Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5221_Библиотеки_им_академика_М_И_Перельмана.pdf
1
The Psychiatric Phenomena
of Encephalitis Lethargica
Paul Bernard Foley
Introduction
In early 1917, Constantin von Economo dened encephalitis lethargica on the basis of
seven patients he had attended in the Psychiatric and Neurological Clinic in Vienna.
One hundred thousand cases were subsequently documented around the world, most
in Europe and North America, but the number of mild and asymptomatic infections
was at least an order of magnitude larger. e largest single outbreak, in the United
Kingdom in 1924, was followed by its surprising disappearance as an epidemic disease.
Its pathogen was never identied; both major suspects, the inuenza and herpes simplex viruses, had been eliminated as candidates by 1930. Mortality varied between outbreaks, but the overall level during 1917 through 1924 was about 20 percent.
e oen- bizarre symptomatology of acute encephalitis lethargica— dominated
in 1917 by lethargy and oculomotor pareses, but supplanted from 1920 by contrastingly hyperkinetic features— persisted for days to months. For survivors, this was
just the beginning: recovery was rarely complete, and few resumed their former lives,
enduring instead a debilitating condition, “pseudo- neurasthenia,” characterized by
restiveness and mental torpor. A few months or years aer the acute illness, their situation deteriorated yet further, as post- encephalitic parkinsonism rapidly crippled
people decades too young for classical Parkinson’s disease (Foley, 2017; Stern, 1928;
von Economo, 1931).
Although its neuropathology was limited to the brainstem, encephalitis lethargica
also attacked the mind. Its diverse and oen baing psychiatric symptoms provoked
confusion and vigorous discussion throughout the 1920s and 1930s, challenging the
dichotomy of organic and functional mental disorders.
The mental symptoms of acute encephalitis lethargica
e relatively innocuous impairment of consciousness during acute encephalitis lethargica (and the absence of marked fever) distinguished it from other acute infectious and toxic disorders. Despite the peculiar lethargy for which the disorder was
Paul Bernard Foley, The Psychiatric Phenomena of Encephalitis Lethargica In: Medicine
DOI: 10.1093/ oso/ 9780192870414.003.0002
Infectious Disease and Neurocognition
named, the “sleeper” could generally be easily roused, was immediately oriented, and
could answer questions intelligently; they also woke spontaneously to eat or use the
toilet. Consciousness was only mildly impaired by an occupational delirium in which
they picked at their bedclothes or repeated pointless hand movements. Vague anxiety before or during the acute illness was sometimes reported, but profound indifference was typical, as described by a French military physician admitted to hospital
in Paris: “I saw those attending to me hurrying around; I was aware of their concern
and sometimes, despite their discretion, their sorrow. I was neither moved nor frightened by it . . . all my senses were preserved, but I felt no emotion, I witnessed my end
as a spectator” (Delater, 1920, p. 317). In a state described as “lucid catatonic stupor”
(Hesnard, 1920, p. 449), people could be suddenly immobilized in the middle of an action without consciousness being aected, despite their impassive face and muteness.
Some patients experienced hallucinations they knew to be illusions, oen toward evening or only when they closed their eyes (e.g., Geimanovich, 1927; Lhermitte, 1932).
Between acute and chronic encephalitis
lethargica: The pseudo- neurasthenic phase
Even aer the acute symptoms had subsided, the recovered person and their families
oen felt they were no longer the person they once were. Mental exhaustion, irritability, apathy, and a general loss of initiative remained; they dgeted and wandered
about aimlessly, driven by psychomotor restlessness (akathisia). eir intelligence
and memory were unharmed, but they seemed slow, forgetful, or withdrawn because
they needed to concentrate on everything they did. Few could return to work, particularly to mentally or physically demanding occupations, and unemployment added
to the general frustration and mild depression that typied their mood (Calligaris,
1926; Hesnard, 1920; Mäkelä, 1923; Parsons, 1928; Stern, 1928, pp. 186– 190).
e vagueness of these signs and symptoms could be dismissed as nothing more
serious than indications of a tedious recovery, or of conscious or unconscious
malingering; they might be ignored altogether if the acute phase had not been recognized, but the further evolution of the illness sadly claried their signicance. In
certain respects, the pseudo- neurasthenic phase of encephalitis lethargica resembled later chronic fatigue or post- viral fatigue syndromes, as well as some aspects of
the more recent “post- Covid condition,” the nature of which is as yet incompletely
dened (Soriano et al., 2022).
