TWO CASES OF PROGRESSIVE MUSCULAR DYSTROPHY


In this month of October the parents decided to take her to Johns Hopkins as the oracle of last resort. She stayed there in hospital for a week of observation and testing. Then the parents were given a letter signed by two heads of clinics stating the diagnosis, progressive muscular dystrophy, the prognosis, “no known cure,” a few years to live, not beyond ten years of age probably during which time she would become helpless and progressively weaker.


Since cases of progressive muscular dystrophy are rare, it is interesting to have two in one long practice. The first was reported to the I.H.A. in the early 1930s; it is sketched again here for purposes of comparison.

M.C. was four and a half years old when I first saw her in March, 1931. Up to about that time she had seemed to her parents a healthy child but her inheritance was bad. On the paternal side a great-grandmother had had tuberculosis, a grandmother cancer of the uterus, a grandfather died of spinal meningitis after hard drinking for many years. On the maternal side a great grandmother had had a paralytic stroke and a grandmother several strokes, an uncle died from a stroke, a great aunt had “leaky” heart valves, an aunt tuberculosis, an uncle paralysis from drinking jamaica ginger. The outlook from the point of view of heredity certainly was bad.

A year before I saw her, this child had been walking to her sisters school in the morning and again at noon, each way nine- tenths of a mile, making four miles five days a week. Also she took dancing lessons at that time.

At four and a half she was losing weight steadily, losing appetite, had increasingly poor color tending to be bluish in tint, was giving up play more and more to sit in on the sidelines, was developing fear of anything unusual. On physical examination hollows were noted where muscles ought to be, with a white nodular appearance of the skin over the hollow places. She would stumble and fall easily, would get down on her knees like an old person, would pull herself up with her arms, was very slow dressing and undressing. She had slight fever in spells.

I gave her Sulph. 10M in March and Calc. c. at the end of June. At that time her abdomen had become large and hard; she had noisy flatulence; her legs were looking like sticks. After the Calc., vitality and appetite improved, but the atrophy was progressive. By July she was apt to fall prone and strike her forehead. By October she was pulling herself upstairs with her hands on the banisters or hands on knees. She could not get up off the floor alone, could not get into a chair, had to be lifted, but she took more interest in life and in other children.

In this month of October the parents decided to take her to Johns Hopkins as the oracle of last resort. She stayed there in hospital for a week of observation and testing. Then the parents were given a letter signed by two heads of clinics stating the diagnosis, progressive muscular dystrophy, the prognosis, “no known cure,” a few years to live, not beyond ten years of age probably during which time she would become helpless and progressively weaker. So the despairing father and mother brought their child home and in a few days the father committed suicide because he could not face the future. This did not help matters any for the mother was left with two little children and a heavily mortgaged house.

I could think of only two remedies for this case, Calc. c. and Phos. I had her on one from June to November and on the other from November to January, 1932. Each one produced some improvement in strength, appetite and mental vigor, but neither produced a permanent gain or stopped the progress of the disease. By November it had become almost impossible for her to turn over in bed.

Then, on January 21st, I gave her Calc. phos. 10M and by February 17th, she looked decidedly better, had begun to gain weight. The muscles were really harder, more filled out, and she could handle herself slightly better.

From this time on changes for the better were steady and remarkable. In April she could run, picking feet up off the floor. In May she could walk and run normally. By August she could do almost anything other children could do, could go upstairs one foot over the other without holding the railing, could skip on one foot.

The next summer (1933) she was on a farm, climbing fences, excelling in games, called the tomboy of the group.

She has had no trouble with muscles since. She is tall, erect, wiry, with reddish hair and freckles. Tubercular inheritance showed for a time in frequent colds and coughs, also in enlarged lymphatics. Psoric tendencies were expressed in hay-fever for a few years in the spring and in eczema on back and limbs. Sycotic taint showed in large warts and huge callouses on one heel. Dysmenorrhoea is present too.

She looks in blooming health, however. She has been an office worker the last few years and was happily married in April this year.

Last January I received a letter from a lady in Florida whose husband has a large oil plantation. The title son of the Plantation manager began to be clumsy on his feet as soon as he began to walk. Progress from then on was similar to that of the case reported above, except slower, so that at five and a half years he had more muscular power than she had had at four and a half years.

The lady writing to me sent a description of the case to her friend, Dr. Lucy Swanton Clark, after the body had been taken to a child specialist who pronounced it progressive muscular dystrophy and said, “no known cure.” Dr. Clark responded that she knew just one doctor who had cured a case and referred her to me. As a result I flew down to see the child, watched him carefully for two days, saw his maternal grandparents as well as his parents and his two sisters. Then I promised to do my best and returned to my office to study the problem further.

In this case, also, heredity was very bad. In fact there were five cases of this disease in the five generations preceding this little F.E., all transmitted in the female line and all the victims males, dying with it in childhood or early adolescence. The victim in the next generation before F.E. began symptoms, as he did, on beginning to walk.

F.E. had a younger sister who died at four months with a tumor of the brain.

The patient had fractured a clavicle three times simply by falling out of bed. At two years he had an eruption on neck, upper chest and back, “like flea bites,” itching which lasted three weeks. I was told he was fat and husky up to the last year.

I found a thin, rather wasted boy of medium height and dark complexion with pale, rather waxy skin; a sensitive child,already conscious that he is not like other boys and shrinking from their company. He is naturally a stay-at-home, quiet and precocious, fond of books and pictures, of mechanical toys with which he is clever. I was told he learns extremely easily and has a remarkable memory, repeating bits of the conservation of his elders two weeks after he has heard it and teaching an older sister the whole of a verse she is trying to learn. He thinks things over and then asks questions about them.

He is affectionate, thoughtful of others, with a serious outlook on life, a rare but sunny smile. When younger he shrank, from other children and animals as if afraid of them. Hearing is acute. Myopia is pronounced, for he bends his head way down over a book. Perspiration is free all over, except hands and feet, with a sour odor on face and scalp. He is warm blooded, requiring very light clothing. He takes cold easily, mostly a dry, harsh cough. Teeth are poor. Walked at thirteen months.

During the last year, besides losing flesh and strength, his very good color has left him. Walking had been progressively unsteady until, when I saw him, he almost pitched from one side to other. LIke the first case he pulled himself upstairs with one hand reaching forward on the banisters and the other pushing on his other knee. He had great difficulty getting up from sitting cross-legged on the floor. He could climb into a chair, however. He could not jump at all, falling down when he tried. He could not pull the trigger of his toy pistol with one hand but must reinforce it with the other. Nocturnal enuresis returned after two years without it and has lasted for two years. The muscles did not seem so wasted as those of the first case but some of this was visible along the spine.

Naturally, as I watched this boy, I could not help comparing this case with the earlier one; they were so much alike. The boy had several characteristics of Calc. phos. that were lacking in the girl, causing a stronger temptation to prescribe this remedy on the spot. But I did not do it. I came home and made a thorough study of the case, sending Calc. phos. 10M on February 6th. The first report two weeks later began with the opinion of all the family that the child was better, stronger, with better color and much more appetite; more interested in life; more self confidence.

By April 8th, improvement had stopped and he began losing ground slightly. Another dose of Calc. phos. was sent him then. Improvement followed in two days and continued steadily. He was taken to the child specialist who had said, “no known cure.” This doctor tested the body, pronounced him very much better and wanted to know what I was giving him. I sent word to him through the family that I would be very glad to tell him all about it if he would write to me. I learned later that he said he would write after seeing the patient on his return from seeing me.

Julia M. Green