SYRINGOMYELIA. 
BY 
LEO NEWMARK, M.D., 
PHYSICIAN TO THE SAN FRANCISCO POLYCLINIC, SAN FRANCISCO. CAL. 
FROM 
THE MEDICAL NEWS, 
July 22, 1893. [Reprinted from The Medical News, July 22, 1893.] 
SYR! NGOMYPlIA. 
BY LEO NEWMARK, M.D, 
PHYSICIAN TO THE SAN FRANClSCfTTWrCtTWIC, SAN FRANCISCO, CAL. 
In a recent work1 on diseases of the nervous sys- 
tem it is stated that " it is now quite a fashion in 
this country to report so called cases of syringo- 
myelitis." It it be a fashion it is one that seems to 
have but few votaries in the United States. For, 
since the publication of the first case by Dr. Starr,' 
certainly not many more than a dozen American 
cases have been recorded. The paucity of our lit- 
erature on syringomyelia forms a striking contrast 
to the voluminous records of European authors on 
the subject. Schultze, Dejerine, Charcot, Hoff- 
mann of Heidelberg, and many others have been 
enabled to base their studies of this disease on 
such abundant material as to justify the assertion 
that it is scarcely less common than disseminated 
sclerosis. Hoffmann alone recorded five cases in 
1891,3 and recently, in a very elaborate essay,4 
added a report of no fewer than eighteen. 
Assiduously as syringomyelia has been studied for 
1 " A Treatise on Nervous and Mental Diseases," by Landon 
Carter Gray, M.D., 1893. 
2 American Journal of the Medical Sciences, May, 1888. 
3 Volkmann's Sammlung, 1891, No. 20. 
4 Deutsche Zeitschrift fur Nervenheilkunde, Bd. ii'. 2 
several years past, the interest in it does not seem 
to flag. Its pathology and the relations of the 
structural changes in the cord to the clinical man- 
ifestations are still subjects of laborious research. 
The diagnostic difficulties arising from its resem- 
blance to certain other diseases render its study 
especially important. The question of its identity 
with Morvan's disease has not yet been answered to 
the satisfaction of all; and its resemblance to anes- 
thetic leprosy has called forth the sensational asser- 
tion that syringomyelia and anesthetic leprosy form 
"a single morbid entity," varied only by climate 
or the hygienic conditions of the patient? 
In view of the sustained interest in all that con- 
cerns this remarkable malady, the report of the fol- 
lowing case may not be unacceptable. 
The patient, K. F., is an unmarried woman, 
twenty-five years old, a native of Boston, Mass., of 
Irish parentage, and resident in California since her 
second year. She presented herself at the San 
Francisco Polyclinic on the 13th of January last, 
with the statement that her hands were wasting, 
and added that she was insensible to pain or heat in 
the upper extremities. The disease began about 
six or seven years ago with a prickling sensation 
(" like when the foot is asleep ") in the right deltoid 
region; this sensation gradually descended to the 
elbow, and thence down the forearm to the wrist; 
1 Zambaco: Gaz. hebd., 1891. Quoted by Marestang, Revue 
de Med., 1891, p. 783. In La Semaine Medicale, 1893, June io, 
Zambaco maintains that not only Morvan's disease and syringo- 
myelia, but also sclerodermia, sclerodactylia, a'inhum, morphea, 
and local asphyxia with symmetrical gangrene, are " nothing but 
varieties of one and the same prehistoric disease, old as the 
world, leprosy." 3 
it has never been present on the left side. The. 