Nocturnal excitation crises and the postencephalitic behavioral syndrome in children
Children recovering from acute encephalitis lethargica were also restless, but in
much more dynamic fashion. ey experienced sometimes manic “excitation crises”
Encephalitis Lethargica 11
each evening (agrypnia): they were in motion throughout the night, muttering and
singing to themselves, running around, plucking and tearing at things, sucking and
chewing, touching and re- arranging objects, or dressing themselves to go outside to
play; some spat or defecated on the oor and smeared their beds and walls. e few
hours they slept during the day were never enough to overcome the weariness that
dissipated only as night fell (Findlay & Shiskin, 1921; Hofstadt, 1921).
e intensity of the nocturnal crises ebbed within a few months, but they were
succeeded by mental and behavioral changes that ranged from emotional lability to
abrupt and radical shis in character and behavior “otherwise seen only in the various forms of severe congenital psychopathy” (Hofstadt, 1923, p. 1761). e children
were now disobedient, aggressive, and unable to control their emotions or impulses.
Most observers concurred that such children would be unduly punished or spoiled
if le with their families; in any case, many parents felt compelled to seek outside assistance, particularly those in overcrowded homes, where family life might be completely destroyed (Auden, 1925; Parsons, 1928).
e post- encephalitis behavioral syndrome was characterized by emotional volatility, reduced ability to concentrate, and heightened sensitivity to external stimuli
(hypervigilance). Some children were mildly euphoric and mentally and physically
hyperactive, but sometimes depressed and irritable; others were withdrawn and discontented, and oen depressed or anxious; yet others were sociable to the point of
clinginess and eager to impress their doctors, but subject to explosive outbursts of
unmotivated anger or despair. ree phenomena attracted particular attention (reviewed by Auden, 1925; Kirschbaum, 1921; Neustadt, 1927; Dawson & Conn, 1931;
Stallybrass, 1926; Stern, 1924).
Psychomotor hyperactivity (hyperphrenia) encompassed the compulsion to
move, to touch and handle everything (and everyone) in sight, to incessantly chatter
in repetitive mixtures of conversation, enquiry, and displays of “cleverness,” to touch
and hit themselves and others, to tear at their clothes, and to recklessly act upon
each and every impulse as it arose. e activity suggested a grotesque exaggeration of
normal age- related behavior but oen seemed cheerless and forced. Respiratory tics,
continuous coughing, and paroxysmal snorting were common, as were other repetitive behaviors, such as nose picking, head rocking, nger snapping, blowing on ngers, and awkward gaits (Jellie, 1927b; Leyser, 1924; Parsons, 1928; iele, 1926).
Changes in personality were determined by the irritability, emotional lability, and
impulsiveness of the child, manifested as a thoughtlessly antisocial demeanor that
oen contrasted with their pre- illness disposition. In contrast to psychopaths, the
children were still conscious of what was acceptable, but were not guided by this
knowledge; inappropriate behavior reected their reduced capacity for resisting
motor and mental impulses (Bond & Appel, 1931; Borthwick, 1926).
Finally, attention decits could be so severe that defective memory and intelligence might be suspected, although some children could casually recall overheard
conversations or radio programs verbatim, albeit not always with understanding.
But their diculties in sustaining voluntary focus contributed to their impulsiveness
Infectious Disease and Neurocognition
and their uncontrolled responses to both external stimuli and their own uid emotional life (Fleck, 1927).
Infants who contracted encephalitis lethargica generally exhibited intellectual
decits, but whether intelligence suered in older children was ercely debated.
