POLIOMYELITIS OCCURING IN AN EPIDEMIC FORM, FOLLOWED TWELVE YEARS LATER BY PROGRESSIVE MUSCULAR ATROPHY AND LATERAL SCLEROSIS. BY J. T. ESKRIDGE, M. D., OF DENVER, COLO. NECROLOGIST TO THE ARAPAHOE COUNTY AND ST LUKE'S HOSPITALS. FROM THE COLORADO MEDICAL JOURNAL, January, 1896. Reprinted from the Colorado Medical Journal, January, 1896. P OLIOMYELITIS OCCURING IN AN EPIDEMIC FORM, FOL- LOWED TWELVE YEARS LATER BY PROGRES- SIVE MUSCULAR ATROPHY AND LATERAL SCLEROSIS. * BY J. T. ESKRIDGE, M. D., OF DENVER, COLO., NEUROLOGIST TO THE -ARAPAHOE COUNTY AND ST. LUKE'S HOSPITALS. I wish to call your attention to-day to this patient, Edgar V., 38 years of age, male, white, single, born in Indiana, but living in Colo- rado thirteen years, laborer and sheep herder by occupation. His father died of consumption, his mother in childbirth. One sister is supposed to have had cerebro-spinal meningitis, but there are no other nervous or mental troubles in the family history. The patient had some of the diseases common to childhood. At the age of 23 years he suffered from pneumonia, and the following year from an attack of cerebro-spinal meningitis (?). The place in which he lived up to his 24th year was a malarial district and he tells us that the community suffered from an epidemic of spotted fever, or cerebro-spinal meningitis, during which this attack occurred. He says that he was unconscious for four weeks, and when he regained con- sciousness his left arm, thigh and leg, and the right leg below the knee were paralyzed. Subsequently to this nearly all of the muscles of the left arm, those of the anterior portion of the left thigh and calf, and the peroneus group and tibialis anticus of the right side became greatly atrophied. He says that from that time on right foot-drop ex- isted. He suffered nearly every year from malarial fever, and to es- cape this he came to Colorado in 1889. Two years later he suffered from pneumonia and measles. Although greatly disabled from mus- cular weakness and wasting he asserts that he was able to do a man's work until July, 1894. In October, 1894, he injured his right shoul- der in a runaway accident, and since that time the right arm at the shoulder joint has been restricted in its movements. In July, 1895, he entered the hospital feeling quite ill, and on examination it was found that he was suffering from haematuria. He improved under treatment and eighteen days later left the hospital, although he was not entirely well. During the latter part of October he fell, again injuring his right shoulder. He re-entered the hospital early in November. Examination November 7, 1895: There is no ataxia in the left arm or chest muscles. There is complete foot-drop on the right side * A Clinical Lecture Delivered at the Arapahoe County Hospital, November 16, 1895. 2 and he is unable to dorsally flex this foot. The plantar flexion is ex- tremely weak. All the other movements of the right leg are strong and the muscles of the thigh and hip are well developed. The dorsal flexors of the left foot which are composed mainly of the tibialis anti- cus and peroneus groups are strong, and forcibly dorsally flex the foot against considerable resistance, but the soleus and gastrocnemius are greatly wasted, and retain but little power, so that plantar flexion of the left foot is very weak. The left knee is flexed with considerable force, but extension of the leg at this joint is weak on account of the atrophy of the front thigh muscles. We shall find considerable differ- ence in the strength of the arm muscles. Dynamometer, R. 114; 1_. 80. The muscles of the right hand and arm are well developed and strong except the deltoid which was injured by the fall. The move- ments of the left hand at the wrist are made with fair power. The muscles of the left forearm -still retain considerable power, although they are much smaller than those of the right forearm. He is scarcely able to move the left arm at the elbow or shoulder. The left biceps, brachialis anticus, triceps, pectoralis major and deltoid are greatly wasted. Before going farther I wish to call your attention to the ac- tion of the muscular fibers of the pectoralis major. The lower portion acting with the latissimus dorsi depresses the arm when elevated, but the clavicular portion acts with the deltoid and other muscles in ele- vating the arm. while both the upper and lower portions act together in bringing the arm across the chest, when the arm is held in a hori- zontal position in front of the chest. We may have paralysis of the upper or lower portion for certain movements and not for others. When those muscles are wasted with which an individual portion of the pectoralis major acts in association in performing certain move- ments it will be paralyzed for this movement, but it may act for other movements if the tnnscles that are associated in this action are not paralyzed. When the deltoid is paralyzed the clavicular portion of the pectoralis major will not contract on the arm being held in a horizontal position in front of the chest and the patient makes an at- tempt to elevate the arm. We find this condition to exist in this pa- tient. However, when I hold the arm in a horizontal position in front of him and ask him to bring the arm across the cnest, you observe that both the lower and upper portion of the muscles contract. The lower portion of the pectoralis major contracts in attempting to depress the arm as the latissimus with which it is associated in this movement is not