patient found relief from the distressing feeling by 
directing her companions to strike her on the fleshy 
part of the right arm, and the more vigorous their 
blows the greater was the relief. She did not cease 
to be troubled in this way until about a year and a 
half later; in the meantime she had continued her 
occupation as a seamstress. While engaged in sew- 
ing and ironing she often sustained injuries to her 
hands which, although severe, entailed no suffering, 
and thus attracted her attention to the impairment 
of her sensation. She often burned herself and 
did not notice it until it had been pointed out to 
her; once she put her hand into very hot water 
and scalded it, but suffered.no pain. Her fingers 
finally became very sore, so sore that she thought 
" they must rot off," and some of the nails fell off 
two or three times. About four years ago she 
abandoned her occupation and has since then 
injured herself less. While undergoing electric 
treatment the regulation of the strength of the con- 
stant current was occasionally left to the patient by 
her physician, and there being no painful sensation 
to warn her, a large running sore formed in the 
palm of the left hand, which healed very slowly, 
but did not distress her. The disturbance of sensa- 
tion preceded the wasting of the hands. The right 
hand wasted first; there was twitching in the fingers- 
and especially in the thumb, in which there was a 
sensation " like the fluttering of a bird trying to 
escape." The right hand had wasted to its present 
condition when (about three years ago) the atrophy 
of the left commenced. The patient is often an- 
noyed by a jerking of the left lower limb; the 
jerking consists in violent flexion of the thigh and 
extension at the knee; it occurs most frequently 
and is most violent when the patient is recumbent 4 
and, according to the testimony of the mother, 
during sleep. It sometimes occurs while she is 
sitting, and is then arrrested by standing. Fatigue 
after much walking intensifies the jerking. Occa- 
sionally there are cramps in the great toe of each 
foot, in which the toe is forcibly extended. Com- 
plaint is further made of pain at the back of the 
neck and in the palm of the hand. 
Nothing was elicited indicating a family tendency 
to nervous diseases; but there seems to be a tuber- 
culous taint, as the patient's father and sister and a 
maternal uncle are said to have died of pulmonary 
tuberculosis. 
The patient is at present small and anemic. In 
both hands there is advanced muscular atrophy. 
The mterossei are wasted, the absence of the inter- 
osseus primus being particularly noticeable. The 
thenar and the hypothenar eminences are flattened, 
the right thenar less than the left. The distal and 
middle phalanges are flexed, and the proximal pha- 
lanx is extended, whereby the "claw-hand" is pro- 
duced, more markedly, however, on the right than 
on the left side. Flexion and extension at the wrist 
are preserved ; but the atrophy of the intrinsic mus- 
cles of the hands is so great as to render the left 
hand practically useless and to seriously impair the 
use of the right. The advantage of the right hand 
over the left is due to the better condition of the 
opponens and the adductor pollicis in the former, 
and of the muscles in the right forearm. Abduc- 
tion, extension and flexion of the thumbs are fair. 
Both forearms are thin, but the left is thinner than 
the right, the latter measuring 13 cm., the former 
only 12 cm. in the middle. The muscles of the 
upper arms and shoulder appear a little flabby, 
but not more so than might be expected from the 
patient's general nutrition; their functions are in- 5 
tact. The right arm measures 19.5 cm., the left 19 
cm. around the middle. There is no atrophy of 
the trunk or of the lower limbs. 
Fibrillary twitching is seen in the muscles on the 
inner side of the left forearm and in those of the 
hands; there is also a jerky tremor of the hands. 
As to the electric reactions, the muscles supplied 
by the musculo-spiral nerve react on both sides 
equally well and normally to direct and indirect 
galvanic and faradic excitation. The right median 
and ulnar nerves and the muscles of the forearm to 
which they are distributed respond fairly well but 
less promptly than the extensor muscles; in the 
same nerves and muscles of the left limb there is a 
considerable diminution of excitability by either 
current, without modal change, and the reduction 
of irritability is greater in the ulnar than in the 
median nerve and muscles. There is no typical 
reaction of degeneration in the small muscles of the 
hands; in accordance with what has been noted of 
the comparative preservation of motor power in the 
right thumb, contractions of the adductor and the 
opponens pollicis are obtainable by direct excita- 
tion or by excitation of their nerves at the wrist, 
with not very strong currents; in the other muscles 
of both hands the electric reactions are either greatly 
diminished or abolished. 
The tactile, muscular, and stereognostic senses 
are preserved in all parts. 
