An Examination of the Eyes of 
Fifty Cases of Chorea 
of Childhood. 
By y 
G. E. DE SCHWEINITZ, M. D. 
(TTNIV. OF PENN.), 
OPHTHALMIC SURGEON TO THK PHILADELPHIA HOSPITAL, 
TO THK CHILDREN’S HOSPITAL, AND TO THE IN- 
FIRMARY FOR NERVOUS DI8EA8IR. 
REPRINTED FROM 
2T!)c Neto ¥orfe iftSelucRl Journal 
for June 23, 188S. Reprinted from the New York Medical Journal 
for June 23, 1888. 
AN EXAMINATION OF TIIE EYES OF 
FIFTY CASES OF CHOREA OF CHILDHOOD. 
By G. E. DE SCIIWEINITZ, M. D. (Univ. of Penn.), 
OPHTHALMIC SURGEON TO THE PHILADELPHIA HOSPITAL, TO THE CHILDREN’S 
HOSPITAL, AND TO THE INFIRMARY FOR NERVOUS DISEASES. 
The cases of chorea from which this study has been made 
have for the most part been those of patients in attendance at 
the clinics of Dr. S. Weir Mitchell, Dr. Wharton Sinkler, and 
Dr. William Osier in the Infirmary for Nervous Diseases. 
Certain results which should naturally be the outcome of 
the examinations which follow' are not set down, because 
sufficient time has not yet elapsed to render this possible. 
Tbe following points have received special attention : The 
chromatic symmetry or asymmetry of the irides, the devel- 
opment of the face, the condition of the pupils, the acuity 
of vision, the amplitude of accommodation, the sufficiency 
or non-sufficiency of the external eye muscles, the state of 
the refraction, and the appearances of the fundus. A word 
in regard to the manner in which the cases were studied. 
Each patient was placed opposite a window and the color 
of the iris noted, and whether this was alike in each eye or 
whether differences in color, shade, or tone could be de- 
tected. In the same way the two sides of the face and the 2 
AN EXAMINATION OF THE EYES OF 
development of each orbit were noted. The diameter of the 
pupils was measured facing a clear sky, and the hour was 
always the same—between 1 and 2 p. m. For this pur- 
pose Randall’s pupilometer was employed. The acuity of 
vision was determined under a uniform illumination at a 
distance of five metres with Oliver’s test-types, each eye, of 
course, being tried separately. The accommodation was 
tested with D = 05 of Snellen’s type and the nearest point of 
perfectly sharp vision recorded. The muscular relations were 
studied in the manner especially elaborated by Dr. G. T. 
Stevens * and the surgeons who have followed his methods. 
Great care was exercised to have an accurately adjusted 
trial-frame in which the prisms were set. The object looked 
at was a point of light against a dark background, placed 
five metres from the patient. At 30 ctm. or the reading 
distance the test of v. Graefe was employed, but not the 
usual coarse dot and line figured in the text-books, but a 
very fine dot and line, demanding accurate accommodation 
at the distance employed. 
The ophthalmoscopic investigations were all made in the 
dark room of the infirmary, have in each case been repeated 
a number of times, and have all been done by the writer 
himself with Loring’s instrument. In a certain number 
of the cases, twelve in all, a mydriatic correction was 
made, and hence the findings are accurate. In the others 
the refraction was measured as nearly as possible in the 
axis of the eyes. Retinoscopy was often employed as a 
control test. Fully aware that with complete paralysis of 
accommodation only can the true refraction of the eye be 
determined, I am none the less convinced that the findings 
are reasonably accurate. In this connection I am induced 
to quote a sentence, which exactly coincides with my own 
* “ Functional Nervous Diseases, their Causes and their Treatment,” 
by G. T. Stevens, M. D., 1887, p. 194 et seq. FIFTY CASES OF CIIOREA OF CHILDHOOD. 
