OCULAR
FOLLOWING LETHARGICA.*
MANIFESTATIONS
ENCEPHALITIS BY
(1
A. Michael Cmtchley, M.B., Ch.B. Bristol, asualty Officer, Royal Free Hospital; late House Surgeon, Bristol General Hospital.
TV 1
cephalitis Lethargica, from its initial phase final stages of chronic encephalitis, presents j rge variety of ocular signs and symptoms. These e
to a
eye some are so constant as varied, very yet ^ 0 be most valuable aids to diagnosis both in the early arLcl late stages of the disease. The following article is
anges
are
resume of the ocular manifestations of
argica based
upon
Patients. A.
Pupillary Abnormalities.
(1) Unequal Pupils.?Anisocoria
?Ccurrence
encephalitis
personal observations of 72
both
in
acute
and
is
a
fairly
common
chronic
encephalitis. in 29 of his series pupillary inequality ^Uftcan1 ^5 cases, associated in nearly every instance with ?ttie other defect, such as irregularity or impaired found
reactions.
(2) Irregular Pupils.?This group includes cases of "Circular and eccentrically placed pupils. Such gularities have frequently been described, though ent but rarely in my series. *
Barrett Roue Prize
Essay.
114
Mr. A. M. Critchley
(3) Argyll-Robertson Pupils.?The syndrome of a pupil reacting on accommodation, but not to light, is sometimes found in this disease, especially in the late stages, and unless other physical signs are present, difficulty in diagnosis may quite easily arise. The occurrence
in
encephalitis
of
Argyll-Robertson pupils?
knee-jerks, was first recorded in together 1919 by Naef2 of Munich. Since then it has been occasionally reported. with absent
(4) Sluggish Pupils.?In certain cases of chronic encephalitis the pupillary reactions, though present, may be
abnormally sluggish in their time reactions. Moreover, the amplitude of the reflex dilatation and contraction of the pupil may be abnormally smallDuncan found this impaired briskness of reaction h1 28 per cent, of his
(5) Failure
cases.
to React
on
Accommodation.
the most characteristic features
One oi
typical postencephalitic inability pupil to react o11 accommodation, whilst the reaction to light remains brisk. This abnormality is found in all types of chronic encephalitis, and is of immense value from a medicolegal standpoint, as it may be one of the few signs is
present in
a
case
encephalitis lethargica.
pupillary
reaction a
figure
a
of conduct alteration
attack of per cent.,
of
of the
an
on
following
aI1
In Duncan's series
accommodation
which is low
was
compared
lost in ^ with my
own.
B.
Disorders
Almost every some
in the
the
of chronic
Eyelids.
encephalitis presents a diagnosis of the
and
changes eyelids, can frequently be made phenomena alone.
disease these
case
of
from observation oi
Encephalitis Lethargica !?
Ptosis.?Ptosis has been
frequently
115
described in
phases of this malady, and may
either unilateral
or
bilateral.
at the onset of the
eP?rt3 in 1918 2-
The Government
disease.
epidemic encephalitis
on
?ver 50 per cent, of
in any stage be It is most commonly
cases
had
ptosis
states that
at the onset.
Lid Retraction.?The
typical staring gaze of the Parkinsonian is often accentuated by retraction of the eyelids. It gives a look of
Post-encephalitic a
definite
patient, and von Graefe's and ellwag's signs may be positive. This retraction is s?ttietimes variable, and is not always present. It may actually alternate with ptosis, so that at one examination e patient may have ptosis, and when next seen lid
^ophthalmos
to the
k
fraction
may be marked.
3-
Blepharoclonus.?This sign is said to be present on closing the eyelids, the lids are thrown into series of rapid clonic movements, of fairly large These clonic movements are regular in f^plitude. eir rate and amplitude, and persist so long as closure of the ej^elids is maintained. When e eyelids are closed in sleep, no movements are seen. Ccording to Hall4 this tremor is brought out and by an attempt on the part of the patient ^Xaggerated ?pen his eyes quickly. This flickering of the closed
J^untary .yelids
111 1
^ 0
*
resembles that
eing much
seen
in
neurasthenia, but differs
coarser.
