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 Atlas of
RETINAL OCT
This page intentionally left blank
Atlas of
RETINAL OCT
Darin R. Goldman MD
Partner, Retina Group of Florida
Affiliate Associate Professor
Charles E. Schmidt College of Medicine
Florida Atlantic University
Boca Raton, FL, USA

Nadia K. Waheed MD, MPH

Assistant Professor of Ophthalmology
New England Eye Center
Tufts Medical Center
Tufts University School of Medicine
Boston, MA, USA

Jay S. Duker MD
Director, New England Eye Center
Professor and Chairman
Department of Ophthalmology
Tufts Medical Center
Tufts University School of Medicine
Boston, MA, USA

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Notices

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broaden our understanding, changes in research methods, professional practices, or medical treatment
may become necessary.

Practitioners and researchers must always rely on their own experience and knowledge in evaluating
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PART 1: NORMAL OPTICAL COHERENCE TOMOGRAPHY
Section 1: Normal Optic Nerve.............................................1
Chapter 1.1: Normal Optic Nerve..........................1
Section 2: Normal Retina......................................................4
Chapter: 2.1: Time-Domain OCT...........................4
Chapter: 2.2: Spectral Domain OCT......................5
Chapter: 2.3: Swept-Source OCT..........................6
Section 3: Normal Choroid....................................................7
Chapter 3.1: Normal Choroid.................................7
Section 4: Normal Vitreous ..................................................9
Chapter 4.1: Normal Vitreous...............................9
Section 5: OCT: Artifacts and Errors..................................10
Chapter 5.1: OCT: Artifacts and Errors................10
Chapter 5.2: OCT Angiography Artifacts..............13
PART 2: ISOLATED MACULAR DISORDERS
Section 6: Age-Related Macular Degeneration.................16
Chapter 6.1.1: Drusen.........................................16
Chapter 6.1.2: Geographic Atrophy.....................24
Chapter 6.1.3: Isolated Pigment Epithelial
Detachment......................................................28
Chapter 6.2.1: Type 1 Choroidal Neovascular
Membrane........................................................30
Chapter 6.2.2: Type 2 Choroidal Neovascular
Membrane........................................................32
Chapter 6.2.3: Type 3 Choroidal Neovascular
Membrane........................................................34
Chapter 6.2.4: Subretinal Hemorrhage................35
Chapter 6.2.5: Disciform Scar.............................36
Chapter 6.2.6: Retinal Pigment Epithelial Tear.....37
Chapter 6.2.7: Polypoidal Choroidal
Vasculopathy....................................................38
Section 7: Vitreomacular Interface Disorders....................40
Chapter 7.1: Vitreomacular Adhesion..................40
Chapter 7.2: Vitreomacular Traction....................42
Chapter 7.3: Full-Thickness Macular Hole...........47
Chapter 7.4: Lamellar Macular Hole....................50
Chapter 7.5: Epiretinal Membrane.......................52
Section 8: Central Serous Chorioretinopathy....................55
Chapter 8.1: Central Serous
Chorioretinopathy.............................................55
Section 9: Myopic Degenerative Maculopathies...............59
Chapter 9.1: Myopic Choroidal Neovascular
Membrane........................................................59
Chapter 9.2: Myopic Macular Schisis..................62
Chapter 9.3: Dome-Shaped Macula....................64
Chapter 9.4: Posterior Staphyloma......................66
Contents
Section 10: Hydroxychloroquine Toxicity............................68
Chapter 10.1: Hydroxychloroquine Toxicity..........68
Section 11: Vitelliform Macular Dystrophy...........................72
Chapter 11.1: Vitelliform Dystrophy......................72
Section 12: Macular Telangiectasia......................................73
Chapter 12.1: Macular Telangiectasia..................73
Section 13: Isolated Cystoid Macular Edema......................76
Chapter 13.1: Isolated Cystoid Macular
Edema..............................................................76
Section 14: Other Disorders Affecting the Macula..............78
Chapter 14.1: Angioid Streaks.............................78
Chapter 14.2: X-Linked Juvenile Retinoschisis.....79
Chapter 14.3: Oculocutaneous Albinism..............80
Chapter 14.4: Subretinal Perfluorocarbon............82
PART 3: VASOOCCLUSIVE DISORDERS
Section 15: Diabetic Retinopathy.........................................84
Chapter 15.1: Diabetic Macular Edema...............84
Chapter 15.2: Nonproliferative Diabetic
Retinopathy......................................................86
Chapter 15.3: Proliferative Diabetic
Retinopathy......................................................88
Section 16: Retinal Venous Occlusive Disease ..................90
Chapter 16.1: Branch Retinal Vein
Occlusion.........................................................90
Chapter 16.2: Central Retinal Vein Occlusion......92
Section 17: Retinal Arterial Occlusive Disease....................96
Chapter 17.1: Branch Retinal Artery
Occlusion.........................................................96
Chapter 17.2: Central Retinal Arterial
Occlusion.........................................................98
PART 4: UVEITIS AND INFLAMMATORY DISORDERS
Section 18: Noninfectious Uveitis.......................................100
Chapter 18.1.1: Birdshot
Retinochoroidopathy.......................................100
Chapter 18.1.2: Acute Posterior Multifocal
Placoid Pigment Epitheliopathy.......................103
Chapter 18.1.3: Multiple Evanescent White
Dot Syndrome ...............................................107
Chapter 18.1.4: Serpiginous Choroiditis............109
Chapter 18.1.5: Multifocal Choroiditis
and Panuveitis and Punctate Inner
Choroidopathy................................................111
Chapter 18.2: Vogt-Koyanagi-Harada
Disease...........................................................114
Chapter 18.3: Sympathetic Ophthalmia.............116
v
 Section 19: Infectious Uveitis.............................................119
Chapter 19.1: Toxoplasmic Chorioretinitis.........119
Chapter 19.2: Acute Syphilitic Posterior
Placoid Chorioretinitis.....................................121
Chapter 19.3: Tuberculosis................................124
Contents
Chapter 19.4: Posterior Scleritis........................127
Chapter 19.5: Candida Chorioretinitis................129
Chapter 19.6: Acute Retinal Necrosis
Syndrome.......................................................132
PART 5: RETINAL AND CHOROIDAL TUMORS
Section 20: Choroidal Tumors............................................134
Chapter 20.1: Choroidal Nevus.........................134
Chapter 20.2: Choroidal Melanoma...................135
Chapter 20.3: Solitary Choroidal
Hemangioma..................................................136
Section 21: Retinal Tumors.................................................137
Chapter 21.1: Retinal Capillary
Hemangioma..................................................137
Section 22: Retinal Pigment Epithelium Tumors...............138
Chapter 22.1: Simple Hamartoma of
the RPE..........................................................138
Chapter 22.2: Combined Hamartoma of
the Retina and RPE........................................139
Section 23: Metastatic Choroidal Tumors.........................140
Chapter 23.1: Choroidal Metastases.................140
PART 6: TRAUMA
Section 24: Mechanical Trauma.........................................142
Chapter 24.1: Valsalva Retinopathy...................142
Section 25: Photic Maculopathy.........................................145
Chapter 25.1: Laser Maculopathy......................145
Chapter 25.2: Solar Maculopathy......................146
vi
PART 7: INHERITED RETINAL DEGENERATIONS
Section 26: Retinal Dystrophies.........................................148
Chapter 26.1: Retinitis Pigmentosa....................148
Chapter 26.2: Stargardt Disease.......................149
Chapter 26.3: Best Disease...............................152
Chapter 26.4: Cone Dystrophy..........................154
Chapter 26.5: Malattia Leventinese
(Doyne’s Honeycomb Retinal
Dystrophy)......................................................155
Chapter 26.6: Central Areolar Choroidal
Dystrophy.......................................................157
PART 8: VITREOUS DISORDERS
Section 27: Posterior Vitreous Detachment.......................159
Chapter 27.1: Stages of Posterior Vitreous
Detachment....................................................159
Section 28: Asteroid Hyalosis.............................................162
Chapter 28.1: Asteroid Hyalosis........................162
Section 29: Vitreous Hemorrhage.......................................164
Chapter 29.1: Vitreous Hemorrhage..................164
Section 30: Vitreous Inflammation......................................166
Chapter 30.1: Vitreous Inflammation..................166
PART 9: MISCELLANEOUS RETINAL DISORDERS
Section 31: Peripheral Retinal Abnormalities....................170
Chapter 31.1: Tractional Retinal
Detachment....................................................170
Chapter 31.2: Rhegmatogenous Retinal
Detachment....................................................174
Chapter 31.3: Bullous Retinoschisis...................180
Chapter 31.4: Lattice Degeneration...................183
Chapter 31.5: Myelinated Nerve Fiber Layer......186

