=Hawthorne, C. O.—The Eye Symptoms for Locomotor Ataxia, with a Clinical
Record of Thirty Cases=.—_Brit. Med. Jour._, March 3, 1900.
It is now generally recognized that the disease known as locomotor
ataxia may include among its clinical manifestations symptoms other than
those which depend on pathological changes in the spinal cord. A number
of these are associated with the functions of the eyeballs. The
Argyll-Robertson pupil is universally admitted as valuable confirmatory
evidence of a diagnosis of locomotor ataxia; ocular paralyses, if less
frequent, are certainly not less significant; and optic nerve atrophy is
at least so well known in connection with the disease that its
occurrence in any individual case would hardly call for comment.
A further step forward in our knowledge of the clinical possibilities of
locomotor ataxia has been the recognition of the fact that ocular
disturbances may precede the evidences of any spinal lesion. This
advance necessarily means that the occurrence of any one of the ocular
events above mentioned must, unless otherwise explained, generate the
suspicion that the case may in its later events display the phenomena
known to depend upon sclerosis of the posterior columns of the spinal
cord.
It is very difficult to collect the evidence necessary to show in what
proportion of cases this suspicion is justified by the event. For it is
certain that ocular disturbances may long precede the manifestation of
spinal symptoms. In the case of optic atrophy the interval may,
according to Gowers, extend even to twenty years. Thus it can only be in
very exceptional instances that one and the same physician will have the
opportunity of observing at least a number of these cases through all
the stages of their progress. Yet, if true, it is of manifest
importance, for the sake both of exact knowledge and of accurate
prognosis, that it should be clearly recognized that an optic-nerve
atrophy, an ocular paralysis, or a loss of the pupil light reflex,
unless capable of other explanation, belongs in all probability to the
order of events incident to locomotor ataxia, and that any one of these
may well be the introduction to a more widely-spread manifestation of
the disease.
For reasons stated above, the collection of complete histories necessary
to afford actual demonstration of the truth of these propositions is
difficult; and all the more so as there is reason to believe that in
those cases in which the early stress of the disease falls upon the
nervous apparatus of the eyeball the spinal symptoms are apt to be
slight in degree as well as delayed in development. This is certainly
the case when the ocular disturbance takes the form of optic-nerve
atrophy. “In a large number of such cases,” says Gowers, “ataxy never
comes on, the spinal malady becoming stationary when the nerve suffers.”
Of course, in a given case of optic-nerve atrophy without spinal
symptoms the question may fairly be raised whether it is right to place
such a case in the locomotor ataxia group. All that can be said in reply
is (1) that from cases of optic atrophy pure and simple one passes by an
unbroken series of steps through cases with more and more distinct
evidence of locomotor ataxia to, at the end of the series, optic atrophy
in association with characteristic ataxic symptoms, and (2) that, as
already stated, a simple case of optic atrophy may remain unchanged for
many years, and yet in the end display undoubted evidence of the
development of a spinal lesion. But if optic-nerve atrophy may be the
primary symptom in the disease, if the occurrence of spinal symptoms may
follow it after an interval of many years, and if again it may remain
without at any time any existing ataxia, it is not unreasonable to
presume that both the Argyll-Robertson pupil and an ocular paralysis may
each have exactly corresponding relations to the development of the
spinal evidences of locomotor ataxia. The collection of evidence to
support this suggestion is even more difficult than in the case of
optic-nerve atrophy. The latter condition must ere long compel the
patient to seek medical advice, and thus the opportunity for a complete
investigation of the state of his nervous apparatus is afforded at a
relatively early date. But an Argyll-Robertson pupil may exist, and
presumably exist for years, without any inconvenience to the patient.
Such a patient, therefore, will not consult his medical adviser until
spinal or other symptoms display themselves, and thus the precedence of
the pupillary condition cannot be determined. In the case of an ocular
paralysis medical assistance is, no doubt, usually promptly invoked. But
such an occurrence is open to a number of ætiological explanations, for
example, rheumatism, cold, etc., which it is difficult to exclude with
confidence. Hence it is much less precise in its significance than
either a double optic atrophy or the Argyll-Robertson pupil. It must be
by the collection of observations extending over a long term of years
that actual demonstration of the relationship of the ocular disturbances
now in question to the occurrence of spinal disease can be established.
But while falling short of the merit of actual demonstration, the
presentation of the facts displayed by a number of cases which could
only be observed over relatively brief periods is not without value. If
no one case affords a complete history of all the stages of the disease
the picture presented may none the less be fairly complete, provided the
cases are sufficiently numerous, and they are seen at different points
of development. It is believed that in the present series these
conditions are fulfilled. The conclusions they afford, as far as the
present purpose is concerned, are: (1) That an optic-nerve atrophy, an
ocular paralysis, or an Argyll-Robertson pupil may exist as an isolated
symptom for a considerable time, presumably for years; (2) that any two
of these may be associated together, with a correspondingly increased
presumption that the diseased process causing them is of the locomotor
ataxia order; (3) that any one of the three, or a combination of two or
all of them, may exist in conjunction with a greater or less degree of
evidence of spinal disease; and (4) that occasionally a case which
commences with purely ocular symptoms may be seen to develop with
comparative rapidity characteristic symptoms of the spinal lesion of
locomotor ataxia. The cases therefore may be held to justify the view
that an optic-nerve atrophy, an ocular paralysis, or the
Argyll-Robertson pupil (not capable of other explanation) must be
regarded as affording a definite basis for suspicion in reference to a
possible development of spinal disease. On the other hand, it must be
admitted that the prognostic indication, so far as spinal disease is
concerned, is not an absolute one, for the ocular defect may exist
certainly for many years without any evidence whatever of the
involvement of the spinal cord.