3 
view and experience, from Dr. Randall’s paper on “ A Study 
of the Eyes of Medical Students ” * : “ The newer ophthal- 
moscope of Loring is probably the most perfect instrument 
in existence for the measurement of refraction ; and more 
than five years’ incessant use of it under all circumstances 
has given the writer a confidence in its readings that work 
with atropinized eyes and the artificial eye does not show 
to be overweening. Yet any concealment of its fallibility 
would be utterly unjust and would tend to weaken the fair 
claim that this method is the one which, in the absence of 
complete paralysis of the accommodation, will most fre- 
quently approximate accuracy.” The ordinary details of 
the fundus and media of each eye were studied ; the ap- 
pearance of the discs; the absence or presence of the so- 
called conus; the state of the blood-vessels and lymph- 
sheaths ; and the aspect of the retina and chorioid. 
Of the fifty patients f examined, ten were males and 
forty females, or one male to four females. The young- 
est was a child seven years of age and the oldest twenty- 
eight. Twelve were under ten years of age, nineteen 
between ten and fifteen, fifteen between fifteen and twen- 
ty, and four twenty years and over. In twenty-five, or 
50 per cent, of the cases, the irides were equal in color and 
shade; in the remaining twenty-five, or 50 per cent., there 
were differences in shade or tone. In one of these twenty- 
five only was there any true asymmetry of color. This is 
interesting in connection with the investigations of Ch. 
* “ A Study of the Eyes of Medical Students,” by Alexander V. Ran- 
dall, M. D. “ Trans, of the State Med. Soc. Penn.,” 1885. 
j- A considerably greater number than this has been examined, but 
only fifty are recorded, because in these instances the examinations were 
many times and carefully repeated. The results of the examinations 
were condensed in tables, but space is lacking to allow of their publi- 
cation. 4 
AN EXAMINATION OF THE EYES OF 
Fere.* This observer found asymmetry of color when not 
dependent on local conditions a very rare symptom. In 
over six hundred subjects only seven instances were found, 
and these were imbecile, hysterical, or epileptic. Asymme- 
try of tone or shade was not rare, especially among the 
neurotic. In epileptics 26’7 per cent, had this symptom, 
62 out of 76 hysterical patients; in choreic patients, those 
with obstinate facial neuralgia and with sciatica, chromatic 
asymmetry was observed. When this symptom occurred 
in the children of the hysterical, Fere believed it was a sign 
of neurotic tendencies and called for prophylactic treatment. 
The pupils were of equal size and shape in thirty-nine, or 
78 per cent, of the cases, and unequal in eleven, or 22 
per cent, of them. The smallest diameter was 2 mm., the 
largest 6 mm., while the average size of the entire series was 
3-39 mm. In twenty-five, or 50 per cent., of the patients 
the development of the two sides of the face, of the two 
orbits, and the general appearances of the eyes and their 
appendages, appeared symmetrical, while in the remaining 
twenty-five, or 50 per cent., distinct asymmetry in these re- 
spects was readily noticeable. 
It is well known that the pupils, other things being equal, 
are larger in young people, while they become smaller and 
lose both in range and rapidity with advancing years. It 
is also stated that widely dilated pupils, which react feebly 
to light, are frequently a feature in cases of chorea (Dr. G. 
T. Stevens, op. cit., p. 90). Hence it is interesting to ob- 
serve that in this series the pupillary diameter ranged from 
2 mm. to 6 mm., and the average size was only 3-39 mm. 
While it is true that marked inequality of the pupils is un- 
common, except from disease or highly different refraction in 
* “ Chromatic Asymmetry of the Iris, a Symptom of Neurosis.” “ Le 
Progr&s medical,” September 25, 1885. Abst. in the “ Med. News,” De- 
cember 11, 1886. FIFTY CASES OF CHOREA OF CHILDHOOD. 
5 
the two eyes, it is also true that mere differences in the size 
of the pupils are in no way necessarily a sign of disease. 
