Parkinsonism and
its most frequent accompanying
symptoms.
As Parkinsonism I describe here the chronic
form of the amyostatic complex of symptoms of
encephalitic origin. It was in France (Sicard
and Paraf) that attention was first definitely
directed to the frequency of this sequel, and
that it was so named because of its similarity
to paralysis agitans (Parkinson's disease).
Parkinsonism may develop both in immediate
sequence after the amyostatic phenomena of acute
encephalitis lethargica and in the akinetic or
somnolent stage of its hyperkinetic form, as
well as after an apparently complete recovery
from acute encephalitis lethargica following on
a shorter or longer interval of freedom from
symptoms. The gradual and progressive
development of Parkinsonism may also ensue even
although no amyostatic symptom has appeared
during the acute phase. With regard to its
course, it may develop as a chronic form, or as
a relapse with 'intermittent progressions', or
as a genuine sequel. It happens that quite a
number of cases of Parkinsonism have been seen
in places where there had been no very serious
encephalitis lethargica epidemic, as, for
instance, in Buenos Ayres (Dasso).
The sequelae often begin with weakness in
one arm or leg, or with tremor, or with a
peculiar, somewhat stiff posture. On closer
examination it is noticed that the weaker arm or
both arms no longer participate in the finer
pendulous movements coordinated with the act of
walking. These disturbances of motility, as a
first symptom, may prompt the patient to consult
the doctor. The patient complains of physical
and mental exhaustion, decrease of mental
agility; everything seems difficult. Frequently
these complaints are wrongly thought to be
purely functional. Close objective examination,
however, furnishes even at this stage some
isolated slight symptoms which enable a
diagnosis to be made: the manner of speaking
gives an impression of some effort and is
dragging; the swinging movements of the arms in
walking are perhaps already absent, posture and
gait are no longer as free and easy as before.
Gradually in the course of months the stiffness
in one or other extremities as well increases,
and soon extends to the other extremities as
well. The face, too, may become motionless; all
expression is lacking, the space between the
upper and lower lids appears wide, the range of
movement of the lids is slight, and the staring
gaze of the eyes gives to the patients an
expression of stupid bewilderment. As a result
of abnormally free secretion of the sebaceous
glands the face looks shiny ('greasy face'
(Cohn)); the speech is jerky; generally there is
salivation. Genuine compulsive laughing or
compulsive crying I cannot remember to have seen
in these patients, but it has been described.
The posture comes to be distinctly bent; the
neck is inclined, back, pelvic, and shoulder
girdles are carried stiffly as though the joints
did not exist. The gait is short-stepped and
hesitating, there is propulsion and
retropulsion, as in genuine paralysis agitans,
that is to say, the patient, as a result of
deficiency of the finer movements of the
vertebral column and the extremities, once he is
moved forwards or backwards, cannot stop
spontaneously, but moves on with gathering speed
as far as the next obstacle, where he comes to a
passive halt. It often happens that he loses his
equilibrium and falls, because, with the
slowness of his movements, he cannot keep pace
with the momentum of his body.
Sometimes, although rarely, a hyperextension
of the vertebral column backwards or sideways
may develop instead of the bent posture, when
the head or one of the extremities may also be
fixed in a peculiar extreme position (La Torre).
Sometimes, but much more rarely than in
paralysis agitans, tremor may be present, tremor
of the tongue as well as of the extremities;
genuine Parkinson-tremor ('pill-rolling
movement') is rather rare in Parkinsonism. The
peculiar stiffness which seems to hold the
patient imprisoned consists of two elements,
paucity of movement and rigidity, which occur
often separately but more commonly together; it
is advisable, however, to keep these elements in
mind on account of their separate occurrence.
The paucity of movement is clearly shown by the
lack of mimic expression mentioned above and
also by the absence of the other affective
co-movements of trunk and extremities, the
so-called associated movements (Souques). The
more minute expressive movements of the hands
are likewise wanting, and even the coarser
movements of the limbs are less frequent. The
patients generally sit motionless if left to
themselves, even for a period of hours; when
they seize an object the movements are executed
with excessive slowness. If they look round at
all, they do so generally only with the eyes,
without turning the head or adjusting the
trunk.
