the converse of those just mentioned, and they are all present when
the part is seriously involved. The patient will then have a marked
contraction of the pupil, which no longer responds to light or to
irritation of the skin of the neck, but may change slightly in the act
of accommodation. It resists the action of mydriatics. The vessels of
the choroid and retina may be dilated, as well as those of the iris,
in which case the patient will feel a sense of weariness on any
long-continued attempt to use the eyes. There is no actual disturbance
of vision, and the cornea is not usually flattened, as was formerly
supposed. There is a noticeable narrowing of the palpebral fissure,
the upper lid falling slightly as in a mild state of ptosis, and the
lower lid being slightly elevated. This is due to the paralysis of the
muscles of Müller in the eyelids, which are controlled by the
sympathetic. It is present in 90 per cent. of the recorded cases, and
in many the apparent size of the eye is reduced a half. Retraction of
the eyeball is a less constant symptom, and one which develops only
after the disease has existed some time. It is due partly to the
paralysis of the orbital muscle of Müller, and partly to the decrease
in the amount of fat in the orbit behind the eye. A marked symptom,
and one which is constant, is a dilatation of the vessels of the face,
conjunctiva, nasal mucous membrane, ear, and scalp. This is attended
by redness, a subjective sense of heat, and an actual rise of local
temperature, which may exceed that of the other side by 1.5° F.,
measured in the auditory meatus or nose. This vascular congestion has
persisted in some cases for three years. In others it has been
followed much earlier (in nine months) by a partial or complete return
to the normal condition, and {1265} even when the local temperature
remains higher on the affected side, the visible congestion and the
sensation of heat may have disappeared. The dilatation, succeeded by
the contraction (normal tone), of the vessels has led to a division of
the disease into two stages, and in a few cases the affected side has
become paler than the other in the second stage. In both stages the
part affected is less sensitive to changes in the external
temperature.
An increased secretion of tears and of perspiration has been supposed
to accompany dilatation of the vessels of the skin of the head
inevitably. This is not a constant symptom, as the recent cases have
demonstrated. And no definite statement of the effect of disease of
the cervical sympathetic on the occurrence of dryness or dampness of
the face can be made, both conditions having been observed. A
difference between the degree of moisture on the two sides of the face
on exposure to heat is usually present. Palpitation of the heart has
been an annoying symptom to the patient in many cases, and is usually
associated with a marked slowing of the pulse. This was reduced from
74 to 66 in Möbius' case,[63] and remained slow for some weeks. The
frequency of the heart may, however, be increased after the first
period of slowing, but never reaches a very high rate (88 in the case
cited). A slight atrophy of the affected side of the face has been
observed in several cases, appearing after the disease has existed for
some time. The muscles of the cheek feel flabby and are slightly
sunken; but the condition does not approach in severity true facial
hemiatrophy, nor is it sufficiently rapid to be considered due to a
trophic disturbance. Changes in the secretion of saliva, dryness of
the nasal mucous membrane, and symptoms referable to paralysis of the
intracranial vessels, such as might be expected from the result of
physiological division of the sympathetic, have only been occasionally
observed. Glycosuria has been noted in a few cases.[64]
[Footnote 63: _Berlin. klin. Woch._, 1884, No. 16.]
[Footnote 64: Gerhardt, _Volkmann's Sammlung klin. Vorträge_, No. 209,
“Ueber Angioneurosen,” p. 11.]
COURSE.—The course of the disease has been divided into two stages, as
already mentioned, the majority of the symptoms remaining permanently
from the onset. The second stage is characterized by the cessation of
the dilatation of the vessels, by the appearance of retraction of the
eyeball, and by the development of slight facial atrophy. In the cases
where the sympathetic is extensively destroyed by the lesion no
recovery is possible. When it is simply divided by a wound there has
been a considerable degree of recovery, probably due to a spontaneous
union of the divided ends and re-establishment of the function. From
these facts the prognosis can be deduced.