Thus, Iwanow (“ The Question of Inequality of the Pupils in 
Healthy People,” “ Wratsch,” No. 7, 1887, abst. in the “ Ar- 
cliiv. f. Ophthalmology” Dec., 1887, p. 463) found, in his ob- 
servations on one hundred and thirty-four healthy young 
military recruits, equal width of pupil in only twelve. The 
right pupil was larger in forty-nine, and the left in seventy- 
three. As Landolt has said (“ Examination of the Eyes,” 
trails, by S. M. Burnett, 1879), a lack of symmetry in the 
face, one half being Hatter than the other, would lead us to 
suspect anisometropia, and all forms of asymmetry of the 
cranium can cause astigmatism. Individuals with such con- 
formation of features are frequently astigmatic, sometimes 
anisometropic, the refraction being stronger on the side 
which is the more fully developed—this eye being myopic 
or emmetropic, while the other, as a rule, is hypermetropic. 
Distinct asymmetry existed in 50 per cent, of the cases un- 
der consideration ; indeed, the proportion was probably 
higher, as only such cases in which this was readily visible 
were so classified, and no accurate measures were taken.* 
Again, however, mere asymmetry of the face must not be 
looked upon as necessarily a symptom of a diseased state, 
especially a neuropathic tendency. In Iwanow’s series (loc. 
cit.) the face was asymmetrical in one hundred and thirty- 
one of them, the left being larger in ninety-nine, and the 
right in thirty-two. 
Full acuity of vision—i. e., A—was present in both eyes 
in only ten of the cases; in four of them there was normal 
sharpness of sight in one eye and not the other, while in 
thirty-two the vision of each eye was less than normal. In 
* The relation of this asymmetry to the state of the refraction has 
not been worked out. 6 
AN EXAMINATION OF THE EYES OF 
four of the cases it was not possible to record this. In no 
single instance where it was measured was the amplitude 
of accommodation equal to the normal standard for the age 
of the patient. 
The state of the refraction in these cases of chorea was 
the following : 
Hypermetropia in twenty-three, or 40 per cent.; hyper- 
metropia in one eye and hypermetropic astigmatism in the 
other in seven, or 14 per cent.; hypermetropic astigmatism 
in twelve, or 24 per cent.; myopia in one, or 2 per cent.; 
myopic astigmatism in three, or 6 per cent.; mixed astig- 
matism in four, or 8 per cent. In no single instance was a 
refraction error of less than 1 D found. 
As long ago as 1876 Dr. George T. Stevens was at- 
tracted by the frequent occurrence of hypermetropia in the 
eyes of choreic children, and in a paper before the New 
York Academy of Medicine (“Chorea: its Cause and Treat- 
ment,” G. T. Stevens, M. D., “Trans, of the N. Y. Academy 
of Medicine,” sec. series, vol. ii, 1876) announced the fol- 
lowing proposition : “ Chorea is a functional disturbance of 
the nervous system, which may give rise to organic lesions, 
and which arises from irritation dependent upon anomalous 
refraction of the eye, and in a very large proportion of the 
cases to hypermetropia.” In this paper thirty-three cases 
of chorea are discussed, in which the refraction of the eyes 
was as follows : Hypermetropia in twenty-four cases, hyper- 
metropic astigmatism in four cases, myopic astigmatism in 
four cases, unequal myopia in one case. 
In the hands of Dr. Stevens the correction of these 
anomalies of refraction produced excellent results in the 
curing of the nervous disorder. 
About a year later Dr. C. S. Bull analyzed thirty-one 
cases of chorea with reference to this question, and arrived 
at very different results (“ The Connection between FIFTY CASES OF CHOREA OF CHILDHOOD. 
Chorea and Errors of Refraction,” C. S. Bull, M. D., “ Med. 