Optical coherence tomography (OCT) continues to occupy an
ever-expanding role in the ophthalmic community. OCT is widely
available and forms a requisite portion of the comprehensive
ophthalmic evaluation, particularly as it pertains to the retina.
Although still a relatively young technology that continues to
evolve, OCT has become widely accepted. This acceptance is
due to its non-invasive nature, ease of image acquisition, and
wealth of information that it affords. The quantity of information
conveyed within a typical OCT scan is immense, which can be
daunting to both the beginner and experienced clinician.
Atlas of Retinal OCT grew out of the success of Handbook of
Retinal OCT. The Atlas expands on the images and material in
the handbook, while maintaining a similar and consistent layout
that will be familiar to the reader. This atlas was created to serve
as a supplement to the original text, although the atlas certainly
Preface
can stand alone as an independent reference. We sought to
include a breadth of retinal conditions with a focus on those most
applicable to everyday clinical practice. However, a wide array
of pathology is included to also illustrate unique, less common
OCT findings. Each condition is illustrated with numerous, large,
high-quality OCT images to highlight disease pathology and aid
in disease identification. Additional imaging modalities, such as
fundus photographs and fluorescein angiograms, are included
to supplement OCT images where appropriate.
Atlas of Retinal OCT provides the reader with a high quality,
easy-to-follow visual aid to incorporating OCT scans into the
evaluation and care of your patients. The atlas is designed to
make OCT more comprehensible for both the novice and expert
clinician. We hope that the reader finds this to be a handy and
practical addition to your everyday reference armamentarium.
vii
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 Contributors

A YASIN ALIBHAI, MD NORA W. MUAKKASSA, MD

OCT Research fellow, Ophthalmology, New England Eye New England Eye Center, Tufts Medical Center, Hospital de
Center, Tufts Medical Center, Boston, Massachusetts, USA Olhos do Paraná, Curitiba, Brazil

CAROLINE R. BAUMAL, MD CARLOS A. MOREIRA NETO, MD, PhD

New England Eye Center, Tufts Medical Center, Boston, New England Eye Center, Tufts Medical Center, Hospital de
Massachusetts, USA Olhos do Paraná, Curitiba, Brazil

SHILPA DESAI, MD, FRCP EDUARDO A. NOVAIS, MD

Assistant Professor, Ophthalmology, New England Eye Department of Ophthalmology, Federal University of São
Center/Tufts University Medical Center, Boston, MA, USA Paulo, School of Medicine, São Paulo, Brazil

IVANA N. DESPOTOVIC, MD CARL REBHUN, BA

New England Eye Center, Tufts University School of Medicine, New England Eye Center, Tufts Medical Center, Tufts
Boston, MA, USA University School of Medicine, Boston, USA

JAY S. DUKER, MD LUIZ ROISMAN, MD

Director, New England Eye Center, Professor and Chairman,
Department of Ophthalmology, Tufts Medical Center, Tufts
University School of Medicine, Boston, MA, USA
DANIELA FERRARA, MD, PhD
Assistant Professor of Ophthalmology, Tufts University School
of Medicine, Boston, MA, USA
DARIN R. GOLDMAN, MD
Partner, Retina Group of Florida, Affiliate Associate Professor,
Charles E. Schmidt College of Medicine, Florida Atlantic
University, Boca Raton, FL, USA
Department of Ophthalmology, Federal University of São
Paulo, School of Medicine, São Paulo, Brazil
EDUARDO UCHIYAMA, MD
Retina Group of Florida, Boca Raton, FL, USA
NADIA K. WAHEED, MD, MPH
Assistant Professor of Ophthalmology, New England Eye
Center, Tufts Medical Center, Tufts University School of
Medicine, Boston, MA, USA

ix
This page intentionally left blank
 Acknowledgments

A project such as this requires contributions from many different
groups and individuals to be successful. First and foremost,
the images used in this atlas would not be possible without
our many patients. We are very grateful to these individuals
who trust their care in our hands on a daily basis. Additionally,
we rely on the talented photographers and technical staff at
both the New England Eye Center at Tufts Medical Center and
Retina Group of Florida to obtain the majority of the included
OCT images. Their expertise is reflected in the volume of high
quality images available for inclusion in this project. We would
also like to thank the many co-authors who have contributed
to various chapters throughout the atlas. Additionally, thanks
are due to our fellows whose archive of cases and interesting
images were invaluable to this project. Specifically, we would like
to thank Dr. Chris Or, who provided invaluable feedback on the
final chapters. Lastly, the professionalism and expertise of the
staff at Elsevier is unmatched. We want to thank the entire team
at Elsevier who were critical to the completion of this project,
in particular Russell Gabbedy, Humayra Rahman Khan, Joshua
Mearns, and Andrew Riley.

Dedications
To the memory of my dear sister Candice, whose love, strength
and determination live on in all that she touched. And to my
daughter, Rona, who has added immeasurable joy to our lives.
D.R.G.

To Jujie, Memsie and Ammi, without whom none of this would

have been possible.
N.K.W.

To my colleagues at the New England Eye Center who have

assisted me in bringing innovation to eye care for over 3 decades.
J.S.D.

xi
This page intentionally left blank

Spectral domain OCT (SD-OCT) devices have two scan patterns
to analyze the optic nerve head (ONH): volume scans and line
scans.
Volume Scans
Volume scans acquire a volumetric set of data, centered at
the ONH. It delineates the optic disc margin and optic disc
surface contour and is segmented to obtain the retinal nerve
fiber boundaries. Each device has its own scanning protocol.
The Cirrus HD-OCT identifies the center of the optic disc and
creates a 3.46-mm circle on this location and calculates the
thickness of the retinal nerve fiber layer (RNFL). The Heidelberg
Spectralis creates a cylindrical volume with a diameter of 3.4 mm
through and around the ONH (Duker, Waheed & Goldman 2014).
The Optovue RTVue’s protocol for the ONH consists of a grid
pattern with circular and radial scans that acquires a 4- × 4-mm
volume around the optic nerve. Because different machines use
circles of different diameters around the center of the ONH, the
measurement of RNFL between machines is not comparable
(Duker et al. 2014).
Retinal Nerve Fiber Layer Thickness (RNFL)
OCT devices calculate RNFL thickness as the distance between
the internal limiting membrane and the outer aspect of the RNFL
(Fig. 1).
Ganglion Cell Complex
The ganglion cell complex (GCC) consists of the thickness of
three inner retinal layers: the NFL, the ganglion cell layer, and the
inner plexiform layer. The scan is centered at the fovea, and the
software presents the results as a color-coded map, comparing
to a normative database (Fig. 2).
SECTION 1: NORMAL OPTIC NERVE
Normal Optic Nerve
Carlos A. Moreira Neto | Carl Rebhun 1.1
Optic Nerve Morphology
SD-OCT devices also calculate optic nerve diameter, area, cup,
and rim measurements (see Fig. 1). Each measurement varies
according to age (Cavallotti et al. 2002) and ethnicity (Girkin 2008).
According to Budenz et al. (2007), the mean RNFL thickness in
a normal population is 100.1 µm. Thinner RNFL measurements
were associated with older age. Caucasians had slightly thinner
RNFL thickness than Hispanics or Asians. Persons with smaller
optic disc areas also have thinner RNFL thickness.
Line Scans
Aiming to obtain a higher resolution visualization of structure and
anatomic anomalies at the ONH, line scans provide a single or a
series of high-resolution B-scans similar to the scans obtained
in the macula (Fig. 3).
OCT angiography (OCTA) (Fig. 4) allowed for a greater under-
standing of optic disc vasculature and peripapillary vessel density.
This information provides insight into the role of this vascular
bed in the functioning of the RNFL.
REFERENCES
Budenz DL, Anderson DR, Varma R, et al. Determinants of normal retinal
nerve fiber layer thickness measured by Stratus OCT. Ophthalmology.
2007;114(6):1046–1052.
Cavallotti C, Pacella E, Pescosolido N, et al. Age-related changes in the
human optic nerve. Can J Ophthalmol. 2002;37(7):389–394.
Duker JS, Waheed NK, Goldman DR. Scanning Principles. Handbook of Retinal
OCT. St Louis: Elsevier; 2014.
Girkin CA. Differences in optic nerve structure between individuals of predomi-
nantly African and European ancestry: Implications for disease detection
and pathogenesis. Clin Ophthalmol. 2008;2(1):65–69.
1
 ONH and RNFL OU Analysis: Optic Disc Cube 200¥200 OD OS
RNFL Thickness Map ! OD OS
350
Section 1: Normal Optic Nerve

Average RNFL Thickness 102 m X

RNFL Symmetry X

Rim Area 1.88 mm2 X

175
Disc Area 2.17 mm2 X

Average C/D Ratio 0.36 X

Vertical C/D Ratio 0.32 X

0 m
Cup Volume 0.009 mm3 X

RNFL Deviation Map

Neuroretinal Rim Thickness
m OD
800

400

0
TEMP SUP NAS INF TEMP

Disc Center (0.09,0.12) mm

RNFL Thickness
Extracted Horizontal Tomogram
m OD

200

100

TEMP SUP NAS INF TEMP

Extracted Vertical Tomogram
Diversified:
121 Distribution of Normals

S NA 95% 5% 1%

86 T N 72

I RNFL
Quadrants
RNFL Circular Tomogram 129
93
162 109
113 80

64 65 RNFL
Clock
80 70 Hours
174 74
137

FIG. 1. Normal peripapillary RNFL, neuroretinal rim thickness, and disc area measurements using SD-OCT.

2
 GCC Right / OD
1.1
Thickness Map Signal Strength Index 73 NDB Reference Map
250 6 mm × 6 mm

Average Thickness Thickness (m)

Total 89.28
Superior 88.52

Normal Optic Nerve

Inferior 90.03
200 Intra Eye Difference (S-I) –1.51
FLV (%) 1.058
GLV (%) 6.029

150

T N

Fovea
100

50

p > 5% Within Normal

0 m p > 5% Borderline
p < 1% Outside Normal

FIG. 2. Normal color-coded ganglion cell complex (GCC) thickness using SD-OCT.

