ljaralgsk 
Win. W , aged 58 years, began to complain in 
May, 1869, that the saliva dribbled from bis month 
when speaking or reading. One month afterward he 
said he was conscious of a difficulty in the pronuncia- 
tion of words, although at this time it was so slight as 
not to be perceptible to those with whom he conversed. 
The following autumn there was a very evident hesita- 
tion in his speech; he talked slowly and deliberately, 
but correctly, excepting a defect in the articulation of 
certain words, and his voice had assumed a nasal tone. 
In February, 1870, he visited some friends in the 
northern part of New York, and was able to travel 
alone. While in the country he placed himself under 
the charge of a physician, and electricity was employed 
but without benefit, as in his letters to his family he 
stated that he was growing worse. 
He returned to Albany, in June, 1870, in the follow- 
ing condition: complete loss of speech ; difficulty in de- 
glutition, any effort to swallow being attended with par- 
oxysms of coughing and suffocation; paralysis of the 
tongue, which could only be protruded a quarter of an 
inch beyond the edge of the teeth ; body well nourished 
and appetite good; partial loss of motion in both arms, 
especially on the left side; pain in the thumbs, arms, 
and back of the neck; mental action unimpaired, ex- 
cept that the emotions were easily excited so that he 
laughed and wept without sufficient cause; could write 
perfectly with his elbow resting upon the table, but 
was unable to raise his arm so as to feed himself; could 
walk without difficulty. 4 
In July it was noticed that lie dragged Ms feet a 
little, but lie could still walk alone for a considerable 
distance. After this time, the movements of the arms 
and legs gradually became weaker, until in November 
he was unable to get up and down stairs, while at the 
same time the difficulty in deglutition increased. His 
writing became more and more illegible, and in the lat- 
ter part of November he had to give it up altogether, 
and communicated his wishes to others by pointing out 
letters with a stick placed in his hand for the purpose. 
In December he had a severe chill, which was fol- 
lowed by acute pain and complete loss of motion in the 
left arm, which became oedematous, and remained so 
until a few days before his death. He could walk only 
a few steps, and had great difficulty in doing even this. 
During December the flexor muscles of his hands and 
arms began to contract, and in the latter part of the 
month his feet became oedematous. Sight, hearing, 
and appetite remained good throughout. 
I saw the patient through the politeness of Dr. Fow- 
ler, his attending physician, from whom I obtained the 
above notes, on the morning of January 4th, 1871. 
He was then sitting in a chair propped up by pil- 
lows, being unable to lie down on account of dyspnoea; 
complete loss of motion, except a slight nodding of the 
head and a little movement of the right hand; sight 
and hearing appeared unimpaired; speech entirely lost; 
mouth partly open and lips immoveable, except a slight 
twitching of the left angle of the mouth; cheeks flac- 
cid; tongue completely paralyzed and lying on the 
floor of the mouth; respiration feeble and occasional 
coughing; pulse 90 per minute and regular; both arms 
paralyzed and slightly flexed and attempts to straighten 
the fingers caused pain; lower extremities completely 
paralyzed, and feet and ankles oedematous; defecation 5 
natural; micturition slow and frequent; attempts to 
swallow occasioned distressing cougli and suffocation, 
and the aliments were often rejected through the nose. 
He remained in this condition until the afternoon of 
the same day, when an attempt to swallow some por- 
ridge brought on severe coughing and strangling. At 
seven o’clock that evening he died without a struggle. 
Autopsy twenty hours after death. 
External appearances. Rigor mortis well marked. 
Body spare, but not emaciated. No very evident signs 
of muscular atrophy. 
Head. Scalp very dry. Scull cap removed with great 
difficulty, owing to adhesions of the dura-mater, which 
was torn in trying to separate it from the bone. Dura-, 
mater very much thickened. Arachnoid normal, with 
considerable serous infiltration of the sub-arachnoidean 
connective tissue. Pia-mater much injected. The cere- 
bral substance both cortical and medullary appeared to 
be of normal color and consistency, but exceedingly 
hypergemic. The following condition of the cranial 
nerves was found. Ist. Olfactory, normal. 2d. Optic, 
normal. 3d. Motor oculi, normal. 4th. Patheticus, 
small. sth. Trigeminus, on the left side flattened, gray 
and softened; on the right side, larger and very hyper- 
semic. 6th. Abducens atrophied, especially on the left 
side. 7th. Facial atrophied, and gray on both sides. 
