muscular mass, whatever it is, is common to all cases, and that when
myalgia occurs in a healthy man after extraordinary muscular effort or
exposure to cold damp when fatigued, or in a delicate child who has
played too long, or in a poorly-fed weaver working long hours over his
loom, or in the consumptive whose cough gives him no rest, or in
connection with any chronic disease or acute disease, whether
tonsillitis or bronchitis or fever or rheumatism, it is the same
thing--the expression of muscles or groups of muscles overworked. It is
not a disease; it is not a symptom of disease. It is an accident of
many diseases--of any disease that lowers nutrition. And it is not less
an accident of health when such muscular effort is demanded as is
beyond the capacity of health.
The essential pathology of myalgia is obscure. It is not an
inflammation, as that term is generally understood, but there is ground
for the opinion that the lesions are of the nature of a subinflammatory
process within the muscle. The not uncommon instances in which an
injury or contusion--in short, traumatism--has been followed shortly
after the recovery by severe myalgia are of further value as
illustrating this theory.
The obstacles in the way of precise histological investigation in cases
of acute myalgia are so great that it seems probable that further
knowledge is to be reached for the most part by way of clinical work.
DIAGNOSIS.--The fundamental question for consideration in this place is
whether we are dealing in any given case with local manifestations of a
constitutional disease or with purely local phenomena. That the latter
is the correct view seems to the writer to admit of no further
discussion in this article. This position being assumed, and due regard
having already been paid to the differential diagnosis between myalgia
and rheumatism, neuralgia and inflammatory myositis, it seems useless
to enter upon the consideration of the diagnosis between this and other
painful affections to which it bears but slight and superficial
resemblances. Spinal irritation, hypochondriasis, locomotor ataxia,
alcoholism, syphilis, gout, and lithiasis are on the one hand attended
by pains which are clearly not myalgic in character, and on the other
hand peculiarly predispose those subject to them to this affection of
poorly-nourished and easily-overworked muscles. Each of these diseases,
however, presents a complexus of {538} symptoms in which that which is
essential and characteristic is readily to be distinguished from that
which--as myalgia--is accidental.
A few words concerning the diagnosis of some of the varieties may not
be amiss.
In pleurodynia the ordinary physical signs of pleural, pulmonary, and
cardiac disease are absent, the painful points characteristic of
intercostal neuralgia are not found, and there is little or no
constitutional disturbance.
The diagnosis of myalgia lumbalis is, as a rule, unattended by
difficulty. The muscular pain in the loins is characteristic. It is
greatly increased by efforts to rise or to turn in bed, and is
associated with diffused slight tenderness upon pressure, but never
with the acute localized soreness of neuralgia or abscess. The
practitioner must, however, guard against the danger of mistaking the
back pains of more serious affections for lumbago by the careful
examination, in all cases, of the back and abdomen, and by the
investigation of the condition of the urine. The possibility that pain
in this region may be caused by spinal meningitis, lumbar abscess from
spinal caries, sciatica, inflammatory affections of the hip-joint,
renal calculus, perinephritis, abdominal aneurism, diseases of the
pelvic viscera, and the onset of certain of the acute infectious
diseases must not be overlooked.
PROGNOSIS.--Under satisfactory conditions as regards hygiene and
treatment the prognosis is always favorable. It becomes in chronic
cases unfavorable as regards complete recovery when by reason of
poverty, unhealthy occupations, unwholesome surroundings, or
established wasting diseases the nutrition of the muscles and their
physiological rest are permanently interfered with, and the balance
between their power and work permanently deranged.
TREATMENT.--The indications are threefold: (_a_) relief of pain; (_b_)
physiological rest for the affected muscles; (_c_) restoration of the
balance between the nutrition of the muscle and the work it has to do.
(_a_) Relief of pain is often secured by rest in a posture that permits
the complete relaxation of the muscles involved. In acute cases due to
overwork pure and simple, and where complete rest is attainable, little
other treatment is required. In the course of a few hours or days the
function of the muscles is fully restored and their contractions are
performed without pain. Where, however, complete muscular relaxation is
impracticable or fails to afford relief, anodynes are necessary.