While the muscles of the upper extremities were 
being tested by faradism the patient gave no indica- 
tion of pain even when strong currents were applied 
with a very small electrode. Other tests, however, 
disclosed that the analgesia was not absolute in these 
parts, deep punctures and strong pinches, particu- 
larly in the right hand and shoulder eliciting evi- 
dence of painful sensation. (Figs. 1 and 2.) The 6 
analgesia is more profound throughout the left supe- 
rior extremity than upon the right.1 It is most pro- 
Fig. i. 
Fig. 2. 
Analgesia. 
The dots, slanting lines, horizontal lines, and crossed lines, 
denote different degrees of sensory impairment, the intensity 
increasing in the order in which they are mentioned. 
found on the left side of the head (except a part of 
the face), on the left half of the neck and the left 
shoulder. It is slight, but still evident in compari- 
1 By a lamentable oversight the four illustrations produced in 
the reprint of the article were omitted from the article as it 
originally appears in The Medical News. 7 
son with the other side, on the median half of the 
left side of the face and on the left side of the 
Fig. 3. 
Fig. 4. 
Thermo-anesthesia. 
The dots, slanting lines, horizontal lines, and crossed lines, 
denote different degrees of sensory impairment, the intensity 
increasing in the order in which they are mentioned. 
mouth and tongue. The upper and lower regions 
of the chest are slightly analgesic, while the breasts 
are normal or nearly so. Ammonia held to the 
nostrils produces equal irritation on both sides. 
The analgesia in the hands is not as great as the 
statements of the patient would lead one to believe. 8 
The ulnar nerves are not sensitive to strong pressure 
at the elbows. 
The insensibility to heat and cold coincides for the 
most part in distribution and degree with the anagle- 
sia. (Figs. 3 and 4.) In the right hand it is less than 
in the left, and is far from absolute, although the 
impairment of the thermic sense is beyond doubt; 
but it is less than would be inferred from the history 
of the patient's experience. A piece of ice can be 
retained in the left hand almost indefinitely without 
causing discomfort, whereas in the right a stinging 
sensation of cold is announced after it has been 
held a short time. Water which was quite hot when 
applied to the right hand for the purpose of elec- 
tric testing was not felt to be at all warm, whereas 
subsequently a test-tube filled with moderately warm 
water was distinguished from one filled with water 
of about 60° F.; and the distinction was made 
between the wooden handle and the metal part 
of a percussion hammer, when held in the right 
hand, by the temperature of the metal. The patient 
herself, however, when informed of the result of the 
tests, remains convinced of the insensibility of her 
hands to painful impressions, heat and cold, under 
ordinary circumstances. A piece of ice taken into 
the left side of the mouth is recognized as cold, but 
is immediately felt "several degrees colder" when 
shifted to the right side. An analogous difference 
obtains in regard to warm water. Dissociation of 
the temperature-sense itself was not observed. 
Trophic changes are not pronounced apart from 
the muscular atrophy. A few insignificant scars 
indicate the sites of former injuries. The nails are 
not abnormal. 
Nor are the vasomotor disturbances marked. 
On the chest and back the vasomotor irritability 
seems to be increased ; this is especially noticeable 9 
when the finger is passed along the row of spinous 
processes. The hands are, as a rule, subjectively 
and objectively quite cold. 
The iris-reflex is normal in either eye. The left 
conjunctival reflex is absent; the right one is pres- 
ent. The pharyngeal reflex is preserved. A brisk 
jaw-jerk can be obtained. The triceps-tendon re- 
action can be elicited in either arm. On percussion 
the supinator longus on each side contracts actively. 
No periosteal or tendon-reflexes exist at the wrists. 
The epigastric reflexes are absent. The knee-jerks are 
both much exaggerated, and are equal on the two 
sides. The tendo Achillis reflexes are quite active. 
The front-tap contraction is obtainable on either 
leg. Ankle-clonus was elicited on the left side at 
one examination, but has not been produced since. 
The plantar reflexes are absent. 
