CHAPTER IX

SYMPTOMS AND PHYSICAL SIGNS

=Special=

Our conception of arteriosclerosis as a degenerative process affecting the vascular tree rather than a disease, removes the possibility of discussing special symptoms. As a matter of fact, we know of very few organs where even profound pathologic changes in the vascular system produced during life any symptoms which could be laid to these arterial changes. Kind nature has given to us such an excess of organs of every kind that the destruction of large portions of any organ seems to affect the function but little. So only particular groups of organs, which show symptomatic changes as the result of arteriosclerotic processes, will be discussed. It is realized that this may not give Teutonic completeness to the discussion, but it certainly saves paper and has a distinct practical value to the long suffering reader.

Although arteriosclerosis is a disease which affects the whole arterial system, it nevertheless never reaches the same grade all over the body. The difference in the structure and functions of the various organs determines to great extent the eventual symptomatology. Endarteritis obliterans of a small sized artery in the liver or leg would lead to no marked symptoms, as the circulation is so rich that the anastomoses of the blood vessels would soon establish a collateral circulation that would be perfectly competent to sustain the function of the part. Quite different would it be should one of the small arteries of the brain, the lenticulo-striate, for example, which supplies the corpus striatum, become the seat of a thrombosis or embolism caused by arteriosclerosis. The arteries of the brain are terminal arteries and the blood supply would be cut off entirely with a resulting anemic necrosis of the part supplied by the artery and a loss of function of the part. What would be of no moment in the leg or arm might prove even fatal in the brain.

The further symptomatology, therefore, of arteriosclerosis depends entirely on the organ or organs most affected by the interference with the blood supply. The following groups may be recognized:

1. Cardiac.

2. Renal.

3. Abdominal.

4. Cerebral.

5. Spinal.

6. Local vasomotor effects.

7. Pulmonary.

=Cardiac=

Most cases of arteriosclerosis sooner or later present symptoms referable to the heart. When the organ is hypertrophied and is already working against an enormous peripheral resistance, a slight excess of work put upon it may cause a dilatation of the chambers with the resulting broken compensation. There is dyspnea on slight exertion, possibly some precordial distress, slight edema of the ankles and lower legs and possibly scanty urine. With proper care, a patient with such symptoms may recover, but the danger of another break in compensation is enhanced. The next attack is more severe. The edema is greater, there may be signs of edema of the lungs, effusions into the serous cavities may occur. The heart shows marked dilatation. There is gallop or canter rhythm and there are loud murmurs at the apex. When a patient is first seen in this stage, it may be quite impossible to state whether or not there is true valvular disease of the heart. The muscle is usually diseased in that there is fibroid degeneration of more or less extensive character. This factor causes the heart to lose much of its elasticity and increases the tendency to permanent dilatation. Such cases must be watched before one can say that true valvular insufficiency is not present. The fatal termination of such a case is quite like that of true valvular disease. There is increasing dyspnea, increasing anasarca, and the patient usually succumbs to edema of the lungs, drowned in his own secretions.

A very rare complication of the fibroid degeneration of the heart muscle is aneurysm of the heart wall. (Fig. 59.) The apex of the left ventricle is most commonly the site of the aneurysm and rupture occasionally occurs. Such an accident is rapidly fatal. In the arteriosclerotic process which occurs at the root of the aorta, the coronary arteries become involved both at the openings and along the courses of the vessels. A branch or branches or even one artery may become blocked as a result of obliterating endarteritis. The arteries of the heart are not terminal vessels but as a rule blocking of a large branch leads to anemic infarct. These areas become replaced by fibrous tissue which in the gross specimen appears as streaks of whitish or yellowish color in the musculature. Anemic infarcts may not occur. In such cases the anastomosis between branches of the coronary arteries is unusually free. Through arteriosclerosis of the coronary vessels extensive fibrous changes may occur that lead to a myocardial insufficiency with its attending symptoms--dyspnea, irregular and intermittent heart, gallop rhythm, edema, etc. One of the most distressing and dangerous results of sclerosis of the coronary arteries and of the root of the aorta is angina pectoris. While in almost every case of angina pectoris there is disease of the coronary arteries, the contrary does not hold true, for most extensive disease, even embolism, of the arteries is frequently found in persons who never suffered any attacks of pain. This symptom group is more common in males than in females and as a rule occurs only in adult life. "In men under thirty-five syphilitic aortitis is an important factor." (Osler.)

