constitutional symptoms are less prominent than the local ones. In
some cases there are noticed a certain degree of mental disturbance, a
condition of depression with a tendency to sigh and cry without cause,
disturbed sleep with unpleasant dreams, irritability, and headache. A
loss of appetite and disorders of digestion may follow, and then the
local symptoms appear. In other cases, which seem to be the majority,
the local condition develops without any such premonitory disturbances
of the nervous and digestive systems, although these may ensue. The
local symptoms first noticed may be paræsthesiæ or pain in all the
extremities, usually limited to the tips of the fingers and the toes.
These are continuous and severe, and are immediately followed (and
occasionally preceded) by an appearance of ischæmia or of cyanosis or
of erythema, in the order of frequency named.
(1) The fingers may look pale and dead, presenting the appearance of
the so-called digiti mortui, and may be cold, painful, and anæsthetic.
If this condition is moderate in degree, a certain amount of blood
will continue to flow through the contracted arterioles, and then it
corresponds to the description given by Raynaud of syncope locale. If
it is extreme, the part may be wholly deprived of arterial blood, and
then a true local asphyxia is present. In this stage the patients
usually suffer considerably, although some do not complain of pain
until the next stage. The ischæmia is attended with an impairment of
sensation to touch, temperature, and pain, and finer motions become
clumsy on account of the subjective numbness and actual anæsthesia. At
the same time, the fingers look shrunken, the skin being thrown into
folds, as if the hand had been soaked in hot water, or they may appear
as if frozen, the skin being hard and immovable.[59] The secretion of
perspiration may be increased, and the fingers feel damp as well as
cold, or it may be suspended. The local temperature is lowered. If the
part is cut, little or no blood will flow. At this stage the arterial
spasm may suddenly relax and the part return gradually to its normal
condition, the cessation of the constriction of the arteries and the
return of blood being usually accompanied by burning pain, which may
last for some hours. The duration of such an attack may vary from a
few moments to several days. If it continues longer, this stage is
usually succeeded by the second stage, of cyanosis.
[Footnote 59: Finlayson, _Medical Chronicle_, 1885, No. 4.]
(2) The stage of cyanosis results from one of two conditions: either
the arterial spasm is so complete that no blood passes into the part,
in {1259} which case venous blood from lack of vis a tergo or in
response to gravitation regurgitates into the capillaries, distending
them and producing a state of blueness; or a venous spasm occurs,
preventing the exit of blood from the part, which then becomes
actively congested, and the blood in the capillaries, from want of
renewal, soon becomes venous and produces the cyanotic appearance. The
stage of ischæmia may be so short that it is hardly noticed, so that
the patient's attention is first attracted by the swollen, blue, and
extremely painful condition. The skin may be stretched, the tissue
infiltrated with products of exudation, which can be pressed out, as
can also the venous blood, and the surface may itch as well as be
painful. Anæsthesia is rarely present in this stage, and there may
even be hyperæsthesia. The part is cool from the increased radiation
of heat and cessation of the processes of metabolism, the local
temperature being lowered. The small vessels on the surface will be
visibly injected, and capillary ecchymoses may rarely be seen. There
is less liability to difficulty in movement in this stage than in the
former one, as the sensations of the part are not benumbed, but if
present it is due to the swelling. This condition, like that in the
former stage, may cease suddenly, the recovery of the normal
appearance being, as a rule, slower than after a simple ischæmia. The
duration of this stage has varied from a few seconds to several days.
It is usually followed by gangrene.
(3) The condition of local erythema is described here because it may
lead to gangrene, and has therefore been considered by Weiss as one of
the early stages of the disease. As a rule, however, it is not
followed by the death of the part, and the affection in these cases is
probably one of erythromelalgia rather than of symmetrical gangrene.
Like the stage of ischæmia, the stage of erythema may appear suddenly.
The part presents a bright-red or a mottled appearance—is hot and
swollen, and painful. The vessels are visibly injected, the local
temperature is raised, the secretion of sweat may or may not be
increased, and the patient feels a burning sensation rather than pain.
Hyperæsthesia to touch and temperature and pain is usually present, or
the sensations are normal. The blood can be pressed out, but returns
immediately. In this condition of hyperæmia slight injuries lead
frequently to an inflammatory process, ulcers may form in the pulps of
the fingers or around the nails, and the eschars may appear dark and
even gangrenous; or an actual condition of gangrene may appear in the
tips of the fingers, the exact method of its occurrence being a matter
of dispute. The erythematous condition is much more likely to be
permanent than are the other stages of the disease—another fact which
has led to some hesitation in considering it a true stage. This
condition of erythema may be due to a paralysis of the
vaso-constrictors, the converse of the spasm occurring in ischæmia. It
has also been ascribed to an irritation of the vaso-dilators; and this
appears to be the more probable hypothesis.
