Ataxia

“ There is neither tremor nor ataxia present, and paralysis and anæsthesia are not necessary factors. It is distinguished from vertigo and dizziness by absence of subjective sensations of movement. Staggering may show itself in a general way or in the shape of latero-, retro-, or propulsion (disease of the internal ear, paralysis agitans) . A well-defined variety of staggering is the wrongly-termed cerebellar ataxia. In this the patient, having disease of the cerebellum involving its vermis superior, stands with feet widely separated to increase his base of support ”

“ In patients who cannot walk, ataxia may be tested by ordering repeated pronation and supination of the forearm. Paresis or asthenia may be found in the trunk muscles, or evidenced by weakness of the grip, or drooping of the head to one side. Changes in muscle tone may arise and lead to exaggerated or decreased reflexes, often varying from day to day. Vertigo and nystagmus may also be present, in addition to occipital headache and tenderness on percussion. When one lateral lobe is implicated, the symptoms are referred to the same side ”

“ When such a patient comes into our hands the history, and especially the history of predisposing causes, needs the most careful examination. It is well established that syphilis is a common precedent of ataxia, occurring in at least two-thirds of the cases; it is even more firmly settled that iodide and mercury in large doses do no good in advanced ataxia. I say in advanced ataxia, because a few cases are seen in which the syphilis has been of recent occurrence, or where the spinal symptoms are of decidedly acute character, and in these anti-syphilitic medication is needed and useful ”

“ Women, as I have before observed, although rarely in America the victims of tabes, when they do have it have far less disturbance of balance than men, and this is to be attributed to their life-long habit of walking without seeing their feet. I have found in the few cases of ataxia in women that I have seen that they benefited much more quickly by these balance instructions than did men, though their other symptoms were in no way different. ”

“ The disturbance of co-ordination above spoken of as manifested in the inability of the patient to stand well with his eyes closed is the first step in the development of the characteristic ataxia which marks the full-blown affection. The patient finds that he tires more and more on slight exertion—not because his muscles are weak, but because he has to make more voluntary effort than a person in health. He finds that he stumbles easily—is unable to ascend and descend at the curbstone or to walk over an irregular surface with ease. Going down stairs is peculiarly irksome. ”

“ No cure is sure. Salvarsan, skillfully administered, is as near a specific as any known form of treatment. But we don’t know whether it has any effect at all upon locomotor ataxia or general paralysis, the after effects, which may destroy the patient fifteen or twenty years after the actual disease has been cured. All locomotor ataxia and all general paralysis come from syphilis. And these diseases are not only incurable, but are as nearly a hell on earth as poor humanity is ever called upon to endure. Of course, you know that a man who is base enough to marry with syphilis dooms his children. ”

“ In human cases we find that where the sensibility of one hand is lost or greatly impaired, without paralysis, there is extreme awkwardness and uncertainty in delicate muscular movements, but no ataxia. The staggering exhibited by patients having plantar anæsthesia is largely of this type: they stand fairly well while their eyes are open, but oscillate or [p. 49] fall when they are closed. This variety of inco-ordination may result from toxic conditions (alcohol) , peripheral, neural, or central nervous disease. ”

“ In some examples of its spinal form locomotor ataxia is undoubtedly an extremely chronic disease, with few and imperfectly developed symptoms; and it is thus not unnatural to expect that similar limitations may obtain in the ocular manifestations of the disease. That evidences of grave nervous disease may be limited to the pupil is well seen in Case XVIII., where a syphilitic patient was under observation for three years without the discovery of any abnormality other than paresis of each sphincter iridis. ”

“ The patient has incoördination; one, therefore, does one's best to teach him to co-ordinate his movements by small beginnings and by small increases.
The lessons may be given by the physician at first and be executed under his eye. After a few days any tolerably intelligent patient should be able to carry them out alone, but still each new movement should be personally inspected to make sure that it is done correctly.
In patients in the first stage of ataxia the most striking result of incoördination is the impairment of station. We therefore begin with balancing lessons. ”

“ The loss of the sense of touch in the skin is not, however, the sole or even chief reason that the ataxic man cannot walk. I once saw a patient who could march very well by night or by day, although [Pg 634] the skin of his legs was so dead that a live coal would scarcely awaken it. The sense that is wanting in locomotor ataxia is that form of general sensibility which is known as the muscular sense; that is, that sense or feeling which tells the lower brain exactly how much a muscle is contracted, and when to urge it more, when to let it relax. ”

“ The pain of a gastric neurosis may, indeed, so simulate the gastric crises of locomotor ataxia as to make what is only a case of hysteria seem beyond doubt one of locomotor ataxic. Locomotor ataxia patients are prone to think much about themselves and to fear the recurrence of these painful crises once they have had experience with them. As a consequence they sometimes suffer from what are pseudo-crises, that is, from neurotic painful conditions which simulate genuine crises mainly in the amount of reaction they produce in the patient. ”

“ Yet in the light of the development of an ocular paralysis, it can scarcely be doubted that, whether he develop spinal symptoms or not, his nervous system is the site of diseased processes of the locomotor ataxia order. When to these facts there are added, as in Cases XIV. to XVII., illustrations of the various forms and degrees of evidence of spinal disease that may be associated with the Argyll-Robertson pupil, it seems impossible to resist the conclusions that the condition of the pupil so named may be the first evidence of locomotor ataxia ”

“ In the third, the paralytic stage, which seldom comes on until the symptoms have lasted for years, there is gradual loss of power and ataxia, increasing until he is totally unable to walk. If a patient is not seen until this condition of things has been reached, but little can be hoped from any treatment, though in a few cases energetic measures may bring about a marked improvement, which is rarely lasting. ”