DIAGNOSIS.—The symptoms are so characteristic that there is no
difficulty in reaching a diagnosis. The most important point in any
case is to determine the cause, care being taken to consider all the
possibilities already mentioned in discussing the causation. The
symptoms of lesion are always unilateral.
TREATMENT.—If the cause can be removed, an indication for treatment is
afforded. Sources of reflex irritation are to be eliminated. If the
sympathetic has been divided by a wound, it may be well to unite the
cut ends, as in suture of other nerves, although this has not yet been
attempted; otherwise there is little hope from any method of
treatment. {1266} Electricity has been applied in vain, and
galvanization of the sympathetic in the neck is now regarded by all
good authorities as useless.
Diseases of the thoracic and abdominal sympathetic ganglia and cords
have been suspected, but nothing definite is known of their symptoms
or pathology; the statements which have recently been made regarding
visceral neurosis not being based upon any cases in which post-mortem
lesions were found.
Trophic Neuroses.
TROPHIC NERVES AND NERVOUS CENTRES.—The nutrition of the body depends
upon the nutrition of the individual cells of which it is made up.
Each cell has the power of appropriating from the blood such
substances as will preserve its existence, enable it to perform its
functions, and produce a successor. Whether this power is inherent in
the cell or is controlled by the nervous system is a question upon
which authorities are divided. Those who hold the first position deny
the existence of trophic nervous centres and of trophic nerves from
those centres to the organs and elements of the body, claiming that
this hypothetical trophic system has not been demonstrated
anatomically, and that the facts urged in its support are capable of
another interpretation. Those who believe in the existence of a
trophic system have been able to demonstrate the existence of fine
peripheral nerve-fibres passing to and ending in individual cells of
the skin, glands, and other organs,[65] and have brought forward a
large collection of facts which merit a careful examination.[66] They
are as follows:
[Footnote 65: _Bericht der Section für Dermatologie, Versammlung
Deutscher aerzte_, Strasburg, 1885; _Vierteljahrschrift für
Dermatologie und Syphilis_, 1885, 4 Heft, S. 683.]
[Footnote 66: “Tropho-neurosen,” _Real Cyclopædie f. d. gesammt.
Medicin_, vol. xiv., 1883; Erb, _Ziemssen's Cyclopædia_, xi. pp.
408-423.]
ATROPHY.—When a nerve is cut certain changes occur in it which are
known as Wallerian degeneration.[67] This affects the peripheral end
of a severed nerve, the peripheral end of a severed anterior
nerve-root, and the central end of a posterior nerve-root. To maintain
its integrity a motor nerve must be in direct continuity with a normal
cell of the anterior cornu of the spinal cord; a sensory nerve must be
in connection with the intervertebral spinal ganglion on the posterior
nerve-root. Nerves which pass between two such ganglia do not
degenerate when cut. The degeneration consists[68] in a coagulation of
the myelin in the medullary sheath, a fatty degeneration of the
coagulum, and a gradual absorption of the débris. The axis-cylinder is
compressed, and finally disintegrated, by a mass of protoplasm which
develops about the nuclei of the interannular segments, and after
undergoing fatty degeneration its débris becomes mingled with that of
the myelin, and is also absorbed. The sheath of Schwann, whose nuclei
have in the mean time increased by a process of subdivision, is partly
filled by the protoplasm (from which the new axis-cylinder develops if
regeneration occurs), remaining as a fine {1267} thread of connective
tissue when all other traces of the nerve-fibre have disappeared.
There may be a proliferation of cells of the endo- and perineurium at
the same time which aids in the transformation of the nerve into a
connective-tissue strand. This process of degeneration involves the
terminal plates by which the nerves join the muscles, but the
terminations of the sensory nerves—_i.e._ tactile corpuscles—do not
appear to be affected. The central end of the cut nerve may display a
similar change for a distance not greater than one centimeter; it
usually develops a bulbous swelling of connective tissue, and retains
its conducting power indefinitely.