FIG. 3. Line scan of the ONH. FIG. 4. OCT angiograph image (3 × 3 mm) of the ONH.

3
 SECTION 2: NORMAL RETINA

Time-Domain OCT
Carlos A. Moreira Neto | Carl Rebhun 2.1
The first OCT image, published by Huang et al. (1991), was REFERENCES
captured using a device that detected light echoes using time Huang D, Swanson EA, Lin CP, et al. Optical coherence tomography. Science.
1991;254(5035):1178–1181.
domain detection. In time domain OCT (TD-OCT) the reference Duker JS, Waheed NK, Goldman DR. Scanning principles. In: Handbook of
arm, with a physically moving mirror, and a sample arm undergo Retinal OCT. St Louis: Elsevier; 2014.
interference, which is used to generate an A-scan. Multiple
A-scans obtained linearly are combined to generate a cross-
sectional B-scan (Duker et al. 2014).

4

Summary
In spectral domain OCT (SD-OCT), a spectral interference
pattern between the reference beam and the sample beam is
obtained simultaneously by a spectrometer and an array detec-
tor. Unlike time domain (TD)-OCT, SD-OCT does not require
a physically moving reference mirror, instead using frequency
information to produce interference patterns. This allows for
much faster acquisition and higher quality images than those with
TD-OCT.
The high resolution provided by SD-OCT allows for visualization
of the microscopic anatomy of the retina (Fig. 1) with more detail
than with TD-OCT.
Spectral Domain OCT
Carlos A. Moreira Neto | Carl Rebhun 2.2
Because the retinal pigment epithelium (RPE) is highly hyper-
reflective with OCT imaging, there is limited penetration of light
beyond it, decreasing the resolution of the choroid (Schuman,
Fujimoto & Duker 2013). Normal mean central foveal thickness is
approximately 225 ± 17 µm as measured by SD-OCT, although
this varies with age and retinal status.
REFERENCE
Schuman J, Fujimoto J, Duker J. Optical Coherence Tomography of Ocular
Diseases. 3rd ed. Thorofare NJ: Slack Inc.; 2013.

Ganglion cell Outer

layer plexiform Outer
Inner plexiform layer nuclear
layer Inner layer Nerve
nuclear fiber
layer layer

Photoreceptors
Choriocapillaris outer segments
RPE/Bruch’s
complex
External
IS/OS/EZ Myoid limiting
zone membrane
S
3
T N

FIG. 1. Normal macula imaged using SD-OCT. IS/OS/EZ, Inner segment/outer segment/ellipsoid zone; RPE, retinal pigment epithelium.

5
 Swept-Source OCT
Carlos A. Moreira Neto | Carl Rebhun 2.3
Summary penetration of the choroid (Fig. 1). In SS-OCT, a narrow-band
light source is rapidly swept through a wide range of frequencies.
Swept source OCT (SS-OCT) is a modified Fourier-domain and The interference pattern is detected on a single or small number
depth-resolved technology that offers potential advantages over of receivers as a function of time.
SD-OCT, including reduced sensitivity roll-off with imaging depth,
higher detection efficiencies, improved imaging range, and better

Inner
Ganglion
plexiform Outer cell
layer plexiform layer
Inner layer
nuclear Outer Nerve
layer nuclear fiber
layer layer

Choriocapillaris
Choroidal larger
vessels
External Myoid
limiting zone
membrane
IS/OS/ RPE/
EZ Bruch’s Choroid/
complex sclera
Photoreceptors junction
outer
segments

FIG. 1. Normal retina imaged using SS-OCT. EZ, ellipsoid zone; IS, inner segments; OS, outer segments; RPE, retinal pigment epithelium.

6

Summary
Enhanced depth imaging (EDI) on commercially available OCT
devices allows for higher quality images of the choroid (Fig. 1).
EDI mode moves the zero-delay line of the spectral domain
(SD)-OCT closer to the choroid, enabling better visualization of
choroidal structures and a more precise measurement of choroidal
thickness than standard OCT scanning protocols. This is useful for
diseases such as central serous chorioretinopathy, in which the
choroidal-scleral interface may be difficult to visualize. Studies of
choroidal thickness in normal subjects and those with pathologic
processes have shown a wide variation in measurements (Fujiwara
et al. 2012; Margolis & Spaide 2009).
SECTION 3: NORMAL CHOROID
Normal Choroid
Carlos A. Moreira Neto | Carl Rebhun 3.1
The choroid is divided into three layers, the choriocapillaris
or smaller blood vessels, Sattler’s layer, and Haller’s layer, or
larger blood vessels (Fig. 2).
REFERENCES
Margolis R, Spaide RF. A pilot study of enhanced depth imaging optical
coherence tomography of the choroid in normal eyes. Am J Ophthalmol.
2009;147(5):811–815.
Fujiwara A, Shiragami C, Shirakata Y, et al. Enhanced depth imaging spectral-
domain optical coherence tomography of subfoveal choroidal thickness in
normal Japanese eyes. Jpn J Ophthalmol. 2012;56(3):230–235.
7
Section 3: Normal Choroid

Choriocapillaris
Larger choroidal Choroid
Stroma
vessels
S S
3 3
T N
Lumens of larger blood
T N
vessels
Choroid/sclera
I I junction
A B
FIG. 1. Chorioretinal OCT image not using EDI (A) and using EDI (B).

Larger choroidal vessels

A B

FIG. 2. En face structural OCT images of choriocapillaris (A) and Haller/Sattler layers (B).

8
 SECTION 4: NORMAL VITREOUS

Summary
Until recently, the anatomy of the vitreous could not be imaged
in vivo. With the use of OCT, a better view and understanding
of vitreous structure has become possible. Along with normal
structure, abnormal vitreous processes such as vitreomacular trac-
tion have been revealed (Duker et al. 2013). High dynamic range
imaging as well as enhanced vitreous imaging techniques, present
on most commercially available OCT devices, allow visualization
of the fluid-filled spaces as well as the collagenous and cellular
structure of the vitreous. Secondary features of vitreous debris
are also often identifiable on SD-OCT (Fig. 1).
Normal Vitreous
Nadia K. Waheed 4.1
Key OCT Features
In OCT of a normal retina the following vitreous structures may
be observed:
• Posterior cortical vitreous (posterior hyaloid) (Fig. 2)
• Retrohyaloid space: Created after posterior vitreous detachment
(Fig. 2).
• Premacular bursa: Liquid space overlying the macula, caused
by liquefaction of the vitreous (Fig. 3).
REFERENCE
Duker JS, Kaiser PK, Binder S, et al. The International Vitreomacular Traction
Study Group classification of vitreomacular adhesion, traction, and macular
hole. Ophthalmology. 2013;120(12):2611–2619.

Posterior cortical vitreous (posterior hyaloid)

Retrohyaloidal space

S
1
N T

FIG. 2. Posterior hyaloid and retrohyaloid spaces.

Vitreous

B Premacular bursa Vitrepapillary adhesion

FIG. 1. Vitreous opacity (arrows) demonstrates shadowing on SS-OCT. FIG. 3. Premacular bursa in a normal patient using SD-OCT.

9
 SECTION 5: OCT: ARTIFACTS AND ERRORS

OCT: Artifacts and Errors

Carlos A. Moreira Neto | Carl Rebhun 5.1
Artifacts can occur during image acquisition or analysis because of • The Early Treatment Diabetic Retinopathy Study (ETDRS) grid
patient, operator, or software factors. Accurate image interpreta- usually can be moved to obtain an accurate measure of the
tion depends on the quality of the image and an understanding foveal thickness.
of the various artifacts that can affect an OCT image (Duker,
Waheed & Goldman 2014).
Software Breakdown
Mirror Artifact • OCT segmentation lines are incorrectly drawn because there
is misidentification of the inner or outer retinal boundaries.
• Occurs only on spectral domain (SD)-OCT. • Vitreomacular surface disorders (epiretinal membrane, vitreo-
• Occurs when the area of interest crosses the zero-delay line macular traction) could cause inner line breakdown.
and results in an inverted image. • Outer retinal/retinal pigment epithelium disorders (age-related
• Reasons macular degeneration, cystoid macular edema) might cause
1. OCT device is pushed too close to the eye. outer line breakdown (Fig. 4).
2. Conditions in which the curvature of the retina is such that
it crosses the zero-delay line, such as retinoschisis, retinal
detachment, an elevated choroidal lesion, or high myopia
Blink Artifact
(Fig. 1). • If a patient blinks during image acquisition, loss of data occurs.
• OCT scans and volumetric maps both show black or white
bars (Fig. 5).
Vignetting
• Occurs when the iris blocks a part of the OCT beam. Motion Artifact
• Loss of signal is seen over one side of the image (Fig. 2).
• Occurs when there is movement of the eye during scan
acquisition.
Misalignment
• OCT image shows distortion or double scanning of the
• This occurs when the fovea is not centered during the volu- same area.
metric scan (Fig. 3). • Blood vessels are misaligned (Fig. 6).
• Most common reason is a patient with poor fixation or incorrect • The fovea may be duplicated.
placement of fixation target by operator. • This is much less common due to better eye tracking software
on current OCT machines.

Real image

Mirror image

Fovea

FIG. 1. Mirror artifact occurring in a high myopic eye.