(4) The stage of gangrene is always preceded by that of cyanosis, and
the death of the tissue is due to the arrest of nutrition consequent
upon a stasis of the blood. It is not necessary to invoke the injury
of trophic nerves to explain its appearance. In the tips of the
cyanotic fingers, on their palmar surface, beneath the epidermis, a
small blister appears, filled with a dark serous fluid or with pus or
blood. This soon ruptures, and a dark dry scab forms, beneath which an
ulceration may go on destroying {1260} the corium, but not penetrating
deeper. In the majority of cases the gangrene is limited to a small
area of the pulps of the fingers, and only involves the superficial
layers of the corium. The gangrenous spot is surrounded by a purple
margin. When the sphacelus has separated a scar remains which is
frequently insensitive. In other cases when the sphacelus is thrown
off it leaves a deep ulcer, which may look as if the lost tissue had
been cut out with a punch, and this gradually granulates and heals. In
still other cases the entire skin of the terminal phalanx may become
black and dry, presenting a true gangrenous appearance. Then a line of
demarcation is formed, usually at the junction of the terminal with
the middle phalanx; separation of the gangrenous part occurs, and a
stump is left covered with thin, glossy skin. This extensive gangrene,
involving an entire phalanx, is the exception rather than the rule.
While the gangrenous process is in progress in the tip of the finger
the nails cease to grow, and may become bulbous and rigid; the
epidermis elsewhere may become dry and desquamate, and ulceration
around the root of the nail may take place. With the completion of the
stage of gangrene, which may last from one to five weeks according to
its extent, the local symptoms terminate.
It is the symmetrical distribution of the local symptoms just
enumerated which is the peculiar characteristic of the disease. The
fingers of both hands, the toes of both feet, symmetrically situated
spots upon the back, trunk, thighs, legs, forearms, and arms (in the
order of frequency named), are affected either singly or in
combination. In the majority of cases fingers and toes are affected
together, and a few spots are seen on the trunk. In many cases the
toes escape. In a large number of cases the face has been affected,
spots of cyanosis appearing on the nose or ears or lips. As a rule,
the stage of gangrene only ensues in the tips of the extremities, but
a few cases are recorded in which little areas of skin elsewhere have
passed through all the stages of the disease. Pigmentation occurs in
spots upon the body when the process does not go on to gangrene.
Among the rare symptoms which have occurred in some cases are great
impairment of temperature, pain, and electric sensations in the
affected extremities; swelling, pain, redness about, and effusion
into, the joints; considerable loss of motion in the muscles of the
hands and feet, with diminution of electric excitability, but no
qualitative change; and oculo-pupillary changes ascribed to an
irritation of the cervical sympathetic fibres at their origin in the
spinal cord.
In addition to the constitutional symptoms mentioned, which may usher
in the disease and may continue during its course, there have been
observed temporary albuminuria, glycosuria, and hæmaturia. Fever never
occurs as a symptom of the disease, and if present must be ascribed to
some other condition. The special senses have been affected in a few
cases. In one case a spastic contraction of the retinal arteries
alternated with attacks of ischæmia in the extremities.[60] The
intellect is usually unaffected, but Weiss observed a case in which
transient aphasia occurred, which he attributes to local spasm in the
arteries of the cortex. The patient could find words only after long
thought, and spoke slowly and with difficulty.
[Footnote 60: Raynaud, _Arch. gén. de méd._, 1874, p. 11; Galezowski
examined the discs.]
{1261} COURSE AND DURATION.—The onset of the disease is sudden. The
symptoms in the first two stages may last only for a few minutes and
pass off, or the disease may pass through all the stages and terminate
in gangrene. It is usual for the first stage of ischæmia to last
several days, varying in severity; for the second stage to last
several days; and for the stage of gangrene to occupy about three
weeks. The shortest duration of a single attack has been ten days, the
longest five months. If the gangrene begins simultaneously in all the
fingers, the duration will be shorter than if it proceeds to one after
another. In one-third of the cases a recurrence of the disease within
a year of the first attack has been observed, and it is probable that
the proportion would have been larger had all the patients been kept
under observation. In some cases three and four attacks have succeeded
each other with some rapidity, some of the attacks being much less
severe and shorter than others. In some cases the condition of
gangrene has developed only in one out of three attacks. When the
condition is one of local erythema the duration may be indefinite, the
state becoming chronic and lasting for several years.