Since the valuable experiments of Erlanger on heart block, considerable attention has been paid to lesions of the Y-shaped bundle of fibers, a bundle arising at the auriculoventricular node and extending to the two ventricles, known also as the auriculoventricular bundle of His. Interference with the transmission of impulses through this bundle gives rise to the symptom group known as the Stokes-Adams syndrome, which is characterized by: (a) slow pulse, (b) cerebral attacks--vertigo, syncope, transient apoplectiform and epileptiform seizures, (c) visible auricular impulses in the veins of the neck. Many of the cases which occur are in elderly people the subjects of arteriosclerosis.

So far as we now know all cases of the Stokes-Adams syndrome are caused by heart block which is only another name for disease in the auriculoventricular bundle. Of interest here is the fact that besides gummata, ulcers, and other lesions of the bundle, definite arteriosclerotic changes have been found.

"The investigation of a typical case of Stokes-Adams disease has shown that the symptoms of this case are caused by some lesion in the heart which gives rise to the condition now generally termed heart block. Practically all degrees of heart block have been observed, namely, complete heart block and partial block with 4:1, 3:1, and 2:1 rhythm, and occasionally ventricular silences. These stages occurred during recovery.

"Experiments testing the reaction of the heart to various extrinsic influences demonstrate that when the block is complete the ventricles do not respond to influences presumably of vagus origin, although the auricles still respond normally to such influences, that effects exerted upon the heart presumably through the accelerators still influence the rate of the ventricles as well as that of the auricles.

"When the block is partial the rate of the ventricular contraction varies proportionally with the rate of the auricular contractions but only within certain limits. When these limits are exceeded the block becomes more complete, i. e., a 2:1 rhythm may be changed into a 3:1 rhythm, this into a 4:1 rhythm, and this into complete block, and vice versa.

"The syncopal attacks are, in all probability, directly dependent upon a marked reduction of the ventricular rate. Such reductions of the ventricular rate are always associated with an increase of the auricular rate, and it is believed that the latter is the cause of the former." (Erlanger.)

The epileptiform seizures of the syndrome may be caused by the anemia of the brain resulting from failure of the heart to supply a sufficient quantity of blood.

The apoplectiform attacks are most probably caused by venous congestion when the slowing of the ventricular contractions is not sufficient to cause convulsions, but will just cause complete unconsciousness.

=Renal=

Chronic nephritis, hypertension, arteriosclerosis form a most important trinity. Some stoutly affirm that in all cases of high tension there is chronic renal disease. Certainly the very highest blood pressures which we see occur in the chronic interstitial forms of kidney disease. The cause is most probably to be sought in some poison which is elaborated in the kidney, is absorbed into the circulation and acts powerfully either on the vasoconstrictor center as a stimulus, or directly on the musculature of the small arteries all over the body. Usually hypertension is progressive but it may be temporary.

A man, 43 years old, entered the Milwaukee County Hospital in uremic coma. The systolic blood pressure was 280-290 mm. Hg, the diastolic pressure 220 mm. (Janeway instrument). Under treatment his blood pressure gradually became lower, at the same period the albumin and casts gradually disappeared from the urine. In two weeks from admission he seemed perfectly well, there were no albumin or casts found in the urine, and the systolic blood pressure was 136 mm., not a high figure for a muscular man of the laboring class. It must be admitted, however, that such cases are the exception, not the rule.