FIG. 2. Vignetting: Loss of signal over the left side of the image. FIG. 3. Misalignment error. The fovea is not centered because of an
eccentric fixation.

10
 5.1

OCT: Artifacts and Errors

A

FIG. 4. Software breakdown caused by choroidal neovascularization (A) FIG. 6. Motion artifact.
and geographic atrophy (B).

Blink artifact

FIG. 5. Blink artifact.

11
 Out of Range Error
• Occurs when the B-scan is not centered in the preview screen,
resulting in it being shifted out of the scanning range.
• A section of the OCT scan is cut off (Fig. 7).
Section 5: OCT: Artifacts and Errors

REFERENCE
Duker JS, Waheed NK, Goldman DR. Artifacts on OCT. Handbook of Retinal
OCT. St Louis: Elsevier; 2014.

FIG. 7. Out-of-range error. Due to improper positioning of the machine

during image acquisition, the outer retina and the choroid are cut off.

12
 OCT Angiography Artifacts
Carlos A. Moreira Neto | Carl Rebhun 5.2
Artifacts are very common in OCT angiography, and their Segmentation Errors (Fig. 6)
identification is important for appropriate image interpretation
(Ferrara, Waheed & Duker). • Caused by PED, macular edema, or other pathologic process
that disrupts the horizontal alignment of retinal layers.

Blockage Artifacts (Fig. 1)

• Blockage artifacts are caused by lesions that affect light
penetration through ocular tissues, including both the anterior
and posterior segments.
• Anterior segment blockage can be cause by cataract, inflam-
mation, or corneal scar.
• Posterior segment blockage can be caused by intravitreal
hemorrhage or inflammation, floaters, intraretinal or subretinal
hemorrhage, pigment epithelial detachment (PED), or large
drusen.
Shadowing Artifacts (Fig. 7)
• Usually appear in choriocapillary segmentation.
• Caused by PED, hemorrhage, floaters.
REFERENCES
Ferrara D, Waheed NK, Duker JS. Investigating the choriocapillaris and choroidal
vasculature with new optical coherence tomography technologies. Prog
Retin Eye Res. 2016;52:130–155.
Spaide RF, Fujimoto JG, Waheed NK. Image Artifacts in Optical Coherence
Tomography Angiography. Retina. 2015;35(11):2163–2180.

White Line Artifacts (Fig. 2)

• Caused by transverse ocular movements.
• A major cause of artifacts in OCT angiography.
False Positive Flow
• Ocular movements are in the axial direction (arterial pulsation).
• An OCT dataset may be displaced and may have enough
decorrelation to cause the appearance of flow (Ferrara, Waheed
& Duker 2016; Spaide, Fujimoto & Waheed 2015).

Quilting Defects (Fig. 3)

• Related to software correction of ocular movement.
• Caused by multiple saccades in the horizontal and vertical
directions.

False Negative Flow

• Caused by blood flow below a given threshold.
• Vessels seem absent even if they are present.
Projection Artifacts (Fig. 4)
• Superficial vessels are seen in deep and choroidal slabs when
they are not actually present in those slabs (Ferrara et al.
2016; Spaide et al. 2015).

Vessel Duplication (Fig. 5)

• Result of a breakdown in registration of the X and Y scans.
• Caused by eye movement. FIG. 1. Blockage artifact causing a focal loss of signal.

13
 White line
Section 5: OCT: Artifacts and Errors

FIG. 2. White line artifact. FIG. 3. Quilting artifact.

FIG. 4. Projection artifact on deep plexus. Vessels from the superficial FIG. 5. Vessel duplication.
plexus (arrows) are seen in the deep plexus.

14
 5.2

OCT Angiography Artifacts

FIG. 6. Segmentation error (green line) caused by a pigment epithelial detachment.

FIG. 7. Shadowing artifact (arrow) in the choriocapillaris segmentation.

15

Ivana N. Despotovic | Daniela Ferrara
Summary
Drusen are focal yellow or white deposits of extracellular debris
located between the retinal pigment epithelium (RPE) and
Bruch’s membrane. They occur naturally with age and usually
are asymptomatic. Drusen are the hallmark of age-related
macular degeneration (AMD) and the most common early sign
of nonexudative AMD. Esterified and unesterified cholesterol are
significant components of the lipid-rich lesions associated with
AMD (basal linear deposits and soft drusen) and comprise more
than 40% of hard druse volume (Curcio et al. 2011).
Drusen may range in appearance, size, and location. Hard
drusen are smaller and have distinct margins (Figs. 2, 3, 4, 5, 6,
10, and 12). Soft drusen are larger, mound-like elevations that
may have a diameter greater than 1000 µm, with margins that
are not clearly defined (Figs. 1, 2, 3, 4, 7, 8, 9, and 11). A large
number of round and punctate cuticular drusen give a “stars
in the sky” appearance. Cuticular drusen have a spheroid or
triangular shape on OCT (Fig. 12).
The tomographic features of drusen on OCT have been
extensively investigated in natural history studies as potential
biomarkers for AMD progression, although some features have
yet to be validated. Among drusen characteristics defined on
cross-sectional OCT, drusen shape, internal reflectivity, and
substructures can be cited as some of the relevant biomarkers with
increased risk for development to advanced AMD (Yehoshua et al.
2011; Veerappan et al. 2016). Drusen size and confluency have
been historically associated with the progression of AMD. More
recently, drusen volume has been assessed through automatic
OCT algorithms, which also appears to be relevant in disease
progression (Abdelfattah et al. 2016).
Drusenoid pigment epithelial detachment (PED) is formed by
the confluence of large areas of soft drusen (Figs. 7, 8, and 11)
and is part of the clinical spectrum of AMD (Casswell, Kohen, &
Bird 1985). The natural history of eyes containing drusenoid PED
is characterized by a high rate of progression to both geographic
atrophy (GA) and neovascular AMD (Cukras et al. 2010).
Subretinal drusenoid deposits (SDDs, also known as reticular
pseudodrusen, can be confounded with drusen, but are actually
a clinically distinct entity located above the RPE (Figs. 3, 4, and
10). OCT is considered a fundamental imaging modality to identify
and characterize SDD (Suzuki, Sato & Spaide 2014; Zweifel et al.
2010). SDD in older eyes with a normal macular appearance, as
defined by the Age-Related Eye Disease Study (AREDS) scale,
is a risk factor for further development of AMD (Huisingh et al.
2016). Reduced visibility of cones overlying SDD in adaptive
optics images can be due to several possible causes, including
a change in photoreceptors’ orientation, an alteration of their
cellular architecture, or absence of the cones themselves, imply-
ing decreased cone photoreceptor function (Mrejen et al. 2014).
16
SECTION 6: AGE-RELATED MACULAR DEGENERATION
Drusen
6.1.1
OCT imaging of refractile drusen (drusenoid material containing
small refractile spherules) show hyperreflective dots (many small
spherules rich in calcium phosphate) and appear to be a stage
of drusen regression marked by loss of RPE, thus contributing
to the development of GA (Suzuki et al. 2015).
Key Points
• Small drusen (“drupelets”) are less than 63 µm in diameter,
intermediate drusen are 63 to 125 µm, and large drusen are
greater than 125 µm.
• Small drusen are considered normal aging and do not represent
a risk for progression to advanced AMD.
• Subretinal drusenoid deposits (also known as reticular pseu-
dodrusen) are located above the RPE and are associated with
progression to advanced AMD.
• OCT is valuable in the differential diagnosis of drusen.
• Specific drusen features on OCT are investigated as biomarkers
for AMD progression.
REFERENCES
Abdelfattah NS, Zhang H, Boyer DS, et al. Drusen volume as a predictor of
disease progression in patients with late age-related macular degeneration
in the fellow eye. Invest Ophthalmol Vis Sci. 2016;57(4):1839–1846.
Casswell AG, Kohen D, Bird AC. Retinal pigment epithelial detachments in
the elderly: classification and outcome. Br J Ophthalmol. 1985;69(6):397–
403.
Cukras C, Agrón E, Klein ML, et al. Age-Related Eye Disease Study Research
Group. Natural history of drusenoid pigment epithelial detachment in age-
related macular degeneration: Age-Related Eye Disease Study Report No.
28. Ophthalmology. 2010;117(3):489–499. doi:10.1016/j.ophtha.2009.12.002.
Curcio CA, Johnson M, Rudolf M, et al. The oil spill in ageing Bruch membrane.
Br J Ophthalmol. 2011;95(12):1638–1645. doi:10.1136/bjophthalmol-2011-
300344.
Huisingh C, McGwin G Jr, Neely D, et al. The Association between subretinal
drusenoid deposits in older adults in normal macular health and incident age-
related macular degeneration. Invest Ophthalmol Vis Sci. 2016;57(2):739–745.
doi:10.1167/iovs.15-18316.
Mrejen S, Sato T, Curcio CA, et al. Assessing the cone photoreceptor
mosaic in eyes with pseudodrusen and soft Drusen in vivo using adap-
tive optics imaging. Ophthalmology. 2014;121(2):545–551. doi:10.1016/j.
ophtha.2013.09.026. [Epub 2013 Oct 30].
Suzuki M, Curcio CA, Mullins RF, et al. Refractile Drusen: Clinical imaging
and candidate histology. Retina. 2015;35(5):859–865. doi:10.1097/
IAE.0000000000000503.
Suzuki M, Sato T, Spaide RF. Pseudodrusen subtypes as delineated by
multimodal imaging of the fundus. Am J Ophthalmol. 2014;157(5):1005–1012.
Veerappan M, El-Hage-Sleiman AM, Chiu SJ, et al. Optical coherence
tomography reflective drusen substructures predict progression to
geographic atrophy in age-related macular degeneration. Ophthalmology.
2016;123(12):2554–2570.
Yehoshua Z, Wang F, Rosenfeld PJ, et al. Natural history of drusen morphology
in age-related macular degeneration using spectral domain optical coherence
tomography. Ophthalmology. 2011;118(12):2434–2441.
Zweifel SA, Spaide RF, Curcio CA, et al. Reticular pseudodrusen are subretinal
drusenoid deposits. Ophthalmology. 2010;117(2):303–312.
Another random document with
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disturbances of bladder function have been described as vesical
crises, and recent French observers have observed so-called crises
clitoridiennes in female tabic patients which were characterized by
voluptuous sensations. All of these symptoms have these in
common: that they last but a short time, that their disappearance is
as rapid as their advent, and that they depend for their distribution on
the attitude of the disease in the cord. The vesical crises are more
apt to occur early than late in the disease and where the belt
sensation is in the hypogastric region. The gastric and enteric crises
are usually found when the belt is in the epigastric level, and the
bronchial, cardiac, and laryngeal crises when it is in the thorax and
neck. Some connection has been observed between the occurrence
of the lightning-like pains and these crises. Thus, a sudden cessation
of the former is sometimes the forerunner of the latter. It is also
found that one variety of these crises disappears to give way to
another: this is particularly noticed with the bronchial crises, which
often cease suddenly, to be followed by vomiting.
22 Krause, in a paper read before the Society of Neurologists at Berlin
(Neurologisches Centralblatt, 1885, p. 543), found either laryngeal crises or other
laryngeal symptoms, such as demonstrable ataxia of movement of the vocal cords, in
13 out of 38 cases. This proportion would be far too high for tabes in general; his
cases were probably advanced ones. He established the interesting fact that the
laryngeal crisis may sometimes be provoked by pressure with a probe on the superior
laryngeal nerve at its laryngeal entry-point. Jastrowitz claims to have determined the
existence of actual paralysis of the vocal cords with the crises, but Krause concludes
from the experimental inductibility of the crisis that it cannot be due to a posticus
paralysis. In a discussion on the subject Remak affirms that a unilateral paralysis of
the crico-arytenoideus posticus may be an early or the earliest symptom of tabes. It
seems, however, that in all cases where he determined such paralysis there had been
disturbances of function of other cranial nerves in unusual severity at about the same
time.

