Polioencephalitis Superior (Nuclear Oph- 
thalmoplegia) and Poliomyelitis. 
BY 
B. SACHS, M. D., 
Professor of Mental and Nervous Diseases in the New York Polyclinic. 
FROM 
THE AMERICAN JOURNAL OF THE MEDICAL SCIENCES, 
Sebtfmbkr, 1889. Extracted from the 
American Journal of the Medical Sciences for September, 1889. 
POLIOENCEPHALITIS SUPERIOR (NUCLEAR OPHTHALMO- 
PLEGIA) AND POLIOMYELITIS.1 
By B. Sachs, M.D., 
PROFESSOR OF MENTAL AND NERVOUS DISEASES IN THE NEW YORK POLYCLINIC. 
It is a rare experience in neurological matters to have the pathology 
of a disease unravelled as quickly as was done in the case of those 
clinical groups of symptoms which we know as ophthalmoplegia externa 
and interna. 
The paralysis of the muscular apparatus of the eye was soon dis- 
covered to be due, in most cases, to a lesion or lesions affecting the 
nuclei of the nerves which govern the various ocular muscles. In this 
nuclear paralysis, the nuclei of the oculo-motor nerves play a most 
important role, though the nuclei of the fourth and sixth nerves are 
involved frequently enough. Our knowledge of the pathological pro- 
cesses resulting in such lesions was gained by showing first of all that 
all the cranial nerve nuclei were subject to the same acute and chronic 
affections, and that what we ordinarily call progressive bulbar paralysis 
was, as far as its" pathological character is concerned, exactly the same 
as the progressive paralysis affecting the ocular nuclei. 
The relation of the bulbar process to poliomyelitis was firmly proved 
by cases of bulbar paralysis which were associated with symptoms 
resembling those of a progressive muscular atrophy or a chronic ante- 
rior poliomyelitis, and furthermore by cases of typical progressive 
muscular atrophy which, in their terminal stages, developed bulbar 
symptoms. 
On the strength of this clinical analogy, Hutchinson, Mauthner, 
and Birdsall were struck with the pathological resemblances between the 
diseases affecting the ocular and spinal nuclei; and Wernicke pro- 
posed to call the affection of the oculo-motor nuclei a polioencephalitis 
superior, whence it followed that the bulbar paralysis might well be 
styled polioencephalitis inferior. While the analogy with poliomyelitis 
had been proven beyond the shadow of doubt for the bulbar cases, 
Wernicke’s theory needed further proof as regards the cases of total 
ophthalmoplegia externa and interna. Heretofore but one case has 
been recorded (by Seelignifiller) in which the symptoms of a chronic 
1 Read before the American Neurological Association, June, 1889. 2 
SACHS, NUCLEAR OPHTHALMOPLEGIA. 
poliomyelitis were associated with those of polioencephalitis superior, 
and with the exception of the cases of Henoch and Buzzard1 in which 
an ocular (nuclear) paralysis occurred in the course of an acute 
poliomyelitis anterior, I know of no cases which exhibit this interesting 
association of symptoms. The case which I report here proves rather 
conclusively that the same pathological process may give rise to a 
typical polioencephalitis superior and to chronic or subacute poliomye- 
litis anterior. 
The history of this case is as follows: 
H. M., set. forty, is a man of robust build, unusually intelligent, and 
one who has experienced all the vicissitudes of life. He was born in this 
city, and has been married fourteen years. Has one brother living and 
healthy, and one brother who died from want of water, as the patient 
says, on the desert of Arizona. Both parents are dead ; the father died 
of yellow fever in New Orleans in 1858; the mother died of a paralysis 
which lasted seven or eight years and began by turning-in of both feet. 
The paralysis of the legs became complete ; she died at the age of 
seventy-four. The patient went to school at Syracuse, N. Y. As a boy 
had frequent “ bilious ” attacks associated with headaches and vomit- 
ing, was otherwise in robust health. He attended school until the age 
of twelve ; went to New Mexico at the age of fourteen, and there learned 
a trade. 
When sixteen years of age, one day after reading several hours got 
up to stretch himself, but fell back unconscious against a hot stove, 
burning the left temple severely—the scar is visible at the present day. 