The cases here recorded have all been the subject of detailed and in
most cases repeated examination, and unless the contrary is stated, it
may be taken for granted that the thoracic and abdominal viscera are
normal, to physical and other methods of examination. In all cases, too,
in which no specific statement is made, it is to be understood that the
visual acuity, the visual fields (both for white and colors), and the
fundus oculi have been proved to be normal. This last statement of
course does not apply to cases in which optic atrophy exists. Particular
care has been taken to be accurate in regard to the condition of the
pupils and the knee-jerks. In nearly, if not absolutely in every
instance where a departure from the normal is chronicled, the record has
been confirmed by more than one observer, and in the case of a deficient
knee-jerk the conclusion stated has never been formulated until the
conditions insisted on by Gowers, Buzzard, and Jendrassik have been
fulfilled. With a few exceptions in which only a single observation was
possible, the patients have been watched for months, and in some
instances for several years. The cases are arranged in series, with a
view to show how, from a purely ocular condition, one may pass through
gradually accumulating evidence to the same ocular condition in
association with the characteristic signs of the spinal lesion of
locomotor ataxia.
I.—CASES IN WHICH OPTIC-NERVE ATROPHY IS THE PRIMARY OR DOMINATING
CONDITION.
(_a_) _Optic Atrophy, without Other Evidence of Disease._
CASE I.—W. T., aged twenty-five. Failure of vision extending over two
years, with reduction of visual acuity to the power of counting fingers
at three feet. Double optic atrophy; pupils medium, with distinct light
response; knee-jerks distinct and no evidence of spinal disease, and no
cerebral symptoms other than one or two attacks of giddiness.
Urethritis, but no syphilis.
CASE II.—F. R., aged thirty-eight. Double optic atrophy, with almost
complete loss of vision, the defective sight having been observed for at
least eighteen months; pupils dilated and immobile; no evidence of
spinal disease, unless possibly some degree of failure of sexual power;
no cerebral incidents; no history or evidence of syphilis.
(_b_) _Optic Atrophy, with Other Ocular Evidence Suggestive of Locomotor
Ataxy._
CASE III.—(By permission of Mr. Ernest Clarke, F. R. C. S.) R. C., aged
thirty-nine. Double optic atrophy, reducing right visual acuity to the
power to count fingers at four feet, and left to mere perception of
light; right pupil dilated and three times the size of the left; neither
any light response, but free movement on convergence; entire absence of
symptoms and objective signs of spinal disease; “gleet” twenty years
before, no syphilis.
CASE IV.—A. S., aged twenty-five. Double optic atrophy, with observed
failure of vision for twelve months. V. A. right-hand movements only;
left, 6/18 part; pupils 2.5 mm., no light response, but contract on
convergence; knee-jerks difficult to obtain, but movement, though
possibly wanting in promptness, is normal in extent; no ataxia or other
evidence of spinal disease; mother of three healthy children, no
miscarriages.
(_c_) _Optic Atrophy, with Some Evidence of Spinal Disease._
CASE V.—W. A., aged thirty-seven. Double optic atrophy, with reduction
of visual acuteness to “hand movements;” pupils dilated and immobile;
knee-jerks absent, but no other evidence of spinal disease; venereal
sore when aged twenty; no recognized secondaries, and father of four
healthy children.
CASE VI.—J. G., aged thirty-five. Failure of sight (six months); optic
atrophy, gradually increasing whilst under observation of twelve months;
pupils not definitely abnormal; knee-jerks absent throughout, but no
further appearance of spinal disturbance; urethritis, but no history of
syphilis; father of two healthy children, wife no miscarriages.
CASE VII.—F. L., aged thirty-nine. Double optic atrophy, reducing visual
acuteness to 6/24, pupils very small, and with Argyll-Robertson
phenomenon; subsequent to failure of sight (twelve months) has had
shooting pains in thighs, and failure in retention power of bladder;
knee-jerks distinct; no ataxia or sensory defect in lower limbs;
venereal sore twenty years before; no recognized secondary syphilis;
wife healthy: seven pregnancies, five miscarriages.
(_d_) _Optic Atrophy, with Distinctive Evidence of Spinal Disease._
CASE VIII.—C. H., aged thirty-eight. Failure of sight (two years) from
double optic atrophy; pupils medium, with Argyll-Robertson phenomenon;
moderate double ptosis, but no ocular paralysis; shooting pains in lower
limbs (eight years); knee-jerks absent; considerable ataxia and failure
of control over bladder; syphilis at nineteen years.
CASE IX.—G. S., aged forty. Pallor of disks and peripheral contraction
of visual fields; four months later loss of knee-jerks and gradual
development of ataxia; pupils normal throughout; death at the end of
twelve months with symptoms of meningitis; syphilis at twenty-five
years.