Patients suffering from the association of chronic nephritis with hypertension die slowly, usually. There is gradual development of anasarca. Headache is frequent and severe. Pains all over the body may occur. The sight may suddenly become dim or may even be lost. Dizziness may be complained of and dyspnea is usually marked. Cyanosis comes on, the pulse becomes weak, irregular or intermittent, heart failure sets in, and the patient dies with edema of the lungs.

Another class of renal arteriosclerosis is characterized by a small granular kidney in which fibrous changes of a patchy character have taken place. These scattered areas are the result of obliterating endarteritis of renal arteries here and there with consequent anemia, death of cells, and replacement by fibrous tissue. It occurs as part of a generalized arteriosclerosis in which the whole arterial system is the seat of diffuse (senile) sclerosis. The palpable arteries are usually beaded or even encircled with calcareous deposits and the aorta is the seat of an extensive nodular and ulcerating sclerosis. The heart is usually small, shows extensive fibrous and fatty changes and possibly the condition known as "brown atrophy;" the blood pressure is low. Such cases do not show any special symptoms. They are anemic, short of breath on exertion, have the appearance and show the signs of senility.

In the first group it is, at times, difficult to say whether the kidney disease or the arterial disease is the most important. From a clinical standpoint the decision is not essential as the end results are much the same in both. However, when actual uremic symptoms dominate the picture, it becomes evident that the disease of the kidney is the chief feature in the causation of the symptoms.

=Abdominal or Visceral=

There is an important group of cases to which but little attention has been paid until quite recently. This is the abdominal or visceral type of arteriosclerosis. It has been stated that arteriosclerosis of the splanchnic vessels almost invariably causes high tension. Among others, Janeway has shown that general arteriosclerosis without marked disease of the splanchnic vessels does not cause as a rule increase of blood pressure.

There are cases in which the brunt of the lesion falls upon the abdominal vessels. Such cases have been called "angina abdominalis." It has been suggested (Harlow Brooks) that this type of arteriosclerosis may be determined by constant overloading of the stomach with food, especially rich and spiced food. This causes overwork of the special arteries connected with digestion and so leads to sclerosis of the vessels of the stomach, pancreas, and intestines. Personal habits probably influence to great extent the production of this more or less =localized= condition.

The organs supplied by the diseased arteries suffer from changes analogous to those occurring in general or local malnutrition, such as starvation, old age, or local anemias. These changes are atrophy with hemachromatosis (brown atrophy) or fatty infiltration and degeneration. Following the degenerative changes there result connective tissue growth and further limitation of the functionating power of the affected organs.

Pain is a more or less constant symptom of visceral sclerosis. In the early stages there may be only a sense of oppression, of weight, or of actual pressure in the abdomen or pit of the stomach. There may be only recurring attacks of violent abdominal pain accompanied by vomiting. In some cases symptoms of tenderness in the epigastrium, pains in the stomach after eating, vomiting and backache may suggest gastric ulcer. There may be dyspnea and a sense of anguish accompanied with a rapid and feeble pulse. Hematemesis may make the symptom group even more like ulcer of the stomach, and only the course of the disease with the failure of rigid ulcer treatment and the substitution of treatment directed toward relief of the arterial spasm with resulting betterment, enables one to make a diagnosis. The condition may be present for years and the symptoms only epigastric tenderness with dizziness and sweating on lying down after dinner, as in one of Perutz's patients. The attacks are probably due to spasmodic contraction of the sclerosed intestinal vessels with a resulting local rise in blood pressure. The pains are most probably due to the spasm of the intestinal muscles, and some think they are located in the sympathetic and mesenteric plexuses.

This result of arteriosclerosis is not so uncommon, and by keeping this cause of obscure abdominal pain in mind we are now and then enabled to save a patient from operation.

An autopsy on a case which for many years had attacks of abdominal pain and cramp-like attacks, with high blood pressure and heart hypertrophy, showed extensive sclerosis of the abdominal aorta, superior mesenteric and iliacs. These vessels were calcified. Hypertrophy of the left ventricle was found. The kidneys were microscopically normal. There were no changes in the ascending aorta but in the descending portion there were scattered nodules and small calcified plaques.