paralyzed. This "associated paralysis of muscular fibers'' as it has been termed by Beaver, of England, is supposed to be evidence of 3 spinal lesion, We notice further in this patient that most of the left shoulder muscles are paralyzed, including the infra-spinati and supra- spinati, rhomboidei and serratus magnus. The latissimus dorsi, how- ever, still preserves considerable strength, although it is remarkably wasted. The left levator anguli capsulse muscle is very weak. The neck muscles, both anterior and posterior, are strong. There is no in- volvement of the face or tongue. Measurement of the foot at the instep: Right, 8 inches; left, Ankle, right. left, Calf, right 12; left, n. Thigh, 22 inches above the heel, right, 13% inches; left, 27 inches above the heel, right, left, 18. Gluteal region fairly well developed. Upper arm in contraction, right, left, Relaxation, right, 9%; left, When the patient is at rest no tremor is observed, but as soon as he makes any muscular effort a tremor begins which is rather fine in character, and if he makes considerable exertion it seem to affect most of the muscles of the body, The tremor does not begin on first mak- ing muscular movements, but as soon as the muscles become fatigued it is apparent, and continues after the muscular effort ceases until the effects of the exhausted condition passes away. Ordinarily his tremor on exertion is limited to the waste groups of muscles. On stroking the left arm above the elbow a very fine fibrillary tremor is observed. Reflexes: Knee-jerks are greatly increased, but not much influ- enced by reinforcing. Ankle-clonus present. Tendo-Achillis both greatly increased. Plantar, cremaster and abdominal reflexes all ab- sent Deep reflexes of the arms; extensors of the forearms; right in- creased; left slight. Flexors both increased. Triceps, biceps, deltoid and pectoralis major reflexes absent, and so are the masseters. All general sensory phenomena are normal. Special senses: Hearing, watch; right, %; left %. Tuning fork heard better in the closed ear, show- ing that the impaired hearing is due to middle-ear trouble. Smell and taste are normal. The external and internal ocular muscles act well aud the discs and fundi are normal. The haematuria which you have heard mentioned in connection with this man's history probably plays but little part in the disease from which he is suffering. It is a condition which is not infrequently met with in persons who have lived in a malarial district. When he entered the hospital a few days ago there was still slight traces of blood in the urine, but these are rapidly passing away under treatment. Before discussing the nature of this man's trouble we will test the 4 electrical irritability of the muscles. As the muscles are wasted in the left arm, and not in the right, a comparative, test of these two limbs will be sufficient. For testing the faradic irritability I will use a large size Fleming battery, taking the precaution to have the electrodes thoroughly covered with absorbent cotton and moistened in an alka- line solution. The strength of the battery is increased by tapping the current at different buttons, numbered from i to 4, the weakest being 1 and the strongest 4, also by withdrawing the cylinder which sur- rounds the iron rod. For the comparative test the Roman numerals are used for the button tapped, and the Arabic after a plus mark to denote the extent to which the cylinder is withdrawn: Deltoid, R., I-|-2; L,. will not respond to the strongest current. Biceps, R., I+i; E., IV+4. Triceps, R., I-j-3; L. will not respond to the strongest current. Extensors of forearm, R., I; I/., II-J-2. Flexors of forearm, R. I; L., II. The galvanic irritability cannot be determined as the battery is out of order. The points of interest in connection with this case are the sup- posed acute cerebro-spinal meningitis from which he suffered several years ago, followed by paralysis and marked atrophy of the muscles, but without any affection of the special or general sensory phenomena; paralysis of the dorsal flexors of the right foot and of most of the mus- cles that move the left arm at the shoulder and elbow; development of tremor, but the exact date of the beginning of the latter is apparently not known to the patient; increased weakness with wasting of other muscular groups, especially of the legs, increase of the deep reflexes, and retention of the faradic irritability by certain muscles that are greatly wasted. The patient tells us that the wasting of the muscles has not increased since his attack of fever nearly twenty years ago. but the results of the electrical examination prove him to be mistaken in this statement. Muscles that have been absolutely paralyzed and so greatly wasted from a spinal lesion are not to be able to con- tract for a period of nearly twenty years will not preserve as much ir- ritability to the faradic current as we find in most of these wasted muscles, so that we may conclude that a condition of progressive mus- cular atrophy has supervened. Further points in the history of this case are that there have never been any sensory disturbance, and the sphincters of the bladder and bowels have never been involved. The nature of this man's trouble at present seems to be quite evi- dent. The marked muscular wasting, reaction of degeneration in the 5 mnscles and bilateral character of the symptoms would separate it at once from the cerebral lesions. The random character of the paralysis, with the proximal portions of