Diminished Blinking.?Parkinsonians
blink less
are
often than the normal individual.
surface of the
eyeball
is
not
found As
a
adequately apt to injure the foreign bodies JUrictiva ; hence blepharitis and conjunctivitis may ^ ound as complications of this state. The infrequent lr*king is another factor in the production of the lcated,
and
are
116
Mr. A. M. Critchley
typical
The
Parkinsonian stare.
impression thus given
fixity of gaze ; suggesting, in turn, fixity of ideation or mental concentration. Such an impression
is
one
of
the may be quite false, for in the post-encephalitic facial expression frequently belies the true emotional content.
5.
Sign
Blinking
not
phenomenon, individuals, is
syndrome.
Wilson,5
in
unknown
though highly characteristic
Kinnier
Deviation.?This
Lateral
on
of the
who first
normal
Parkinsonian
reported
n>
described it
as follows in his Croonian Lecture f When the Parkinsonian is asked to make a series o successive right and left conjugate deviating movements of the eyeballs he very frequently blinks his "
...
eyelids position
the eyes pass from one extreme lateral to the other. The blinking movement,
as
which he appears to be unaware, is sometimes quick' In more than one case sometimes slow and deliberate.
quick double eyelid flicker has been distinctly and repeatedly observed." This sign may depend on the dryness of the conjunctiva through diminished blinking' a
necessitating the act of blinking before they can move easily. 6.
On
Normally
the
Convergence when
a
eyes converge, the gracefully lower.
to lubricate the
Eyelids
person looks at
pupils contract, In
a
remain
eyeS
up-
object, the
near
and the upper
encephalitis cad1 lS movement complex
chronic
component of this co-ordinate upset: the eyes do not converge, accommodation of the pupil is often lost, and the eyelids reman1 elevated. C. 1.
Disorders
Limitation
post-encephalitics
of are
of
Movements.
Movement. unable to
The
majority
converge, which
0f lb
Encephalitis Lethargic a ?f course but it is
|s
a so
limitation in the range of movements, frequent and important a sign that it
reserved for
^ability
J^Qve
to
a
some
converge,
their
lateral
eyes
in
a
deviation,
Punctuate
?ften of the
"
?l?Wness
cases
are
unable
particular direction.
only upward
movement may
less
rarely
?Wnward deviation. 2-
In addition to
separate paragraph.
Citation of full ocular
111
117
on
Movements.? Generalised
cog-wheel
"
to
This occur
and
rigidity,
type, together with
a
of movement (or bradykinesia), is generally und in Parkinsonism. A similar type of movement 18 present in the voluntary muscles of the eyeball, so ^at the eye moves in a characteristically jerky
smon. This phenomenon is best observed during eral movements of the It may possibly be eyes. e expression of a similar hypertonus in the extrinsic
1
e^ular muscles. 3-
Diplopia.?Often the only clue to the date of encephalitis lethargica is a history of "influenza" a cold," which was accompanied by a brief spell of uble vision. This diplopia is caused by a disturbance ^ synergic action of the muscles rather than by isolated Paralyse or paralyses. If the diplopia persists, and is Present in the chronic stages, there is then found some ?nset of '
Paralysis, though
rarely associated with strabismus. Ambers6 has pointed out that in some of these cases e diplopia is only present with distant fixation. lere is much to suggest that distant fixation is an a?tive function carried out by the simultaneous and
Anergic
contraction of both external recti, with a relaxation of the internal recti. Diplopia
^responding ay in such eakness 0l"
of
cases,
therefore, be the expression of
divergence.
No. 168.
a
118
Mr. A. M. Critchley 4.
Strabismus.?Strabismus is quite
common
in the
stages, but in the chronic phase is less often seen. Frequently the squint only occurs when looking in a acute
certain direction, and is found to be due to degree of paresis of a single muscle.
a
mild
5. Paralysis of Convergence.?One of the chief distinctions between Parkinsonism due to lethargica and that due to paralysis agitans is that m the latter there is never any difficulty in converging*
encephalitis
subject be very advanced in years, whilst the former convergence is lost. On attempting to converge, the eyes may look towards the nose temporarily, but almost immediately one eye W# deviate outwards again. unless the
in
6.