Morphine hypodermically is very useful, but this altogether
independently of any local action. Continuous dry or moist heat by
means of flannels, flaxseed poultices, spongio-piline, etc. may be
applied. Various anodyne lotions are useful. Liniments containing
aconite, belladonna, chloroform, or chloral also afford relief. The
compound belladonna liniment of the British Pharmacopoeia is especially
to be recommended. So also are plasters of belladonna, conium, and
mustard. Galvanism occasionally gives prompt relief. The same statement
may be made of the use of static electricity. The pain sometimes
disappears under gentle and long-continued massage.
(_b_) Rest is usually enforced by the intensity of the pain attending
movement. In severe cases the bed is a necessity. In affections of the
respiratory muscles, as pleurodynia, firm support of the side, by means
of {539} overlapping strips of plaster drawn from the spine downward
and forward in the direction of the ribs to the median line in front,
is sometimes necessary and always comfortable.
(_c_) The balance of nutrition is restored by rest. Local means to
further this end are such as relieve pain--heat, anodyne and
stimulating frictions, massage, and galvanism. The parts must be
protected from sudden changes in temperature by extra thicknesses of
flannel or sheets of wool or cotton batting--if necessary covered with
a piece of oiled silk or fine gum-cloth. In old cases prolonged massage
with passive movements, shampooing, and the slowly interrupted galvanic
current, alternating with rapid faradic currents, are followed by good
results.
As a constitutional measure a Dover's powder at night, followed by mild
purgation in the morning, is often indicated. Purgation is especially
called for in plethoric or gouty persons, in whom also Turkish or vapor
baths are of great service, while poorly-nourished, anæmic subjects
demand quinine, iron, lime, and cod-liver oil. If the attack linger,
full doses of ammonium chloride, and in old cases potassium iodide in
moderate doses well diluted and long continued, are advocated; and in
stubborn cases Anstie recommends deep acupuncture of the muscle near
its tendinous attachment. In cases marked by a tendency to spastic
rigidity the repeated hypodermic injection of atropine may often be
relied upon as the speediest means of cure.
Where the general nutrition is poor the local trouble is apt to be
obstinate, and often yields only to measures that restore the general
health.
{540}
PROGRESSIVE MUSCULAR ATROPHY.[1]
BY JAMES TYSON, A.M., M.D.
[Footnote 1: From the view taken by the author as to the nature of the
disease under consideration, it is evident that its proper position
would be under affections of the nervous system. But as this view has
not been established to the satisfaction of all who have studied the
disease, it seems appropriate to place it in the intermediate position
selected for it by the Editor, between muscular and nervous diseases.]
SYNONYMS.--Chronic anterior poliomyelitis; Spinal form of progressive
muscular atrophy; Adult form of progressive muscular atrophy; Wasting
palsy (Roberts); Cruveilhier's atrophy; Amyotrophia spinalis
progressiva (Erb).
DEFINITION.--Progressive muscular atrophy is a gradually progressive
wasting of a group or groups of voluntary muscles, independent of
primary functional inactivity and of local lesion to nerve or muscle.
HISTORY.--We are indebted to William Roberts[2] for the best historical
account of this disease up to the date of publication of his monograph.
Van Swieten seems to have described the first case, in 1754, but
without comment. Cooke in his work _On Palsy_,[3] published 1822,
relates a case which had been under the care of Cline--that of an
officer, first attacked in 1795. Caleb H. Parry[4] reported another
case in 1825, and Sir Charles Bell[5] three cases in 1830. Abercrombie
described a marked case in 1828,[6] Dorwall[7] three striking cases in
1831, and Herbert Mayo[8] two evident cases in 1836. In 1849, Duchenne
presented to the Institute of France his memoir on _Atrophie musculaire
avec Transformation graisseuse_. In the next year Aran published his
essay entitled _Recherches sur une Maladie non encore décide du Système
musculaire_ (_Atrophie musculaire progressive_),[9] in which he claimed
priority in description. He reported in all eleven cases, and regarded
it as a primary muscular affection. Aran's researches were very
important, and have caused his name to be intimately associated with
the disease along with that of Duchenne.
[Footnote 2: _An Essay on Wasting Palsy_, London, 1858.]
[Footnote 3: London, 1822, p 31.]
[Footnote 4: _Collected Works_, London, 1825, p. 523.]
[Footnote 5: _The Nervous System of the Human Body_, London, 1830.]
[Footnote 6: _On the Brain and Spinal Cord_, 1828, p. 419.]