Bth. Auditory, normal. 9th. Gilosso-pharyngea], nor- 
mal. 10th. Pneumogastric, atrophied on both sides. 
11th. Spinal accessory much atrophied. 12th. Hypo- 
glossal so much atrophied on both sides as to resemble 
mere threads or filaments of connective tissue. The 
corpora striata and optic thalami were normal. The cer- 
ebellum was very hypersemic, but otherwise presented 
nothing unusual. The pons Yarolii and medulla oblon- 
gata appeared to be of firmer consistency than usual. 6 
Spinal cord. Spinal meninges mucli injected. The 
anterior spinal roots were atrophied especially on the 
left side. Transverse sections of the cord showed 
the anterior cornua of gray matter, as well as the 
left anterior and right lateral columns, to he of a dark 
rose color as if very hyperaemic. 
Thorax and abdomen were not examined on account 
of objections on the part of relatives of the deceased. 
Portions of the brain, cerebellum, and spinal cord 
were immersed in absolute alcohol, preparatory to mak- 
ing sections for microscopic examination. When suffi- 
ciently hardened thin sections were made, stained 
with carmine, rendered transparent with benzole, and 
mounted in balsam. 
The sections of the brain revealed nothing abnormal. 
The sections of the cerebellum a very hypersemic condi- 
tion of the part and a granular degeneration of the 
large ganglionic cells forming the middle layer of the 
cortical portion. 
Thirty sections were made at various levels of the 
medulla oblongata involving the roots and nuclei of 
implantation of the cranial nerves, especially those of 
the facial and hypoglossal. Careful microscopic exam- 
ination of these specimens with objectives varying from 
15 to 900 diameters, demonstrated that the portion of 
medulla forming the floor of the fourth ventricle was the 
seat of several pathological lesions. 
There was a decided hypertrophy or overgrowth of 
the connective tissue, which appeared to have en- 
croached upon and to some extent replaced the several 
groups of ganglionic cells which form the nuclei of 
implantation, for the facial and hypoglossal nerves. 
The individual cells comprising these groups were sep- 
arated from one another and in many instances had 
lost their stellate appearance, their radiating processes 7 
having heen destroyed so that each cell remained iso- 
lated and disconnected from its neighbors. These cells 
had also undergone a degenerative process which in 
many cases rendered them simply a collection of fine 
grannies, and a deposit of brownish yellow pigment 
had taken place to such an extent as to give the cells 
an appearance almost precisely similar to those which 
are normally found in the locus niger of Soemmering; 
they were fewer in number than usual and diminutive 
in size. 
Sections of the cord made in the cervical, dorsal, and 
lumbar regions showed a sclerosis with increase of 
connective tissue in the anterior and lateral columns, 
which was most marked in the left anterior and right 
lateral columns. The multipolar ganglionic cells situ- 
ated in the anterior cornua of gray matter were fewer 
in number than usual, and many of them appeared 
granular and very much pigmented. 
The form of disease of which the above is a typical 
example, has been described by Trousseau and Du- 
chenne, and a number of cases have been reported in 
various medical journals. It commences with a paraly- 
sis of the lips and tongue which is followed by a paral- 
ysis of the soft palate and pharynx; in many cases 
the disease does not stop here, but gradually attacks 
the muscles of the trunk and extremities. There is no 
muscular atrophy, but simply a loss of motor power. 
In those cases where an autopsy has been made, the 
roots of the hypoglossal and some of the other cranial 
nerves have been found to be atrophied, but little or 
nothing has been said of the condition of the central 
origin of these nerves, except in one case described by 
Dr. Samuel Wilkes, in the last volume of Guy’s Hospi- 
tal Reports. Dr. Wilkes says that “ there was very 
obvious gray red change of the calamus scriptorius 8 
from the nib upward and outward for half an inch.” 