Every movement, every action is strikingly
slowed down; even to speak takes the patients a
long time; their reaction-times, in spite of
intact consciousness, are enormously prolonged;
this is described as bradyphrenia (Bianchi,
Naville). They masticate a piece of food for
hours with long pauses and are hardly able to
direct it into the pharynx to swallow it
(dysphagia). Their speech is low-voiced, jerky,
strikingly monotonous, without any modulation or
accompanying facial expression. This 'paucity of
movement' or akinesis also occasionally occurs,
as I have repeatedly emphasized, without any
rigidity (Gerstmann and Schilder, Bychowski,
Verger and Hesnard). We shall revert to this
circumstance on discussing the mental symptoms.
Compare also the section on acute amyostatic
states (vide Mayer-Gross).
Generally, however, this akinesis is
combined with genuine muscular rigidity, so that
repeated attempts have been made to explain the
akinesis, erroneously, as a result of the
hypertonia (Hauptmann).
On examining the patient we find an increase
of muscular tone, an increase of the normal
elastic resistance against passive movements.
This stiffness of the musculature is more marked
when dealing with the large proximal joints than
the distal, smaller ones; it is, for instance,
more marked in the shoulder-joint than in the
wrist and the fingerjoints, thus differing from
the so-called pyramidal increase of tone, in
which, as it occurs, for instance, in
hemiplegias through lesion of the pyramidal
tract (volitional tract), rigidity is more
particularly marked as time advances in the
distal joints. Further, as a characteristic
difference, there is no exaggeration of
tendon-reflexes. The patient himself feels this
stiffness very distinctly. Sudden passive
movements overcome the increased tonus more
easily, whereas moderately slow ones make it
more obvious. In the opening stages of the
disease we can satisfy ourselves that the tonus
is a static postural tonus-increase of the
static postural reflexes (Stertz, Jakob,
Froment)-that is to say, when, for instance, the
patient leans against something, is supported or
sits, the rigidity, which could be observed
before in arm or hand or when standing without
support, vanishes. In the further course of the
disease, however, the tonus becomes permanent,
and in patients confined to bed we find arms and
legs in a state of continuous tonus, the head,
in consequence of rigidity of the musculature of
back and neck, lifted from its support and
carried freely in the air. But the character of
the rigidity varies in different cases. The
general characteristics which distinguish it
from the rigor of pyramidal paralyses are:
1. The rigidity, generally also present
during rest, lessens in most cases on intention
of movement (pyramidal rigidity increases).
2. The big proximal joints are generally
more affected by the rigidity than the distal
small ones (in pyramidal rigidity the reverse
obtains).
3. Flexors and extensors are equally
affected by the rigidity (in pyramidal rigidity
the flexors are more affected in the upper
extremities, the extensors more in the
lower).
4. Reflex-disturbances are absent (in
pyramidal rigidity there is an increase of
reflexes).
Now the rigidity of Parkinsonism may
sometimes deviate considerably from these rules,
and in accordance with this we distinguish with
Gerstmann and Schilder: (a) the plastic type,
which displays the tension already well marked
during rest. Passive movements do not cause any
considerable increase in tone, but there is a
tendency to retain any posture imparted or
spontaneously assumed by muscular action
(flexibilitas cerea); (b) the proprio-reactive
type with a slight degree of 'rest-form'
tonicity ('Ruheformtonus') and no flexibilitas
cerea, where passive movements, particularly if
repeated, increase the rigidity; (c) the
reactive type with hardly any' rest-form'
tonicity and no flexibibitas cerea; hence
passive movements suddenly (though only at
times) provoke the tonus, stimulation of the
skin producing the same result; in this type
active movements do not, as under (a) and (b),
decrease the tonus, but rather evoke it; and
finally (d) the reflectory type, in which though
active movement removes tension, the slightest
passive movement provokes at once a maximal
counter-spasm. It is important to add that types
(a) and (b) are more irquent and typical,
whereas (c) and (d) already, approach more
closely to the pyramidal type; furthermore, the
types may occur together in the same patient,
different limbs being affected by different
types.
It is a very interesting fact that akinesis
and hypertonia are not unconquerable symptoms.