Oculo-gyral
Crises (Tonic Eye Fits).? These of immense interest, and the theories as t? their pathogenesis have caused considerable controversy* The crises consist in a spasmodic deviation crises
are
conjugate
of the eyes usually in an upward in paroxysms, and lasting for a few seconds to hours. or rather are part of, attacks and
are a
not
are
late
direction, coming 011 * period varying from
They
are
often
generalised convulsion. These complication of epidemic encephalitis?
common.
found only three cases of three hundred encephalitis >'
Bing7
tonic eye fits amongst Wimmer8 found five cases from
patients.
There is
some
becoming phenomena appears are
of
Ocular Symptoms.
found in my series of 72 percentage incidence as
was
and the
encephalitics, Young's9
of
suggest that they A fuller discussion of these
table shows the number of times
ocular manifestation with
similar number
later.
Incidence
following
a
evidence to
commoner.
The
accompanied by?
a
tables
ead1
chromc
compare^
:?
Encephalitis Lethargica
119
Table I. Number of Cases.
^lepharoclonus
Punctate eye
95
92
64
88
64
63
87
59
82
81
50
69
64
47
65
67
37
51
41
19
26
15
20
9
12
9
12
8
11
7
9
Diminished blinking
Loss of convergence Loss of accommodation "
Lid retraction
^togis
Unequal pupils Limitation of movements -^upil reaction poor
or
Strabismus ^uggish pupils
lost
.
Young's
69
movements
Blinking sign diplopia
Per-
centage. percentage
?
13
6
Oculogyral crises
4
?Irregular pupils
4
Argyll-Robertson pupils Nystagmus ^pil reaction to light poor
9
or
lost
The ocular signs may accompany any type of chronic eilCephalitis. My cases were of the following types :? Table II.
Parkinsonism Parkinsonism Parkinsonism Parkinsonism palilalia
Sleep
.
..
..
..
..
..
inversion
only Conduct change only
.
.
with respiratory disorder with conduct change with conduct change and ..
.
.
..
..
..
..
..
..
..
53 10 4 1 1 4
The value of the less obvious and finer ocular
^Ils
the
of chronic encephalitis in nonwill be seen from the following cases :?
diagnosis
^arkinsonians a'S
reactng6dto light but not Jjer
clon
the
beeil
on accommodation ; cannot converge. and on closing them there is blepharoinking is less frequent, and she blinks on looking to When looking to the right or to the left her eyes "ef?re her head. For the last twelve months she has
are
retra,cted,
to frequent oculogyric crises, occurring t^i^hject^uring her stay in hospital. When she is
e*cit
once
ri
she gets premonitory feelings
every tired or that she must look up;
122
Mr. A. M. Critchley
finally, she is impelled to gaze in an upward direction, and is then unable to lower her eyes. An attack lasts from half a minute to half an hour. During these crises she cannot close her eyes, which lacrimate freely. Coarse nystagmus in a The eyelids are retracted, her lateral direction occurs. forehead wrinkled, her head hyperextended, and there is a tremor of the cheeks. The mouth remains open, and she gives a Her whole body lS yawn and a sigh every few minutes. rigid and tremulous, especially marked on the left side. The patient is very agitated by these attacks, which at the time cause her pain at the back of the eyes, in the frontal region* and sometimes in the ear. All objects appear to be moving from side to side, but there are no visual hallucinations. When an attack comes on she lies down in a darkened room with something thrown over her eyes, and after about fifteen minutes she becomes able to lower her eyes. She then sleeps for several hours, as she feels too exhausted to pursue her normal occupation. 2.?Male, aged 25. This patient is a soldier, and gives no history of an acute attack of encephalitis lethargic?' but states that he was quite well until one day when on parade his eyes became fixed in an upward direction. He is now in a state of Parkinsonism. The pupils are regular, equal, reacting to light but not on accommodation. No convergence. Eye movements jerky. Lid retraction. Blepharoclonus. blinking on looking to side. His attacks occur daily, usually about two o'clock in the afternoon, just after he has returne Case
eyelid8
?
from exercise. His eyes turn up, but he can close his on doing so his eyes come down, only to go up again on his lids. These crises last for about eight hours. The pa^i?11 can terminate his attacks by going to sleep, and on awaking his eyes are normal. The paroxysms cause him no pain an no visual disturbance.
openi11^
3.?Male, aged 21. Onset of encephalitis, 1924. presents typical Parkinsonian appearance, and has disorder. Pupils equal, regular, reacting to light and not Case
respirat0^
accommodation. movements
jerky
Failure to converge. Double ptosis; blepharoclonus. The oculogyral crises
;
this case were of a peculiar type. They came on chiefly meal-times. His eyes would turn up, and his eyelids close tightly. This spasm of the orbiculares palpebrarum ^v0 persist for about ten minutes. These attacks occur sev times in the course of a day.