23 They must be extremely rare: they have not occurred in a single one of my 81
private cases, nor do I recall one in clinic or dispensary experience.
There is some analogy between the exceptionally-occurring mental
disturbance of tabic patients and the crises. Like them, it resembles
a disturbance of innervation, in this instance the centres regulating
the cerebral circulation appearing to be at fault. It also seems as if in
cases of this character the direction of the mental disturbance were
determined in some sense by the emotional relations of the
oblongata, for the insane outbreak usually consists in a brief but
extreme outburst either of a depressed and melancholiac or an
expansive maniacal or delirious outburst. It is a very rare occurrence,
and usually limited to the latest stages. Much more common is the
development of paretic dementia, but this is to be regarded rather as
a complication than an integral feature of tabes. Most tabic patients
retain their mental equilibrium to the last moment; some develop
truly heroic resignation to their terrible sufferings and gloomy
prospects; and a few, becoming irritable, petulant, and abnormally
selfish, show the effect of invalidism manifested equally with other
chronic diseases.

In addition to the visceral crises there are other disturbances of

innervation of a nutritive or trophic character which are characteristic
of tabes. There is scarcely one of the eruptions or other changes
which are attributable to central nervous disease which may not
occur at some one or other period of this disease. Thus, herpetic
eruptions may occur independently or in conjunction with lancinating
pains, in one case even corresponding to the area of the belt
sensation. Discoloration of the skin or hair, the latter turning gray or
white in circular24 or in irregular patches, spontaneous falling of the
nails, spontaneous hemorrhages in the matrix of the nail,
ichthyosis,25 purpura, diffuse œdemas, localized perspiration
sometimes associated with sudamina and accompanied by
ecchymoses, muscular atrophies, bullæ, and pemphigus-like
eruptions, have been noted by various observers. As yet, they are to
be regarded rather as clinical curiosities than as constant features,
as diagnostic criteria, or as presenting special indications for
treatment. The trophic disturbances affecting the bones and joints
and a peculiar ulcerative process known as the perforating ulcer of
the foot are better studied, and, ranking among the most formidable
and striking manifestations of tabes, merit special consideration. The
tabic joint affection, first described by Charcot as a trophic
manifestation of tabes, is usually located in the hip or knee, but
almost any joint, including the temporo-maxillary articulation, may be
its seat. The earlier or lighter forms consist in serous exudations with
or without active local manifestations resembling those of synovitis;
but the commoner and graver form consists in actual deformity of the
articular surfaces, such as enlargement or atrophy, sometimes
supplemented by the formation of osteophytes. In addition,
spontaneous dislocations occur in a manner which is often quite
surprising.26 As a type of such an occurrence the following may
serve: A tabic patient presented a synovial swelling of the left knee
of truly enormous dimensions; as soon as this became reduced a
distinct crepitus was elicited, and both the femoral and tibial articular
surfaces found enlarged. The whole process occupied only a few
days; the lateral ligaments were then found so relaxed that the leg
could be freely moved in every direction.
24 Rossolymmo, Archiv für Psychiatrie, xv. p. 727. I have never observed this
symptom as beautifully developed as in a case of spinal irritation, in which the
possibility of the existence of any organic disease could be positively excluded.
Bulkley has observed albinism.

25 In three cases by Ballet et Dutil, Progrès médicale, May 19, 1882.

26 Boyer (Revue de Médecine, 1884, p. 487) records a case where the hip became
luxated spontaneously as an early symptom.

The connection between the morbid process in the spinal cord and
these remarkable arthropathies is as yet unexplained; and as
Charcot's original proposition, that they can be always referred to
lesions in the anterior horns of the gray matter, has not been
sustained, some of the German clinicians, notably Strümpell, are
inclined to attribute them, in part at least, to ordinary results and
accidents due to the anæsthesia and its disturbing effect on
voluntary and automatic joint protection. A controversy arose which
was participated in by the Clinical Society of London,27 and evoked
opinions from nearly all the eminent neurologists of Europe. The
result of this thorough discussion has been to establish the joint
affection as a true tabic symptom due to the same morbid process
which underlies the less enigmatical symptoms of the disease. One
of the best reasons for regarding these joint lesions as of trophic
origin is the fact that they are associated with textural bone-changes
by which they become either unduly soft or brittle, and therefore
exposed to apparently spontaneous fracture. Cases of fracture of
almost every long and some of the short bones are recorded, among
the most remarkable being one by Dutil,28 where fracture of the
radius occurred without adequate cause in the initial period of tabes;
and one by Krönig,29 in which the patient, who had been tabic for
eight years, broke a lumbar vertebra while catching himself in the act
of falling down stairs.
27 The question was formally raised by Morrant Baker at the December meeting in
1884, and the discussion participated in by Charcot himself. Among the opponents of
Charcot's theory were Jonathan Hutchinson and Moxon, the latter of whom
administered a sound and well-merited criticism regarding some of the premature, if
not sensational, announcements of the distinguished French neurologist. He
compared the joint lesions of tabes to decubitus: just as the latter can be avoided by a
proper protection of the exposed parts, so the former would not occur if the joints
could be kept in a, surgically speaking, normal state. Moxon seems to have forgotten,
in suggesting this comparison, that there is a form of decubitus which will occur
independently of the greatest care and in spite of every measure taken to arrest it,
and which can be attributed only to an obscure but active perversion of nervous
nutritive control. Barwell, Paget, Herbert Page, Broadbent, and McNamara agreed
that the joint lesions are not of surgical or rheumatic origin, but essentially signs of the
nervous affection. Although Barwell's claim, that the rheumatic and tabic joint
diseases are essentially different, because the former is hyperplastic and the latter is
atrophic, is not borne out by all cases of tabic joint disease, some of which are
certainly hyperplastic, yet the other reasons advanced for regarding these affections
as distinct, and considering the joint affection of Charcot as a trophic disorder, far
outweighed those advanced by the opponents of this view. In fact, the only ground the
latter had to stand on was the fact that Charcot's asserted anatomical foundation was
found to be chimerical.

28 Gazette médicale de Paris, 1885, No. 25.

 29 Zeitschrift für klinische Medizin, 1884, vii., Suppl. H.

Another reason for considering the arthropathies as of central origin

is their chronological association with other signs whose trophic
origin cannot be disputed, such as unilateral sweats and œdemas
and visceral crises. They are also found to correspond in many
cases to the lancinating pains both in location and time.

The feet of an advanced tabic patient usually appear deformed; the

extremities of the bones, particularly at the metacarpo-phalangeal
articulation, are thickened, and the axis of the foot as a whole is
bent. It is a question how far this deformity may be due to persistent
faulty innervation of the muscles going to the foot and to ensuing
mechanical influences. A most pronounced deformity found in a few
cases has been designated by Féré as the tabic foot.30 It consists in
the thickening of the tissues on the inner side of the foot, obliterating
its arch in such a manner that in standing the plantar surface is
everywhere in contact with the floor. With this the tarso-metatarsal
and the metacarpo-phalangeal joints of the great toes appear greatly
swollen.
30 Pied tabétique—Revue de Médecine, 1884, p. 473.

