columns and of the posterior nerve roots. Microscopically the posterior
columns are seen to be the seat of sclerosis, their normal structure having been
replaced by connective tissue. The same condition obtains in the posterior
nerve roots, a degenerative neuritis may involve the sensory fibres of the
larger nerve trunks and the finer sensory nerves may contain degenerated
fibres. The process usually begins in the lateral zone of the column of Bvu -
dach and in the column of Lissauer at about the level of the second and third
lumbar segments, thence it spreads through the middle zone of Burdach's
column to the column of Goll and finally to the posterior zone of the column
of Burdach. The disease at the same time is extending upward, and al-
though the process may take years, eventually the entire area of the posterior
columns is involved, together with the posterior nerve roots, the posterior
horns of the cord and the medullated fibres of Clarke's column. Ultimately
there may be changes in the brain itself.
Symptoms. These are best classified according to the time of their inci-
dence, the disease developing in three more or less distinct stages.
a. The Stage of Pain. Most of the symptoms of this stage are sensory.
There are various paraesthesiae, such as numbness and tingling of the feet,
a sensation as if cotton were being trodden upon, sensations of burning or of
cold and pruritus over various areas, notably the scrotum and at the anus.
Pain is an early and prominent symptom and may continue throughout the
course of the disease. It usually begins in the thighs or lower legs and extends
as the disease progresses. In certain cases it may be slight or wholly absent,
particularly when the onset of the disease is characterized by optic nerve
atrophy. The pain is sharp, lightning-like, lancinating, cutting or boring in
character, often beginning on the outside and anterior surface of the thigh
and later extending to the feet, and usually appears in repeated paroxysms
lasting from a second or two to half a minute, or it may occur in attacks lasting,
without remission, for hours or days. There is no tenderness. There may
be aching pain in the back and loins, or girdle sensations about the legs,
ascending to the body as the disease involves higher levels of the cord. The
patellar reflex is lost (Westphal's symptom) ; spinal myosis (contracted pupil)
LOCOMOTOR ATAXIA. 747
may be present; the pupil reacts in accommodation but not to light (Argyll-
Robertson pupil); the control of the bladder may be partly lost; this symptom
progresses until complete incontinence results. Sexual impotence is not
b. The Stage of Ataxia. The symptoms of the first stage persist and in
addition disturbances of sensation appear. Sensitiveness to pain is lessened
and delayed, a pin prick may not be felt until after a perceptible interval,
or hyperalgesias may be present in rare instances, pain being caused by even
the touch of a finger. The temperature sense is disturbed, the patient becom-
ing very susceptible to cold, while heat is felt less than normal; ultimately
the limbs become wholly anaesthetic.
The muscular sense is impaired, the position of the body in space is not
correctly perceived, the patient consequently walks unsteadily, especially
in the dark when sight is unable to assist the muscular sense, and is unable to
accurately touch objects when his eyes are shut; for instance he has difficulty in
placing his finger upon the tip of his nose when blindfolded; finer move-
ments of the hands and fingers are performed inaccurately; difficulty is experi-
enced in picking up a pin or other small object, in buttoning the clothes and
in writing. The gait is characteristically ataxic, the patient walks with the
legs widely separated and the body poorly balanced, he lifts the feet too high
and plants them too forcibly, bringing down the heel with a stamp, the steps
are irregular in length, he staggers and cannot walk in a straight line, especi-
ally if the eyes are closed. When the feet are placed closely together and
the patient stands with eyes closed, he reels and may fall (Romberg's symp-
Various ophthalmic disturbances may occur, such as optic nerve atrophy,
with resulting blindness, ocular nerve paralyses with consequent ptosis and
strabismus, convergent with contracted pupil when the sixth nerve is affected,
divergent with dilated pupil with involvement of the third; unilateral deafness
may develop due to auditory nerve atrophy. The other cranial nerves may
During this stage the so-called "crises" may appear. These are probably
due to a complicating pneumogastric neuritis and are of various types. The
gastric crisis is the most frequent and is characterized by sudden severe
pain and vomiting which may persist for several hours or even a day or twoj
intestinal and rectal crises are associated with pain, diarrhoeal tenesmus or a sen-
sation of a foreign body in the rectum; laryngeal crises are characterized by sud-
den cough, stridor, glottic spasm and dyspnoea; cardiac crises resembling attacks
