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THE DIAGNOSIS OF ONE FORM OF INTRA CRANIAL SYPHILIS.'
By LANDON CARTER GRAY, M.D.,
PROFESSOR OF NERVOUS AND MENTAL DISEASE IN THE NEW YORK POLYCLINIC.
The literature of syphilis is enormous, and probably equalled by that of no other subject in medicine except tuberculosis. Writing some years ago upon the subject, I made this comparison : Prof. Max Müller, the well-known philologist, informs us that the whole of the Sanscrit literature, running, as he expresses it, like a high mountain-path of literature through the whole history of India, and extending over a period of three or four thousand years, is contained in about 10,000 manuscriptsmore, the same authority asserts, than the whole classical literature of Greece and Italy put together. I have been able to count up five hundred different articles that have been written on the subject of syphilis in the last thirty years, and there are probably many more. This is one-twentieth of the whole Sanscrit literature or of the combined classical literature of Italy and Greece, and therefore in order that the literature of this one subject should equal these great national literatures it would only take the time of about six hundred years, or one-seventh to one-fifth the time of the Sanscrit writings." Notwithstanding the vast interest in the subject, however, that is manifested by these figures, the diagnosis of syphilis of the nervous system is still in a state of great uncertainty. The pathological lesions of the brain, of the cord, and of the peripheral nerves have been well studied, so that I need not pause to dwell upon them before an assemblage of this nature. But the clinic cal symptoms of these pathological alterations are involved in considerable uncertainty unless there has been a clear history of the initial syphilitic lesion, and of its sequelæ in skin, bones, and mucous membranes, For example, we may be able to affirm that we have before us the symptoms of an intra-cranial, a spinal, or a peripheral nerve lesion, because our knowledge of diseases of the central and peripheral nervous system has advanced to great certainty within the last few years; but when the further question arises as to whether this lesion of the brain, the spinal cord, or the peripheral nerves is due to syphilis, we must in the vast majority of cases fall back upon the history of the initial lesion or its sequelæ, or wait for the proof that may be contained in the success or non-success of treatment by iodide and mercury. Unfortunately it is a fact that the history of the initial lesion or its sequelæ is often very difficult to obtain. The primary sore being very slight, it may
. easily be overlooked, as we all know, by one who is not aware of its characteristics ; and this may even happen to one who is upon the alert, as all syphilographers will testify. The wife may be infected by the husband, and innocently enough, too. Several years ago I heard a lady
1 Read before the American Neurological Association at the Congress of American Physicians and Surgeons at its meeting in Washington, September, 1891.
I telling some friends how she had been afflicted with an obstinate headache and subsequent inflammation of the eyes, and as I knew that this lady's husband had died a short time before of intra-cranial syphilis, I went to her attending physician to inform him of the fact, and found him entirely ignorant of the etiology of his patient's symptoms. The infection may also come through a surgical operation, so that I presume there are very few communities of any size in which physicians cannot be found who have infected themselves in this way; indeed, I know at the present time of four physicians of whom this is true. Or the infection may come through a lesion innocently acquired about the buccal cavity, and even perhaps through articles of clothing. The tendency to concealment, which so often actuates patients from motives of shame and family pride, constitutes another great source of error for the physician. It is therefore apparent that some method of diagnosis independent of the history of the initial lesion and its sequelæ would be of great value, and all recent writers upon this subject have recognized the need, although no one has ventured to outline any pathognomonic symptoms. Rumpf's great book of some 600 pages, published in 1887, casts absolutely no light upon this question of positive diagnosis of syphilitic nervous affection, although it is a most industrious and painstaking compilation of pathological, clinical, and therapeutical memoranda. For many years I have been keenly aware of this defect in our clini. cal knowledge, and I have been endeavoring to obtain some further light upon the subject, which I am now prepared to definitely offer to the profession, and to which I first called attention some four and a half years ago in a paper read before the Philadelphia Neurological Society. I have become convinced that in many, if not most cases of intra-cranial syphilis the following group of symptoms is to be obtained : namely a cephalalgia that is apt to be peculiar in a quasi-periodicity that mani. fests itself in a tendency to return at a certain time in the twenty-four hours, most frequently at or towards night, less frequently in the afternoon or morning; marked insomnia, usually at the outset, lasting a few weeks; a sudden cessation of the cephalalgia and insomnia upon the supervention of any paralytic or convulsive symptoms. Hemiplegia in an adult individual under forty years of age, even when not confined with the foregoing cephalalgia and insomnia, is also, in my opinion, apt to be syphilitic, exclusion being made of trauma, tumor, and nephritis. Through a period of some eight years I have satisfied myself of the value of this symptom.group.