There is less dispute concerning the nature of the so-called

perforating ulcer of the foot. This lesion occurs only in cases where
the central or peripheral nervous apparatus is diseased; thus, it may
occur with neuritis, and it is therefore unanimously regarded as due
to morbid nervous influences. In tabes it may follow an exacerbation
of the fulminating pains. It consists in a swelling of the tissues of the
foot; the skin becomes firm and thickened; deep sinuous ulcers are
then developed, showing but slight if any tendency to granulate, and
which extend down to the bone, the latter usually remaining
unaffected.31 In a few cases more remarkable accidents due to
disturbed nutrition have been noted. Thus, J. Hoffmann records a
subcutaneous rupture of the Achilles tendon, and in another case the
spontaneous discharge of all the teeth of the upper jaw within about
a week's time.32
 31 Like most of the manifestations of advanced tabes, the perforating ulcer may occur
exceptionally as a pre-ataxic symptom. Thus, Suckling (British Medical Journal, 1885,
April 4, p. 693) mentions its preceding the development of tabes proper by one year.

32 Berliner klinische Wochenschrift, 1885, No. 12. In this unique case the loosening
and falling out of the teeth preceded the tabes by a year. Demange, who observed the
same phenomenon in two cases, found that the ascending root of the fifth pair was
involved. The only analogous observation in my experience relates, like the bleaching
of the hair in circular patches, to a case of spinal irritation: here the gums and alveolar
borders underwent atrophy, exposing the roots of the teeth to beyond the normal
alveolar border: first the right upper row, then the right lower row, and finally the teeth
on the left side in the same order, showed this condition; only the most posterior fell
out.

COURSE.—Tabes dorsalis is to be regarded as an extremely chronic

affection. Its development, as previously stated, is insidious,33 and
the symptoms demonstrating the advent of the disease may be so
slight for many years that the patient does not regard himself as ill or
as likely to become so. In this condition he may remain for ten or
more years; and there are cases recorded where the exact signs of
the incipient stage were well marked, and yet no ascertainable
progress was made in a period of such long duration that the
inference naturally follows of a much longer prospective period in
which the patient would not have become ataxic. There may be
distinguished two forms of invasion: the more frequent is marked by
the development of the terebrating, fulgurating, and fulminating
pains, to which, sooner or later, the abolition of the pupillary and
certain other deep reflexes is added. In the vast majority of cases
they are found absent when the physician's attention is directed to
the question of the existence of spinal disease. In about one-fifth of
the cases pain is at no time a prominent feature of the disease, and
may be, as is established by the cases cited, entirely absent. In
these patients the first symptoms noted are muscle-tire on slight
exertion. Usually, the evidences of disturbed sensation and
innervation begin in one extremity or preponderate in it, but
symmetry is soon established. The same is noticed in the extension
of the morbid process: when the ulnar distribution is involved—which
is the first danger-signal on the part of the cervical enlargement of
the cord—usually one side is first involved, but the other soon
follows, and becomes affected in equal or nearly equal degree. In
the overwhelming majority of cases the disease manifests itself in
the lower extremities first, remaining limited to them for a long
period, and if extending to the upper extremities doing so in much
slighter intensity than in the lower. Gull, Leyden, and others have
observed cases where the arms were first and chiefly involved; but
this is extremely rare. Cases of a simultaneous involvement of the
upper and lower extremities are more frequently noted. The
symptoms detailed above are developed in varying order in different
patients, and the line has been already indicated which separates
the progressive from the episodial features of the disease. Even in
the ataxic period the patients may remain in a comparatively
stationary period for many months and years.34 The general bodily
nutrition is usually good, and the muscular strength, as such, rarely
affected to any noteworthy extent until the extreme phase of the
disease is reached. Even the formidable-appearing crises and
trophic disturbances, though occasionally fatal, are not always so.
The direct danger from the disease proper, so far as life is
concerned, is in the possible development of cystitis and pyelitis, of
the malignant bed-sore, and of delirium and coma, the latter of which
is occasionally found to be due to cerebral hemorrhage—a lesion
which some writers regard as possibly due to some impalpable
influence of the spinal disease on the cerebral circulation.35
33 Cases of acute locomotor ataxia have been described. In no instance are they pure
or typical, and the last case of the kind published in America, beginning, as it is
evident from the account, with signs of general myelitis, illustrates the impropriety of
utilizing one or a few symptoms as determining nomenclature.

34 Thirteen patients are now under my observation, or I have been able to obtain
reliable medical information concerning their condition, who have been in the ataxic
period since the date of my first examination, varying from a year to seven years ago.
Not one of these patients is materially much worse than at that time. Two only died,
both being bedridden at the time they came under observation. Of the first group,
 three have shown more or less lasting improvement in respect to special symptoms,
as will be detailed in connection with the treatment.

35 In a case of sudden death, with an asthma-like seizure, of a tabic patient under the
charge of T. A. McBride, I found an intense injection of one (the right) vagus nucleus,
the color of the ala cinerea and of the nucleus on section being almost black.

MORBID ANATOMY AND PHYSIOLOGY.—Our knowledge of the structural

disease underlying the symptoms which comprise the clinical picture
of tabes dorsalis is perhaps the clearest in the domain of spinal
pathology. Not alone the commencement of the morbid process and
every stage of its subsequent encroachment on the spinal cord, but
also its relations to special symptoms, have been demonstrated in
numerous cases by some of the most careful German and French
investigators.

In advanced cases of tabes the disease of the spinal cord is so

evident that it was noted even by earlier observers, the region of the
cord lying between the posterior nerve-roots being altered in color,
consistency, and dimensions. It is gray instead of white, hard instead
of soft, and considerably shrunken. The gray discoloration of the
surface appears to be complete in the lumbar and lower dorsal
region; it is less intense in the upper dorsal and cervical part of the
cord; and often shows a division into three streaks, of which one, in
the middle line, occupies both columns of Goll, while on either side
another extends in the inner side of the posterior root-entry. The
posterior roots themselves are frequently atrophied, and exhibit a
firmer consistency and darker color than in health. On making cross-
sections of the spinal cord it is found that the gray discoloration
extends inward, involving more or less of the posterior columns in
well-defined areas. Microscopic examination shows that the myelin
of the nerve-tubes is either destroyed or atrophied, and the axis-
cylinder is found to be affected in the same way, usually presenting
alterations in its refraction and diameter where it is not entirely
destroyed. In the maximum foci of disease the conducting elements
of the cord are nearly destroyed, and their place is occupied by a
firm connective substance made up of wavy bundles, enclosing here
and there a few atrophied axis-cylinders with wasted myelin-sheaths.
The blood-vessels participate in the morbid process, at least as far
as the larger vessels are concerned: these are sclerotic, their lumen
appears contracted, the adventitial sheath is hypertrophied and
occupied by a nucleated, fibrillar connective substance. The
capillaries are sometimes normal, or participate in the morbid
process to the extent of a thickening of the extra-endothelial sheaths
which is either hyaline or fibrillary, and may show inflammatory cell-
proliferation. It is evident that the increase of connective tissue in the
posterior columns is not merely a relative one, due to the
condensation of the normal neuroglia after the disappearance of the
normal nerve-tubes, but is the result of actual proliferation. Opinion is
somewhat divided as to whether this is to be considered as an
inflammatory interstitial proliferation or as a more passive
development of tissue compensating for the destroyed conducting
elements. My own observations incline me to the belief that in
syphilitic tabes the morbid process is essentially interstitial, while in
non-syphilitic tabes it is parenchymatous, the disease beginning in
the nerve-tubes proper. Perhaps the controversies between
pathologists as eminent as Schultze, Tuczek, Rumpf, Zacher,
Kahler-Pick, Rosenstein, and Adamkiewicz—who are rather evenly
divided into opposing camps, one maintaining the parenchymatous,
the other the interstitial, origin of the disease—may be solved when
we learn to distinguish between the non-syphilitic and syphilitic
cases, which is clinically, as yet, impossible. The difficulty of deciding
what is a parenchymatous and what is an interstitial disease of the
cord is considerably enhanced by the fact that the neuroglia, which
by some at least is regarded as an interstitial connective tissue, is
derived, like the nerve-substance proper, from the upper or neuro-
epidermic germ-layer of the embryo, and not from the mesoblast,
which furnishes all other connective substances in the body.

It is regarded as well established that the sclerosis of the areas of

the spinal cord which are affected is preceded by a stage of granular
degeneration.36 This is supported by the facts that the sclerotic areas
are surrounded by a zone in which sclerosis has not yet developed,
but which is in a state of granular degeneration, and that in more
advanced tabes this belt is also found sclerosed. In paretic
dementia, a disease whose complicating cord affection closely
resembles that of locomotor ataxia, a granular degeneration of the
posterior columns is very common in earlier periods, while in later
periods a sclerotic tissue is found in the same locality.
36 Westphal, Archiv für Psychiatrie, ix. p. 725.