Rec.,” May 20,1877). Of his patients, fifteen were emmetro- 
pic; two were emmetropic in one eye and hypermetropic in 
the other; eight were hypermetropic of the same degree in 
each eye; two were hypermetropic of unequal degrees in 
the two eyes; two were simple hypermetropic astigmatism 
of the same degree in two eyes; one was hypermetropic in 
one and simple hypermetropic astigmatism in the other; 
and one was simple hypermetropic astigmatism in one eye 
and compound hypermetropic astigmatism in the other. This 
result, emmetropia in fifteen out of thirty-one cases, is un- 
usual, but must be accepted as correct, as Dr. Bull’s patients 
were examined under atropine. Again, Dr. Ranney (“ N. 
Y. Med. Journal,” Jan. 14, 1888), speaking of chorea, says: 
“I have not found a single case where either manifest or 
latent hypermetropia did not exist.” The writer is cer- 
tainly in accord with this statement—at least in accord with 
a statement that anomalies of refraction, in his experience, 
hkve always existed, a single case excepted. Dr. G. T. 
Stevens further classifies in his work on “Functional Nerv- 
ous Diseases,” p. 91, 113 cases of chorea, according to the 
refraction, as follows: Simple hypermetropia existed in 78 ; 
hypermetropic astigmatism in 13; mixed astigmatism in 5 ; 
myopia, unequal in the two eyes, in 6; myopic astigmatism 
in 11. 
Adding these various examinations together, we have 
227 cases of chorea, of which 212 were amctropic and 15 
emmetropic. Of the ainetropic cases, hypermetropia or hy- 
permetropic astigmatism was found in 177, or 77'9 per 
cent.; myopia, or myopic astigmatism, in 26, or 11*5 per 
cent., and mixed astigmatism in 9, or 3-9 per cent. The 
remaining 15, or 6'7 per cent., were emmetropic.* This 
* It is not evident whether the 113 cases classified in Dr. Stevens’s 
book include the 33 cases in his original paper or not. If they do, then 8 
AN EXAMINATION OF THE EYES OF 
high percentage of refraction error, especially of hyperme- 
tropia and hypermetropic astigmatism, among choreic chil- 
dren is certainly suggestive. It may be interesting to com- 
pare these results with the prevailing refraction in children’s 
eyes. To do this, it is only necessary to turn to that most 
praiseworthy paper by Dr. Randall, “ The Refraction of the 
Human Eye ” (“ Amer. Jour, of the Med. Sci.,” July, 1885), 
where we find in conclusion No. 2 the following: “ Hyperme- 
tropia is the enormously preponderating condition in infancy 
and early childhood, and the first years of school life witness 
little reduction in its proportion. . . . It was found in 91-26 
per cent, of the eyes of infants examined, in 81 ’75 per cent, 
of the eyes of young children, and in 76 per cent, of the 
3,358 eyes of children in the elementary school years. So, 
also, in the higher schools, it constituted at least 56 per 
cent, of the whole number of eyes studied.” Further com- 
ment on this point would seem needless, except to point 
out the singularly close correspondence in the percentage 
of hypermetropic refraction in the eyes of children in the 
elementary school years to the percentage of hyperme- 
tropia and hypermetropic astigmatism in the eyes of choreic 
children. 
An imperfect equilibrium of the eye muscles was found 
in every case examined. In a few instances strabismus was 
present, while in the remainder some form of insufficiency 
of the ocular muscles was always manifest. Inasmuch as 
the terms introduced by Dr. Stevens are convenient, they 
have been employed to designate the muscular defects, 
which were as follows: Exophoria alone in sixteen, or 32 
per cent., of the cases; esophoria alone in thirteen, or 26 
per cent., of the cases; hyperphoria alone in one, or 2 per 
we have 194 patients in all, of which 149, or 76*7 per cent., had liyper- 
metropia or hypermetropic astigmatism as the existing refraction error. 
All of the emmetropes were found in Dr. Bull’s series. FIFTY CASES OF CHOREA OF CHILDHOOD. 
9 
cent., of the cases ; cxophoria with hyperphoria in six, or 12 
per cent., of the cases; esophoria with hyperphoria in five, 
or 10 per cent., of the cases; exophoria in accommodation, 
witli esophoria and hyperphoria for the distant point in 
one, or 2 per cent., of the cases; convergent strabismus in 
six, or 12 per cent., of the cases; divergent strabismus in 
two, or 4 per cent., of the cases. 