[Footnote 7: _London Medical Gazette_, vol. vii., 1830-31, p. 201.]
[Footnote 8: _Outlines of Human Pathology_, London, 1836.]
[Footnote 9: _Archives générales de Méd._, t. xxiv., Sept. and Oct.,
1850.]
Cruveilhier's studies were commenced as early as 1832, but his results
were not published until March, 1853,[10] when he read his memoir
before the Academy of Medicine of Paris. He seems to have made the
first autopsy, and was much surprised at the absence of any apparent
lesion of the {541} spinal cord. So enthusiastic and so exhaustive was
his study of the disease that his name, too, has become almost
inseparably associated with it, and the term Cruveilhier's atrophy is
one of those by which it is known. He concluded from his earlier
autopsies that the lesions were solely in the muscular system, which is
progressively destroyed, while the brain and spinal cord may remain
perfectly normal. In a later case (his third), terminating January,
1853, he found atrophy of the anterior roots of the spinal nerves, and
then concluded that the disease resided "not in the muscles themselves,
but in the anterior roots of the spinal nerves." But after the
termination of his fourth case, in which an autopsy was also secured,
he placed the primary lesion in the gray matter of the cord, whence he
considered the anterior roots take their origin.
[Footnote 10: _Ibid._, May, 1853, p. 561.]
Thouvenet,[11] an interne of Cruveilhier's, published in 1851 a thesis
based on some cases collected in the Charité, and was the first to
claim that the disease resides primarily in the peripheral nerves, and
that it must be classed among rheumatic affections.
[Footnote 11: _Gaz. des Hôp._, Nos. 143 and 145, 1851.]
In December, 1851, E. Meryon[12] read a paper before the
Medico-Chirurgical Society of London entitled "Granular and Fatty
Degeneration of the Voluntary Muscles." His observations appear to have
been made quite independently of any preceding researches. He argues
that the primary morbid change is a default of nutrition in the
muscular fibres.
[Footnote 12: _Med.-Chir. Trans._, vol. xxxv. p. 73.]
Subsequently, cases were published in 1853 by Bouvier, Landry, Burg,
and Niepce in France; in 1854 by Chambers in England, Guérin and Robin
in France, Cohn, Virchow, and Betz in Germany, and by Schneevogt in
Holland; in 1855 laborious essays were published by Oppenheimer,
Wachsmuth, and Eisenmann, and cases by Hasse, Valentiner, Virchow,
Meyer, and Diemer in Germany, and Gros in France. Duchenne's work on
_Local Application of Electricity_, also published in 1855, contains
much information on the subject.
Since 1855 the reports of cases and papers on the subject have been so
numerous as to make it unprofitable to enumerate them. Among the most
notable are those of Eisenmann, published in _Canstatt's Jahresbericht_
for 1856; Roberts's classic work on _Wasting Palsy_, in 1858; the
papers of Lockhart Clarke in 1866 and 1867,[13] and of Swarzenski in
1867;[14] Kussmaul's clinical lecture[15] and Friedreich's treatise[16]
in 1873; and Eulenburg's article on "Progressive Muscular Atrophy" in
_Ziemssen's Cyclopædia of Practical Medicine_, published in German in
1875 and in English in 1877. An important case, in consequence of the
careful post-mortem study of the nervous tissues, is one recently
reported by Wood and Dercam.[17]
[Footnote 13: _Med.-Chir. Transactions_, xlix., 1866, p. 171, and l.,
1867, p. 489.]
[Footnote 14: _Die Progressive Muskelatrophie_, Berlin.]
[Footnote 15: "Ueber die fortschreidende Bulbärparalyse und ihr
Verhältniss zur progressiven Muskelatrophie." _Sammlung klinische
Vorträge_, liv.]
[Footnote 16: _Ueber progressive Muskelatrophie, über wahre und falsche
Muskelhypertrophie_, Berlin, 1873.]
[Footnote 17: _Therapeutic Gazette_, March 16, 1885.]
ETIOLOGY.--The cause of this affection in a large number of cases is
quite unknown. That hereditation plays an important part seems well
determined by numerous observations, among which may be mentioned those
of Roberts, Friedreich, Hemptenmacher, Trousseau, Meryon, {542}
Eulenburg, Sr. and Jr., Naunyn,[18] Hammond, and Osler.[19] In the Farr
family, reported by Osler, 13 individuals in two generations have been
affected, 6 females and 7 males--a larger proportion of the former than
is common in this disease. Of these 9 had died at date of publication
of paper. With the exception of two, all occurred or proved fatal after
the age of forty. Of the 10 instances in the second generation, 5 are
the offspring of males and 5 the offspring of females. The disease has
not yet appeared in the third generation, which promises between forty
and fifty individuals, several of whom are over thirty years of age.
