sphincter is attached to
the iris border in a circumferential pattern and is inner-
vated by the parasympathetic nervous system. The iris
dilator muscles are weaker, radially arranged and inner-
vated by the sympathetic nervous system. The pupil is
constantly moving, responding to changes in ambient
lighting and innervation. This constant movement
(hippus) is normal and needs to be differentiated from
the early release of a RAPD.
ANATOMY
c
PUPILLARY REACTION TO LIGHT1
Afferent pupillary function depends on the integrity of Figure 1.4 Slit-lamp examination may reveal an obvious
the retinal receptors, the retinal ganglion cells and their ophthalmologic cause of the abnormal pupil–iris sphincter
axons in the optic nerve, chiasm and tract (Fig. 1.5). Like atrophy (a), iritis with posterior synechiae (b) or rubeosis
the visual fibers, approximately one half of the fibers iridis (c).