He was insensible for several hours and was then put to bed ; knew 
nothing of what had happened until he saw doctors around ; no paralysis 
followed. A second similar attack, again after reading, occurred three 
months later, from which he quickly recovered. A third attack 
occurred, but the date of this he cannot recall. No further sickness 
until the age of twenty, when he was in Peru and was steward on a 
United States steamer. While on shore he fell from a horse, striking the 
right elbow' and injuring the arm. Recovered completely from this fall. 
At the age of twenty-five was in Europe ; felt one day a severe pain 
in the left eye ; engaged passage at once for New York, reaching there 
a few weeks later. By that time the left eye was closed. Went to sea 
again (to Australia on a sailing vessel), and during this trip noticed that 
the right lid was also beginning to droop. He was treated in Mel- 
bourne by electricity, but the condition remained stationary for several 
months. After that a slight improvement is said to have set in in the 
left eye, but the right eve grew wrorse; both pupils were dilated (physi- 
cian’s statement). Had double vision all the time, and small ulcers 
formed on the left eye. The Australian physicians suspected tumor ot 
the brain. He had severe headaches at the time ; the left eye became 
inflamed and was in such a bad condition that the physician advised 
enucleation, but the patient objected. After a short trip at sea, the 
inflammatory condition was at an end. 
It was about this time that he began to be suspicious of his legs, for 
1 Quoted by E. Blanc, Arch. gen. de Med., Janv. 1887. SACHS, NUCLEAR OPHTHALMOPLEGIA. 
3 
one day while walking on the deck his right knee gave way. A few 
days later the same accident occurred. Nevertheless he joined a ship 
from Australia to California. When thirty days out, he had to refuse 
duty as steward, as he could not move his right thigh, leg, or toe a single 
inch. The captain ordered hot steam bath, and gave him blue pill and 
black draught. He went to Oregon next—now thirteen years ago. He 
remembers that when there he could not hold water nor contain feces. 
His left leg was not affected at any time. Iodide treatment was pro- 
posed; patient objected on the ground that he had never had any 
syphilitic affection. In consequence of the paralysis of the right leg he 
was compelled to go about on crutches. The doctor who examined him 
found a sensitive point between the shoulder-blades and ordered blisters, 
and strychnine internally. In six months time he was able to walk with 
the assistance of a stick; he could use the hip and knee joint, but could 
not move ankle or toes. The eyes remained in about the same condi- 
tion ; he was not worried by them. He undertook contract work on a 
railroad in Panama, and there contracted severe malaria. 
One year and a half ago he was stricken down with prolonged fever. 
As soon as he recovered from this he travelled about considerably ; finally 
went to Jacksonville, where he got a thorough drenching, which was fol- 
lowed by severe chill. One day he tried to read the papers but could not 
see anything. Last year, on his return to New York he was examined by 
Dr. E. Griming, who performed an iridectomy of the right eye which 
did not improve vision. Later on, Dr. Griining raised the left eyelid 
by operation and restored vision to that eye. Went to Panama in May, 
1888, and returned to New York about seven weeks ago. 
For the past four weeks he has been an inmate of the Montefiore Home 
for Chronic Invalids, where I have had the opportunity of studying the 
case. He denies ever having had gonorrhoea or syphilis, and an examina- 
tion of his body reveals no symptoms of the latter. Has been moderate 
in sexual matters and has never been a hard drinker. Has smoked innu- 
merable cigarettes for years. No loss of consciousness has occurred since 
the attacks recorded above. Does not suffer from headaches and but 
for the condition of his eyes and of his right leg would feel entirely well. 
Present condition.—Strong, well-built man ; heart sounds normal; no 
enlargement of liver, slight enlargement of spleen ; other thoracic and 
abdominal organs normal. The most striking feature of the patient’s 
appearance is the double ptosis, at present more marked on the right 
than on the left, in consequence of the operation on the left eyelid. 
Slight lateral nystagmus of right eye; in this eye also maculte > orneae, 
old iritis with exclusion of pupil, small colo&oma upward. Right pupil 
is not visible, left pupil dilated. A transparent, thin membrane has 
grown upward, covering nearly one-half of left pupil. The results of 
my examination of the eyes, which were kindly corroborated by Dr. E. 
Fridenberg, are as follows: 
O. D.—Paretic—rectus internus, rectus externus, and inferior oblique. 
Paralyzed—levator palpebrarum, rectus superior, obliquus superior, and 
rectus inferior. Associated movements with the left eye do not differ 
from those attempted singly. 