[Footnote 18: _Berliner med. Wochenschrift_, Nos. 42 and 43, 1873.]
[Footnote 19: _Archives of Medicine_, vol. iv., No. 3, Dec., 1880.]
The over-use of the muscles involved seems to be a well-determined
cause in certain cases of true muscular atrophy. The following
interesting illustrations are given by Eulenburg:[20] Betz observed
atrophy of the side three times in the cases of smiths and saddlers,
who had to do heavy work with the right hand; Gull, in a tailor after
excessive exertion; Hammond reports a case apparently due to excessive
use of one thumb and finger in playing faro; Friedreich, one of a
dragoon who may have exhausted his left hand in holding the bridle
while riding; another in a morocco-leather worker, who used to press
hard with his left hand; and a musician who played several hours a day
on the bass viol. Schneevogt names two cases of primary atrophy of the
shoulder-muscles, especially of the deltoid of the right side--one of a
sailor who had to pump for days together on a leaking ship, and the
other of the left side in a woman who always carried her child on the
left arm while suckling it. Continued threshing and the handling of a
musket have both been followed by it in the muscles called into play by
these exercises. Roberts was able to trace the effects of over-muscular
exertion in producing the disease in 35 out of 69 cases. As a
determining cause, at least, therefore, we must admit the over-use of
muscles.
[Footnote 20: _Op. cit._]
There is reason to believe, too, that this form of atrophy is one of
the consequences of senility--that the tendency to connective-tissue
overgrowth which characterizes old age operates to produce, in a way to
be presently explained, an atrophy of groups of muscles. In a woman
aged seventy, now under my care, the fingers of both hands are
clawed--became so inappreciably almost, and the condition is still
increasing.
In addition to the above-named causes, long-continued exposure to cold,
and especially to the action of very cold water, has been named.
Traumatic influences, such as injuries to nerve and muscle, have been
called upon to account for localized and progressive atrophy, but these
are excluded by our definition from the category of true progressive
muscular atrophy.
Cases have also occurred in the course of convalescence. Typhoid fever,
rheumatism, measles, scarlet fever, cold during salivation,
vaccination, childbed, excessive venery, syphilis,--have all been held
responsible for a certain number of cases.
AGE AND SEX.--In examining the literature of acute muscular atrophy it
is found that cases are reported at all ages. Thus, Wachsmuth, quoted
by Eulenburg, found among 49 cases 13 under the age of fifteen, 8 from
fifteen to twenty, 22 from twenty to fifty, and only 6 over fifty
years. On the other hand, Roberts--who, following Aran, divides the
disease into the general form and partial form--says the latter very
rarely falls on {543} individuals under adult age or over fifty, while
the average age of the instances of the partial form studied by him was
thirty-two years and four months. In 10 instances of the general form
the patients were under twelve, and 2 more are reported as children; 1
was said to be sixty-nine and another fifty-four, the average being
twenty-eight years and three months. Of Eulenburg's own cases, 7
acquired the disease before the age of ten, 6 before the twentieth
year, 2 before the thirtieth, 8 before the fortieth, 5 before the
fiftieth, and none later. The latter observer also finds that whenever
the disease is hereditary it occurs earlier, usually before the close
of the twentieth year. This was certainly not the case in the Farr
family, reported by Osler.
I am inclined to believe, especially in the light of Charcot's[21] and
of Erb's[22] recent studies, that the true spinal form of progressive
muscular atrophy is a disease of adult life, and that the majority of
cases reported as occurring in early life are instances either of what
Erb calls the juvenile form of progressive muscular atrophy or of
pseudo-hypertrophic paralysis.
[Footnote 21: "Revision nosographique des Atrophies musculaires
progressive," _Le Progrès méd._, No. 10, 1885, i. 314-335.]
[Footnote 22: "Ueber die Juvenile Form der Progressive Muskelatrophie
und ihre Beziehungen zur sogenannten Pseudohypertrophie," _Deutsches
Archiv für klin. Med._, xxxiv. 1884, S. 467.]