of angina pectoris, and vaginal crises, characterized by pain and copious
mucous discharge, have also been observed. Trophic disorders, evidenced
by the so-called " Charcot joint," an affection probably having its origin in a
traumatism which is disregarded by the patient because of disturbance of his
748 DISEASES or the nervous system.
pain sense, and which is characterized by entire absence of pain, great swelling
and copious effusion, occur. The knee is most frequently affected but the
condition may involve the ankle, elbow or WTist. The ligaments, cartilage
and bone are gradually destroyed and spontaneous fracture or dislocation
may result. Perforating ulcer of the foot, resulting from a neglected injury
often of slight character, may occur. Other trophic disturbances are rar-
efaction of the bones, with resulting fractures, irregular atrophy of muscles
and various skin eruptions.
c. The Stage of Paralysis. The ataxia gradually increases until the patient
becomes helpless; the bladder and rectum are incontinent and bed sores are
apt to develop; cystitis with consequent pyelitis may result from the use of
the catheter; the patient is confined to his bed, even though his general nutri-
tion remains good.
The prognosis. The course of the disease is chronic and may extend over
twenty or thirty years. The first stage lasts from a few months to fifteen
or ttt^enty years, the second may develop slowly or rapidly and the third stage
is soon terminated by death. The prognosis should always be guarded; it is
believed that recovery has never taken place but the progress of the disease
may be stopped or at least delayed. The thoroughness with which syphilitic
disease of late years has been treated seems to have borne fruit in that mild
t^-pes of locomotor ataxia are becoming more frequent.
Treatment. In all cases in which there is suspicion of syphilis, appropriate
treatment in the form of mercurial inunctions — one drachm (4.0) of the oint-
ment should be thoroughly rubbed into the inner aspect of the thigh or arm
daily until the gums become sore, when mercury bichloride may be substi-
tuted — gr. ^ (0.003) three times a day — and the internal administration
of potassium iodide in ascending doses should be begun. The dosage of the
latter must be governed by its effect, as much as possible being given without
inducing iodism; after the limit is reached the doses should be diminished by
about one-half and continued for several months. If the primary specific
lesion is not of recent date certain authorities are inclined to omit the mer-
cury and depend chiefly upon the potassium iodide, others consider the use
of the former drug to be contraindicated in cases with optic nerve atrophy
on the ground that it may hasten the process. Mercury biniodide may at
times be employed with good effect in doses of gr. ^ (0.003) three times
a day and a change of the potassium iodide to the strontium salt (gr. xx to
XXX — 1.33-2.0 — three times a day) or to the swup of hydriodic acid, i
drachm (4.0) at the same interv^als, may prove beneficial.
The disadvantages of antis}^hilitic treatment are that it may cause diges-
tive disturbances which result in an impairment of nutrition and that excessive
administration of merciu*}^ may produce distressing neurasthenic conditions.
When after thorough trial it is certain that no benefit from this form of treat-
LOCOMOTOR ATAXIA. 749
ment is to be expected it should be stopped; if it acts favorably it shoiild
be continued, the mercury being alternated with the iodide, a period of three
months being devoted to each, or the two should be given simultaneously for
three months, omitted for a like period, then repeated and so forth.
Various other drugs have been from time to time exploited as efhcacious
in locomotor ataxia; of these those most likely to be affective are arsenic, in
the form of Fowler's solution rq_ v(o.33), arsenic trioxide, gr. -3L to gV (o-oo2-
0.003) or sodium arsenate, gr. J^ to -^ (0.002-0.006) three times a day,
aluminum chloride gr. iii to x (0.2-0.66) three times a day, strychnine, gr.
-3V (0.002) or less thrice or four times daily, calcium glycerophosphate, gr.
iii to V (0.2-0.33) and calcium h}^ophosphite, gr. X to xxx (0.66-2.0). Silver
has been advocated but it, barium, gold, ergot and physostigma are prob-
ably of no effect and are little used.
Much can be done in this disease toward mitigating the symptoms; the
pains may be controlled by the coal tar analgesics, antipyrine, acetanilide,
acetphenetidine, salophen, salip^Trine, or aspirin in the usual doses; this symptom
at times may yield to a drachm (4.0) of sodium bicarbonate; codeine, gr.