Psychomotor restlessness and attention problems (and, in many cases, parkinsonism) hampered both schooling and formal intelligence testing that required focused performance and the timely cooperation of the child. Sympathetic examiners,
however, oen found that intelligence, judgment, and imagination were reasonably
normal even in children uninterested in acquiring new knowledge or skills (Auden,
1925; Dawson & Conn, 1931; Lange, 1934; Parsons, 1928; iele, 1926).
More disturbing were problems of a sexual or violent nature. Psychosexual development could be accelerated (or halted) as early as the agrypnia phase, but sexual
urges were not always accompanied by the physical maturation required to act upon
them. Public masturbation, exhibitionism, and indecent language and gestures
were common, particularly among girls (Runge, 1928). Violence was impulsive and
opportunistic rather than purposeful, oen the result of momentary curiosity; an
8- year- old boy, for example, pushed another into the harbor “just to see someone
drown” (Runge, 1928, p. 604). Cruelty to animals was popular, as were vandalism,
pyromania, and other acts of destruction; older children might commit criminal
acts, including rape and murder, but minor delinquency was more typical (Auden,
1925; Hall, 1939; Runge, 1928; Wimmer, 1930). Self- mutilation included compulsive
picking at the skin wounds typical for encephalitis lethargica, sometimes leading to
perforation of the nose or cheek, gnawing of lips, ngers, or toes, or, as extreme examples, teeth extraction and enucleation or complete removal of eyes (Goodhart &
Savitsky, 1933).
Other antisocial manifestations included impulsive stealing (kleptomania),
whereby the usefulness of the stolen items played no role; in any case, they were usually abandoned shortly aer their capture. Impulsive lying, running away and aimless wandering, and Witzelsucht- like behavior (pathologic inability to be serious)
were generally irritating rather than consequential (Auden, 1925; Neustadt, 1927;
Royal Society of Medicine, 1925; iele, 1926).
e unstable temperament of children and young adults aer encephalitis lethargica caused diculties both for them and for those around them. Impulsive antisocial acts— including vagrancy, the, assault, and sexual misbehavior— drew the
attention of social and legal commentators, but trivial transgressions were more frequent than serious crimes. e legal status of children aer encephalitis lethargica
was nevertheless discussed in many countries. e British Ministry of Health noted
in 1924 that “it is possible that some instances of motiveless crime and of violent behavior are the result of this disease, and not, as might be supposed, of inherent vice”
(Ministry of Health, 1924); e Times reported this nding under the title, “A disease
which makes criminals” (Medical Correspondent, 1924). e United Kingdom
Mental Deciency Act (1913) was subsequently amended to recognize that people
of any age might be rendered legally incompetent by encephalitis lethargica, and in
Encephalitis Lethargica 13
most European countries similar insights prevailed. A variety of behavioral, psychological, and pharmacological approaches (primarily sedation) were tried in special institutions for children with post- encephalitic behavioral problems, without
marked success (Foley, 2012, pp. 449– 461).
e restiveness of the behavioral syndrome was driven by an “utterly amorphous,
objective, and non- directional discharge tendency” (iele, 1926, p. 55), in contrast to genuine compulsions, in which achieving a specic goal provides temporary relief. e child acted thoughtlessly, but not selshly. ey rarely attempted to
conceal their misdeeds, whether mischievous or more serious, and were genuinely
remorseful aerward; they did not attempt to justify themselves and oen confessed
to undiscovered misdeeds. Children described their inability to resist urges they
perceived as external to themselves, but not as voices or hallucinations. With time,
however, the once unremitting turmoil was broken by ever longer phases of critical insight and regret (Bostroem, 1930; Gerstmann & Kauders, 1924; Leyser, 1924;
Menger, 1929; iele, 1926).
e behavioral and mental changes in children with encephalitis lethargica
were suciently consistent and distinct from other conditions to identify their etiology. Nascent schizophrenia might be considered in the dierential diagnosis, but
hypersalivation, moist eyes, and a history of sleep problems were usually sucient to
clarify the diagnosis, even when the acute illness had been missed. One in ten adults
experienced similar behavioral changes following acute encephalitis lethargica, but
it was assumed that immaturity of the adolescent brain explained their greater frequency in children (Bonhoeer, 1922; Ebaugh, 1923; Gerstmann & Kauders, 1924;
Homburger, 1926; Lange, 1934; Neustadt, 1932).
