| Reprinted from 
The Philadelphia Polyclinic, 
Vol. IV, June 8, 1895, No. 23.] 
ATHETOSIS. 
A. O. J. KELLY, A.M , M.D. 
Clinical Assistant in the Neurologic Department, Philadelphia Polyclinic ; Pathologist to St. Agnes' Hospital ; 
Assistant to the Medical Dispensary at the Hospital of the University of Pennsylvania. 
Symptomatic only though it usually is, 
being rarely a disease sui generis, of suf- 
ficient interest nevertheless is the subject of 
athetosis. First described by Hammond in 
1871, it has since been the subject of re- 
peated investigations and careful study, until 
to day its symptomatology is as well under- 
stood as its pathology is obscure. A report 
of the following case, presenting, as it does, 
the symptoms typically, may not be devoid 
of interest. 
The patient, J. M., aged 18 years, male, 
single, white, a native of the United States, 
presented himself at the Philadelphia Poly- 
clinic, March 6, 1895. His mother died of 
right-sided hemiplegia, his father, one sister, 
and three brothers are living and well. The 
patient was apparently healthy until he was 
one year old, when he had a convulsion, 
which is said to have come on suddenly, and 
is supposed to have been caused by teething. 
No acute infectious disease preceded the con 
vulsion, nor is he known to have been par- 
ticularly ill at this time. Beginning thus, 
these convulsions, occurring once or twice 
every two months or thereabouts, gradually 
increased in frequency until they would occur 
once a week or oftener. When thirteen years 
of age, having been under medical treat- 
ment more or less all his life, the convulsions 
ceased, and have not recurred. As he now 
recalls the attacks, his first sensation was that 
of a " lightness in the head," followed by 
winking of the left eye, twitching of the left 
side of the face, and drawing of the head to 
the right side. Then came on unconscious- 
ness and convulsion affecting the entire left 
side of the body. Unconsciousness would 
continue for from ten minutes to half an 
hour; then supervened a condition of drowsi- 
ness and sleep lasting two hours, or more. 
On different occasions during the convul- 
sions he bit his tongue, but never had any in- 
voluntary voiding of urine that he can remem- 
ber. He presents himself now to ascertain if 
the condition of the left side of his body, par- 
ticularly of his hand, can be improved. The 
hand is the seat of mobile spasm (Gowers) ; 
of movements slow, involuntary, and of some 
regularity ; movements of adduction and ab- 
duction, of flexion and hyperextension of the 
fingers, and of pronation and supination of 
the hand and forearm, follow each other in a. 
sequence more or less orderly. (One of the 
most frequent positions of the hand is repre- 2 
sented in the accompanying figure). This 
condition has endured since the patient and 
his elder sister can remember. The hand is 
at times for a while quiet. Attempts at vol- 
untary motion always induce the mobile 
spasms, which become very much exaggerated 
and markedly inco-ordinate upon emotional 
or other excitement. Occasionally he can 
exercise a slight momentary control over the 
movements of his hand ; whether or not these 
movements persist during sleep he cannot say. 
He has little or no power in his left hand, 
and but little in the forearm; good power in 
the upper arm. The muscles of his arm are 
well preserved, as are also apparently the 
muscles of his forearm and hand. The left 
arm and hand • is somewhat smaller and a 
trifle shorter than the right. The deep and 
superficial reflexes are slightly exaggerated ; 
there is no disturbance of sensation. The 
left leg is a little less thick and a trifle shorter 
than the right; good muscular power; no 
disturbance of sensation ; patellar-tendon re- 
flex slightly exaggerated ; no ankle clonus ; 
no spasms of toes, nor can he move his toes 
at all. The face is probably unaffected, 
although it occasionally seertis, especially 
when he talks or smiles, as though there were 
an earlier and some slight overaction of the 
•muscles of the left side. There is slight 
tremulousness of the tongue on protrusion, 
•but no disturbance of speech. There is no! 
numbness of the affected side, nor any pains 
at the seats of spasms. The patient sleeps 
well, hasseldom headache, and never vertigo. 