J to -J (0.016-0.032) may prove effectual, as also may extract of cannabis
indica (gr. -j to | (0.016-0.032) or cocaine, hypodermatically, gr. -gtoj (o.oii-
0.016). The Paquelin cautery applied once or twice a week is a useful agent
in the severe pains; cups, leeches and blisters may also be employed with
good effect. Morphine in the severe pain may become necessary, it should
be given under the skin and only after all other means have failed, on account
of the danger of establishing the habit. The insomnia may be relieved by
the bromides, veronal, gr. x to xv (0.66-1.0), sulphonethylmethane in the
same doses or siilphonmethane, gr, xx (1.33). Hydrated chloral may in ex-
treme cases be combined with the bromides.
The bladder disturbances may be rendered less distressing by the adminis-
tration of hyoscyamus combined with the balsams, and small doses of strych-
nine, gr. y-i-g- (0.0006), may be given if sexual weakness is present. Neu-
ralgic pains in the rectum often disappear after an enema and a thorough
evacuation. Suppositories containing iodoform, codeine and belladonna
may be efficacious.
In the crises a hypodermatic injection of morphine is often the only thera-
peutic measure which will relieve the patient.
From the beginning of the treatment the physician should insist upon the
importance of systematic rest. It may be advisable to put the patient to bed
for a number of weeks. If so drastic a measure is not necessary a regular
life should be prescribed, with moderate exercise and avoidance of all mental
and physical labor. The excessive use of alcohol and tobacco must be for-
bidden as well as more than moderate venery. Institution treatment for a
number of months each year is often of great beneiit.
75° DISEASES OF THE NERVOUS SYSTEM.
Electricity is useful for its tonic effect upon the muscular and nervous
systems and if employed perseveringly may benefit the lesion itself. Galvan-
ism (15 to 30 milliamperes) may be administered by applying the anode
to the side of the neck and moving the kathode up and down the spine and
limbs. Both the constant and the interrupted currents may be employed
in this manner; the former may be effective in the pains and the latter is
indicated in vesical vi^eakness.
Hydrotherapeutic measures are often beneficial but have disadvantages,
in that the patient often fails to react after the cool bath and the warm bath
is likely to prove enervating. Oftentimes, however, a daily lukewarm bath
lasting for fifteen or twenty minutes, after which a cool douche is applied
to the spine and the patient is vigorously rubbed, is an excellent procedure.
Wet compresses to the limbs are often useful to ease the pains and douches
neither hot nor cold often affect the patient favorably. Spa treatment with
systematic bathing may confer benefit but probably rather from the regular
life led at such resorts than from any effect of the bathing itself.
Treatment by suspension was much advocated a mmiber of years ago. The
patient was harnessed about the neck and shoulders with a specially constructed
apparatus and was by this means lifted from the floor. This was done for a
minute or two, two or three times a week for eight or ten weeks, and after an
interval of several months, the procedure was repeated. This method seemed
to benefit some cases but is little employed at present. It is useless in the
stage of paralysis. Treatment by means of education of the muscular sense
(the method of Fraenkel) is often valuable. This consists of systematic exer-
cises calculated to educate certain nerve fibres of the cord so that they may
be able to perform the function of those which have been destroyed. The
idea is to train the patient in certain motions requiring coordination. The
exercises may be greatly varied, among those applicable are to require the
patient to walk a straight line drawn upon the floor, to draw sets of parallel
lines with one hand, then with the other, etc. For an extended list the reader
is referred to Dana's Textbook of Nervous Diseases. This method, while
it does not influence the lesion, often enables the patient to use his limbs to
better advantage and is particularly effective in patients in the latter part
of the iirst or early in the second stage.
There is no special diet to be prescribed. The patient should eat regularly
a sufficient quantity of nourishing and easily digestible and assimilible food.
Fats, starches and proteids are all allowable.
Patients should be encouraged to use every means to combat their disease
and to carry out a systematic treatment. Those who have the courage and
energy to do this will, after a time, reap the benefit of their perseverance
and reach a condition in which they may lead comparatively comfortable
Friedreich's ataxia. 751
Synonyms. Hereditary Ataxia; Hereditary Ataxic Paraplegia; Family
Ataxic Paraplegia; Friedreich's Disease.
