when Brown-Séquard, observing that certain lesions of the spinal cord
were accompanied by symptoms resembling those which he witnessed in
animals after section of one lateral half of the cord, recognized it
as a special affection. Although some of the accompanying phenomena of
such a section had likewise been observed by Stilling, Budge,
Eigenbrodt, Tuerk, Schiff, Von Bezold, and Van Kempen,[1] nevertheless
this whole group of symptoms, as belonging to the same disease, was
first clearly recognized and anatomically demonstrated by
Brown-Séquard.[2] According to this physiologist, a section or a
destruction of a small portion of a lateral half of the spinal cord in
its cervical region gives rise to the following phenomena: namely, on
the injured side is observed a paralysis of voluntary motion, of the
muscular sense, and of the blood-vessels; the latter, manifesting
itself by a greater supply of blood and a higher temperature of the
parts, may continue to exist for some years. There is, furthermore, an
increased sensibility of the trunk and extremity to touch, prick,
heat, cold, electricity, etc., owing to vaso-motor paralysis, though
in some cases a slight anæsthesia may exist in a limited zone above
the hyperæsthetic part, and also in certain parts of the arm, breast,
and neck. Besides these symptoms, vaso-motor paralysis of the
corresponding side of the face and of the eye, manifested by an
elevated temperature and sensibility, partial closure of the eyelid,
contracted pupil, slight contraction of some of the muscles of the
face, etc., may also be present. On the opposite side of the injury an
anæsthesia of all kinds of sensation, excepting the muscular sense, is
observed in both extremities; there is also an absence of motor
paralysis. The anæsthesia on this side is owing to the decussation of
the sensory nerves in the spinal cord.
[Footnote 1: Eckhard, “Physiologie des Nervensystems,” in _Handbuch
der Physiologie_, edited by L. Hermann, 2d part of vol. ii. p. 165.]
[Footnote 2: “On Spinal Hemiplegia,” _Lancet_, Nov. 7, 21, and Dec.
12, 26, 1868, reported in _Virchow and Hirsch's Jahresbericht_ for the
year 1868, vol. ii. p. 37.]
If the hemisection of the cord is made in the dorsal region, the
functional disturbances are limited to that part of the body below the
point {1166} of division, and a hemiparaplegia, or paralysis of the
corresponding lower extremity, will be the result.
From these facts it will be readily understood that a lesion occurring
in any portion of one lateral half of the spinal cord of man must be
followed by some or all of the above-mentioned symptoms, and that the
phenomena produced by physiological experiments on animals constitute,
in reality, the pathological basis of unilateral spinal paralysis in
man. They will be more clearly understood by calling to mind the
course of the musculo-motor, vaso-motor, and sensitive tracts in the
spinal cord. Thus, the musculo-motor tracts, after having descended to
the crura cerebri, cross one another in the pyramids of the medulla
oblongata and adjoining upper portion of the spinal cord, forming the
so-called decussation of the pyramids; they then descend through the
spinal cord to supply the muscles of the same side of the body.[3] A
section of one lateral half of the cord therefore causes motor
paralysis on the same side. The vaso-motor tracts remain uncrossed,
and pass, each, through one lateral half of the cord to supply the
vessels on the same side; some regions of the body are stated to make
an exception to this rule. According to Brown-Séquard, the sensitive
tracts conducting the different kinds of sensation, with the exception
of the muscular sense, on the contrary, cross over to the opposite
half of the spinal cord soon after their entrance into it, and thence
pursue their further course to the brain. A section of one lateral
half of the cord, therefore, will be followed by a loss of sensation
of touch, pain, heat, tickling, etc. on the other side of the body.
[Footnote 3: Though, in the majority of cases, a complete decussation
of the motor tracts probably takes place in the pyramids, the
researches of Flechsig have shown (_Die Leitungsbahnen im Gehirn und
Rückenmark des Menschen_, p. 273) that there are a number of others in
which the decussation is not complete, but where a part of these
tracts passes to the spinal marrow uncrossed on the inner surface of
the anterior white columns.]