The attacks of pain from which this patient suffered for many years, the hypertrophy of the left ventricle and the increased blood pressure were thought to be directly due to the sclerosis of the abdominal vessels.

=Cerebral=

It has been stated that arteriosclerosis is a general disease, yet certain systems of vessels may be affected far more than others, and indeed there may be marked sclerosis at one part of the body and none demonstrable at another part.

In advanced sclerosis there may be one or more of a series of accidents due to embolism, thrombosis, or rupture of the vessels. Such conditions as transient hemiplegia, monoplegia or aphasia may occur. The attacks may come on suddenly and be over in a few minutes; what Allbutt calls "Larval apoplexies." They may last from a few hours up to a day, and are very characteristic. A patient aged 64 years with pipe stem radials and tortuous hard temporals would be lying quietly in bed when suddenly he would stiffen, the eyes would become fixed and the breathing cease. In a few seconds consciousness returned, the patient would shake himself, pass his hand over his brow and ask, "Where am I? Oh, yes, that's all right." He had as many as thirty of these attacks in twenty-four hours, none of them lasting over one minute. To just what such attacks are due, it is hard to say. Some have attributed them to spasm of the smaller blood vessels of the brain, but there have never been demonstrated in the vessels any constrictor fibers.

There is a well recognized form of dementia caused by arteriosclerosis. In general paralysis of the insane and in senile dementia the blood vessels are always diseased. Milder grades of psychic disturbances are accompanied by such symptoms as mental fatigue, persistent headaches, vertigo, memory weakness and fainting. Aphasia, periods of excitement and mental confusion occur in some. Later stages are at times accompanied by inclination to fabulate, loss of judgment, disorientation, narrowing of the external interests, episodes of confusion and hallucinatory delirium.

The hemiplegias, monoplegias and paraplegias may occur again and again and last for one or two days. Unless there has been rupture of the vessels, there is complete recovery as a rule.

In persons who have arteriosclerosis with high tension attacks of melancholia are seen. There are at the same time fits of depression, insomnia, irritability, fretfulness, and a generally marked change in disposition. When the tension is reduced by appropriate treatment these symptoms disappear, to recur when the tension again becomes high. On the contrary, attacks of mania are accompanied by low blood pressure. The dizziness and vertigo in cerebral arteriosclerosis are probably due to the stiffness of the vessels which prevents them from following closely the variations of pressure produced by position, and thus, at times, the brain is deprived of blood and a transient anemia occurs.

Arteriosclerosis of the cerebral vessels is always a serious condition. The greatest danger is from rupture of a blood vessel. Another of the dangers is gradual occlusion of the arteries bringing about necrosis with softening of the brain substance. The latter is more apt to be associated with psychic changes, dementia, etc.; the former, with hemiplegia. It is curious that a small branch of the Sylvian artery, the lenticulo-striate, which supplies the corpus striatum, should be the one which most frequently ruptures. Where the motor fibers from the whole cortex are gathered together in one compact bundle, a very small hemorrhage may and does cause very serious effects. A comparatively large hemorrhage in the silent area of the brain may cause few or no symptoms.

=Spinal=

It is conceivable that arteriosclerosis of the vessels of the spinal cord might cause symptoms which would be referred to the areas of the cord where the process was most advanced. The lesions would be scattered and consequently the symptoms might be protean in character.

True epileptic convulsions dependent on arteriosclerotic changes are also seen and are not so uncommon.