Hearing, taste, and smell are normal. The eyes 
were kindly examined for me by Dr. Pischl. V. 
with + 1.5 - 6/vi in both eyes. The field of vision 
determined for white and colors is normal. The 
ophthalmoscope reveals in the right eye a band of 
opaque nerve-fibers encircling the inner half of the 
papilla and equal in width to three-fourths the 
diameter of the disc. In the left eye a small patch 
of such fibers is seen at the upper, and another at 
the outer, edge of the disc. The papilla appears a 
little redder than is normal, which is probably due 
to the contrast with its white environment. There 
is a slight narrowing of the left palpebral fissure, its 
width being 8.5 mm., while that of the right is 
9.0 mm. 
The secretion of tears is more abundant in the 
left eye than in the right. As has already been 
mentioned, the left conjunctival reflex is abolished. 
The pupils react to light and in accommodation, 
and are equal. There is no nystagmus. 10 
The spinal column is not tender to pressure. 
There is no scoliosis. On passing the finger down 
the row of spinous processes a peculiarity is felt in 
the shape of some of them in the lower dorsal and 
upper lumbar regions. The ninth dorsal spinous 
process is normal; the tenth is clubbed; the eleventh 
and twelfth dorsal and the first lumbar are clubbed 
and bifid; the second lumbar is like the tenth dorsal 
and the rest are normal. 
The treatment since the patient has been under 
my care has consisted chiefly in the exhibition of 
iron. Her general health has improved, and she is 
now free from the pain in the back of the neck, 
but still complains occasionally that the hands ache. 
The combination of symptoms such as have been 
described in the foregoing notes, consisting in pro- 
gressive muscular atrophy of the Duchenne-Aran 
type and dissociation of sensory impressions, stamps 
this as a classical case of syringomyelia. If it be at 
all necessary to differentiate this malady from Mor- 
van's disease, the absence of painless whitlows will 
be conclusive of the diagnosis. 
How close the clinical resemblance between syrin- 
gomyelia and anesthetic leprosy may be, despite 
the difference in pathology, is evident from the de- 
scriptions and illustrations furnished by Marestang. 
Dissociation of sensation has been observed by this 
author in leprosy; but only in one case among over 
one hundred was there retention of tactile sense 
with analgesia and thermoanesthesia, while in two 
cases there was retention of pain and of the thermic 
sense with tactile anesthesia. On the other hand, in 
syringomyelia loss of sense of contact has not in- 
frequently been met with (which is not surprising, 11 
in view of its pathology). Such dissociations, how- 
ever, are exceptional in leprosy, and the preservation 
of tactile sensation in syringomyelia is the'rule. 
The muscular wasting in the extremities is similar 
in the two diseases, and even the atrophy of facial 
muscles, often seen in leprosy, has been observed in 
four cases of syringomyelia. Nevertheless a proper 
appreciation of what is frequent and what is rare in 
each, or the recognition of some characteristic of 
leprosy, such as anomalies of pigmentation, loss of 
hair, nodular thickening of nerves, the bacillus, 
etc., will generally lead to a diagnosis. But that 
errors may occur has been recently shown by Pitres1 
in his report of the case of a man who, presenting 
trophic disturbances and the sensory dissociation of 
syringomyelia, was for a long time considered to be 
suffering from that disease until the detection 01 
Hansen's bacillus in a cutaneous nerve proved him 
to be affected with leprosy. This man had lived 
for three years in Martinique, and it is in countries 
which are in frequent communication with endemic 
foci that special caution is required. The Pacific 
coast is exposed to the occasional intrusion of lepers 
from Hawaii or China, and in the public eleemosy- 
nary institutions of San Francisco there are now 
thirteen lepers. But in the present instance, besides 
the absence of anything in the patient's antecedents 
likely to excite a suspicion of leprosy, and the lack 
of any symptom characteristic of that disease, the 
1 Gaz. des Hopitaux, 1892, No. 137 ; abstract in Neurolog. Cen- 
tralblatt, 1893, P- IO2- See also Kalindero : Wiener med. Presse 
1892, No. 39. 12 
insensitiveness of the nerves to pressure, the fact 
that the disturbance of sensation is less profound at 
the peripheral parts of the upper extremities than 
in certain other regions, the fibrillary twitching, the 
augmented knee-jerks and the narrowing of the left 
palpebral fissure, decide in favor of syringomyelia. 