[Footnote 67: Waller, _Philosoph. Transactions_, 1850, ii. p. 423;
_Comptes rendus de l'Acad. de Sci._, 1852-55.]
[Footnote 68: Ranvier, _Leçons sur l'Histologie de Système nerveux_,
Paris, 1878; Von Recklinghausen, _Pathologie der Ernahrung_, 1883.]
Degeneration in the tracts of the spinal cord occurs after various
forms of lesion,[69] and is similar in its processes to degeneration
in the peripheral nerves. The increase in the connective-tissue
elements is more noticeable in contrast with the parts unaffected, and
from the density of the tract involved the result has been called
sclerosis. The recent researches of Homen[70] have shown that the
process of degeneration begins in the entire length of the affected
tract, and does not proceed from the point of lesion onward, as was
formerly supposed.
[Footnote 69: See Vol. V., “Myelitis—The Secondary Scleroses,” p. 892;
Schültze, _Arch. für Psych._, xiv. 2.]
[Footnote 70: _Fortschritte der Medicin_, 1885, No. 9.]
When a muscle is separated from its connection with the central
nervous system, either by a division of the nerve passing to it or by
a destruction of the cells in the anterior cornu of the spinal cord
from which that nerve arises, it undergoes an atrophy which is
peculiar in being immediate and rapidly progressive, thus contrasting
strongly with the gradual and slighter atrophy from disuse in cases of
cerebral paralysis where the cells mentioned and the nerve-fibres are
intact. There is at first a simple diminution in the number of the
fibrillæ of which the muscular fibre is made up, together with an
increase in the interstitial connective tissue nuclei. Then an
albuminoid and fatty degeneration of the muscular elements occurs,
with a proliferation of muscle-corpuscles or nuclei, and a gradual
absorption of the débris. The interstitial connective tissue then
increases rapidly, forming fibrous bands through the degenerated
muscle which compress the few muscular fibres remaining, until as a
result the muscle is transformed into a mere ribbon of connective
tissue without any power of contractility.[71] As these changes go on
the electrical reactions change, the three degrees of reaction of
degeneration corresponding to the three stages of atrophy
described.[72] These phenomena of nerve- and muscle-degeneration are
observed in traumatic or idiopathic neuritis, in acute and chronic
poliomyelitis anterior, in general myelitis involving the anterior
cornua, and in bulbar paralysis.
[Footnote 71: Hayem, G., _Recherches sur l'Anatomie pathologique des
Atrophies musculaires_, Paris, 1873; Ross, _Diseases of the Nervous
System_, vol. i. p. 238.]
[Footnote 72: See Vol. V., “Electric Reactions.”]
The influence of the nervous system on the nutrition of the bones has
also been ascertained. When a bone is developing, a lesion of the
nerve to it, or of the deeper portion of the anterior cornua of the
spinal cord from which these nerves arise, will modify and partly
arrest its growth. This is often seen in anterior poliomyelitis and in
hemiatrophy of the face occurring in children. In the adult a no less
marked effect is produced, although the results are less noticeable. A
condition known as {1268} osteoporosis is caused, consisting of an
enlargement of the Haversian canals and an infiltration of fatty
matter into them and an actual decrease in all the inorganic
constituents of the bone, which loses in weight, becomes thinner and
more fragile, so that spontaneous fractures may occur.[73] This
condition has been noticed more frequently in diseases of the spinal
cord than in neuritis; it is said to occur in locomotor ataxia. It has
been found in a few cases of long-standing hemiplegia and also in
dementia paralytica, no explanation of its pathogeny in these
instances, however, being offered. In a case of ataxia with a lesion
in the medulla which involved the nuclei of the fifth, ninth, tenth,
and eleventh nerves on one side, all the teeth of the upper jaw on
that side fell out within a few weeks, those in the lower jaw
remaining.[74] Changes in the nutrition of the bones have also been
recorded in cases of progressive muscular atrophy in the paralyzed
limbs.[75]
[Footnote 73: Weir Mitchell, _Amer. Journ. of the Med. Sci._, 1873, p.