^
^ j
Encephalitis Lethargica
123
Case 4.?Male, aged 17. Encephalitis lethargica three and half years ago ; since then has developed a pronounced
^arkinsonian
condition. Pupils regular, equal, reacting to light accommodation. Convergence poor. Bilateral Ptosis. Blinking less frequent, blinks on looking to the side. epharoclonus. His attacks last about thirty minutes, during flich the eyes are deviated vertically, and with great difficulty e eyes can be brought down. There are no visual hallucinations, , ut he gets frontal headaches while an attack is in progress. ls crises occur two or three times a week.
^
not
on
There are numerous theories as to the pathology of these oculogj'ral crises. Many observers regard tliem as Marinesco12 and Radovici entirely functional. sP^ak of them as crises hysteriformes," as they found ^at attacks could be precipitated in their patients by Ejections of sterile water or by watching other Patients. Certainly there may be a superimposed Actional element in these crises, but the other signs definite organic disease which occur in the ^rkinsonian syndrome do not warrant our calling ese attacks hysterical. The paroxysmal nature of the tonic eye fits makes ?^e think that they are of the same origin as the yperkinesias, such as spasmodic torticollis, athetosis, cThese extra-pyramidal hyperkinesias are considered ^0 "
be due
to lesions in the neostriatum.
Bing, Popowa and Schwartz. ^Pmionis of striatal for
Such is the
Stertz considers
conjugate eye movements, Bing and Schwartz the hyperkinesias are Please phenomena due to failure of the inhibitory Pulses passing from the neostriatum to the pallidum. ^ is aiso possible that the oculogyral crises are ^ irritative phenomena. Although there is no 0;vi1 cortical centre subserving upward ocular devia^ a
centre
^0re ^cording to
"
k^nsient
cortical nidus is well known, which, will evoke deviation of the eyes in a
nevertheless
Simulation,
a
124
Encephalitis Lethargica
lateral direction. crises
One
can
conceive the lateral
oculogyral
from stimulation of such
resulting Russell,13 acting
as
centre.
a
0f
upon the suggestion stimulated the cortical oculomotor Hughlings Jackson, centre, having previously severed the external rectus
Risien
eye and the internal rectus of the other. The result was a tonic upward movement of the globes. The hypothesis was made of a common oculomotoi muscle of
one
centre in the
cortex, stimulation of which
in animals at least?the
deviation in picture these of
a
strongest
produced?"
effect, namely One can therefore
motor
lateral direction.
encephalitic oculogyral crises as the result impulses arising in a previously diseased cortical
centre for the
I wish to
representation of eye
movements.
express my thanks to the Honorary the Bristol General Hospital and of Southmead Hospital for giving Ilie
of
Physicians Dr. Phillips permission to
examine their my observations.
patients and
to
publish
REFERENCES. 1 2
Duncan, Brain, 1924, vol. xlvii., p. 7G. Naef, Muncli. Med. Woch., vol. Ixvi., 1919.
3
No.
Report of Local Government Board 121, London, 1918. 4 Hall, Lancet, April 14th, 1923.
5 6
on
Encephalitis
Kinnier Wilson, Croonian Lecture, Lancet, 1925, pp. Chambers, Brit. M. J., 1925, ii. 507.
LethargicCl' 1, 53,
1^'
7
Bing
8
A. Wimmer, Act.
9
No. 29, July, 192'* 84, p. 1,489. PaschefT, Arcliiv. d? Ophtalmologie, No. 12, 1925. Marinesco, cited after Wimmer. Risien Russell, Journal of Physiol., 1894, vol. xvii., p. 1.
10 II 12 13
and Schwartz, U Encephale, No. 3, 1925.
Young,
Psychet Netir., vol. i., Psycho., vol. viii.,
Jour. Neur. and
Hohman,
Jour. A. M.
A., 1925,
vol.
Ease. 2, 1926.
