1879 The Lancet  
2 unable to stand, with contracted ankles and wasted limbs, There is no other instance of the disease in the family. Before showing you the second patient I should like tc recall to your recollection the two cases I showed you a few months ago. They were, as you may remember, twc brothers, named S-, aged respectively four and seven years. I show you now their photographs. That of the youngest, Harvey S- (Fig. 1 ), would not suggest to you FIG. 1. FIG. 2. the idea of disease. There is no obvious
more » ... There is no obvious muscular wasting or enlargement, and yet, as you may remember, his movements were greatly impaired. He could only just succeed in rising from the floor, getting first his toes upon the ground, then placing first one hand upon his knee, then the other, and so working himself up. The other boy, William S-, aged seven (Fig. 2) , presents, as his photograph indicates, very distinct enlargement of the calves. His thighs are small, the back thin, hollow in the lumbar region, the angles of the scapulse prominent, the muscles of the upper limbs thin, except the deltoids, which are rather large. The pectoral muscles are normal, but the clavicular part of the sterno-mastoid is scarcely to be detected. You will remember his shuffling gait, and that, although he had less difficulty in rising from the ground than his younger brother, and could do so without putting his hands on his knees, yet his tendency was to do this, unless prevented. I would draw your attention to this fact, that in a slight but undoubted case of the disease the patient exhibited merely a tendency to this action, being able to rise without it if he tried. The eldest brother, Arthur S-, aged twelve, presented a few years back the same difficulty in rising and the same enlargement of the calves. He is now unable to stand, his calves are still large, but they are contracted so that the heels are drawn up and the feet are in a line with the legs. The muscles of his arms and back are very thin, and possess but little power. Besides these three brothers, a fourth, aged three years, presents a difficulty in movement, which reminds his mother only too forcibly of the early symptoms presented by his brothers who are affected. Besides these children there are two other brothers-one aged ten, quite healthy, and one aged four, who is also believed to be perfectly healthy, although he is a twin-brother of Harvey, whose symptoms are, for his age, so marked. This does not, however, exhaust the remarkable history of the family. The father and mother are healthy. The mother had four sisters and one brother. The latter had large calves, which ultimately wasted, and he died at the age of fifteen. One of the sisters suffered from similar symptoms, and also died at the age of fifteen. The other three sisters were healthy ; they married, and their children have presented no evidence of the disease. Thus we have evidence of six cases in the family. The other patient whom I have to show you is a lad, fifteen years of age, who, as you see, has almost reached man's stature, being about 5 ft. 3 in.-in length rather than in height, for he can no longer stand, and presents a painful extremity of helplessness. Except "weak knees," nosymptoms were noticed till he was seven years of age, when difficulty in getting up attracted attention, and this has. since steadily increased. Two years ago he was able to stand. His arms were thin and weak, and there was marked atrophy of the muscles of the back. The calves were unduly large: right llg in., left 11 in. in circumference. He lost the power of standing at Christmas last. Now the extreme wasting and weakness of the back at once arrest attention. As he sits his lumbar spine is extremely curved, but in the opposite direction to that observed in the other patients when standing, the convexity being backwards. There is a slight rotation of the spine, and the wasting of the extensors is so great that you can feel the anterior surfaces of the transverse processes and even the bodies of thevertebrse on the left side. This curve is solely dependent on the muscular weakness, and disappears if the boy is raised by the arms. His latissimi, rhomboids, trapezii, and serrati are almost gone, and the consequent mobility of thescapulae is extreme. The lower parts of the greater pectorals, are gone, and even the upper parts are thin. The deltoids. are of moderate size, but their power is greatly impaired. The arm muscles, however, are, although thin, not extremely wasted. Those of the upper arm are weak, but those of the hand and forearm possess fair power. The right thigh isthin, but in the left the vasti are distinctly enlarged in their lower parts. The knees can be flexed or extended, but with little force. The muscles moving the hip-joint are extremely weak. The calves are not now large or hard ; they possessmoderate power, and are not much contracted; the anklejoint can be flexed to a right ankle. On the left side of theneck and trunk there are some peculiar spots of warty growth. They are not, however, increasing, and probably, cannot be associated with the disease. (To be continued.) Clinical Remarks ON THE
doi:10.1016/s0140-6736(02)47620-9 fatcat:jbaib3w5vbh77azipwsdtahyju