Sometimes patients who can walk only with
difficulty may dance quite well to musical
accompaniment, or may in a state of excitement
run after and jump on board a moving vehicle. A
musical female patient, who on account of her
rigidity could no longer write, was able to play
waltzes and other melodies quite well on the
piano. Moreover energetic commands may, at least
temporarily, break through the akinesis. On
discussing the mental condition we shall again
return to these phenomena. The fact, repeatedly
observed by us, that high fever (even
artificially produced) may in some cases abolish
rigidity and akinesis, although unfortunately
only for a few days, after which the previous
condition re-establishes itself, may prove of
possible importance for future therapy.
Other disturbances of motility also occur in
Parkinsonism, if not regularly yet occasionally,
as, for instance, pareses of one or other
extremity; but these are not paralyses with
reflex-differences but diminished motility, not
as a result of rigidity, but of inability to
execute more powerful movements. Also
hemipareses are repeatedly observed. Genuine
paralyses with reflex-disturbances, however,
occur only in exceptional cases. I have also
repeatedly seen flaccid pareses of the muscles
which keep the head in position, so that it
dropped on the chest. Such pareses are usually
transitory; probably they are rather to be
looked upon as atonias, belonging to the group
of the atonie paralyses of chorea, than as motor
paralyses of a possibly neuritic nature. The
reflexes in such states of weakness of the
musculature may be diminished; generally they
are unchanged and only very rarely
exaggerated-hardly ever to such a degree as in
pyramidal lesions, though occasionally
Babinski's sign may be seen. In the latter case
a combination of centrally caused motor and
tonic disturbances is likely.
The muscles feel often peculiarly inelastic,
'doughy'. The mechanical muscular irritability
is in general distinctly increased, a phenomenon
which is probably caused by general
disturbances, perhaps disturbances of
metabolism; also the formation of an
idiomuscular wheal is repeatedly found.
Mechanical hyperexcitability of nerves,
particularly Ohvostek's sign in the field of the
facial nerve, is not infrequently witnessed. The
galvanic excitability is often, though not
always, distinctly increased, in particular
increase of the anodal excitability should be
looked for (Stern). Sometimes a peculiar faradic
after-contraction in a muscle electrically
stimulated, which may even last for more than
half a minute; has been seen (Vincent,
Jarkowsky).
Parkinsonism is not generally associated
with tremor, so that we are faced by the picture
of paralysis agitans sine tremore. Though this
is the rule, tremor is not infrequently found in
any of its varieties. The pillrolling movement,
and tremor during rest which ceases on intended
movement, are hardly ever seen; all other kinds
of tremor are more frequently met with, for
instance violent shaking of the extremities
which may spread over the whole body. In other
cases again tremor occurs only on the initiation
of movements or on mental excitement.
Repeatedly one sees attacks of tremor,
tremor of the jaw, of the lower lip, of the
muscles of mastication, less often of the head
or any other kind of trembling in general.
Clonic spasms and fascicular twitches do not
occur in the chronic forms.
Since the discovery of lenticular
degeneration by Wilson we attribute this kind of
static rigidity, tremor, and the whole of the
complex of symptoms surrounding paralysis
agitans, the choreiform and athetotic movements,
and the so-called extra-pyramidal disturbances
of locomotion, such as we find so well developed
in Parkinsonism, to affections of the basal
ganglia, the nucleus caudatus, and nucleus
lentiformis, in more recent times also of the
subthalamic nuclei and the substantia nigra. We
speak also of the striatal or, better, with
regard to the last-named localizations,
extrapyramidal complex of symptoms. Post-mortem
examination of the cases which die of
Parkinsonism tells largely in favour of this
supposition. In this region of the basal grey
cerebral masses there is probably located a
series of centres which exercise an influence on
progression of movements, rhythm, volume of
progressive movements, timing, and many other
processes, quite independently of the question
whether these movements are of a mainly
automatic nature or are originally volitional
and executed under the control of attention and
have only later become partly automatic. This
points to some direct influence acting upon the
course (so far as it is automatic) of volitional
acts and in its further interpretation to a
possible indirect action on purely mental
processes. Under the heading of this group of
disturbances I should feel inclined to classify
quite a number of phenomena which, as
post-encephalitic phenomena, are generally
associated with other signs of Parkinsonism or
accompany a fully developed state of
Parkinsonism, but which occasionally may occur
as a separate syndrome of sequelae by themselves
or in common with other quite different
post-encephalitic symptoms even without
Parkinsonism. One might describe them as
disturbance of the automatisms.