Tt should be remembered that these were the manifest 
muscular errors, and have no reference to insufficiencies 
which may have been latent. It must equally be remem- 
bered that, inasmuch as the refraction error was corrected 
in only a part of these patients, the precaution to examine 
for muscular defects through the correcting glasses could 
not nearly always be exercised. What modifying tendency 
such correction would have had upon the results can only 
be surmised. In Dr. Bull’s series of thirty-one cases (loc. 
cit.) insufficiency of the intend existed in twenty-three. In 
a considerable number of Dr. Stevens’s cases (loc. cit. ; the 
percentage is not given) more or less muscular disability 
was found, and insufficiency of the lateral recti muscles was 
found in five unassociated with any marked degree of re- 
fraction error. The presence of imperfect equipoise of the 
eye muscles in cases of functional nervous disease, and the 
belief in some ndnds that this may be the cause of the dis- 
order, is too well known to need further repetition here. 
Before deciding the significance of the relation of the high 
percentage of insufficiency of the ocular muscles in cases of 
chorea, it may be well to note for a moment its percentage 
in the eyes of young people generally. In Risley’s admira- 
ble statistical paper, “Weak Eyes in the Public Schools of 
Philadelphia ” (“ Trans, of the State Med. Soc. of Pennsyl- 
vania,” vol. xiii, 1881), among 554 children of an age rang- 
ing from twelve to twenty, and of an average age of seven- 
teen years and one third, insufficiency of the intend was 10 
AN EXAMINATION OF THE EYES OF 
found in 37'2 per cent. Touching this point, Beselin * has 
reached some noteworthy results by the examination of 
530 cases, of which 363 were Em., 116 H., and 51 M. 
All having a refraction error of 1 D. or less are called 
Em. Equipoise in this particular was found in only 
140 = 38*5 per cent, of the Em., in 32 = 27'6 per cent, 
of the II., and in 12 = 23’5 per cent, of the M. Insuffi- 
ciency of the interni with tendency to divergence for far 
or for near, or for both, was met in 155 = 42-7 per cent, 
of the Em., in 52 = 44‘8 per cent, of II., and 32 = 62'7 
per cent, of M. The contrary condition, with excess of con- 
vergence for near or far, was found in Em. 34 = 9T per 
cent., II. 27 = 23‘2, and M. 1 = 2 per cent. There were 
further 2 II. and 1 Em. with divergence for distance and 
excess of convergence for the near, while of the contrary 
condition, excessive convergence for distinct and insufficient 
for near was met in 33 = 9 per cent. Em., 3 = 2’5 per 
cent. H., and 6 = 11‘4 per cent. M. The tests used were a 
candle-flame for distance and a white Graefe test for the near. 
Further comment here is needless, except to point out 
the large percentage of muscular errors which occur among 
children who are not and have not been choreic. It is per- 
fectly proper to note that in these school investigations the 
tests for hyperphoria were apparently not made, and it is to 
this defect that special attention has recently been drawn. 
In regard to the fundus details and the condition of the 
media, the following points were observed : 
In two instances opacities of the media (cornea and 
vitreous body) were observed. These, of course, hear no 
reference to the disease. In two, coarse changes in the 
* “ Untersuchen iiber Refraction und Grundlinie der Augen und iiber 
die dynamischen Yerhiiltnisse der lateral wirkenden Augen-Muskeln an 
Miidchen von 5-18 Jahren,” von Dr. Otto Beselin, “ Archiv f. Augen- 
heilkunde,” Bd. xiv, zweiter Heft, August, 1884, p. 132. FIFTY CASES OF CHOREA OF CHILDHOOD. 