I regard these observations as opposed to the assumption of

Strümpell that tabes is a system-disease of the cord in the sense in
which he employs that term. In his fascinating and suggestive thesis
on the systemic affections he cites the observations of Tuczek made
on the posterior sclerosis ensuing from ergotism as confirming his
view that special systems of nerve-fibres are separately vulnerable
to special morbid influences. The remarkable correspondence
between the topographical distribution of the lesion of ergotin tabes
described by Tuczek and the characteristic areas of sclerosis found
in the column of Burdach in ordinary tabes, seems to justify a very
different conclusion. Ergot exerts its morbid influence through a
disturbance of nutrition. Just as it produces gangrene by constriction
of the nutritive vessels of the fingers and of whole extremities, so it
produces interference with nutrition of those nerve-elements in the
cord which are most liable to suffer from general arterial constriction.
These are presumably those portions which are supplied by the
longest and narrowest arterial stems—a condition obtaining in
precisely those parts of the cord which are affected both in ergotin
tabes and in ordinary tabes.

It has also been supposed that the morbid process began in the
posterior roots and crept in with these, thence extending upward.37
This view is opposed by the fact that there is no constant relationship
between the root lesion and the cord lesion; the sclerosis of the root-
zones within the columns of Burdach I found to be absolute in at
least one case where the outer nerve-roots were not distinctly
affected.38
37 Takacs is the most recent defender of this view.
 38 The root lesion may, like that of the column of Goll, according to a minority of the
interpreters, be a secondary process, for in ergotin tabes (Tuczek) both are usually
intact.

In typical tabes the sclerotic process begins in a special triangular

field of the posterior column in the lumbar enlargement of the cord.
The innermost of the posterior rootlets run through this field, which
has been designated by the French school as the posterior root-
zones, and the rootlets become exposed to all the vicissitudes which
the conducting tracts are destined to undergo in consequence.
Throughout the remainder of the cord it is the involvement of the
root-zone which indicates the advance of the affection upward. At a
time when the lesion is comparatively slight in the cervical level,
manifesting itself by a slight grayish or reddish streak to the naked
eye, a faint, pale discoloration in chromic-acid specimens, a deeper
stain in carminized, and a pallor in sections stained by Weigert's
method, the affected part in the lumbar region has undergone great
shrinkage; scarcely a single myelin-tube is preserved to receive
Weigert's stain within the affected area; the latter involves nearly the
entire field between the cornua, and absorbs carmine deeply, so that
it is difficult to differentiate the gray and white substance in
carminized sections. A small part of the posterior column which most
closely adjoins the posterior (gray) commissure remains free in all
cases; so also does a small laurel-leaf-shaped field bisected by the
posterior median septum. Examination with higher magnifying
powers, aided by the modern improved methods of preservation and
staining, reveals that the gray substance of the posterior segment of
the cord is usually more or less diseased. In advanced cases the
gelatinous as well as the spongy part of the posterior gray horn, and
even the posterior gray commissure, undergo such considerable
atrophy that they may occupy but half the normal area. In some
cases the columns of Clarke appear to be involved quite early in the
disease.39
39 Leyden discovered the degeneration in the fibres which in part make up these
columns and correctly traced its origin to the posterior rootlets. Lissaner and Zacher
(Archiv für Psychiatrie, xv. p. 437) confirmed him, the latter finding, as Leyden was the
 first to discriminate, that the cells may escape while the fibres are materially
diminished. Krause claims that the columns of Clarke are constantly affected, but not
necessarily in their entire length, the higher levels appearing quite normal at a time
when the lower are severely involved (Neurologisches Centralblatt, 1884, p. 50).

The triangular field in the lumbar part of the posterior column, which
is one of the typical starting-points of the affection, contains those
ascending nerve-bundles which in their cephalic course emancipate
themselves from the column of Burdach and constitute the slender
columns of Goll. The result is that the degenerative process creeps
up these columns at the same time that it ascends in the root-zones
and deep portions of Burdach's columns. Some authorities regard
this as a mere extension by contiguity;40 others incline to consider it
a secondary degeneration. It may extend to the medulla oblongata,
becoming lost in the level where the nucleus of the column of Goll
terminates, and is accompanied, at least in those advanced cases in
which the upper extremities are involved, by a comma-shaped area
of degeneration in the adjoining part of the column of Burdach, which
similarly extends into the oblongata and terminates slightly more
cephalad. In typical advanced tabes, therefore, the cross-section of
the cord exhibits a characteristic distribution of the sclerosis in each
level. As this distribution is associated with certain constant
symptoms, it is permissible to attempt bringing certain features of the
lesion in relation with special features of the disease symptoms. The
posterior gray horns and the posterior white columns, together with
other fibre-systems connected with them, are much more
complicated in structural and physiological relations than the
corresponding anterior structures. The relations of the anterior
rootlets to the gray substance, and those of the motor ganglionic
elements to their controlling tracts, are comparatively simple; those
of the posterior roots are very intricate. They run up, in great part, at
an angle to the longitudinal tracts; a few pass in directly, and still
fewer dip to a lower level. The result is that a section of the cord
made in the longitudinal direction through the root-zones, so as to
pass from the root-entry to the anterior commissure, shows the
column and root-fibres to be woven into each other like a plait.
Trabeculæ of connective tissue, dragged in as it were with the
posterior roots, fill up the interstices of this labyrinth. They are
particularly dense in the lowest part of the lumbar enlargement of the
cord, constituting the so-called posterior processi reticulares. It is
reasonable to suppose that the overlapping of ascending and
descending root-fibres, associated with the presence of an extra
amount of connective tissue, imbedded as this fibre-maze is in that
part of the cord which is most distant from its lymphatic emunctories,
affords a favorable soil for slow inflammatory trouble. This is the
primary field of tabic sclerosis, and in it the disease may remain most
intense for years, extending but slowly and with diminishing intensity
upward, hand over hand, as it were, on the natural ladder which the
intertwined fasciculi and their matrix constitute. The longitudinal
tracts which lie in and near the root-zones belong to the so-called
short fibre systems, uniting the segments of higher and lower levels
of the cord with each other. As the sclerotic process ascends it
involves the caudal ends of these systems: they consequently
undergo secondary degeneration, and, shrinking in their turn, affect
the caudal part of the next system above in the same manner. The
morbid process in the column of Burdach may therefore be
considered as a combination of inflammatory and degenerative
changes, the inflammatory products causing a series of short
ascending degenerations, and the vulnerable path thus established
being followed by a cirrhotic condition in which the connective and
vascular structures participate actively. With regard to the reasons
for regarding the degeneration of the column of Goll and that of the
comma-shaped field near it as a secondary process due to the
cutting off of its apparent nerve-supply at the caudal end, and of the
posterior nerve-roots or their provisional terminations, they may be
stated in this way: When the lesion of the primary field is limited to
the lower lumbar or sacral part of the cord, the degeneration of the
column of Goll is limited to its postero-internal part; when the upper
lumbar and lower dorsal cord is involved, the entire tract is affected;
and when the cervical portion is diseased, the supplementary
comma-shaped area degenerates. In other words, the projection
tract of the sciatic nerve, as far as it is represented in Goll's column,
suffers in the first, that of the crural nerves in the second, and that of
the brachial nerves in the third instance. In all advanced cases of
tabes the affection of the column of Goll is in direct proportion to the
altitude of the lesion in the primary field. Symptomatically, it bears an
equally constant relation to the ataxia.41 No case is on record in
which these columns were totally degenerated without some motor
inco-ordination of the lower extremity having been observed during
life; and no case is recorded in which brachial ataxia had been a
marked and persistent feature in which the comma-shaped area—
area of the column of Burdach—was healthy.
40 It is held by them that the histological character of the change of the columns of
Goll is not different from that in the column of Burdach. Zacher (Archiv für Psychiatrie,
xv. p. 435) urges that it does not resemble true secondary degeneration, beginning in
the vessels and connective substance instead of the nerve-fibres. Schultze (ibid., xiv.
p. 386), on the other hand, recognizes a primary involvement of the nerve-fibres in
both of the areas of fascicular degeneration in tabes. The observation of intact axis-
cylinders by Babinski in the sclerotic fields is in conflict with the latter's claim, and the
various differences of observation and interpretation seem to be reconcilable only on
the assumption that there are two different modes of origin, both leading to nearly the
same results and occasionally combined in one and the same case.

41 Krause's case and others show that the ataxia of movement is not influenced by
lesion of the column of Clarke; but we are not informed as to the static equilibrium of
the patients in whose cords these columns were found intensely affected.

It is scarcely necessary to seriously consider the suggestion of

Strümpell, that the lesion of the column of Goll is in relation with the
bladder disturbance.42 Cases are on record by Wolff and others
where this lesion was intense and there was little or no bladder
disturbance.43
42 Archiv für Psychiatrie, xii.

43 The column of Goll is not present in those mammals which, like the porpoise, have
no developed hind limbs, but these animals have urinary bladders.

If the disease of the column of Goll were a primary systemic affection

independent of the disease of the root-field, it would be difficult to
understand why it, as well as the likelihood of finding a
corresponding degeneration of the direct cerebellar tract, increases
with the extent to which this field is involved. This occurrence
becomes quite clear when we remember that both the direct
cerebellar tract and that of Goll, being centripetal, are under the
trophic dominion of the posterior nerve-roots. The opinion is not
distinctly expressed, but implied in some writings, that the column of
Goll degenerates because of a general transverse cord lesion at a
low level; this is not the case in the tabic cord. There is a difference
in appearance between that part of the primary field which
corresponds to the column of Goll in the lumbar cord and the
surrounding sclerosis in early cases: it is more intensely
degenerated, more homogeneous in appearance, and more evenly
stained. The other part of the triangular field presents a more
trabecular appearance. In the specimen represented in the
accompanying figure this is easily recognized: the darker field
corresponds exactly with the ascending degeneration, which follows
compression of the cauda equina,44 and is the sciatic equivalent of
the column of Goll.
 FIG. 31.