The symptoms above mentioned must of course vary according to the
extent, the intensity, and the particular nature of the lesion, as
well as the height at which it is located in the spinal cord.
DEFINITION.—The chief characters of unilateral spinal disease are
motor paralysis, hemiplegia, or hemiparaplegia, paralysis of the
muscular sense and of the blood-vessels on the side of the lesion, and
paralysis of sensation with preservation of the muscular sense on the
other side of the body. These symptoms may vary, and be accompanied by
other phenomena according to the particular seat, extent, and depth of
the lesion.
SYMPTOMS.—According to the nature of the lesion, the symptoms of
unilateral disease of the spinal cord may be developed suddenly, as,
for instance, when caused by traumatic injuries; or in a gradual and
slow manner, when they may be preceded by premonitory symptoms, such
as vertigo, pain on the side of the lesion, etc. The most prominent
clinical phenomena, as before mentioned, are motor paralysis on the
side of the lesion, and anæsthesia on the opposite side of the body.
The motor paralysis on the side of the lesion may, according to the
seat of the latter, manifest itself in either the form of a hemiplegia
or hemiparaplegia, and even extend in a light form to the opposite
side of the body. In typical cases, however, in which the injury or
disease is strictly confined to one lateral half of the cord, the
motor power on the other side {1167} of the body remains entirely
undisturbed. At the same time, the muscular sense on the injured side
is paralyzed or considerably diminished, and in some cases (Fieber,
Lanzoni, Allessandrini) the electro-muscular excitability also has
been found lowered, while in others it has remained normal. There is
furthermore observed, on the side of the lesion, a vaso-motor
paralysis, manifesting itself by a greater supply of blood to, and a
higher temperature of, the paralyzed trunk and limbs, giving rise to
an increase of sensibility (hyperæsthesia) of touch, prick, heat,
cold, electricity, etc. in these parts. If the seat of the lesion is
sufficiently high up in the cord, this paralysis extends, moreover, to
the corresponding side of the face and eye, where it also causes an
elevation of temperature, increase of sensibility, partial closure of
the eyelid, contracted pupil, slight contraction of some of the
muscles of the face, etc. In a number of cases at the boundary of the
hyperæsthetic region a narrow anæsthetic zone is observed to exist on
the breast, neck, or arm. This anæsthesia is owing to the division, at
the level of the section, of some nerves of sensation on their way to
the other half of the spinal cord. An increase of the reflex
irritability of the tendons has in some cases (Erb, Schulz, Revillons)
been observed, while in one case (Glaeser) the reflex was found to be
absent. Swelling and œdema of the paralyzed limbs have also been met
with (Glaeser), and in one case (Allessandrini) even swelling and pain
in all the joints of the injured side were observed before death,
while masses of coagulated blood in these joints, particularly in the
knee, were revealed by the autopsy. The inflammatory affection of the
knee-joint of the paralyzed leg has, moreover, been observed by
Viguès, Joffroy, and Solomon.[4] Frequently, atrophy of the paralyzed
muscles takes place, especially in chronic cases. In one case (Fieber)
even atrophy of the upper extremity of the uninjured side of the body
was observed.
[Footnote 4: Erb, “Diseases of the Spinal Cord, etc.,” _Cyclopædia of
the Practice of Medicine_, edited by H. v. Ziemssen, Amer. ed.]
The most prominent symptoms observed on the side of the body opposite
to the seat of the lesion are anæsthesia of every kind of sensation,
preservation of the muscular sense, and absence of motor paralysis.
Reflex action and electro-muscular contractility generally remain
normal, though in one case (Fieber) the latter was found increased.
Although the anæsthesia of the skin generally comprises every kind of
sensation, three cases were observed (Fieber) in which the sensation
of heat remained unimpaired, while the electro-cutaneous sensibility
appeared to be lost. As a general rule, there is no vaso-motor
paralysis on the uninjured side, though in some cases (Erb,
Allessandrini) an elevation of temperature has been observed.
Besides the above symptoms, some others, less characteristic in
nature, are now and then observed in individual cases. They are
painful sensations on one or the other side, or even simultaneously on
both sides of the body, and also a feeling of constriction at the
level of the lesion (Erb). Disturbances of the functions of the bowels
or bladder are also met with, though in other cases they are absent.