113; Charcot, _Arch. de Phys._, 1874, p. 166.]
[Footnote 74: Demange, _Rev. de Médecine_, 1882, p. 247.]
[Footnote 75: Friedreich, _Progressive Muskelatrophie_, p. 347, 1873.]
The condition of the skin and its appendages is influenced decidedly
by changes in the nervous system, either in the nerves, in the spinal
ganglia, or in the central gray matter. Here it is the sensory nerves
which convey the trophic influence, not the motor nerves, as in the
cases hitherto considered; and when the lesion producing trophic
changes in the skin is central, it is situated in the posterior cornua
of the spinal cord or in the gray matter near the central canal. The
glossy skin seen on the fingers after injuries to the nerves is a type
of such atrophy from disturbance of trophic impulses. Glossy fingers
present a smooth, shining appearance, are dry from the diminution in
the secretion of sweat, feel soft and satin-like to the touch from the
marked thinning of the skin, and frequently show a defective or
irregular growth of the nails, which may be ridged, curved, or
deformed.[76] They are red and mottled from accompanying vaso-motor
paralysis, and are usually hot and painful. Changes in the
pigmentation of the skin and hair are recorded as a not infrequent
accompaniment of severe neuralgia and as a result of great mental
anxiety. Thus in several cases of supraorbital neuralgia the eyebrow
on the affected side has turned white; in infraorbital neuralgia the
beard has become gray; and in both the hair has been observed to fall
out.[77] The sudden turning white of the hair is ascribed to a
swelling of the hair by air within it.[78] In one case, frequently
cited, the hair and nails fell out after a stroke of lightning.
[Footnote 76: Weir Mitchell, _Injuries of Nerves_. See also Vol. IV.
p. 683.]
[Footnote 77: Seeligmüller, _Lehrbuch der Krankheiten d. Peripheren
Nerven_, p. 157, 1882.]
[Footnote 78: _Arch. f. Path. Anat._, xxxv. 5, 575, Landois.]
When a gland is cut off from its nervous connection with the cord or
cerebral axis by section of its nerves, its function is impaired and
its nutrition suffers, so that after a time it loses weight and
undergoes a progressive total atrophy. This has been proven
experimentally in animals in the submaxillary gland. It has been
observed in the testicle in man after division of the spermatic nerve
(Nélaton) and after destruction of the spinal cord by traumatic and
idiopathic myelitis (Klebs, Föster).[79] The sweat-glands are known to
be under the control of a central {1269} nervous mechanism, as cases
of hyperidrosis, anidrosis, and chromiodrosis prove;[80] and an
atrophy of them and of the sebaceous glands has been observed[81]
after nervous lesions.
[Footnote 79: Cited by Samuel, _Realcyclop., loc. cit._ See also
Obolensky, _Centralblatt für med. Wissen._, 1867, 5, 497.]
[Footnote 80: See Vol. IV. pp. 583-586.]
[Footnote 81: See Vol. IV. pp. 683 _et seq._]
Progressive hemiatrophy of the face is treated elsewhere. The
following case of progressive hemiatrophy of the entire body may be
mentioned here: A boy, aged fourteen, dislocated his ankle, which in a
few days became swollen, red, hot, and painful. The inflammation
extended up the leg, but did not involve the knee, and soon subsided.
After a short time the foot began to atrophy. The atrophy extended up
the leg, and involved the thigh; it then progressed to the trunk and
the arm, and lastly to the face on the affected side, until in the
course of two years there had developed a unilateral atrophy of the
entire body. Muscles, fat, and bones were all affected, but no
difference in the skin or hair of the two sides was noticed.
Fibrillary tremors were present in the muscles. The electric reactions
were not altered, but were gradually lost. There was a
hypersensitiveness to touch and to cold, but no other sensory
disturbance. The boy was alive and fairly well when the case was
reported.[82] It is unique.