Hysteria, that arch-mimic of organic nervous 
disorders, has also been known to simulate syringo- 
myelia. Cases of this kind have been reported by 
Charcot1 and by Rossolimo.2 The latter tells of a 
girl, aged twenty, with interosseal paresis, analgesia, 
and thermoanesthesia, but only slight diminution 
of tactile sensation, in her hands. This combination 
of symptoms had recurred six times, in the course 
of a year and a half, at regular intervals, and dis- 
appeared promptly after an hypnotic seance. To 
discuss the possibility of the hysterical nature of 
the case now under consideration would appear like 
raising objections to a diagnosis simply for the 
pleasure of refuting them. Nor does it seem neces- 
sary to enumerate the features distinguishing cervical 
hypertrophic pachymeningitis from syringomyelia, 
the anesthesia in the district of cranial nerves alone 
sufficing to exclude the former. 
The possibility of diagnosticating syringomyelia 
during life is not yet universally conceded. In the 
discussion of a case presented before the Neurological 
Society of New York, in 1890, the president, Dr. 
L. C. Gray, said : " The only way to make a diag- 
nosis of syringomyelia was to make an autopsy." 3 
1 Legons du Mardi, 1888-1889, p. 518. 
2 Neurolog. Centralblatt, 1892, p. 493. 
3 Boston Med. and Surg. Journal, 1890, vol. cxxiii, p. 570. 13 
And the same view is expressed in his recent Treat- 
ise on Nervous and Mental Diseases,1 although he 
acknowledges " that we are on the right track and 
that we shall be able to make a diagnosis in the 
early future." The fact is, however, that the diag- 
nosis has been repeatedly made intra vitam and 
verified post mortem. Charcot2 stated in 1889 that 
Schultze had twice and Kahler once found the diag- 
nosis justified at the autopsy. Moreover, Dejerine,3 
in 1890, and again in 1891/ and with Sottas,5 in 
1892, reported cases in which the diagnosis of 
syringomyelia, which, during life, had been based 
on the muscular atrophy and the sensory dissocia- 
tion, was corroborated after death. Verified diag- 
noses have also been recorded by Gyurman,6 Critz- 
mann,7 and Bernstein.8 In a report of a case of 
syringomyelia by Hughlings Jackson and Galloway9 
mention is made of an instance in which a diagnosis 
of Ferrier's was proved to be right at the necropsy. 
In Hoffmann's10 Cases IX and XIV the disease had 
been recognized during the lifetime of the patients. 
Finally, the reviewer of Dr. Gray's book in the 
American Journal of the Medical Sciences for 
1 Pp. 267 and 268. 
2 Lemons du Mardi, 1888-1889, p. 488. 
3 Societe de Biologie, February 8, 1890. 
4 La Semaine Medicale, 1891, No. 6. 
5 See Neurolog. Centralblatt, 1892, p. 710. 
6 Abstract in Neurolog. Centralbl., 1891, p. 504. 
7 Essai sur la Syringomyelic, 1892; Neurolog. Centralbl., 1892, 
p. 708. 
8 Neurolog. Centralbl., 1893, No. 3, abstract. 
9 Lancet, Feb. 20, 1892. 
10 Deutsche Zeitschrift fur Nervenheilkunde, Bd. iii. 14 
March, 1893, states that two cases of syringomyelia 
have been diagnosticated intra vitam in Philadel- 
phia, and that the autopsy confirmed the diagnosis; 
the cases had, however, not yet been published. 