“ In some cases the disease attacks the membranes of the brain and the small blood vessels giving rise to a still different type of mental disorder. Practically all patients with locomotor ataxia owe their condition to an antecedent syphilis. Moreover it is one of the important causes of arterio-sclerosis, or hardening of the blood vessels, and is also a prominent factor in certain forms of heart-disease, as well as by no means an unimportant cause of blindness in children. ”

“ Treatment and Prevention of Late Syphilis of the Nervous System.—Locomotor ataxia and paresis, even more than other syphilitic diseases of the nervous system, are extremely hard to affect by medicines circulating in the blood, and for that reason do not respond to treatment with the ease that syphilis does in many other parts of the body. Early locomotor ataxia can often be benefited or kept from getting any worse by the proper treatment. For paresis, in our present state of knowledge, nothing can be done once the disease passes its earliest stages. ”

“ The most striking peculiarity about ataxic movements is the defect in the harmonious action of the various [p. 48] (antagonistic) muscles employed, resulting in jerky, oscillatory, but non-rhythmic movements, constantly made worse by greater tension of the will. In simple tremor volition may for a few moments stop the movements (as best shown in paralysis agitans) , while in ataxia the more the patient tries to achieve the act, the greater becomes the disorder. The anatomical fault is mostly in the spinal co-ordinating mechanism ”

“ Again, whenever we endeavor to estimate accurately how heavy a body is, we raise it again and again into the air, evidently that we may feel the muscles contract again and again, and by repeated efforts judge how much of force they put forth.
The phenomena of ataxia are of great importance as corroborating the experimental proofs spoken of a few pages back, which show that walking is automatic and not simply performed by a conscious power of the will. Thus the ataxic may be able to walk with his eyes open, when he cannot even balance himself erect with his eyes shut. ”

“ Stage of Ataxia (Disturbance of motion) .—The disturbance of motion (ataxia) is very marked, especially in the lower extremities; the walking becomes difficult and uncertain; there is difficulty in rising or rapid turning; the legs are wide apart; feet lifted too high and come down too forcibly; the length of the steps is irregular, and the body is imperfectly balanced. If the patient stands with his feet together and eyes closed he begins to sway, (Romberg's symptom) , which is due to a defect in controlling the muscles from impairment of sensation. ”

“ Locomotor ataxia, and, above all, general paralysis develop, as a rule, in men who, having had syphilis, have some occupation in life that calls for considerable mental strenuosity, and involves excitement and worry. Actors, brokers, soldiers and sailors, speculators of all kinds, race-track gamblers, these are the classes from {510} which victims of paresis and locomotor ataxia are particularly recruited. ”

“ There is more than ataxia, however; for the hand grasp is so enfeebled that the patient may have a difficulty in holding his rice bowl as well as in feeding himself. There is no tremor of the hands; and never or very rarely is there any paresis of the ocular muscles, or of the muscles of the face, of mastication, of the tongue, or of the pharynx. The sphincters and bladder operate satisfactorily, and the functions of the alimentary canal are carried on fairly well, although there is often some dyspeptic distension and oppression after food. ”

“ Although this muscular ataxia may be observed, even if not constantly, in the prodromal period of general paralysis, it is usually well marked only when the symptoms have become well developed. There is also a fibrillary tremor of one group of muscles or of one set of fibres after another when these muscles are exerted, and increasing as they become wearied, as they soon do, from the exercise. ”

“ For the hands and arms, which, except in those very rare cases where the ataxia first shows itself in the upper extremities, seldom exhibit much incoördination in the primary and middle stages, the movements are the picking up of a series of different-shaped small articles, arranging objects like dominoes, marbles, or the kindergarten sticks in patterns, bringing the fingers of the two hands one after another together, or touching a finger to the ear or the nose, at first with open and then with shut eyes. ”

“ While certain cases, or even epidemics, of malarial fevers are attended by remarkable adynamia, often manifesting itself from the very incipiency of attacks, it differs widely from that utter nervous ataxia which characterizes typhoid fever. Again, the adynamia of malarial attacks is generally ascribable to some cause not essential to those affections. Imperfect reaction from a chill, long persistent hyperpyrexia, diarrhoea or vomiting, or chronic paludal cachexia, or, it may be, some epidemic influence, may produce it. The ataxia of typhoid fever is part of its morbid process. ”

“ These changes are due to disturbance of the blood and lymph supply of the nervous tissue. Once the changes have taken place, there is no hope of the patient ever recovering the normal use of his limbs. Not infrequently he becomes bedridden and can not walk at all because he is not able to steady himself. He may not suffer in his general health, however, to any serious extent, and may live on for twenty years, though usually his resistive vitality is lowered and he is carried off by some intercurrent disease.
At times locomotor ataxia begins with very severe pain seizures, known as crises. ”

“ The inability to feel the contact of the ground or floor with the feet occasions the difficulty in walking. The causes of this disease are somewhat obscure, but unquestionably exposure to cold and dampness, and over-mental work, are largely instrumental in its production. Scrofula and syphilis favor its development, while abuse of the nervous system, such as results from over-indulgence of the animal and reproductive instincts, are frequent sources of the nervous changes that lead to ataxia. ”

“ When the disease originates in the spinal cord the symptoms are similar to those of locomotor ataxia, and it is now believed that general paralysis and locomotor ataxia are one and the same disease; in the one case the cord, in the other the brain, being the primary seat of lesion. The early physical symptoms are generally motor. The patient loses energy, readily becomes tired, and the capacity for finely co-ordinated motor acts, such as are required in playing games of skill, is impaired. ”