FOLLOWING LETHARGICA.*
MANIFESTATIONS
ENCEPHALITIS BY
(1
A. Michael Cmtchley, M.B., Ch.B. Bristol, asualty Officer, Royal Free Hospital; late House Surgeon, Bristol General Hospital.
TV 1
cephalitis Lethargica, from its initial phase final stages of chronic encephalitis, presents j rge variety of ocular signs and symptoms. These e
to a
eye some are so constant as varied, very yet ^ 0 be most valuable aids to diagnosis both in the early arLcl late stages of the disease. The following article is
anges
are
resume of the ocular manifestations of
argica based
upon
Patients. A.
Pupillary Abnormalities.
(1) Unequal Pupils.?Anisocoria
?Ccurrence
encephalitis
personal observations of 72
both
in
acute
and
is
a
fairly
common
chronic
encephalitis. in 29 of his series pupillary inequality ^Uftcan1 ^5 cases, associated in nearly every instance with ?ttie other defect, such as irregularity or impaired found
reactions.
(2) Irregular Pupils.?This group includes cases of "Circular and eccentrically placed pupils. Such gularities have frequently been described, though ent but rarely in my series. *
Barrett Roue Prize
Essay.
114
Mr. A. M. Critchley
(3) Argyll-Robertson Pupils.?The syndrome of a pupil reacting on accommodation, but not to light, is sometimes found in this disease, especially in the late stages, and unless other physical signs are present, difficulty in diagnosis may quite easily arise. The occurrence
in
encephalitis
of
Argyll-Robertson pupils?
knee-jerks, was first recorded in together 1919 by Naef2 of Munich. Since then it has been occasionally reported. with absent
(4) Sluggish Pupils.?In certain cases of chronic encephalitis the pupillary reactions, though present, may be
abnormally sluggish in their time reactions. Moreover, the amplitude of the reflex dilatation and contraction of the pupil may be abnormally smallDuncan found this impaired briskness of reaction h1 28 per cent, of his
(5) Failure
cases.
to React
on
Accommodation.
the most characteristic features
One oi
typical postencephalitic inability pupil to react o11 accommodation, whilst the reaction to light remains brisk. This abnormality is found in all types of chronic encephalitis, and is of immense value from a medicolegal standpoint, as it may be one of the few signs is
present in
a
case
encephalitis lethargica.
pupillary
reaction a
figure
a
of conduct alteration
attack of per cent.,
of
of the
an
on
following
aI1
In Duncan's series
accommodation
which is low
was
compared
lost in ^ with my
own.
B.
Disorders
Almost every some
in the
the
of chronic
Eyelids.
encephalitis presents a diagnosis of the
and
changes eyelids, can frequently be made phenomena alone.
disease these
case
of
from observation oi
Encephalitis Lethargica !?
Ptosis.?Ptosis has been
frequently
115
described in
phases of this malady, and may
either unilateral
or
bilateral.
at the onset of the
eP?rt3 in 1918 2-
The Government
disease.
epidemic encephalitis
on
?ver 50 per cent, of
in any stage be It is most commonly
cases
had
ptosis
states that
at the onset.
Lid Retraction.?The
typical staring gaze of the Parkinsonian is often accentuated by retraction of the eyelids. It gives a look of
Post-encephalitic a
definite
patient, and von Graefe's and ellwag's signs may be positive. This retraction is s?ttietimes variable, and is not always present. It may actually alternate with ptosis, so that at one examination e patient may have ptosis, and when next seen lid
^ophthalmos
to the
k
fraction
may be marked.
3-
Blepharoclonus.?This sign is said to be present on closing the eyelids, the lids are thrown into series of rapid clonic movements, of fairly large These clonic movements are regular in f^plitude. eir rate and amplitude, and persist so long as closure of the ej^elids is maintained. When e eyelids are closed in sleep, no movements are seen. Ccording to Hall4 this tremor is brought out and by an attempt on the part of the patient ^Xaggerated ?pen his eyes quickly. This flickering of the closed
J^untary .yelids
111 1
^ 0
*
resembles that
eing much
seen
in
neurasthenia, but differs
coarser.