[Footnote 82: Heuschen, _Schmidt's Jahrbuch._, vol. cxcviii. p. 130.]
These various instances of atrophy cannot be ascribed to simple
disuse, since they differ markedly in their pathological changes and
in the rapidity of their progress from such atrophy. Nor are they to
be referred to vaso-motor disturbances, since in many cases no
vascular changes are evident. Their distribution in the body often
corresponds exactly with that of peripheral nerves, and they accompany
nerve lesions too frequently to be explained on any theory of
coincidence. There are many authorities, however, who refuse to
ascribe them to a lesion of trophic nerves.[83] In regard to the
degeneration of nerves it is said that each nerve axis-cylinder is a
part of the nerve-cell from which it arises, and hence destruction of
the cell or division of the cylinder, by disturbing the unity of
existence, results in the death of the part. The fibre shares all the
changes of nutrition which the nerve-cell undergoes, and if separated
from it necessarily perishes. To this it is replied that trophic paths
and motor paths are distinct at some points in their course, at least
in the central nervous system, since each can be affected alone. Erb,
who has studied this subject carefully,[84] believes that trophic are
distinct from motor centres in the spinal cord, but that both impulses
may be conveyed by the same axis-cylinder in the peripheral nerves—a
middle ground which is widely accepted. It is now known that each
axis-cylinder is made up of several fibrils, so that this theory gains
probability. This would also explain the occurrence of atrophy in the
muscles, the trophic centres being affected when the muscle atrophies,
and unaffected when it is paralyzed without atrophy. Mayer, however,
denies this explanation of the muscular atrophy, holding that the
motor system, cell, nerve, and muscle-fibre, forms a nutritive as well
as functional unit, and that the simple suspension of function, by
interfering with the special {1270} conditions of nutrition attendant
upon physiological excitement, is competent to cause a pathological
change. To this it is replied that the parts of the motor system are
not interdependent, since disease of the muscle does not produce
degeneration of the nerve and of the cell, and the fact of a
degeneration in a peripheral direction alone is evidence of central
trophic influence. The attempt to ascribe trophic changes in the skin,
nails, and hair to vaso-motor disturbance has been equally
unsuccessful in covering all the observed cases.
[Footnote 83: See Handfield Jones, _St. George's Hospital Reports_,
1868, vol. iii. pp. 89-110; Sigmund Meyer, _Hermann's Handbuch d.
Physiol._, ii. Th. 2, “Trophische Nerven,” 1879; Gowers, _Diseases of
the Brain_, 1885, p. 4.]
[Footnote 84: _Arch. f. Psych._, v. S. 445, 1875; also _Ziemssen's
Cyclo._, vol. xiii. p. 117 (Amer. trans.); also _Deut. Arch. f. klin.
Med._, v. S. 54.]
[Illustration: FIG. 58. Diagram of the Arrangement and Connection of
Motor and Trophic Centres and Fibres in the Spinal Cord and Motor
Nerve (after Erb): _a_, motor fibre of spinal cord from the brain to
_d_, the motor cell, which is joined to the muscle _m_ by the motor
nerve; _b_, trophic cell in the spinal cord for the muscle, to which
it is joined by the trophic fibre _bʹ_; _c_, trophic cell in the
spinal cord for the motor nerve, to which it is joined by the trophic
fibre _cʹ_; _s_, a fibre bringing sensory (reflex) impulses to the
cell.
If _d_ is destroyed, the fibres from _b_ and _c_ perish with it, and
the result is paralysis and atrophy of the muscle and degeneration in
the motor nerve—_e.g._ poliomyelitis anterior. If _b_ is destroyed,
the muscle atrophies, and paralysis is a secondary result—_e.g._
progressive muscular atrophy. If _c_ is destroyed, the nerve
degenerates, and paralysis and atrophy of the muscle are secondary
results—_e.g._ neuritis with reaction of degeneration. If _a_ is
destroyed, voluntary power is lost, but reflex power remains, and no
atrophic changes occur—_e.g._ lateral sclerosis. If the motor nerve is
cut between _d_ and _m_, the result is the same beyond the division as
when the motor cell is destroyed.]