O. S.—Paretic—rectus externus, rectus internus, and superior oblique. 
Paralyzed—levator palpebrarum, rectus superior, rectus inferior, and 
obliquus inferior. Paralysis of iris flight reffev abolished), ciliary mus- 4 
SACHS, NUCLEAR OPHTHALMOPLEGIA. 
cle normal. Accommodation reflex good. Media apparently clear in 
20 
both eyes. Vision, left eye, — Right eye = 0. 
Ophthahnoscopic examination.—Left papilla normal; right papilla 
cannot be examined. 
No change in the facial distribution. Hearing normal /m right side ; 
on left side somewhat diminished, but normal bone conduction. Tongue 
protruded straight, slight fibrillary movements. Sensation of face and 
tongue normal in every particular. Smell and taste normal on both 
sides. The left arm appears to be slightly larger than the right, but 
grasp is equally strong on both sides. Sensation normal to touch and 
pain. Distinguishes numbers written on arm with greatest ease. No 
reflexes to be obtained in upper extremities. No difficulty in respiratory 
or abdominal muscles. 
Fig. 1. 
Fig. 2. 
Subject attempting to look to extreme right. 
Showing atrophy of right leg. 
Lower extremities.—Marked atrophy of right leg from hip downward. 
Largest circumference on right side; hip, 14f inches; left side, 19\ 
inches; right calf, 10 inches; left calf, 12£ inches. Patient can flex 
kr /e very little, but cannot move toes of the right foot. Walks by ex- 
r _usive use of posterior thigh and leg muscles. Muscular excitability 
iost in right thigh. No disturbance in sensation except apparent diminu- 
tion of pain sense on inner aspect of right thigh. The left thigh and leg 
muscles show normal myotatic excitability and absolutely normal sensa- 
tion. No change to be noted in any respect in leg of left side. There 
is no ataxia of either leg and none in the upper extremities. No Rom- 
berg symptom. The knee-jerks are lost on both sides and cannot be 
elicited by Jendrassik’s method. All cutaneous reflexes sluggish but 
present. 
The electrical examination reveals no changes in any of the muscles SACHS, NUCLEAR OPHTHALMOPLEGIA. 
5 
of the face, of the upper extremities, or of the trunk, nor in the left leg, 
but marked degeneration reaction exists in the anterior thigh and leg 
muscles. The vasti and anterior tibial muscles are atrophied to such an 
extreme degree that no reaction could be obtained with currents at 
command. 
This history can be summarized in a few words: A man in perfect 
health, without any specific alcoholic or hereditary taint, is affected with 
a slowly developing paresis or paralysis of all of the ocular muscles. 
This condition is scarcely fully established before a weakness of the right 
leg is noticed by giving way of the knee. This weakness is developed 
into a most marked paralysis associated with extreme atrophy. The 
symptoms remain restricted to the right leg, become retrogressive, and 
have not to this day affected the opposite leg. The arms remain entirely 
normal. The transitory bladder and rectal symptoms were probably 
due to an extension of the inflammation of the gray matter, and do not 
imply, to my mind, the existence of a transverse myelitis, acute, subacute, 
or chronic. No other interpretation can be put upon these symptoms 
except to say that in the course of a chronic nuclear paralysis of the 
eyes a subacute poliomyelitis set in. Both in the eyes and in the leg 
the disease developed in the same fashion and has practically remained 
stationary for years. 
It will hardly be necessary in this paper to prove the diagnosis of 
subacute poliomyelitis in this case, and considering the rarity of polio- 
myelitis in the adult it would be strange indeed if the occurrence of 
such an affection in the course of a polioencephalitis superior were a 
mere coincidence. It seems to me to prove positively that the ganglion 
cells of the anterior horns of the spinal cord are subject to the same 
pathological changes as the large nuclear cells on the floor of the third 
and fourth ventricles. 