NATURE.—The nature of the disease is a matter of deduction from the
study of the symptoms, no autopsies having as yet been made. As
already stated, the symptoms are explained on the theory of a
vaso-constrictor irritation in the stages of ischæmia and cyanosis—of
a vaso-dilator irritation in the stage of erythema. Whether this
irritation is the direct result of abnormal processes going on in the
vaso-motor centres in the spinal cord, or is the reflex result of
irritation arising elsewhere, is undetermined. Raynaud held that it
must be of central origin, since in his cases galvanization of the
spinal cord modified the arterial spasm. The latter observation has
not been confirmed by other observers. Weiss believes that the
condition may occur in response to irritation arising in the skin, in
the viscera, or in the brain, and thus prefers the theory of reflex
origin. This theory is adopted by several observers, who find a source
for such irritation in the female genital organs in their cases.
ETIOLOGY.—The disease occurs in adult life, only two cases having been
observed in persons fifty years old. It is most frequent between the
ages of fifteen and thirty, although children and adults beyond the
age of thirty are about equally liable. Females are more liable to it
than males, four-fifths of the recorded cases having been in women. It
occurs more frequently in the winter months, exposure to cold being a
common exciting cause. Other exciting causes are nervous exhaustion,
especially occurring in those who are predisposed to nervous diseases
by heredity; general weakness from anæmia, malnutrition, or the
occurrence of acute fever or exhausting disease; and mental agitation,
a fright having preceded the attack in several cases. In women
menstrual disorders and uterine disease have been considered as
etiological factors. Occupation has something to do with its
occurrence, since washerwomen, waitresses, and chambermaids are the
class most often affected. In many cases, however, no cause of local
irritation can be found.
DIAGNOSIS.—The diagnosis rests upon the development of vaso-motor
symptoms in the extremities, situated symmetrically, going on to
gangrene, in a person not afflicted with cardiac disease or with
endarteritis of any kind, and not having been exposed to frost-bite or
ergot-poisoning. The age of the patient, the symmetrical position of
the symptoms, the {1262} persistence of the pulse in the main
arteries, and the limitation of the gangrene to the tips of the
extremities distinguish it readily from senile gangrene. The history
of the case, the absence of itching, and the presence of pain during
the arterial spasm which passes off when the spasm ceases, serve to
separate it clearly from chilblains. Congenital cyanosis is produced
by cardiac anomalies, and the entire body is affected. Ergot-poisoning
can be ascertained by the history.
PROGNOSIS.—Life is not endangered by this disease, no fatal cases
having been recorded. Recovery from an attack is certain, but the
duration cannot be stated, as it will depend in any case on the
character, the extent, and the severity of the symptoms. The
possibility of a recurrence of the attack should be stated to the
patient.
TREATMENT.—The methods of treatment have varied, and none are wholly
satisfactory. If the causes can be met—_e.g._ anæmia, nervous
exhaustion—they should be treated. If not, the disease itself may be
attacked by means of electricity. Or the symptoms may be treated as
they demand it. Electricity has been used by almost all observers. The
faradic current produces an aggravation of all the symptoms except in
the stage of erythema, and has been discarded. The galvanic current
may be employed in several ways. Two methods are in use. In the first
the positive pole is applied over the cervical region, and the
negative pole over the lumbar region, a descending current being thus
sent through the spinal cord. The current should be of moderate
strength, not above twenty-five milliamperes, few patients being able
to endure the strength implied in Raynaud's statement that he used
sixty-four cells of a Daniel battery. The duration of the application
should be ten minutes, and the electricity may be applied once daily.
In the second method the anode is applied over the brachial or lumbar
plexus, as the case may be, and the cathode passed over the affected
extremity, the current being constant and care being taken not to
break it suddenly. The strength, duration, and frequency should be the
same as in the first method. From these two methods, separately or
combined, Raynaud claimed to have seen favorable results. His
assertions have not been confirmed by other observers who have
followed his directions closely, and hence considerable doubt at
present prevails as to the efficacy of the electric current. The
so-called electrical application to the cervical sympathetic is
certainly useless. In the stage of erythema a very weak faradic
current applied to the hands in a bath may be of service.
Many observers have found that the progress of the case to recovery
was quite rapid if the limb were put at rest in an elevated position,
were kept warm by cotton batting or similar bandaging, and were kept
clean with antiseptic lotions when the stage of gangrene set in.
Massage is to be used in all cases, the limbs or affected parts being
gently rubbed with the dry hand or with aromatic liniments or oils.
All local injury, however, and especially counter-irritation, are to
be carefully avoided. General tonic treatment, especially iron and
cod-liver oil, is to be used in all cases.
The pain occurring in the early stages is often so severe as to
require the use of opium or other narcotics. And when the nervous
symptoms are especially aggravated, and irritability and insomnia give
the patient discomfort, bromide and chloral may be employed.
{1263} Diseases of the Cervical Sympathetic.
ETIOLOGY.—Diseases of the cervical sympathetic ganglia or cord may be
of two kinds—either irritative or destructive.[61] They are produced
by pressure upon the cervical ganglia or upon the sympathetic cord
between these ganglia, by tumors, especially aneurisms, and enlarged
glands; by abscesses; and by cicatrices of old wounds in the neck.