Definition. A chronic disease of the spinal cord, occurring hereditarily
and characterized by ataxia and paraplegia.
etiology. The direct causative factor of this disease is unknown. It
seems to occur rather more frequently in males than in females; it may occur
in infancy but the greater number of cases develop between the ages of
three and fifteen years. It very rarely appears after the age of twenty-five.
The condition is probably due to lack of, or to poor development of certain
tracts in the spinal cord. It sometimes follows an acute infectious disease,
and alcoholism, syphilis and the neurotic tendency in the parents, seem in
some measure to be predisposing factors.
Pathology. The lesions of Friedreich's disease are found in the posterior
and lateral columns of the cord and consist of a degeneration involving these
tracts and at times also the columns of Clarke and Gowers. On examination
the cord is found to be smaller than normal and the pia, over the posterior
columns especially, is thickened. The normal tissue is seen to be degenerated
and to some extent replaced by neuroglia, the cells in the anterior and poste-
rior cornua are atrophied and this change affects also the anterior and posterior
Symptoms. These are, as would be expected, a combination of those of
lateral sclerosis and locomotor ataxia — paraplegia with ataxia. Pains at the
onset are infrequent, the first symptom noticed being a gradual loss of coor-
dination first affecting the legs. The patient totters and with diflSculty
maintains his equilibrium, he sways in walking and may fall. Romberg's
symptom may be present; the patellar reflex is usually absent, but rarely may
be exaggerated. The ataxia of the arms is characterized by choreic and
irregular movements; in grasping objects there seems to be an excess of move-
ment. As the disease progresses the jerky movements involve the head and
there may be accompanying tremor, nystagmus is present when the eyeballs
are moved but is absent when they are at rest. The pupils are usually normal
and optic atrophy is very rare. Scanning speech with elision of syllables
may occur late in the disease.
The paresis appears later than the ataxia and chiefly affects the legs; the
power of the muscles, the flexors more than the extensors, is impaired and
consequent talipes may occur; if the muscles of the body are affected, curva-
tures of the spine result. The nutrition of the muscles is little if at all
There is a lack of sensory symptoms except, at times, cramp-like muscular
contractions in the earlier stages which may cause discomfort. Rarely there
752 DISEASES OE THE NERVOUS SYSTEM.
are disorders of the pain and temperature senses and sensation mav be slightly
The mind is not materially affected, but children in whom the disease
appears early in life, seldom attain normal development in this regard.
The prognosis as regards recover}^ is utterly imfavorable but the progress
of the affection is slow and may be interrupted by periods of remission; patients
who are^ttacked in the earlier years of life seldom survive to attain maturitv.
Treatment is of little avail. With regard to prophylaxis, we should, when
the disease has occurred in one member of a family, endeavor to impress upon
the parents the necessit}- of especial care in the feeding and general manage-
ment of the others. Breast-feeding should be insisted upon, all infections
and traumatisms guarded against and excessive physical exertion avoided.
Patients affected -n-ith the disease should receive nourishing and easily-digest-
ible food and live in as hygienic a manner as possible. Nerve tonics may
be employed and may be of some benefit, suspension may be tried and has
seemed to act favorably in certain cases. The therapeutic means applicable
to locomotor ataxia are worthy of trial. Tendency to contractures and defor-
mity may be combated by orthopedic methods and apparatus.
HEREDITARY CEREBELLAR ATAXLA..
This is a condition characterized by all of the symptoms of Friedreich's
disease with the exceptions that in the former aff'ection there are various ocu-
lar disturbances such as atrophy and paralyses, together with an increased
patellar reflex. The condition usually appears after the age of twenty
and is the result of congenital defect in or incomplete development of the
Recent research seems to tend to draw cerebellar ataxia and ataxic para-
plegia more closely together as regards lesions and symptoms and it is quite
possible that they "^-ill come to be regarded as types of the same disease.
Like Friedreich's ataxia, this disease is progressive and -with, regard to
treatment one can hardly do more than maintain the patient's nutrition.
Otherwise the affection may be managed in a manner identical with that
described under the treatment of the former condition.
Synonym. Glosso-labio-lanmgeal Paralysis.
Definition. A progressive paralysis invohdng the tongue, lips, throat and
larynx and in advanced cases the muscles of the lower part of the face.