In the case which Seeligmiiller described, the upper and lower 
extremities were the parts first to be affected, and secondarily an affec- 
tion of the eye, right and left oculo-motor nerves, set in. In this case 
there would seem to be a spreading by contiguity; and yet the third, 
fourth, and sixth nuclei were affected before the seventh, tenth, eleventh, 
and twelfth nuclei. The absence of such contiguity in my case does not 
argue, I think, against the supposition that the leg and eye symptoms 
are dependent on the same pathological process. It is a peculiarity of 
this morbid process that it is selective, that in some cases it affects parts 
contiguous with one another, but that again in other cases it does not 
spread in this manner. In some instances the upper extremities are 
affected and with these the ocular muscles, showing that the bulbar 
nuclei have not been similarly affected. The bulbar nuclei and oculo- 
motor nuclei may be affected without an involvement of the sixth and 
seventh nuclei, and among the various cell groups constituting the 6 
SACHS, NUCLEAR OPHTHALMOPLEGIA. 
oculo-motor nuclei we find that the pathological process may attack 
portions of the nuclei which are situated at the extreme anterior and 
posterior limits of the nucleus and skip cell groups lying in between. 
This is in close keeping with the pathological condition we meet with in 
cases of poliomyelitis anterior, for we see too many of these cases not to 
know that contiguous muscles often escape paralysis and atrophy, simply 
because their representative ganglion cells in the spinal cord have not 
been affected like their immediate neighbors. 
The involvement of the iris in the one eye (the condition of the other 
could not be examined) takes my case out of the category of cases of 
ophthalmoplegia externa. According to most authors, an ophthalmo- 
plegia externa, with paralysis of the iris, would compel one to refer the 
lesion to the base of the brain, but since Westphal and Spitzka have 
plausibly shown that the nuclei for the accommodation and light reflex 
lie anteriorly and away from the remaining oculo-motor nuclei, it is 
readily seen that these nuclei also, one or both, may be affected by the 
extension of the inflammatory process. It is in this way that I explain 
the affection of the iris in this case. Since the accommodation reflex 
remained normal, it is natural to infer that the ciliary and iris nuclei 
must be some appreciable distance apart. We must be careful, however, 
not to be too positive in such assertions, for Thomsen has recorded 
cases in which there were distinct paralyses of various ocular muscles 
with only the slightest involvement of a few of the nuclear cells, and, 
strangest of all, one case of paralysis of associated vision upward due 
to a gummatous infiltration of the oculo-motor root fibres, whereas the 
nuclei were found entirely normal. It is for this reason also that 
I believe that the determination of the exact location of the various 
subdivisions of the oculo-motor nucleus on clinical grounds only,1 has 
been carried too far. This question can be settled in no other way but 
by the experimental method, or by noting to what extent clinical and 
post-mortem records tally. 
One other point in the case demands explanation: the knee-jerk 
is absent on both sides. The first suspicion was that of an accompanying 
tabes dorsalis, as in Westphal’s well-known case; but this supposition 
must be abandoned, since a close examination with this end in view has 
showni the absence of every other important symptom of tabes. The 
absent reflex on the left side must therefore be regarded as the only 
evidence of the extension of the process in the spinal cord to the left 
half of the cord, but at the same time the normal condition of the 
muscles, the normal electrical reactions, and the total absence of atrophy 
prove that that side can be affected but very little. 
The chief value of my case is, that it proves the close relationship 
1 Starr, Journal of Nervous and Mental Diseases, May, 1888. SACHS, NUCLEAR OPHTHALMOPLEGIA. 
7 
between the gray matter at the floor of the third and fourth ventricles 
and the anterior gray horns of the spinal cord. 
Wernicke choose the term polioencephalitis superior wisely enough; 
but Striimpell’s polioencephalitis, a supposed cortical disease, has caused 
some confusion. Striimpell’s theory and disease lack proof, and for the 
present we need not decide whether we shall have to add a polioencepha- 
litis suprema to polioencephalitis superior. 
Bibliography. 
Hutchinson: Med. Chirurg. Trans.,vol. Ixii , 1879. 
Mauthner: Vortrage. 
Birdsall: Journal of Nervous and Mental Diseases, 1887. 
Wernicke : Lehrb. d. Gehirnkrankh., ii. p. 229, iii. p. 460, 1883. 
Seeligm'uller: Neurolog. Centralbl., March 15, 1889. 
Westphal: Arch. f. Psych., vol. xviii. p. 846. 
Spitzka: Journal of Nervous and Mental Diseases, July, 1888. 
Thomsen : Arch. f. Psych., vols. xviii. and xix. THE AMERICAN JOURNAL 
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