Diminished Blinking.?Parkinsonians
blink less
are
often than the normal individual.
surface of the
eyeball
is
not
found As
a
adequately apt to injure the foreign bodies JUrictiva ; hence blepharitis and conjunctivitis may ^ ound as complications of this state. The infrequent lr*king is another factor in the production of the lcated,
and
are
116
Mr. A. M. Critchley
typical
The
Parkinsonian stare.
impression thus given
fixity of gaze ; suggesting, in turn, fixity of ideation or mental concentration. Such an impression
is
one
of
the may be quite false, for in the post-encephalitic facial expression frequently belies the true emotional content.
5.
Sign
Blinking
not
phenomenon, individuals, is
syndrome.
Wilson,5
in
unknown
though highly characteristic
Kinnier
Deviation.?This
Lateral
on
of the
who first
normal
Parkinsonian
reported
n>
described it
as follows in his Croonian Lecture f When the Parkinsonian is asked to make a series o successive right and left conjugate deviating movements of the eyeballs he very frequently blinks his "
...
eyelids position
the eyes pass from one extreme lateral to the other. The blinking movement,
as
which he appears to be unaware, is sometimes quick' In more than one case sometimes slow and deliberate.
quick double eyelid flicker has been distinctly and repeatedly observed." This sign may depend on the dryness of the conjunctiva through diminished blinking' a
necessitating the act of blinking before they can move easily. 6.
On
Normally
the
Convergence when
a
eyes converge, the gracefully lower.
to lubricate the
Eyelids
person looks at
pupils contract, In
a
remain
eyeS
up-
object, the
near
and the upper
encephalitis cad1 lS movement complex
chronic
component of this co-ordinate upset: the eyes do not converge, accommodation of the pupil is often lost, and the eyelids reman1 elevated. C. 1.
Disorders
Limitation
post-encephalitics
of are
of
Movements.
Movement. unable to
The
majority
converge, which
0f lb
Encephalitis Lethargic a ?f course but it is
|s
a so
limitation in the range of movements, frequent and important a sign that it
reserved for
^ability
J^Qve
to
a
some
converge,
their
lateral
eyes
in
a
deviation,
Punctuate
?ften of the
"
?l?Wness
cases
are
unable
particular direction.
only upward
movement may
less
rarely
?Wnward deviation. 2-
In addition to
separate paragraph.
Citation of full ocular
111
117
on
Movements.? Generalised
cog-wheel
"
to
This occur
and
rigidity,
type, together with
a
of movement (or bradykinesia), is generally und in Parkinsonism. A similar type of movement 18 present in the voluntary muscles of the eyeball, so ^at the eye moves in a characteristically jerky
smon. This phenomenon is best observed during eral movements of the It may possibly be eyes. e expression of a similar hypertonus in the extrinsic
1
e^ular muscles. 3-
Diplopia.?Often the only clue to the date of encephalitis lethargica is a history of "influenza" a cold," which was accompanied by a brief spell of uble vision. This diplopia is caused by a disturbance ^ synergic action of the muscles rather than by isolated Paralyse or paralyses. If the diplopia persists, and is Present in the chronic stages, there is then found some ?nset of '
Paralysis, though
rarely associated with strabismus. Ambers6 has pointed out that in some of these cases e diplopia is only present with distant fixation. lere is much to suggest that distant fixation is an a?tive function carried out by the simultaneous and
Anergic
contraction of both external recti, with a relaxation of the internal recti. Diplopia
^responding ay in such eakness 0l"
of
cases,
therefore, be the expression of
divergence.
No. 168.
a
118
Mr. A. M. Critchley 4.
Strabismus.?Strabismus is quite
common
in the
stages, but in the chronic phase is less often seen. Frequently the squint only occurs when looking in a acute
certain direction, and is found to be due to degree of paresis of a single muscle.
a
mild
5. Paralysis of Convergence.?One of the chief distinctions between Parkinsonism due to lethargica and that due to paralysis agitans is that m the latter there is never any difficulty in converging*
encephalitis
subject be very advanced in years, whilst the former convergence is lost. On attempting to converge, the eyes may look towards the nose temporarily, but almost immediately one eye W# deviate outwards again. unless the
in
6.