PATHOLOGICAL ANATOMY.—The pathological changes taking place in the
spinal cord of patients affected with unilateral spinal paralysis must
vary in different cases according to the particular nature of the
lesion giving rise to the characteristic symptoms. In those cases
reported to {1168} have terminated by a gradual disappearance of the
symptoms with or without therapeutic interference it is very probable
that the exciting cause was a hyperæmia or a myelitis of a small
portion of one lateral half of the spinal cord, sufficiently high in
degree to impair the conducting power of the nerve-fibres passing
through it. In some cases the myelitis may lead to a degeneration of
the nerve-fibres, or even extend to the other half of the cord, and by
calling forth additional symptoms render the case more complicated. In
syphilitic cases the disease depends upon syphilitic deposits or
neoplasms in the affected portion of the spinal cord; these cases,
however, generally yield to treatment. In the same manner may
circumscribed sclerosis give rise to the disease. Another cause may be
found in the compression of the cord caused by meningeal tumors or by
the fractured portions of some of the vertebræ. Chronic disease of the
vertebral bones themselves (Pott's disease) may also, by encroaching
upon the spinal cord, become an exciting cause.
The most typical cases, however, are those depending upon traumatic
injuries, by which one lateral half of the spinal cord is forcibly
divided. These lesions resemble in nature the division of the cord in
the physiological experiments on animals, and are most frequently
caused by a stab from a knife penetrating to the cord through the
intervertebral spaces.
DIAGNOSIS.—In those cases in which the symptoms of unilateral spinal
paralysis appear soon after an external injury to the spine, it
becomes obvious that the latter is the exciting cause. In cases of a
more chronic character, in which the symptoms appear gradually, the
nature of the exciting cause can only be correctly determined by the
observation of certain collateral symptoms characteristic of such
causes as might give rise to the symptoms of the disease in question.
As regards the diagnostic symptoms of unilateral spinal paralysis
themselves, they are sufficiently characteristic to be easily
distinguished from those of other forms of hemiplegia or
hemiparaplegia. Thus, cerebral hemiplegia may be distinguished from
the disease under discussion by the sensory disturbances being either
absent or on the same side as the paralysis; furthermore, by the
one-sided paralysis of the face and of the tongue and by the affection
of various cranial nerves. The hemiplegic form of spasmodic spinal
paralysis is distinguished by the absence of sensory disturbance, etc.
Lastly, hemiplegia depending upon lesion of one side of the cauda
equina is distinguished from unilateral spinal disease by the
paralysis and anæsthesia being confined to the same side, and by
generally affecting certain nervous districts of the lower
extremities.
PROGNOSIS.—In unilateral spinal lesions the prognosis depends
obviously on the particular nature and intensity of the exciting
cause. On the whole, there are quite a number of cases reported, even
of traumatic origin, which have terminated favorably.
TREATMENT.—The treatment of unilateral spinal paralysis depends, like
the prognosis, upon the nature of the exciting cause. The principles
upon which it is to be pursued of course are the same as those upon
which the treatment of the various lesions causing the disease—such as
hyperæmia, myelitis, sclerosis, wound of the spinal cord, etc.—is
based.
{1169}
PROGRESSIVE LABIO-GLOSSO-LARYNGEAL PARALYSIS.
BY H. D. SCHMIDT, M.D.
SYNONYMS.—Chronic progressive bulbar paralysis; Progressive muscular
paralysis of the tongue, soft palate, and lips.
HISTORY.—Although the particular group of symptoms constituting this
disease must have been met with and known to the older medical
observers, they were nevertheless first recognized as a special
variety of paralysis in 1841 by Trousseau,[1] who named the affection
labio-glosso-laryngeal paralysis. But as the memorandum prepared by
this distinguished physician at the time when, in consultation with a
medical colleague, he had observed the particular symptoms of this
affection, unfortunately remained unpublished, twenty years more
elapsed before the first accurate and detailed description of the
symptoms and progressive nature of this disease under the name of
progressive muscular paralysis of the tongue, soft palate, and lips
was rendered by Duchenne. The writings of this author directed at once
the attention of other medical men to this disease, and since that
time a large number of cases have been reported and discussed,[2]
while the microscopical examination accompanying the autopsies of many
of them finally revealed that the seat of the lesion giving rise to
the phenomena of this disease was to be sought in the nervous nuclei
of the medulla oblongata. Hence at the present time the pathology of
this disease is thoroughly understood.