Trans-section of Upper Lumbar Cord of a Patient moderately advanced in

Tabes: f, ventral or anterior fissure; g, caput gelatinosum; r r′, entry and
deep course of the posterior rootlets; d, degenerated field, including the
origin of the tracts which in higher levels form the columns of Goll; n, field
near the posterior commissure which remains free from degeneration,
both in the lumbar and cervical cord; s, sharply marked leaf-shaped field,
bisected by posterior septum, which, as claimed by Strümpell and shown
in this case, remains free from disease; z, zone of nearly normal
consistency around it; a, diseased field, suspected to be related to the
analgesia, not usually affected in early tabes; v, fibres running up and
down in front of the gelatinous substance; *, region where the tabic
process sometimes begins.

44 I have also found that this field corresponds to the column of Goll in its myelinic
development: the lumbar part of this column—designated as such by Flechsig—is an
entirely different tract, which enjoys a remarkable immunity from disease in tabes.

As illustrating the bearing of the lesion of the column of Goll on the motor ataxia I may
refer to two cases which happen to be related side by side by Strümpell (Archiv für
Psychiatrie, xii. p. 737, Cases 1 and 2). As far as the lumbar segment of the cord is
 concerned, the distribution of the lesion is similar; but in the one presenting marked
motor ataxia the triangular field was slightly diseased, and there was no upward
extension of the lesion in the column of Goll. In the other, with marked ataxia, the
triangular field was intensely diseased, and ascending degeneration (?) occurred in
the sciatic fields of the latter.

In support of the view that the comma-shaped area in Burdach's columns is

homologous with the fibres of the column of Goll, it is to be advanced that
degeneration of this field bears the same relation to symptoms in the distribution of
the ulnar nerve that degeneration of the column of Goll bears to sciatic and crural
symptoms. Where the initial pains and subsequent tactile and locomotor disturbance
were severe, this field was found affected, and most so in the side where the
symptoms had been most intense (Friedreich-Schultze's cured cases, Archiv für
Psychiatrie, xii. p. 234). This area has no direct connection with the root-fields.
Secondly, in a primary system disease of the column of Goll, associated with
degeneration of the nucleus of the column of Goll, described by Scoli, an irregular
encroachment of the column of Burdach was noted. Third, the innermost fibres of the
column of Burdach (those belting the nucleus in the oblongata) have the same
relation to the interolivary layer which the column of Goll has through its provisional
nucleus of termination.

While the evidence of high lesion of the cerebral continuation of the column of Goll,
and, what I regard as its homologue, the comma-shaped area of Burdach, together
with the constant association of marked degeneration of these columns with motor
ataxia, is strong positive proof of its relation to this symptom, there is equally strong
evidence negativing its relation to any other of the prominent symptoms of tabes
dorsalis. Thus Babesin (Virchow's Archiv, lxxvi. p. 74) found degeneration of the
posterior columns limited to the column of Goll, and the patellar reflex was not
destroyed; the root-fields at the upper lumbar levels were intact. That the columns of
Goll have been found profoundly affected without bladder disturbance has been
stated previously, and constitutes a stronger argument against Strümpell's view than
the frequent observation of bladder trouble in spinal diseases, along with which these
columns may be entirely free.

Among the various constituents of the posterior columns which

appear to present a relative immunity to the disease, aside from the
area near the posterior commissure and the laurel-leaf-shaped area
of the posterior septum, Strümpell noted one which is situated at the
periphery of the cord, bordering on the entry-line of the posterior
roots and the inner contour of the posterior roots. In the few cases
where it was found destroyed there was, what is a comparatively
rare thing in moderately severe tabes, complete analgesia. It is not,
however, certain that there is a necessary connection between the
pathological and clinical fact here. The nerve-roots themselves are
involved within the diseased area of the root-zones. The lesion is
one of a kind which, affecting a nerve-trunk, would produce first
irritation of, then impediment to, and ultimately destruction of, its
function. The clinical parallel to this is the occurrence of the lightning-
like pains in the earlier phases of the disease, which are followed by
delayed pain-conduction, and finally by loss of sensation. Of the
rootlets or fibres subservient to the various sensory and reflex
functions mediated by the posterior roots, those which convey the
centripetal impression normally evoking the patellar jerk appear to be
the most vulnerable, or, because of their limited number, the earliest
to be destroyed or compressed, with the result of total functional
paralysis.45
45 It is now conceded that, as Westphal claimed, the patellar jerk is always abolished
when the upper lumbar level of the root-zones (bandelettes externes of Charcot) is
involved. Tshirijew has shown that the translation of the knee-jerk reflex occurs in a
single segment of the rabbit's cord at the homologous level. In transverse sections a
distinct fascicle may be seen coursing from the innermost root-fibres toward the
antero-intermediate cell-group of the anterior horn; it furnishes a pictorial substratum,
if not anatomical proof, for the patho-physiological observation, and harmonizes with
the fact that it is the innermost rootlets which usually suffer first. Perhaps the delicacy
of this tract accounts for the frequent disappearance of the jerk in old people as a
result of senile sclerosis.

With regard to the interpretation of the various tactile sensory

disturbances of tabes and the delayed pain-sense conduction little
positive advance has been made. That the lesion of the root-zones
and gray substance is responsible for them seems to be the general
conclusion of French and German observers. Recent researches
have shown that disease of the peripheral nerves (multiple neuritis)
may produce sensory phenomena which it had previously been
customary to regard as pathognomonic of disturbance of their
intraspinal terminations and continuations. At the same time, we are
confounded by the observation of Erb, that even retardation of
conduction of the pain-sense, which the dictum of Schiff taught us to
regard as a sign of disturbed function of the spinal gray matter, is
also produced by peripheral neuritis.

Immediately adjoining the gelatinous apex of the posterior horn there

is a column of vertical fibres which bear a relation to it resembling
that of the ascending root of the fifth pair in the oblongata to the
tuber cinereum of Rolando. Sclerosis of this column, as well as of
the ascending root in question (Demange), has been found in tabes,
and usually in association with pronounced trophic disturbances. In a
number of cases (Oppenheim, Eisenlohr, and others) where
spasmodic laryngeal crises had been a marked feature during life,
lesion of the floor of the fourth ventricle, or atrophy of the
pneumogastric nerve, or even of its nucleus, was found. In one case
with marked gastric crises I found sclerotic changes of the arteries in
the ala cinerea.

With regard to the involvement of the peripheral nerves proper, aside

from the optic, opinion is somewhat divided. The discovery of
multiple neuritis, and the recognition of the fact that it had been
erroneously confounded with tabes, led Dejerine to claim that tabes
might be of peripheral origin. He even proposed to account for the
oculo-motor trouble on the basis of an affection of the oculo-motor
nerves. This explanation has been repudiated by Westphal and
sound authorities generally. That the peripheral nerves are
occasionally involved in tabes was already known to Friedreich in
1863,46 and later Westphal found the cutaneous branches in an
advanced tabic patient to present similar changes to those
discovered by Friedreich: these findings are confirmed by his
Japanese pupil, Sakaky. The nerve-tubes are atrophied, the axis-
cylinder being often preserved, and the interneural connective
substance is proliferated. But there is no constant relation between
these changes and the symptoms of the disease; in one of Sakaky's
cases the nerves of an extremity which had been the seat of severe
sensory symptoms were entirely normal. The findings in the
peripheral nerves of tabic subjects lose much of their value in view of
their recent discovery in marantic persons47 advanced in life, but who
had no nervous disease whatever.
46 Virchow's Archiv, vol. xxvi. pp. 399-452.

47 Krause, Neurologisches Centralblatt, 1885, p. 53.

It has been attempted to bring the tabic process in relation to a

supposed primary meningitis. Tabes is a rather common nervous
affection, and primary spinal meningitis is one of the very rarest. The
leptomeninges are found considerably thickened in one out of ten
tabic cords, and those who defend the meningitic origin of the
disease base their theory on this inconstant finding, and allege that
in the cases where it is absent the meningitis has disappeared while
the cord lesion progressed. It is a fatal objection to this view that the
part of the posterior columns immediately adjoining the pia is often
quite free from disease. A meningitic affection, either as an
etiological or a complicating factor, can be admitted in those cases
only where there is a marginal sclerosis.

The changes in the optic nerve resemble those of the white columns
of the cord in their naked-eye and minute character as well as in the
controversial nature of the various interpretations made. When
affected, the nerve is found to be firmer than normal, and discolored;
later it becomes quite gray, and may eventually shrink to two-thirds,
and even less, of its normal diameter. It is generally believed that, as
in the cord, the myelin undergoes wasting before the axis-cylinder
disappears, and that the latter may survive a long time, thus
explaining why the patient may retain his visual power for a
considerable period after the ophthalmoscope determines the
existence of atrophy. No satisfactory explanation has as yet been
offered for the optic-nerve affection of tabes. There is no direct
continuity of the spinal and optic sclerosis. Two theoretical
possibilities suggest themselves. The first is that the lesion of the
cord exerts a remote effect upon the physiological, and through this