This is on the whole a rare condition, much less common than arteriosclerosis of the cerebral vessels. Collins and Zabriskie report the following typical case:

"H., a fireman, fifty-one years old, was in ordinary good health until toward the end of 1902. At that time he noticed that his legs were growing weak and that they tired easily. Later he complained of a jerking sensation in different parts of the lower extremities and at times of sharp pain, which might last from several minutes to two or three hours. The legs were the seat of a heavy, unwieldy sensation, but there was no numbness or other paresthesia. About the same time he began to have difficulty in holding the urine, a symptom which steadily increased in severity. These symptoms continued until March, 1903, i. e., for three months, then he awakened one morning to find that he was unable to stand or walk, and the sphincters of the bowels and bladder relaxed. There was no complaint of pain in the back or legs, no difficulty in moving the arms, in swallowing or in speaking. He says he was able to tell when his lower extremities were touched and he could feel the bed and clothes. He was admitted to the City Hospital three weeks later and the following record was made on April 21, 1903.

"The patient was a frail, emaciated man of medium height, who had the appearance of being 55-60 years of age. He was unable to stand or walk. When he was lying, he could flex the thigh and the legs slowly and feebly. There was slight atrophy of the anterior and inner muscles, more of the left than of the right side. The knee jerks and ankle jerks were absent. Irritation of the soles caused quite a typical Babinski phenomenon. The patient had fair strength in the upper extremities, but the arms tired very soon, he said. The grip was moderate and alike in each hand. The motility of the face, head, and neck was not noticeably impaired. There was no difficulty in swallowing, and articulation was not defective. Tactile sensibility was slightly disordered in the lower extremities, although he could feel contact of the finger, the point of a pin, and the like. Sensibility was not so acute as normal; there was a quantitative diminution. Sensory perception was not delayed. There was a distinct zone of slight hyperesthesia about as wide as the hand above the femoral trochanters. Above that, sensibility was normal. There was no discernible impairment of thermal sensibility. No part of the body was particularly tender on pressure. A bedsore existed over the sacrum, and there was excoriation of the genitals from constant dribbling of urine.

"Examination of the chest showed shallow respiratory movements. The heart was regular, weak, there were no murmurs, the second sound was accentuated. Examination of the abdomen showed that the liver and spleen were palpable, but were not enlarged. The abdominal reflexes, both upper and lower, were sluggish. The patient was slow of speech, likewise apparently of thought. He did not seem to show an adequate interest in his condition, still he was fully oriented and seemed to have a fair memory. His mental reflex was slow. There were indications in the peripheral blood vessels and heart of a moderate degree of general arteriosclerosis. The peripheral vessels such as the radial, were palpable, the walls thickened, the blood pressure increased.

"The patient did not complain of pain while he was in the hospital, a period of four weeks, nor was there any particular change in the patient's symptoms, subjective and objective, during this time. His mental state remained clear until forty-eight hours before death, when he became sleepy, stuporous, and comatose, dying apparently of cardiac weakness, which had set in simultaneously with the clouding of consciousness."

At autopsy, except for a few small hemorrhages in the posterior horns of the lower dorsal segments on the right side and a similar condition of the left anterior horns, there was nothing noticed. On microscopic examination, there was found widespread sclerosis of the vessels of the cord to a marked degree with only slight thickening of the vessels of the brain. There were secondary degenerations of ascending and descending type particularly marked at the ninth dorsal segment. They included portions of all the tracts, the pyramidal tract as well. The symptoms in brief were: (1) weakness and easily induced fatigue of the legs; (2) peculiar sensations in the lower extremities, described as jerky, numbness, heaviness, and occasionally sharp pain; (3) progressive incontinence of urine; (4) progressive paraplegia.

Since one of the chief manifestations of syphilis is sclerosis of the arteries, neurologic cases characterized by irregular symptoms and signs which can not be placed in any of the definite system disease groups, are possibly due to irregularly scattered areas of sclerosis throughout the spinal cord caused by obliterating arteritis. Such cases are not so very uncommon. Several have come under my observation. Further studies of the spinal cords of these cases at autopsy are necessary before a final opinion can be given as to their dependence on arteriosclerosis of the spinal vessels.