^Etiology. This disease occurs most frequently in middle age. Syphilis,,
ACUTE ASCENDING PARALYSIS. 753
exposure, and mental and physical over-work have been considered possible
causative factors but the direct jstiology of the condition is unknown.
Pathology. The morbid change which characterizes this disease consists
in degeneration of the nuclei of origin of the motor nerves which supply the
lips, tongue, throat and lar}'nx. In certain cases the lesion spreads to the
cranial nerve nuclei on the floor of the fourth ventricle of the medulla in-
volving the vagus, the fifth in its motor fibres, the seventh, the ninth, the
twelfth, and exceptionally the abducens and motor-oculi. The degenerative
change is characterized by a wasting of the normal nerve tissue and a re-
placement of it by neuroglia, with connective tissue increase in the walls of
Symptoms. These usually begin with disturbance of the speech, there is
difficulty in articulating the linguals, R, L, D, T, the voice is nasal in quality,
the tongue becomes atrophied and paralyzed; swallowing is difficult and
mastication imperfect. Wlien the muscles of the lips become involved the
articulation of the labials, B,F,P,V, becomes imperfect and the lips are thin
and tremulous, the facial expression is changed and the saliva dribbles. With
involvement of the phar}Tix and larynx swallowing becomes impossible,
food may be regiirgitated, or drop into the larynx and cause foreign body
pneumonia; the voice becomes low, monotonous and is finally lost. Fine con-
tractions of the involved muscles may be observed. There are no changes of
sensation and taste is not affected. The course of the disease is chronic
and may last over a period of several years.
The prognosis is hopeless, death taking place from obstruction of the
lar}Tix by food particles, septic pneumonia or exhaustion.
Treatment consists in the employment of all possible means to maintain
the patient's strength, and in the use of tonics, such as iron, arsenic, quinine
and strychnine. \Vlien there is a s>T)hilitic element in the history the use of
mercury and potassium iodide is indicated. Electricity may confer some
benefit; the galvanic current may be employed by placing the electrodes,
one at each mastoid process, and f aradism may act as a tonic upon the affected
muscles. Galvanism may aid the patient in swallowing, the anode being
placed at the back of the neck and the cathode at the side of the lar}^nx; when
the latter is moved along this organ it causes a reflex deglutatory act.
It is often advisable to begin to feed the patient by means of the stomach
tube early in the disease.
ACUTE ASCENDING PARALYSIS.
Synonym. Landry's Paralysis.
Definition. An acute disease characterized by rapidly advancing paralysis
beginning in the legs, progressing upward to the body and finally reaching
the arms, causing death by involving the respirator}^ center in the medulla.
754 DISEASES OF THE NERVOUS SYSTEM.
Etiology. This disease affects males more often than females and is usually
seen in healthy individuals in the prime of life (twenty to forty.) Cases have
been observed in which the abuse of alcohol has seemed to be a considerable
factor in predisposing to the condition and the same may be said of syphilis.
Of late there is more and more inclination to attribute the affection to a toxic
infection of the peripheral nerves and spinal cord, since it, at times, occui's
as a sequela of microbic diseases such as enteric fever, erysipelas, epidemic
Pathology. The lesions found post mortem differ but it seems sure that
the peripheral motor neurons are the seat of chief involvement; changes are
also observed in the spinal cord resembling those of acute general myelitis
or very similar to those occturring in peripheral neuritis.
Symptoms. Prodromata, such as loss of appetite, rise of temperature,
pains in head and back, general malaise and tingling sensations in the extrem-
ities, lasting from a few hours to several days, are usually present. Follow-
ing these a rapidly augmenting weakness of the legs appears, which, within a
few hours or a few days, may become a complete paralysis. The paralysis
soon extends to the trunk and in a day or two, or even less time the arms are
involved. As the muscles of the neck and face become affected breathing
is interfered with, the speech becomes indistinct, swallowing is difficult and
other symptoms of bulbar paralysis develop and death occurs from respiratory
paralysis. The reflexes are diminished or lost but may later return and become
exaggerated, the sphincters remain continent, there is no musciflar atrophy
or tendency to bed sores and the electric reaction usually remains normal
but exceptionally there is loss of faradic excitability. As a rule sensation
is not affected, except for the tingling occurring at the onset and the develop-