some limbs being affected more than the distal parts and, the absence of all sensory disturbance, distinguish it from multiple neuritis. We then have a spinal lesion, and as the symptoms are typical of poliomyelits, and could not be accounted for from a lesion in any other portion of the cord, we must conclude that this man suffered from poliomyelitis originally, instead of cerebro- spinal meningitis. As this case illustrates some of the difficulties of the diagnosis of the disease in its acute stage, and in its chronic condition presents two of the sequalae that are sometimes observed years after all the acute symptoms have subsided, we may spend to advantage several minutes in discussing the diagnosis of the disease in its acute and chronic conditions. In the acute variety the chief symptoms of the disease are muscu- lar paralysis and wasting, without sensory disturbance or affection of the sphincters of the bladder or bowels, but with abolition of the deep reflexes of the muscles involved. The paralysis is not symmetrical, but is random in its distribution, not infrequently the proximal por- tion of the limbs may be affected and the distal portions escaping. The most common form is paralysis of the legs, either one or both. In a number of instances the leg on one side and the arm on the opposite side are affected. The rarest form of all, is that in which the arm and leg of the same side are affected, presenting a condition of hemiplegia, without facial involvement. There is no objective sensory disturb- ance, and but little of a subjective character in the infant. In the adult and latter part of childhood pain is not infrequently complained of in the affected linibs. Most commonly they are of a dull, heavy character and are spoken of as rheumatoid. The joints are not tender, but some of the muscles, especially when the electrical changes are well marked, are tender to pressure. The faradic irritability begins to lessen from the fourth to the tenth day in the severer cases, but at the same time the increased irritability of the muscles to the galvanic cur- rent is observed, and it is only in the very lightest cases that no elec- trical changes are observed. It has not been generally recognized by many of the profession that poliomyelitis sometimes comes on in the form of an epidemic, with many symptoms, especially delirium and unconsciousness, simu- lating cerebro-spinal meningitis. One or two such epidemics have been observed in this country and a number in continental Europe. It 6 is probable that when the disease assumes an epidemic form that cere- bral complications, not infrequently seen in the sporadic variety, are often common and pronounced. It does not seem that much difficulty in diagnosticating the disease frdm cerebro-spinal meningitis should exist if the cases are carefully studied. A much more common mis- take in diagnosis and one to which I wish to direct your attention to- day especially, is mistaking poliomyelitis for some acute febrile dis- ease. Often poliomyelitis comes on suddenly, with little or no febrile manifestation. At other times the invasion may be more gradual, at- tended by a temperature of two to four degrees, or more, above normal and the fever may extend over a period of from ten days to two or three weeks. In children the fever in some respects simulates that of typhoid. As the elevated temperature may be the principal symptom to which the attention of the physician is attracted he may not ob- serve the condition of the limbs until the fever subsides, and then he is amazed to find that his little patient is paralyzed. Only a short time ago I heard a prominent physician of this city remark that polio- myelitis, in his experience, was a very common sequela of typhoid fever in children. You have only to remember that poliomyelitis may come on with fever to prevent your mistaking it for febrile conditions. The paralysis as a rule comes on early and may be observed the first two or three days of the febrile stage. You have but to observe the limbs of your patient from day to day and your attention will be at- tracted as soon as the flaccid character of the paralysis manifests itself. I have seen a number of cases of typhoid fever, but I cannot call to mind a single case of poliomyelitis that I have observed as a sequela of typhoid fever. It may occur, however, but it is less frequent than many physicians would have us believe. As the disease begins in adults with rheumatoid pains, soreness in the muscles, fever and a general malaise condition, it has not infre quently been mistaken for rheumatism. ' If paralysis occurs in acute rheumatism, it is inhibitory in character, and due to the severe pain in the joint, but the weakness is temporary in duration. In poliomyeli- tis the paralysis is flaccid, there is no involvement of the joint and the reflexes are abolished. In rheumatism paralysis is absent, the joints are especially involved, and the reflexes are unaffected or increased. The disease is distinguished from myelitis from the fact that in the latter sensory disturbance is well pronounced, and is observed, like motor paralysis, to affect the trunk muscles up to the highest level of the cord lesion, and the sphincters of the bladder and bowels are involved. The arms usually escape, except when the cervical re- gion is affected, when we usually find all the muscles of the trunk, as 7 well as those of the arms and legs paralyzed. A spinal hemorrhage coming on suddenly, especially in a child, producing