11 
chorioid were found (atrophic retino-chorioiditis and dis- 
seminated chorioiditis), bearing also probably no reference 
to the chorea. In one case there was slight but evident optic 
neuritis; suspicion, however, was not lacking that this child 
had also a brain tumor, although Gowers has several times 
observed optic neuritis associated with chorea. In one 
case the discs were greenish and of diminished capillarity, 
the eyes distinctly amblyopic without corresponding refrac- 
tion error, but the vessels were unchanged in size. Atrophy 
of the optic nerve following chorea has been described by 
William G. Syme * ; both sets of vessels were very small, and 
the trouble was probably due to a former embolus plugging 
the retinal artery. A similar case is on record by Forster, 
and Swanzy has seen embolism of the central artery of the 
retina at the commencement of the attack. Argyll Robert- 
son observed atrophy of one optic disc follow a severe at- 
tack of chorea in a young woman. While in the remainder 
of the cases no perfectly typical, healthy fundus was found, 
the changes were such as were entirely compatible with 
systemic health. Quite universally one or another form of 
conus was present; the veins were frequently distended, 
often markedly tortuous; coarse retinal striation was quite 
generally to be found, and tbe chorioids presented changes 
ranging from mere woolliness or absorbing interspaces to epi- 
thelial chorioiditis. Usually both arterial and venous lymph- 
sheaths were distended, and many lymph-reflexes were a 
common feature. In other words, the changes, sometimes 
slight, sometimes amounting to decided accommodative 
retinitis and retmo-chorioidal disturbances, were such as 
should be reasonably expected in young, often asthenopic, 
eyes, suffering, in most instances, with decided errors of 
refraction. It was, in short, not possible to observe in this 
* “ Edinburgh Med. Jour.,” March, 1888. 12 
AN EXAMINATION OF THE EYES. OF 
scries any fundus change that was in any way characteristic 
of the disease chorea. 
The following conclusions seem justifiable: 
1. The irides of choreic children quite commonly pre- 
sent chromatic asymmetry in shade, just as the same condi- 
tion has been found in other forms of nervous disorders. 
2. Slight differences in the width of the pupils may be 
observed, but not more frequently—in fact, not as frequently 
—as these have been noted in perfectly healthy individuals. 
3. Facial asymmetry is present in about one half of the 
cases, just as this is present in cases of high refractive er- 
ror, and also in individuals perfectly free from nervous dis- 
orders. 
4. Hypermetropia and hypermetropic astigmatism are 
vastly the preponderating conditions of refraction in the 
eyes of choreic children, being found in about 77 per cent, 
of the cases, exactly as hypermetropic refraction is the pre- 
ponderating condition in childhood generally, being found 
in 76 per cent, of the eyes of children in the elementary 
school years. 
5. Imperfect equipoise of the eye muscles is found in 
the great majority of the cases, but imperfect equipoise of 
the eye muscles is very frequently present in the eyes of 
school-children free from chorea or neuropathic tendencies. 
6. Embolism, atrophy of the disc, and optic neuritis 
may occur during or after attacks of chorea, but appear- 
ances in the fundus oculi characteristic of the disease have 
not been found. 
7. As Octavius Sturges remarks : “ It s3ems certain that 
a fairly constant proportion of chorea is directly connected 
with what may be called injudicious schooling, . . . but 
such nice adjustment as shall prevent overstrain on the one 
hand and overindulgence on the other is practically unat- 
tainable.” Certainly an endeavor to lessen the overstrain FIFTY CASES OF CHOREA OF CHILDHOOD. 
13 
of the eyes should be made. Hence the refraction errors 
and muscular defects in these children should be carefully 
and fully corrected by glasses, by prisms when necessary, or 
even by judicious surgical interference, and thus a probable 
exciting element removed ; just as w6 should perform the 
same service for eyes similarly afflicted in children who are 
not choreic; just as we should improve the hygiene, re- 
move the anaemia, treat the disabled circulatory apparatus 
in children who are choreic. Evidence, however, seems 
quite as lacking that hypermetropic refraction is the basal 
cause of chorea as it is that the chorea is the cause of the 
hypermetropia. 
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