He has no symptoms referable to his thoracic' 
or abdominal viscera, examination of which 
is negative. 
The diagnosis was made of athetosis fol- 
lowing acute cerebral palsy of childhood.' 
Unfortunately, as is often the case, he came 
to us as a dernier resort, and equally unfortu- 
nately for him, we could prognosticate no 
more favorable course than that which the 
affection had heretofore run. He was given 
sodium iodid, and ordered to report regu- 
larly for electric treatment, in the hope that 
some benefit might be derived therefrom. 
Cases of athetosis are interesting, especially 
from a diagnostic and pathologic point of 
view. In the diagnosis we must first distin- 
guish between idiopathic and symptomatic 
athetosis. The morbus sui generis is com- 
paratively unfrequent, and the causes to 
which it is usually ascribed-e g., traumatism, 
fright, cold,-are often either absolutely er- 
roneous, or must be accepted with great 
reserve by an impartial critic. Jacoby, 
Strumpell, and others speak of a congenital 
athetosis. Symptomatic athetosis may be 
attendant upon a variety of nervous disorders. 
Originally described in connection with epi- 
lepsy and severe psychoses, it is as hemi- 
athetosis post hemiplegica, that we most com- 
monly meet with it; not so often, indeed 
rarely, following the ordinary hemiplegias, 
but especially after the acute cerebral hemi- 
plegias of children. Osler, Gowers, Oppen- 
heim, Strumpell, etc., mention this connec- 
tion, and assert that by far the majority of 
infantile hemiplegias show sometime or other 
during their course more or less well devel- 
oped athetosic movements. 
The only tremor with which athetosis is 
likely to be confounded is that of chorea, and 
with care even this should not happen. The 
athetosic movements are to be distinguished 
from those of chorea by their localization and 
by their character. By their localization, in 
that they are usually confined to the periph- 
eral parts of the extremities, the hands and 
feet, the choreic spasms being not thus lim- 
ited. Marked athetosic implication of the 
face is very uncommon. Indeed, if the face 
alone be affected, the disorder is almost cer- 
tainly choreic. In character, the slow, more 
or less regular and monotonous movements 
of athetosis serve to distinguish it from the 
quick, eminently irregular, and purposeless 
movements of chorea. A relationship be- 3 
tween the two can, however, not be denied. 
Leube reports a case of idiopathic athetosis 
which gradually assumed the character of a 
typical chorea. Bernhardt cites a case of 
post hemiplegic chorea, in which the move- 
ments became athetosic. In this connection, 
Leube says that post-hemiplegic chorea, and 
post-hemiplegic athetosis are identical. Op- 
penheim mentions that he saw cases in which 
the clinical picture of the upper extremity was 
that of chorea, while that of the lower extrem- 
ity was athetosis. 
The pathology of the affection has not, as 
yet, been satisfactorily explained. Within 
late years there has been a seeming tendency 
among some to accept the view that a lesion 
anywhere in the course of the fibers of the 
pyramidal tract may be followed by the pro- 
duction of athetosis. Strumpell, however, 
says, that concerning the nature of athetosis, 
concerning the region where the irritation 
occurs, and the manner in which it develops, 
nothing is as yet known. He is disposed to 
believe that it is always due to some cerebral, 
perhaps cortical, disturbance. But he men- 
tions a personal observation of his,-a case of 
well-developed athetosic movements of the 
arm and hand, which developed idiopathi- 
cally in an aged woman, and in which the 
post-mortem examination of the brain gave 
absolutely negative results. Those believing 
that disease affecting any part of the pyramidal 
tract may cause athetosis, base their assertions 
on the great variety in the localization of the 
lesions found post mortem. Demange has 
reported a case proving conclusively that dis- 
ease limited to the cortex may cause athetosis. 