Many years ago I proved the existence in the
substantia nigra of an important shuntingstation
for automatic rhythm of movement and progression
of acts of mastication and swallowing. Now in
post-encephalitic disturbances we repeatedly
find a rhythmic repetition or tic-like
recurrence of movemente of mastication and
swallowing or convulsive
yawning and
sounds of clucking and sucking. But other tics
and compulsive movements occur as well,
particularly in those groups of muscles in which
the nonencephalitic tics locate themselves by
preference and not, as might be expected, in
those muscles which are generally affected by
the acute encephalitic hyperkinesis (Wagner y.
Jauregg). We see therefore blepharospasms,
mimetic tics, tics of clucking and hissing, also
torticollis and tics of the upper and lower,
extremities, but we find also fits of yelling
and yawning,
&c. Often these tics may be influenced by
the position of the body, for instance
thày cease on lying down; but not
infrequently they may be influenced
bysuggestion, for instance by hypnosis, 'or may
be suppressed for some time by will-power.
But unfortunately these improvements by
suggestion or education do not last for long. To
this group probably belong also the respiratory
disturbances which we often find in
post-encephalitic patients (Vincent and Bernard,
Babinski and Charpentier, van Bogaert, S. E.
Jelliffe, W. A. Smith, Westphal, Stem, Hess).
The respiratory disturbances of the acute phase
have already been discussed on p. 50. Relatively
speaking, the most frequent disturbances are
attacks of hyperpnoea with forced expiration.
Often these attacks lead to quite grotesque
paroxysms with the oddest accompanying
movements, which the patients feel to be
compulsive. A patient in this condition opened
his mouth wide, twisted his trunk and arms
backwards in the most extreme fashion, stamped
at the same time with his feet, and uttered a
snorting sound.
On being questioned he did not perhaps give
dyspnoea as the reason for these weird acts, but
said: 'Well, I must twist my arms that way.' As
a matter of fact there is actually as a rule no
dyspnoea present. Sometimes there seems to be a
diminished amount of bicarbonate in the
blood-serum (Happ and Mason), but at other times
this is not observed. Quite occasionally,
however, genuine dyspnoeic attacks occur
(Bériel). But this seems rather a
disturbance of rhythm and 'progression of
movement' and volume of movement in proximal
centres, whereby the automatism of the distal
respiratory centre loses part of its proper
regulation through the absence of central
inhibition. These attacks may be released by
emotion (Insabato), but at other times they may
themselves cause states of fear (Kennedy).
Hyperpnoea may also exist as a condition of some
duration, sometimes in conjunction with peculiar
accompanying movements, for instance,
contractions of the biceps with every breath.
The hyperpnoea may be accompanied by tetanoid
symptoms as a result of hyperventilation (Barker
and Sprunt), also bradypnoea, apnoea, irregular
respirations, nervous cough, spastic cough,
sighing, tics of respiration, &c.,
occur.
These purely nervous functional disturbances
are primarily caused by organic lesions. They
can bring sensations or complexes of sensations
and emotions in their wake, which on the one
hand may be primary, that is, of the same
generic order as the accompanying movements so
frequently seen and caused by the same
disturbance as the disturbance of motility
itself, or on the other hand possibly secondary.
Thus, for instance, appreciation of the position
in which he is placed may cause emotions of fear
or embarrassment in a patient. Certain mental
fixations may therefore lastingly associate
themselves with these motor disturbances and
lend them a neurotic imprint; this neurotic
element asserts itself (Jelliffe) in dreams or
otherwise in the behaviour of the patients. The
neurotic aspect of these patients is then still
further enhanced by the apparent possibility,
mentioned above, of influencing or even
suppressing their tics with their associated
mental (primary as well as secondary) symptoms
by suggestion.
Encéphalite léthargique
Cruchet, Moutier, Calmettes Soc méd hop
Paris 27 avril 1917