Oculo-gyral
Crises (Tonic Eye Fits).? These of immense interest, and the theories as t? their pathogenesis have caused considerable controversy* The crises consist in a spasmodic deviation crises
are
conjugate
of the eyes usually in an upward in paroxysms, and lasting for a few seconds to hours. or rather are part of, attacks and
are a
not
are
late
direction, coming 011 * period varying from
They
are
often
generalised convulsion. These complication of epidemic encephalitis?
common.
found only three cases of three hundred encephalitis >'
Bing7
tonic eye fits amongst Wimmer8 found five cases from
patients.
There is
some
becoming phenomena appears are
of
Ocular Symptoms.
found in my series of 72 percentage incidence as
was
and the
encephalitics, Young's9
of
suggest that they A fuller discussion of these
table shows the number of times
ocular manifestation with
similar number
later.
Incidence
following
a
evidence to
commoner.
The
accompanied by?
a
tables
ead1
chromc
compare^
:?
Encephalitis Lethargica
119
Table I. Number of Cases.
^lepharoclonus
Punctate eye
95
92
64
88
64
63
87
59
82
81
50
69
64
47
65
67
37
51
41
19
26
15
20
9
12
9
12
8
11
7
9
Diminished blinking
Loss of convergence Loss of accommodation "
Lid retraction
^togis
Unequal pupils Limitation of movements -^upil reaction poor
or
Strabismus ^uggish pupils
lost
.
Young's
69
movements
Blinking sign diplopia
Per-
centage. percentage
?
13
6
Oculogyral crises
4
?Irregular pupils
4
Argyll-Robertson pupils Nystagmus ^pil reaction to light poor
9
or
lost
The ocular signs may accompany any type of chronic eilCephalitis. My cases were of the following types :? Table II.
Parkinsonism Parkinsonism Parkinsonism Parkinsonism palilalia
Sleep
.
..
..
..
..
..
inversion
only Conduct change only
.
.
with respiratory disorder with conduct change with conduct change and ..
.
.
..
..
..
..
..
..
..
53 10 4 1 1 4
The value of the less obvious and finer ocular
^Ils
the
of chronic encephalitis in nonwill be seen from the following cases :?
diagnosis
^arkinsonians a'S
reactng6dto light but not Jjer
clon
the
beeil
on accommodation ; cannot converge. and on closing them there is blepharoinking is less frequent, and she blinks on looking to When looking to the right or to the left her eyes "ef?re her head. For the last twelve months she has
are
retra,cted,
to frequent oculogyric crises, occurring t^i^hject^uring her stay in hospital. When she is
e*cit
once
ri
she gets premonitory feelings
every tired or that she must look up;
122
Mr. A. M. Critchley
finally, she is impelled to gaze in an upward direction, and is then unable to lower her eyes. An attack lasts from half a minute to half an hour. During these crises she cannot close her eyes, which lacrimate freely. Coarse nystagmus in a The eyelids are retracted, her lateral direction occurs. forehead wrinkled, her head hyperextended, and there is a tremor of the cheeks. The mouth remains open, and she gives a Her whole body lS yawn and a sigh every few minutes. rigid and tremulous, especially marked on the left side. The patient is very agitated by these attacks, which at the time cause her pain at the back of the eyes, in the frontal region* and sometimes in the ear. All objects appear to be moving from side to side, but there are no visual hallucinations. When an attack comes on she lies down in a darkened room with something thrown over her eyes, and after about fifteen minutes she becomes able to lower her eyes. She then sleeps for several hours, as she feels too exhausted to pursue her normal occupation. 2.?Male, aged 25. This patient is a soldier, and gives no history of an acute attack of encephalitis lethargic?' but states that he was quite well until one day when on parade his eyes became fixed in an upward direction. He is now in a state of Parkinsonism. The pupils are regular, equal, reacting to light but not on accommodation. No convergence. Eye movements jerky. Lid retraction. Blepharoclonus. blinking on looking to side. His attacks occur daily, usually about two o'clock in the afternoon, just after he has returne Case
eyelid8
?
from exercise. His eyes turn up, but he can close his on doing so his eyes come down, only to go up again on his lids. These crises last for about eight hours. The pa^i?11 can terminate his attacks by going to sleep, and on awaking his eyes are normal. The paroxysms cause him no pain an no visual disturbance.
openi11^
3.?Male, aged 21. Onset of encephalitis, 1924. presents typical Parkinsonian appearance, and has disorder. Pupils equal, regular, reacting to light and not Case
respirat0^
accommodation. movements
jerky
Failure to converge. Double ptosis; blepharoclonus. The oculogyral crises
;
this case were of a peculiar type. They came on chiefly meal-times. His eyes would turn up, and his eyelids close tightly. This spasm of the orbiculares palpebrarum ^v0 persist for about ten minutes. These attacks occur sev times in the course of a day.