Kahler and Peck have endeavored to demon- 
strate that all disturbances of motion follow- 
ing hemiplegia are due to disease of the pyra- 
midal tract between the optic thalmus and the 
lenticular nucleus, the various sorts of disor- 
der of motion to be explained upon the sup- 
position of destruction, partial or complete, 
or simple irritation of the fibers. And many 
cases have been reported in which there were 
post-mortem evidences of • disease in this 
region. In some cases it has been disease of 
thethalmus; again, disease of the lenticular 
nucleus ; or the fibers themselves of the pyra- 
midal tract have been implicated. The two 
former would have affected the latter indi- 
rectly. 
Not the least plausible argument is that of- 
fered by Gowers, who suggests that the athe- 
tosis is due rather to the quality of the lesion 
than to its site. He bases his supposition upon 
two important factors in the etiology: firstly, 
that the disorder is much more frequent after 
cerebral softening from vascular occlusion 
than after cerebral hemorrhage; secondly, 
that it follows the hemiplegias of childhood 
much more frequently than it does those of 
adult life. 11 The probable significance of the 
first fact is that in softening slight damage to 
the cerebral tissue is more extensive than the 
actual destruction, and the spontaneous spasm 
must be referred to the overaction of gray 
matter, which is in a state of altered nutrition 
and function. Hence we can understand the 
occurrence of this symptom from a lesion 
which involves extensive slight damage. The 
significance of the second fact-the frequency 
with which the condition follows infantile 
hemiplegia-is probably the greater facility 
with which the growing and developing nerve 
cells recover, and their greater susceptibility 
to disorder of function where their develop- 
ment is perverted." (Gowers.) The lesion is 
thus one of quality-impairment of the nutri- 
tion of growing motor nerve cells. But the 
uncertainty is well expressed : " We cannot 
yet give any trustworthy explanation of the 
mechanism of the spasm in the limbs which 
so constantly accompanies the hemiplegia. 
It seems to follow lesions of various kinds, 
degrees and seat, in the cortex as well as the 
central ganglia. The far greater frequency 
(almost constancy) of the symptom after a 
lesion in early life makes it probable that it 4 
is in some way due to the disordered action 
of centers that remain, and not to the direct 
effect of the disease itself." (Gowers.) Re- 
garding idiopathic athetosis, or athetosis oc- 
curring without hemiplegia, the supposition 
is that it is caused by a lesion not sufficiently 
severe nor extensive enough to produce a 
paralysis, the lesion being more irritative 
than paretic producing. 
Not without interest in the case reported, 
and in many similar ones, is the occurrence 
of convulsions, which in all essential particu- 
lars resemble true epilepsy, and in their mode 
of origin simulate focal or cortical epilepsy. 
These are the convulsions occurring during 
the course of a vast majority of the cases of 
infantile hemiplegia, and constitute not an 
inconsiderable proportion of the cases of epi- 
lepsy as we ordinarily meet with them. They 
frequently, as in the case reported, begin in 
some one particular locality, and are usually 
confined to the paralyzed side. At times, ap- 
parently as in our case, the convulsions may 
commence at the time of the occurrence of 
the hemiplegia, and continue, recurring at 
irregular intervals; in the majority of cases, 
however, there is usually quite an interval be- 
tween the attack of hemiplegia and the devel- 
opment of the epilepsy. It is not uncommon 
to hear of the hemiplegia having been ac- 
quired in early childhood, the development 
of the epilepsy being postponed until the so- 
called epileptogenic period of puberty. Not 
to pass unnoticed in the case reported is the 
sudden cessation of the convulsions when the 
patient was thirteen years of age, since which 
time he has been entirely free from them. He 
still occasionally takes some of the medicine- 
probably bromides-which are supposed to 
have stopped them. 
But little is to be said regarding treatment. 
The nature of the lesions producing the affec- 
tion, so far as they are known, is such as to 
almost exclude the possibility of any beneficial 
influence being exerted by medicines. Elec- 
tricity is of but slight service. Some cases of 
improvement have been reported in which 
galvanism, bromides, iodides, etc., were used. 
But unfortunately we can promise little. 
In conclusion, to Dr. Chas. K. Mills, I 
desire to express my appreciation of his uni- 
form kindness to me, both in permitting me 
to report this case, and for the photograph.