paralysis without fever might simulate the symptoms produced by acute poliomyelitis coming on in a few moments time, were it not for the fact that spinal hemorrhage is attended with sensory as well as motor disturbance and the sphincter of the bladder and bowels are affected. Acute spinal meningitis should present no difficulty in the diagnosis, although I have seen one or two cases in which the mistake of pronouncing a case of spinal meningitis, poliomyelitis, has been made. The rigidity in- stead of flaccidity of the muscles, pain in the back, radiating down the limbs, and sensory disturbance all aid in making the diagnosis easy. The danger of confounding poliomyelitis with multiple neuritis is great in some cases. In a typical case of multiple neuritis the pro- nounced sensory disturbance, with the symmetrical condition of the disease, and its limitation to the distal portion of the extremities, sep- arate it widely from the symptoms of poliomyelitis. It is rather in the atypical forms of the former disease, especially in that variety in which motor symptoms are present and pronounced and sensory slight, if not almost entirely absent, that the greatest difficulty is experienced in distinguishing the peripheral from the spinal cord lesion. In multiple neuritis the symptoms are usually symmetrical and limited to the dis- tal portions of the extremities, with a more gradual onset of symptoms than is observed in poliomyelitis. The disease must be diagnosticated in its chronic stage, especially where it has existed since childhood, from spastic paraplegia and from progressive muscular atrophy. In simple spastic paraplegia great at- rophy of the muscles with the reaction of degeneration never exists. There is rigidity of the muscles with increase of the reflexes, and elec- trical changes are not observed. The marked muscular wasting, the flaccidity of certain muscles, and the electrical changes separate the disease from which this man is suffering, widely from cerebral diple- gia of childhood. The history of the patient's condition will serve, as a rule, to dis- tinguish the disease in its chrortic stage from progressive muscular at- rophy, even when the latter exists at the time of the examination. We must remember that one of the sequela of poliomyelitis is progres- sive muscular atrophy. Chronic poliomyelitis is distinguished from progressive muscular atrophy from the fact that paralysis in the for- mer precedes the wasting, whereas in the latter they occur gradually at the same time. The electrical chafiges are greater in poliomyelitis than in progressive muscular atrophy. When, however, a case of poliomyelitis several years after the initial symptoms, begins to mani- 8 fest symptoms of progressive muscular atrophy, in which the patient claims, as in the case here, that the wasting of the muscles has not increased since childhood, how are we to determine that the atrophy and weakness of the muscles are increased? This patient's condition illustrates very typically the means by which we may make this diag- nosis. In the cases of poliomyelitis which are followed by progres- sive muscular atrophy, the most wasted muscles will usually show marked electrical changes if the trophic disturbance dates back a number of years, but on the other hand if the muscular wasting has been gradual, electrical changes will be a great deal less than are found in poliomyelitis, especially when the latter has existed for ten or fifteen years. You remember that some of the muscles that are considerably wasted in this case respond well to the faradic current, while others presenting only a little greater degree of atrophy will not respond to the strongest current. • Preservation of considerable faradic irritability in muscles greatly wasted in a case of chronic poliomyelitis would be almost positive proof of progressive muscular atrophy having been added. If we needed any further proof in this case we have it in the man's statement in which he says he finds the left leg is getting weaker, so that for a period of eighteen months he has not been able to be on his feet a great deal. The chronic rheumatoid pains so common in some cases of progressive muscular atrophy are well marked in the left leg. The fibrillary tremor observed in this case is a common symptom of progressive muscular atrophy, but is not found in poliomyelitis. You remember the examination showed that all the deep reflexes of the legs are increased, even to the point ofthe development of ankle- clonus, and that some of the muscles of the arms manifested in- creased myotatic irritability. This is certainly not a symptom of po- liomyelitis, but on the contrary the affected muscles in this disease lose their normal irritability. The deep reflexes are not increased in uncomplicated cases of progressive muscular atrophy. How then, are we to explain the presence of a condition for- eign to the original disease, and absent in this sequela, progressive muscular atrophy ? The increased myotatic irritability is due to the invasion of the lateral columns of the cord by the same degenerative process that has taken place in the anterior horns of the grey matter. , We not only have a condition of progressive muscular atrophy added to the original lesion, but a lateral sclerosis has begun to develop. The intimate relations of the anterior cornua to the lateral columns of the cord is probably the reason why the degenerative process has ex- tended to these columns and not to other portions of the spinal marrow.