=Local or Peripheral=

When the arteriosclerosis in the peripheral arteries reaches a stage where endarteritis obliterans supervenes, there is usually no chance for a compensatory or collateral circulation to be established. The area supplied by the vessel undergoes dry gangrene. A portion of a toe or finger or a whole foot or hand may shrivel up. It is more common to see the spontaneous amputation take place in the lower extremities. The same effect may be produced by the plugging of a vessel with a thrombus. There may be much pain connected with the sudden blocking, whereas the gradual obliteration of the blood supply of a toe or foot is not as a rule at all painful. The condition is at times revealed more or less accidentally when a patient injures his toe or foot and discovers that there is no sensation in the part and that the wound instead of healing is inclined to grow larger.

Other interesting vasomotor phenomena are frequently connected with arteriosclerosis. Such a one is the curious condition known as Raynaud's disease, a vascular disorder which is divided into three grades of intensity: (1) local syncope, (2) local asphyxia, (3) local or symmetrical gangrene. This is not the place to describe this condition except to say that the condition called "dead fingers" is the most characteristic feature of the first stage. Chilblains represent the mildest grade of the second stage. The parts are intensely congested and there may be excruciating pain. Any one who has ever had chilblains knows how painful they can be. The general health is not impaired as a rule, although the attacks are apt to come on when the person is run down. The third stage may vary from a very mild grade, with only small necrotic areas at the tips of the fingers, to extensive multiple gangrene.

Another and very rare condition in which chronic endarteritis was the only constant finding is the disease described by S. Weir Mitchell and called by him erythromelalgia (red neuralgia). This is "A chronic disease in which a part or parts--usually one or more extremities--suffer with pain, flushing, and local fever, made far worse if the parts hang down." (Weir Mitchell.)

Probably the most frequently seen result of arteriosclerosis in the leg arteries is the remarkable condition, first described by Charcot, known as intermittent claudication. Persons the subject of this disease are able to walk if they go slowly. If, however, any attempt be made to hurry the step, there results total disability accompanied at times by considerable cramp-like pain. The condition is much more prone to occur in men than in women, and Hebrews seem more frequently affected. The cause is most probably to be sought in the anemia which results from the narrowing of the channels through which the blood reaches the part. The stiff, much narrowed arteries allow sufficient blood to pass along for the nutrition of the part at rest or in quiet motion. Just as soon as more violent exercise is taken, calling for more blood, an ischemia of the part supervenes, for the stiff vessels can not accommodate themselves to changes in the necessary vascularity of the part. A rest brings about a gradual return of blood and the function of the part is restored. Pulsation may be totally absent in the dorsal arteries of the feet and when the legs are allowed to hang down there is apt to be deep congestion.

In this connection a curious case reported by Parkes Weber will not be out of place. The patient, a male, aged 42 years, complained of cramp-like pains in the sole of the left foot and calf of the leg occurring after walking for a few minutes and obliging him to rest frequently. When the legs were allowed to hang over the side of the bed, the distal portion of the left foot became red and congested looking. No pulsation could be felt in the dorsal artery of the left foot or in the posterior tibial artery. There was no evidence of cardiovascular or other disease. An ulcer on the little toe had slowly healed, but cramp-like muscular pains still occurred on walking. The disease had lasted about five years without the appearance of gangrene.

Weber calls this case one of arteritis obliterans with intermittent claudication.

=Pulmonary Artery=

In the symptomatology of sclerosis of the pulmonary artery the clinical signs and symptoms are mostly referable to the obliterating endarteritis of the smaller vessels, while the physical signs are more apt to reveal the involvement of the main trunk. A history of severe infection in the past is frequent, especially smallpox, and accompanying aortic sclerosis with insufficiency of the mitral valve or stenosis of this valve is the rule. Striking cyanosis is an early symptom, while there is little if any dyspnea and edema. Intermittent dyspragia is common. There seems to be no tendency to clubbed fingers. Repeated hemorrhages from the lungs without the formation of infarcts may occur. There is usually an area of dullness at the upper left margin of the sternum and nearby parts, sensitive to pressure and to percussion, and the heart dullness extends unusually far towards the right. The diagnosis of the right ventricular hypertrophy may be substantiated by a fluoroscopic examination.