They are also due to extension of inflammation from a thickened pleura
in phthisis and chronic pleuritis of the apex. They may be caused by
injuries, such as stab-wounds, gunshot wounds, etc. Any disease which
produces marked irritation of peripheral branches of the sympathetic
in the neck, or of the cerebro-spinal cervical nerves, may cause
reflex phenomena resembling the symptoms of actual disease. From such
phenomena it is not justifiable to conclude that the sympathetic cord
and ganglia are the seat of lesions, and the only cases which will be
considered here are those in which actual disease was proven to be
present by an autopsy.
[Footnote 61: Ogle, _Medico-Chirurgical Transactions_, xli. 397-440,
1858, 27 cases; Poiteau, “Le Nerf sympathetique,” _Thèse de Paris_,
1869, 19 cases; Eulenburg and Guttmann, _Die Pathologie der
Sympathicus_, 1873; Nicati, _Le Paralysie du Nerf
sympathique-cervicale_, 1873, 25 cases; Seeligmüller, _Inaug.
Dissertation_, 1876; Mitchell, _Injuries of Nerves_; Mobius,
“Pathologie der Sympathicus,” _Berlin. klin. Woch._, 1884, Nos.
15-19.]
Inasmuch as the cervical sympathetic is in close anatomical connection
with the spinal cord, especially with the eighth cervical to the
second dorsal segments (the so-called cilio-spinal centre of Budge),
and as the functions of the sympathetic are dependent upon the
integrity of the spinal cord, it is evident that any lesion of the
nerves uniting it with the cord, or any lesion in the cord itself at
the levels mentioned, may produce symptoms which resemble closely
those of disease of the sympathetic. Thus, cervical pachymeningitis,
myelitis (especially from injury of the cord, or hæmato-myelia), and
diseases of the cervical vertebræ which produce either or both
conditions, may cause a train of symptoms somewhat similar to those to
be described.[62] A careful distinction must be made between primary
and secondary disease of the sympathetic, between reflex and direct
symptoms, between lesions in its substance and lesions in its
governing centres in the spinal cord. The symptoms produced by
affections of a reflex or central nature are rarely as numerous as
those of disease of the sympathetic itself. An example of such a
secondary affection is the combination of sympathetic symptoms
occurring in progressive muscular atrophy. And, finally, since mental
action of an emotional nature may cause flushing or pallor of the
face, with profuse sweating and variations in the size of the pupil
and prominence of the eyeballs, as well as palpitation or arrest of
the heart, there is reason to believe that symptoms of sympathetic
disease may be produced by cerebral lesions.
[Footnote 62: Ross, _Diseases of the Nervous System_, 2d ed., i.
686-688.]
PATHOLOGY.—The pathological anatomy of the cervical sympathetic is
obscure. This is probably owing to the fact that the ganglia are
rarely examined, and pathologists have not been familiar with their
histology. Lesions of the cervical sympathetic have been described in
almost every imaginable form of disease, and at one time, when many
obscure conditions were blindly termed sympathetic, the records were
filled with descriptions of fatty degeneration or interstitial
inflammation or pigment deposit in the ganglia. As no actual symptoms
of disease of the cervical {1264} sympathetic, as now understood, were
present in such cases, it is impossible to believe that the lesion was
other than hypothetical.
The conditions which have been observed in a few carefully-studied
cases of primary disease have been—(1) A parenchymatous inflammation
of the cells of the ganglia, attended by swelling, loss of nuclei,
granular and fatty degeneration, and by atrophy, together with a
degeneration of the fibres issuing from the cells. (2) A sclerotic
process in the connective tissue in and about the ganglia and in the
nerves, resulting in such an increase in the interstitial tissue as to
compress and injure the cells and axis-cylinders. These may be
observed together in the later stages of the disease. (3) In a number
of cases the capillaries within and about the ganglia have been found
dilated, tortuous, and varicose, and hemorrhages from them are not
rare.
SYMPTOMS.—The symptoms of irritation of the cervical sympathetic are
dilatation of the pupil, widening of the palpebral fissure, protrusion
of the eyeball, pallor of the entire side of the face and head, with
slight fall of local temperature and possibly an increased secretion
of perspiration, and an increased frequency of the heart. It is rarely
that these are all observed in any case, dilatation of the pupil with
slight pallor and rapid pulse being the only signs of irritation as a
rule. Such irritation is a less common occurrence than might be
supposed, many lesions which produce pressure even of a slight degree
on the sympathetic having caused symptoms of a suspension of its
function rather than of an increased activity. This is doubtless due
to the non-medullated structure of the fibres, which thus lack
protection from injury.