One of them is, perhaps, identical with that very 
recently recorded by J. H. Lloyd.1 
This list makes no pretensions to being exhaustive, 
but it will suffice to show that the diagnosis may be 
ventured with some degree of certainty. Not all of 
the cases conformed to the most frequent type, but 
the pathognostic value of progressive amyotrophy 
with impairment, and generally dissociation of sen- 
sation, was sufficient to guide the observers to the 
recognition of the disease. The assumption of 
syringomyelia explains the course and the symptoms 
of the case with which we are dealing. The pro- 
cess of gliosis, taking its origin in the gray substance 
behind the central canal in the lower cervical 
region and extending into the posterior cornua, pro- 
duced the subjective and objective disturbances of 
sensation in the upper extremities; ascending in 
the left posterior horn into the central regions of 
the occipital nerve and of the sensory part of the 
trigeminus, it impaired sensation in the left side of 
the head and face, and, descending into the dorsal 
part of the cord, rendered sensation on the trunk 
abnormal; the invasion of the right and left ante- 
rior cornua, successively, in the lowest cervical and 
first dorsal segments, gave rise to atrophy of the 
small hand-muscles in the same order, and, from 
the wasting of the muscles of the left fore-arm, it 
is evident that the destructive process is extending 
1 University Medical Magazine, March, 1893. 15 
into the anterior horn higher up. The distressing 
jerking in the left lower limb may possibly be due 
to irritation of the lateral pyramidal tract on the 
same side. It may be mentioned that a similar 
symptom was noted in one of Schultze's1 cases. 
The loss of the left conjunctival reflex is due to 
the sensory disturbance in the distribution of the fifth 
nerve, and the slight narrowing of the left palpebral 
fissure is ascribable to the implication of sympathetic 
fibers in the lesion at the lowest cervical and upper- 
most dorsal segments. There is no concentric 
limitation of the field of vision. According to 
Hoffmann,2 limitation of the visual field has been 
observed by Dejerine and Tuilant in seven, and 
by Morvan in five cases of syringomyelia, without 
hysteria; by other authors it has been found in five 
patients with syringomyelia in whom hysteria was 
either certain or at least probable; and it was 
absent in 26 cases. Including the present instance, 
therefore, its absence has been noted in 27 out of 44 
cases of syringomyelia. Its comparative frequency 
in the French cases is conspicuous. 
The opaque nerve-fibers in the retina merit some 
attention. This anomaly is rare. Manz,3 of Freiburg, 
states that he found this opacity mentioned only 154 
times in the records of half-a-million patients with 
ocular diseases. But among 113 psychopathic male 
inmates of a Freiburg institution opaque nerve- 
fibers were observed in the eyes of four. Manz had 
1 Virchow's Archiv, Bd. 102, 
2 Deutsche Zeitschrift fiir Nervenheilkunde, iii, p. 16. 
s Berliner klinische Wochenschrift, 1890, p. 955. 16 
already previously1 called attention to the possible 
value of this, as well as other congenital anomalies of 
the eye, as indicating a neuropathic predisposition, 
although they are not necessarily pathologic ; and 
he suggested that they were likely to be found asso- 
ciated with certain diseases dependent on congenital 
abnormalities of the central nervous organs. Now, 
according to the prevailing theory, the origin of 
syringomyelia is to be sought in a defect in the de- 
velopmental tendency of the spinal cord, or, as 
Hoffmann more definitely expresses it, in a defec- 
tive closure of the embryonic canal, whereby germ- 
inal cells are retained in the line of closure which 
subsequently proliferate and lead to primary gliosis. 
In support of the view that a defect in the original 
constitution of the central nervous system is re- 
sponsible for the development of syringomyelia 
Hoffmann adduces some cases in which that disease 
was combined with other nervous diseases generally 
considered as arising from a congenital predisposi- 
tion, and in others in which at the autopsy abnor- 
mal anatomic features were found that must be 
regarded as developmental errors. Among the 
latter are mentioned an abnormal strand of white 
fibers in front of the anterior commissure, hetero- 
topia of gelatinous substance in a posterior column, 
heterotopia of gray matter, etc. The presence of 
medullated (opaque) nerve-fibers in the retina is 
submitted as an addition to this list. 
In conclusion, the peculiar form noted in some of 
the spinous processes may be alluded to as perhaps 
indicating some developmental irregularity. 
1 Arch. f. Psychiatric, 1884, xv, p. 837. The Medical News. 
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