I have demonstrated its correctness many times to my classes, it has been verified in my clinic by my assistants, and I shall detail histories of twenty-seven cases, although I have as many more that I cannot see the use of recounting at length.
1 Medical News, July 9, 1887.
In one case, Case IX., there was no history of insomnia, but this is an exception proving the rule.
CASE I.-Male, aged thirty-nine years. About six months ago he began to have severe headache which was frontal, and which has lasted ever since, but shifts from one part of the head to the other, being gone on some days, but never leaving him throughout one single day. Has had emesis only on the first day. For the first month he did not obtain more than three or four hours' sleep each night, but has since then slept well. A careful microscopical and chemical examination detected no renal lesion, nor has he any outward symptoms of such. During the first six weeks it is said he had a shutting gait. About six months before coming to me it was noticed that he had a distinct lisp in his speech. He has used both tobacco and liquor excessively, but bas ceased using either for some eight months. Has at times been mentally confused, but not during the last six months. At the first onset of the affection he acted very flightily. The face flushes readily. There is no tremor of the tongue, facial muscles, or extremities. Has optic neuritis which is well marked upon one side. Before coming to me he had been for some time under the care of Dr. R. W. Taylor, who kindly writes me that he could detect no evidences whatever of syphilis. The patient, however, admitted that he had had a chancre, although he knew of no sequelæ; and the vigorous iodide and mercurial treatment to which Dr. Taylor subjected him cured him, for after coming under my care I did nothing more than give a few placebos, and yet he made an excellent recovery, which has now persisted for three years. CASE II.-Male, aged twenty-nine years. Has been ill for six weeks,
. although it is stated that there had been for a long time certain prodromal symptoms, of which I can obtain no definite description. He imagines that his fellow workmen conspired against him, that he is going to be taken away, that the cat has brought bad luck; wishes to get away, and gets up imagining he hears carriages at the door, brought there to remove him. He has been complaining for over four years.
Has been intemperate in his habits until some three years ago. He was sent to me by Dr. J. C. Kennedy, of Brooklyn, who writes that he had syphilis, and that four years ago he had a chancre, the only sequel of which was the characteristic sore throat. Patient tells me himself that about six weeks before coming to me he began to get confused and worried, and then had his delusions, from which he says he has recovered. Then had optic hallucinations of darkness mixed with lightning, but no auditory ones. At the outset of the affection he had severe headache coming on at night, and obstinate insomnia. Exactly how long these symptoms lasted I have been unable to ascertain. Face flushes at times greatly, has had temporary difficulty in speaking, and during the last two weeks it has been noticed that he stumbled in walking, especially when he got up in the morning. The night before coming to me he had a slight convulsion, copsisting of slight loss of consciousness and drawing back of the head and eyes, and slight sidewise movements. There is a neurotic heredity. The retinæ are perfectly normal except that there is some fulness of the vessels in the left fundus. None of the
cranial nerves are affected. There is no trenor of facial or tongue muscles or of the extremities, nor any paresis. The tendon reflexes are abnormal in that they are somewhat spasmodic, although not exaggerated in the extent of the knee-jerk. The patient improved somewhat on large doses of iodide with mercurial inunctions, but then passed away from Dr. Kennedy's observation.
CASE III.—Male, aged thirty-five years. Had a hemiplegia in 1884, with slight motor aphasia, from which he incompletely recovered. Had another in 1886 with more marked motor aphasia, from which he has also incompletely recovered. A violent cephalalgia preceded the first attack, was greatly diminished upon the supervention of the first hemiplegia, disappeared entirely in three days, and was absent for about eight weeks, when it appeared again temporarily, no cephalalgia preceding the second attack. Also had obstinate insomnia preceding the first attack of hemiplegia, this lasting about ten weeks. At the present time is paretic upon both sides, the result of his double hemiplegia. I have had this patient under my observation for some three years, and can therefore state positively that his mental impairment consists only of an occasional emotional condition and a tendency to great irritability. He has had no implication of the cranial nerves, and no vesical, rectal, sensory, or spinal symptoms. One pupil is much larger than the other and responds very sluggishly to light, but fairly well to accommodative movements. This patient had a chancre in 1880, followed by roseola, and was under the care successively of several physicians of eminence who made a diagnosis of syphilis at the start and treated him for such.