^
^ j
Encephalitis Lethargica
123
Case 4.?Male, aged 17. Encephalitis lethargica three and half years ago ; since then has developed a pronounced
^arkinsonian
condition. Pupils regular, equal, reacting to light accommodation. Convergence poor. Bilateral Ptosis. Blinking less frequent, blinks on looking to the side. epharoclonus. His attacks last about thirty minutes, during flich the eyes are deviated vertically, and with great difficulty e eyes can be brought down. There are no visual hallucinations, , ut he gets frontal headaches while an attack is in progress. ls crises occur two or three times a week.
^
not
on
There are numerous theories as to the pathology of these oculogj'ral crises. Many observers regard tliem as Marinesco12 and Radovici entirely functional. sP^ak of them as crises hysteriformes," as they found ^at attacks could be precipitated in their patients by Ejections of sterile water or by watching other Patients. Certainly there may be a superimposed Actional element in these crises, but the other signs definite organic disease which occur in the ^rkinsonian syndrome do not warrant our calling ese attacks hysterical. The paroxysmal nature of the tonic eye fits makes ?^e think that they are of the same origin as the yperkinesias, such as spasmodic torticollis, athetosis, cThese extra-pyramidal hyperkinesias are considered ^0 "
be due
to lesions in the neostriatum.
Bing, Popowa and Schwartz. ^Pmionis of striatal for
Such is the
Stertz considers
conjugate eye movements, Bing and Schwartz the hyperkinesias are Please phenomena due to failure of the inhibitory Pulses passing from the neostriatum to the pallidum. ^ is aiso possible that the oculogyral crises are ^ irritative phenomena. Although there is no 0;vi1 cortical centre subserving upward ocular devia^ a
centre
^0re ^cording to
"
k^nsient
cortical nidus is well known, which, will evoke deviation of the eyes in a
nevertheless
Simulation,
a
124
Encephalitis Lethargica
lateral direction. crises
One
can
conceive the lateral
oculogyral
from stimulation of such
resulting Russell,13 acting
as
centre.
a
0f
upon the suggestion stimulated the cortical oculomotor Hughlings Jackson, centre, having previously severed the external rectus
Risien
eye and the internal rectus of the other. The result was a tonic upward movement of the globes. The hypothesis was made of a common oculomotoi muscle of
one
centre in the
cortex, stimulation of which
in animals at least?the
deviation in picture these of
a
strongest
produced?"
effect, namely One can therefore
motor
lateral direction.
encephalitic oculogyral crises as the result impulses arising in a previously diseased cortical
centre for the
I wish to
representation of eye
movements.
express my thanks to the Honorary the Bristol General Hospital and of Southmead Hospital for giving Ilie
of
Physicians Dr. Phillips permission to
examine their my observations.
patients and
to
publish
REFERENCES. 1 2
Duncan, Brain, 1924, vol. xlvii., p. 7G. Naef, Muncli. Med. Woch., vol. Ixvi., 1919.
3
No.
Report of Local Government Board 121, London, 1918. 4 Hall, Lancet, April 14th, 1923.
5 6
on
Encephalitis
Kinnier Wilson, Croonian Lecture, Lancet, 1925, pp. Chambers, Brit. M. J., 1925, ii. 507.
LethargicCl' 1, 53,
1^'
7
Bing
8
A. Wimmer, Act.
9
No. 29, July, 192'* 84, p. 1,489. PaschefT, Arcliiv. d? Ophtalmologie, No. 12, 1925. Marinesco, cited after Wimmer. Risien Russell, Journal of Physiol., 1894, vol. xvii., p. 1.
10 II 12 13
and Schwartz, U Encephale, No. 3, 1925.
Young,
Psychet Netir., vol. i., Psycho., vol. viii.,
Jour. Neur. and
Hohman,
Jour. A. M.
A., 1925,
vol.
Ease. 2, 1926.