[Footnote 1: _Clinique médicale de l'Hôtel Dieu de Paris_, vol. ii. p.
334.]
[Footnote 2: A very considerable number of cases of this disease, and
discussions thereon, will be found reported in _Virchow and Hirsch's
Jahresbericht über die Leistungen und Fortschritte der Gesammten
Medizin_, for the years 1866-80, vol. ii., section “Krankheiten des
Nervensystems.”]
DEFINITION.—That form of labio-glosso-laryngeal paralysis to be
treated in the following pages is characterized by a progressive
paralysis and atrophy of the muscles of the tongue, lips, palate,
pharynx, and larynx, interfering in a greater or lesser degree with
the articulation of words and sounds and with the functions of
mastication and deglutition—affecting, furthermore, in the later
stages of the disease, the voice and the function of respiration. The
paralysis is caused by a progressive degeneration and atrophy of the
ganglion-cells of those nerve-centres in the medulla oblongata from
which the muscles of the above-named organs receive their supply of
nervous energy, though in most cases the pathological process extends
to, or even beyond, the roots of those nerves {1170} which originate
in these centres and terminate in the respective muscles. In many
cases the pathological process extends to the spinal marrow, and there
causes paralysis and atrophy of the muscles of the trunk, and,
generally, of the upper extremities. Almost in every case the disease,
as its name indicates, slowly progresses until it terminates in death.
There are, however, a number of cases observed which, though
exhibiting the same or similar symptoms, do not, in reality, depend
upon a progressive degeneration and atrophy of the centres and
nerve-roots of the medulla, but, on the contrary, owe their symptoms
to other causes; as, for instance, to tumors, hemorrhages, syphilitic
neoplasms, etc., which, either by pressing upon the medulla from
without, or, if situated within, by deranging in various manners the
individual nervous elements of that part, may give rise to some or
even all of the symptoms of true labio-glosso-laryngeal paralysis.
These symptoms, however, according to the character of the lesion,
may, after remaining stationary for some time, retrograde, and even
disappear, as has been observed in syphilitic cases; or they may
progress, and finally end in death. In order to distinguish these
cases from the chronic or progressive bulbar paralysis some authors
have attached the term retrogressive to this form of the disease.
SYMPTOMS.—As the degeneration of the nerve-centres in the medulla
oblongata, upon which the disease depends, does not proceed in a
regular fixed order, the order in which the clinical symptoms
successively appear also varies in different cases. In the majority of
cases, however, the symptoms appear gradually, manifesting themselves
generally in the form of a greater or lesser impediment in the
articulation of certain sounds or letters depending upon the movements
of the tongue, such as _e_, _i_, _k_, _l_, _s_, and _c_, while at the
same time a difficulty of mastication and deglutition may be
experienced by the patient, due to the progressive development of the
paralysis, which deprives the tongue of its lateral and forward
movements. To this cause also, at this period, the apparently
increased secretion of saliva, running from the corners of the mouth,
must be attributed. With intelligent patients these symptoms are
rendered less prominent by the special effort which they make to
pronounce slowly for the purpose of hiding the deficiency in their
speech. But as the disease advances the difficulty of articulation
increases on account of the paralysis extending to the orbicularis
oris, thus affecting the mobility of the lips and interfering with the
pronunciation of the labial sounds _p_, _b_, _f_, _m_, and _w_. With
the loss of power of articulation the patient's speech becomes
gradually reduced to monosyllables, or even, finally, to
incomprehensible and inarticulated grunts, by which he expresses his
wants to his friends. In consequence of the paralysis of the lips the
patient becomes unable to whistle or blow or to perform any movement
depending upon these organs, while at the same time, through the
disturbance created in the co-ordination of the facial muscles by the
paralysis of the orbicularis oris, the mouth becomes transversely
elongated and drawn downward by the action of the remaining
unparalyzed muscles upon its angles. With the mouth partially open and
the lower lip hanging down, the face of the patient has a peculiar sad
and painful expression, while the voice assumes a nasal sound on
account of the paralysis of the palate.