Case IV.-Male, aged forty-three years. In 1890 this patient had severe cephalalgia over the brows, generally in the afternoon and toward evening, and obstinate insomnia, the headache and insomnia lasting about three weeks. At about the same time he became very vertiginous, these attacks of vertigo coming on suddenly and rendering him temporarily very ataxic. For over a year has suffered from amnesic and ataxic aphasia and still seems doubtful in his memory of the pronunciation of certain words, although a certain part of his speechdefect is due to the fact that he has always stuttered. His general memory had been excellent for about a month before coming to me. There is no paralysis or ataxia of upper or lower extremities, no implication of cranial nerve, no impairment of sensation in the face or extremities, no tremor of the face, tongue, or extremities. He states that he was deaf about six weeks before coming to me, although now there is no impairment of hearing. The optic discs are normal. Tendon reflexes are absent even with the Jendrassik method. Early in the winter of 1889 he tells me that he had what was diagnosticated as a chancre, and he now has a characteristic scar of such. A few months afterward, he states, he had a sore-throat that was attributed to syphilis and relieved by means of mercurials. His headache began about eighteen months after the chancre. This patient had been treated with small doses of the iodide of potash, the doses in the twenty-four hours not exceeding twenty grains, and then sought relief in vain at the Hot Springs, being worse on his return from this trip than he was before he left. I put him immediately upon large doses of the iodide, runniug up to 150 grains in the day, when he commenced to improve rapidly and has been perfectly well and able to attend to his business
during the six months that have since elapsed, although he has lately begun a reduction of his iodides.
CASE V.- Male, aged thirty-one years. Seen in consultation with Dr. D. G. Bodkin. The patient frankly admits that he had chancre about two years ago, although he is not able to give a very definite description of it; there is a characteristic scar upon the penis. I have not been able to ascertain whether he had had any cutaneous sequelæ or not. He has had occasional headache through the last year, at times violent, almost invariably toward evening, with great insomnia and irritability. Dr. Bodkin was only called in to see him about a fortnight before the consultation, when he was suffering from ptosis of the left eye without strabismus, and this was relieved promptly by ten grains of iodide three times daily. The patient was lying in bed, his face much flushed. There was considerable hebetude and thickness of speech. He could not pronounce at all the words“ riding, cavalry, brigade.” Pupils well dilated but responding sluggishly to light and accommodation. Both retina distinctly congested. The left lid droops slightly, right face is paretic, the tongue points straight, the hand-grasp on the right is decidedly weaker than upon the left. There is no paresis in the lower extremities. The right tendon reflex is slightly exaggerated, although the left is normal. The sensory examination was unreliable because of the patient's hebetude. The cephalalgia is extremely severe. The bladder is paretic. He sways markedly in standing with his eyes closed, whether his feet are approximated or separated. No tenderness upon percussion of the scalp. I recommended inunctions of the unguent. hydrargyri and large and increasing doses of the iodide. Under this treatment, so Dr. Bodkin subsequently wrote me, the patient immediately began to improve, and eventually made an excellent recovery, the details of which, however, I am not able to state.
CASE VI.-Female, aged forty years, married. Has given birth to four living children, two of whom died in infancy, and has had two or more miscarriages. Family history is negative. The patient has never suffered from serious illness. Her present condition began to develop about a year before coming to my clinic, with pain in the lower jaw. She had had for several weeks headaches coming on toward night, with marked insomnia, but these were distinct from the pain in the jaw, which latter was intense in degree from the first. Two molars were extracted without benefit, the pain increasing, the right upper jaw and right temporal region becoming involved for about five weeks, when the pain ceased abruptly. Simultaneously with its appearance was noticed a numbness corresponding with the areas into which the pain had previously extended. The numbness was followed almost immediately by paralysis of the right side of the face. The angle of the mouth was drawn down, the facial expression became changed from a loss of the labial folds, there was lagophthalmus even during sleep. There was a loss of power in the muscles of mastication, and the food lodged in the cheek pouch. There has not been any pain since the onset of paralysis. Two months after the onset vision became misty. The eye began to look hazy and keratitis neuro-paralytica set in. In the course of three months vision of the right eye became reduced to a faint perception of large objects only as dim shadows. An examination of the patient at this time showed right facial paralysis involving the seventh nerve in all its branches, as described above; hearing also somewhat impaired