{1271} HYPERTROPHY.—Trophic changes are not limited to the process of
atrophy. There are conditions of hypertrophy of supposed nervous
origin. Samuel considers the hypertrophy of one testicle which attends
atrophy of the other from section of its nerve as an example of this.
It has been ascertained that one kidney hypertrophies when the other
is atrophied or extirpated. When the spleen is removed the lymphatic
glands increase in size. But these facts are capable of another
explanation—viz. that increased demand upon the organ leads to its
increased growth. Hypertrophy of the skin and of the tongue is seen in
cretins in contrast with the deformity of the body and atrophy of the
limbs. A hemihypertrophy of the face has been noticed in several
cases, the counterpart of hemiatrophy; and in one case a unilateral
hypertrophy of the entire body was observed.[85] The local thickening
of the skin known as ichthyosis hystrix, and other hypertrophies of
the skin, certain deposits of pigment, and vitiligo, have been
ascribed to nervous causes. Mitchell has recorded cases of abnormal
growth of the nails and hair after injuries to the nerves, and similar
phenomena have followed central lesions. He has also described a
thickening of the skin of the first three fingers and of the back of
the hand following a wound of the brachial plexus. These conditions of
the skin and its appendages indicate an abnormal activity in the cells
of the affected part, a rapid metabolism and reproduction, resulting
in an undue production of tissue, apparently dependent on nervous
impulses reaching the cells from a distance. The insane ear may be
mentioned in this connection as a trophic disturbance due to central
lesion.
[Footnote 85: Ziel, _Virchow's Archiv_, xci., S. 92.]
MYXŒDEMA (cachexie pachydermique) is a disturbance of nutritive
processes characterized by a production of mucin, which is deposited
in all the tissues of the body, but especially in the subdermal
connective tissue.[86] It is considered by the majority of authors a
trophic neurosis, and is therefore considered here.
[Footnote 86: Sir William W. Gull, “On a Cretinoid State supervening
in Adult Life,” _Trans. Clin. Soc. London_, 1874, vol. vii. p. 170;
Ord, “On Myxœdema,” _Med.-Chir. Trans._, 1878, vol. lxi. p. 57;
Mahomed, _Lancet_, 1881, ii. No. 26; Hadden, _Brain_, 1882; W. A.
Hammond, _Neurological Contributions_, 1881, i. p. 36; Ballet,
_Archives de Neurologie_, 1881, vol. iii. p. 30; _Schmidt's
Jahrbucher_, vol. clxxxix. p. 30, and cxcviii. 264; “The Brown
Lectures,” Victor Horseley, _Lancet_, Jan., 1886.]
PATHOLOGY.—In the few autopsies which have been made an increase in
the connective tissue of all the organs has been found, in the meshes
of which a thick, transparent, slimy substance (called animal gum),
consisting of mucin, is present. This may compress and destroy the
parenchyma of the organs involved. There is also found a thickening of
the coats (adventitia and media) of the vessels. An atrophy of the
thyroid gland has occurred in every case, and experimental extirpation
of the thyroid in animals produces symptoms so nearly identical with
those of myxœdema that this is considered the chief pathological
feature of the disease. Whether this atrophy is due to a compression
by the mucin deposited, or is due to a disease of the trophic centres
of the thyroid in the medulla, or is a primary affection of the gland,
remains to be determined.
ETIOLOGY.—The actual causation is unknown. Cold and mental shock have
been considered exciting causes in some cases. Women are much more
liable to the disease than men, and it develops after the age of forty
in the majority of cases. It may occur in childhood and result in a
{1272} cretinoid state. Syphilis and tuberculosis do not appear to be
etiological factors.