Among more inconstant symptoms, denominated vaso-motor, are, in the early stages, fever and slight elevation in local temperature from 2° to 3° C. Fever is less frequently observed toward the termination of the disease, and at this stage a fall of local temperature, as much as 4° C., has been noted. In the same category of vaso-motor symptoms are classed the skin contractions already referred to, hyperidrosis or excessive sweating, and certain very rare oculo-pupillary symptoms, consisting mainly of contraction of the pupil and slow reaction, but including also, in a case reported by Voisin, flattening of the cornea on both sides and defective sight.
COMPLICATIONS.—Progressive muscular atrophy is not infrequently associated with amyotrophic antero-lateral sclerosis and with labio-glossal or progressive bulbar paralysis. Both affections may result as an extension of the disease from the anterior cornua of gray matter, the former into the antero-lateral columns, the latter into the medulla oblongata, or the affection may be primary in either of these two situations, and extend thence into the anterior cornua of gray matter.
When there is also lateral sclerosis, there is rigidity of the lower limbs in addition to the atrophy of the upper—at first temporary, but afterward permanent. This may extend to the upper also, and the arms become fixed in semipronation and semiflexion.
When there is bulbar paralysis there is difficulty in moving the tongue, in speaking, and in swallowing. The mouth remains open, the lower lip drops, the patient cannot whistle or kiss or blow out a candle; he speaks through his nose. On the other hand, the upper part of the face is natural, the orbicularis palpebrarum muscle and occipito-frontalis acting well. As a consequence, the carrying of the food back into the oesophagus is rendered difficult or impossible; swallowing is imperfectly successful; the food sometimes enters the larynx, and the patient dies of suffocation. The saliva dribbles from the mouth. Later, respiration is embarrassed, and performed principally by the diaphragm; there is difficulty in raising mucus, and if bronchitis supervenes the patient dies of suffocation, because he cannot raise the phlegm. Such was the death of Prosper Lecompte, the historic patient of Cruveilhier.
DIAGNOSIS.—As our knowledge of progressive muscular atrophy increases we realize more and more that there have heretofore been included under this name many cases which must now be relegated to other categories. If we confine the disease, as I think we must, to those cases in which there are degenerative changes in the anterior cornua of the gray matter of the cord, we must endeavor to associate with these lesions a set of symptoms which are sufficiently constant, and exclude all other similar combinations. Such a set of symptoms includes the following: insidious and progressive atrophy of groups of muscles, beginning usually in the hand or shoulder, from which, however, it may extend to others in a diffuse and rarer form of the disease. The atrophy is accompanied by a corresponding loss of power in the affected muscles and partial or complete reaction of degeneration in the same, and by fibrillar twitchings. Along with this, sensibility, the special senses, the reflexes, as a rule, and sphincters always remain normal.
This complex of symptoms is to be distinguished from the so-called juvenile progressive muscular atrophy of Erb, and from pseudo-hypertrophic muscular paralysis. In the first there is also slow, symmetrical, but intermittent and often stationary, wasting and weakness of certain groups of muscles, preferably those encircling the shoulder and upper arm, the pelvis and upper thigh and back—"an atrophy," says Erb, "which is very frequently combined with true or false muscular hypertrophy, with a peculiar toughness of the atrophying muscles, but without fibrillar contraction or any trace of the reaction of degeneration or other lesion in the body, be it of the nervous system, organs of sense, vegetative organs, or external integuments."27 The average age in the juvenile form is much less, Erb's cases ranging from seven to forty-six, or an average of twenty-six and a half, while in the spinal form, or true progressive muscular atrophy, although the age is reduced by reason of the admixture of other cases than those of true progressive muscular atrophy, the average age is much greater. Of Roberts's cases, all of which seem true cases, the youngest was twenty, while the age of the remaining four was thirty-nine, forty-seven, sixty-seven, and thirty-eight.
27 "Juvenile Form der Progressive Muskelatrophie," Deutsches Archiv für klinische Medizin, Bd. xxxiv., 1884, S. 471.
There are certain symptoms in common in progressive muscular atrophy, as heretofore described, and pseudo-hypertrophic paralysis; and I have already said that Friedreich and others are disposed to consider them one and the same disease; but such is not the case. First of all, while there is wasting of muscle, although obscured in the lower extremities by the fatty infiltration, and while there is loss of power, there are in pseudo-hypertrophic paralysis absolutely no alterations in the spinal cord. Pseudo-hypertrophic paralysis always begins in the lower extremities, while progressive muscular atrophy begins for the most part in the upper. Pseudo-hypertrophic paralysis is a disease of childhood, and strikingly hereditary; and while progressive muscular atrophy in its broadest application is also a disease of childhood and hereditary, it is much less so than pseudo-hypertrophic paralysis; and if, with Erb, we separate the juvenile form from muscular atrophy, progressive muscular atrophy is not a disease of childhood, while heredity is almost entirely removed from it.28
28 It cannot but help the reader to get a correct notion of this interesting but still somewhat imperfectly understood disease to be familiar with Erb's formulated conclusions (loc. cit., p. 510):