After a while the difficulty of deglutition, caused by the inability
of the {1171} tongue to properly assist in the formation of the bolus
of food and its propulsion into the pharynx, increases on account of
the paralysis extending to the muscles of the pharynx. The failure of
these muscles in the performance of their special function of grasping
the food and carrying it to the œsophagus obliges the patient to push
it down the pharynx with his fingers. In some cases the difficulty of
swallowing rests with solids, in others with fluids. The defective
deglutition furthermore gives rise to spells of coughing and
suffocation by portions of food getting between the epiglottis and
larynx, while the paralyzed muscles of the palate allow the fluids to
pass through the nose and enter the posterior nares.
As the case slowly proceeds the symptoms grow worse. The paralysis of
the orbicularis oris reaches a point when this muscle is no more able
to close the oral cavity; the mouth of the patient therefore remains
open. The tongue, having now entirely lost its lateral, forward, or
upward movements, rests motionless upon the floor of the mouth,
evincing no other signs of life but occasional slight muscular
twitchings. In some cases a diminution of the sense of taste, and also
of that of touch in the tongue, pharynx, and larynx, has been
observed. Atrophy of the tongue and lips now sets in, and the function
of speech is almost entirely lost. The only letter which the patient
is still able to pronounce is _a_ (broad); all other sounds are
indistinct and can hardly be understood. The paralysis of the tongue
and other muscles of deglutition gives rise, furthermore, to an
accumulation of the now excessively secreted saliva, which, being
retained in the oral cavity, assumes the form of a viscid mucous
liquid dripping from the mouth, extending, in the form of strings or
ribbons, between the surfaces of the lips. Finally, when, through the
progressive paralysis of the orbicularis oris, the patient can no more
close the lips, the flow of saliva from the mouth becomes continuous;
he is then seen engaged in the constant use of his handkerchief for
removing the secretion.
In the latter stages of the disease the pathological process extends
to the centres of respiration, paralyzing not only the muscles of
respiration, but diminishing at the same time the contractile power of
the lungs. A great difficulty of breathing—consisting not only in the
want of depth of the inspirations, but, moreover, in a feebleness of
expiration (manifested by weak, powerless coughing)—then ensues, and
the patient is no further able to blow his nose or clear his bronchial
tubes from the accumulating mucus. The paralysis of the muscles of the
larynx, also occurring at this stage, not only increases the
difficulties of deglutition, but most seriously affects the voice of
the patient by decreasing the innervation of the vocal cords; the
voice then becomes hoarse and weak, to be finally entirely lost.
The decrease in the innervation of the heart—which, lastly, also
occurs—gives rise to irregularities in the action of this organ,
followed by irregularity of the pulse, attacks of syncope, feelings of
extreme weakness, and fears of death; whilst simultaneously the
insufficient performance of the respiratory function renders the
breathing of the patient weaker and weaker. Feelings of great
oppression in the chest then arise, and the patient may die during one
of the now frequent attacks of dyspnœa and suffocation.
No fever or pain is observed during the course of this disease;
sensation {1172} remains unaffected and the mind is perfectly clear.
It is owing to these circumstances that frequently the patient walks
about and attends to the duties of life, until shortly before death he
becomes confined to bed by the great weakness caused by the want of
nutrition. The appetite also remains good, but, unfortunately, in
consequence of the difficulties attending the act of deglutition, the
patient cannot satisfy the demands of his stomach. In the latter
stages of the disease, therefore, when feeding can only be
accomplished by the aid of the stomach-tube, every attempt to relieve
the terrible gnawings and pains of hunger ends in a failure. With
starvation staring in his face, the unhappy victim of the disease
finally sinks into a state of extreme inanition, which, if life is not
suddenly extinguished by an attack of suffocation, slowly leads to
final dissolution.