As to sex, males predominate. Thus, according to Friedreich's
statistics, out of 176 cases but 33 were females, or about 19 per cent.
Of Roberts's collection of 99, 84 were males and 15 females. Of 28
cases noted by Eulenburg, 17 were in men and 11 women. This is
doubtless owing to the fact that men are subjected to the causes of the
disease more than women. For Roberts early noted that women who engage
in needlework, washing, and household service are apparently not less
liable than men similarly employed, and he found that of those whose
labor did not press excessively on any particular sets of muscles
females formed even a majority of cases.
Some singular freaks of selection have presented themselves in the
matter of sex, particularly in the cases which have been ascribed to
hereditation. Thus it will sometimes attack only the male members of a
family. A remarkable instance of this was observed by Meryon, in which
four sons were attacked and six daughters remained unaffected; and,
again, two boys were attacked and two sisters escaped. This may occur
also independent of hereditation. Occasionally the reverse takes place,
the sisters only being attacked, while the brothers escape.
PATHOLOGICAL ANATOMY AND HISTOLOGY.--Two principal seats of change have
been found to exist in connection with progressive muscular atrophy.
The first and easiest recognized is, of course, the alteration in
muscles; the second, that in the nervous system.
The muscular change is simple, and affords a typical instance of what
is known as numerical atrophy. The muscular fasciculi one after another
undergo fatty metamorphosis, succeeded by absorption of the resulting
fat and substitution of connective tissue. The rate of atrophy varies,
but sooner or later the muscle is more or less substituted by fibrous
bands and cords, over which may be traced reddish lines which represent
muscular tissue in a normal state.
The rationale of these changes has not been always the same. The {544}
older observers regarded them as the result of a primary fatty
metamorphosis of muscular fasciculi, followed by absorption of the
resulting fat. Later it was asserted that the atrophy is secondary to a
myositis or inflammation of muscle, beginning as a hyperplasia of the
interstitial connective tissue in its finest ramifications between the
single primitive fibrils. Along with this are seen the results of
irritation in the primitive bundles themselves, shown by swelling and
multiplication of the muscular corpuscles, proliferation of their
nuclei, and sometimes cloudy swelling. Even hypertrophied muscular
fasciculi and dichotomous and trichotomous subdivision have been noted
by Friedreich.
It sometimes happens that the hyperplastic process in the intermuscular
connective tissue is succeeded by a fatty infiltration of the cells of
the connective tissue, and there results a lipomatosis which is
invariably outside of the muscular fasciculi and between them. This
gives rise to an appearance of hypertrophy which is only apparent, for
the muscular fasciculi are themselves wasted, and proportionally
paralytic. This is seen to occur particularly in the muscles of the
calves of the legs, in which is produced an appearance identical with
that in the disease known as pseudo-hypertrophic muscular paralysis,
with which, indeed, the condition under consideration is considered by
some identical. But although we must admit in certain cases a
complication of a certain degree of lipomatosis with progressive
muscular atrophy, the two diseases are essentially different; and it is
quite likely that in some instances pseudo-hypertrophic muscular
paralysis has been mistaken for progressive muscular atrophy.
The changes in the nervous system are not nearly so simple. They have
been noted in the peripheral nerves, both in their trunks and in their
intermuscular branches; in the anterior roots of the spinal nerves; and
in different parts of the spinal cord, including the central gray
matter, the antero-lateral and posterior columns; also in the
sympathetic system. These nerve-changes are not simultaneous, nor have
they been discovered in every case. It is a noteworthy fact, however,
that as methods of examination have improved and the manipulative skill
of observers has increased the number of negative cases has diminished.
First, as to alterations in peripheral nerves in their ultimate
distribution: The character of these is of a kind usually described as
irritative; that is, there is a hyperplastic process in the
connective-tissue sheaths (neurilemmæ) and their internal
prolongations, consisting in nuclear proliferation and thickening of
the tubular membrane or sheath of Schwann. Varicose distortion of the
medullary sheaths and their subsequent disappearance, together with
destruction of the axis-cylinders, also occurs.
The changes in the peripheral nerve-trunks, as studied in the median,
ulnar, radial, and musculo-spinal, are essentially the same, resulting
in thinning of the diameters of the nerves. These changes, however, are
by no means constant.