This change would necessarily implicate the cells of 
implantation of the facial and hypoglossal and proba- 
bly of the spinal accessory roots, and we therefore have 
a central lesion accounting for the peripheral paralysis. 
Dr. Wilkes also states that the anterior half of the 
white matter of the cord was hardened and atrophied. 
In the case above reported not only the anterior, but 
also the lateral columns of the cord were affected so 
that we have a descending degeneration of the motor 
tracts of the cord, consecutive to a primary lesion situ- 
ated in the medulla. This is fully in accordance with 
the views presented by Bouchard in his work on sec- 
ondary degenerations of the spinal cord, and accounts 
for the |>rogressive paralysis of the trunk and extremi- 
ties which follows the original loss of motion in the 
lij>s, tongue, and palate. 
An interesting fact in connection with this secondary 
disease of the cord, is the ultimate permanent contrac- 
tion of the flexor muscles. This contraction with rig- 
idity was present in the case reported, and is also men- 
tioned by Trousseau as having occurred in one of his 
cases. Bouchard speaks of it as an almost constant 
symptom of secondary sclerosis of the anterior columns 
of the cord. 
This case affords an illustration of the aid which the 
study of physiological anatomy may derive from path- 
ology. We have here a very complete destruction of 
the cells of implantation of the facial nerves, and yet 
only a part of the muscles supplied by the facial are 
paralyzed. The orbicular muscles of the eye sare unaf- 
fected, and the patient can wink voluntarily while the 
buccinators and orbicularis oris are perfectly paralyzed. 
This can only be explained by the fact that some of 
the fibres of the facial nerve (as has been stated by 9 
Stilling and Yan Der Kolk) do not pass into their 
proper nucleus, but either pursue an upward course, 
along the raphe of the cord, or else run into small gan- 
glionic groups at a considerable distance from the nu- 
cleus proper. Thus from the fact of the non-paralyzed 
condition of the orbicularis oculi, notwithstanding the 
destruction of the nucleus of the facial nerve, there 
can be but little doubt that the fibres referred to by 
Stilling and Van Der Kolk are those which supply this 
muscle, and it is probable that these fibres terminate in 
ganglionic cells in the neighborhood of those from 
which the fifth pair of nerves originate, and thus we 
have an anatomical arrangement which may aid in the 
explanation of the reflex phenomenon of winking. 
The prognosis in this disease is decidedly unfavora- 
ble. Among the many cases reported, not one case of 
perfect recovery is mentioned, and it is probable that 
the improvement which seems to have taken place in 
some of them was only temporary. The manner of 
commencement may be very sudden or slow and insid- 
ious, but there is but little variation in the ultimate 
termination. Death takes place either as a result of 
suffocation from the impaction of a bolus of food in 
the glottis, or else the slow accumulation of mucus in 
the lungs, which the patient can no longer cough up, 
gradually results in asphyxia. As for treatment there 
is little to be said. Life may be prolonged by the ap- 
plication of galvanism to the upper part of the cord 
and along the tracts of the affected nerves, but nothing 
as yet known has any power to retard the progress of 
the disease in the nerve centres. The following is a 
list of authors with references to where the cases are 
reported: 
Trousseau—Clin. Med., t. 11., p. 334. 
Duchenne—Electrisation localises Doc., p. 640 ; Gaz. Jlebd., 
VIII, 12, 1861. 10 
Dumenil—Gaz. Hebd. de Med. et de Chir., June, 1859, p. 390. 
Samuelson—Berl. Kl. Wochensch., No. 27, 1868 ; Half-yearly 
Compend. of Med., January, 1869. 
Ramskill—Lancet, June, 1869. 
Herard—6r«z. c/es Ilopitaux, No. 20, 1868 ; Rankings Abstract, 
January, 1868. 
Muller—Hospit. Tidend, Nos. 22 and 23, 1861 ; Schmidt's 
Jahrb., vol. 113, p. 169 ; Tear Book of Medicine, 1862. 
Tommasi—L' Union, 114, 1862. 
Wilks— Guy's Hospital Reports, vol. XV. ; Rankin's Abstract, 
July, 1870 ; British Medical Journal, March 19, 1870.