Once the children reached the age of 15 or 16 years, parkinsonism began to limit
their ability to act on momentary impulses. However, observers could be astonished
by the persistence of hyperactivity, if in a new guise: “they seem as sti as an automaton, they run without signicant associated arm movements, more doll- like, like
a wind- up music box, to then fall more or less rapidly into immobility when the
impulse to move has expired” (Hofstadt, 1923, p. 1759). Even as adults with severe
parkinsonism, they could remain roguish and spirited, as shown by some of the residents of the New York Beth Abram Hospital temporarily liberated by L- DOPA (levodopa, L- 3,4- dihydroxyphenylalanine) in the early 1970s (Sacks, 1991). Persistent
behavioral problems of a more serious nature were possible (see, e.g., Makowski,
1983) and in some institutions justied almost continuous sedation or even frontal
lobotomy (Wilson et al., 1952).
Other attention deficit and hyperactivity disorders
e hyperkinetic condition in young children described by Franz Kramer and
Hans Pollnow in 1932, now regarded as the rst report on the attention decit/ hyperactivity disorder, shared some features with the post- encephalitic behavioral
Infectious Disease and Neurocognition
syndrome but lacked marked neurologic symptoms and had a better prognosis
(Kramer & Pollnow, 1932). While the post- encephalitis behavioral syndrome might
have been accommodated in the International Classication of Diseases (ICD)- 10
category “Habit and impulse disorders” (F63)— “characterized by repeated acts that
have no clear rational motivation and that generally harm the patient’s own interests and those of other people. e patient reports that the behavior is associated
with impulses to action that cannot be controlled” (World Health Organization,
1992, p. 517)— the ICD- 11 category “Impulse control disorders” would exclude it
as the patient does not nd the behavior rewarding even initially (World Health
Organization, 2022).
The mental symptoms of chronic encephalitis
lethargica in adults
Parkinsonism dominated chronic encephalitis lethargica in adults, but psychiatric and other mental problems were also prominent. As for children, there was
no compelling evidence that pre- illness factors inuenced the risk of psychiatric
symptoms. Two related phenomena were characteristic of chronic encephalitis lethargica: bradyphrenia and impaired volition.
People with chronic encephalitis lethargica complained that their thoughts no
longer owed freely, that concentration was dicult, and that they experienced
states in which they “had no sense of their own existence” (Bychowski, 1923,
p. 213). Sensory input and interactions with others lacked emotional coloring,
leading to a certain “forgetfulness” and disinterest, to the extent that many seemed
unaware of the gravity of their illness. In 1922, Swiss neuropsychiatrist François
Naville introduced “bradyphrenia” (from Greek, “slow mind”) to encompass this
combination of mental viscosity and apparent apathy, unique to encephalitis lethargica (Naville, 1922). People with bradyphrenia found directing their attention arduous, making it dicult to respond to internal or external stimuli and
crushing creativity; “they collapse into themselves, their relationships are reduced
to a strict minimum, all their needs seem diminished, their instincts of sociability
and their moral personality atrophied, their initiative destroyed, their mind slumbers, nothing concerns them, everything tires them, and their will, however well
preserved, ultimately capitulates” (Naville, 1924, p. 42). Another Swiss neuropsychiatrist, Hans Steck, saw bradyphrenia as an impairment of psychomotor executive power characterized by mental fatigability and apathy that ultimately levelled
the entire personality (Steck, 1931). Cognition was stalled by perseveration and
the perceived need to “want” to think. Response times in tests of free association
and other psychological tests were longer, modifying a response was dicult once
started, and concentration quickly agged. Situational factors could temporarily
overcome bradyphrenia; otherwise slow- moving patients might play cards with
spirit, for example (Fleck, 1930).
Encephalitis Lethargica 15
e personal impact was devastating, as described by one 22- year- old man: “ere
is no action for which I did not consider every consequence . . . every bodily process,
such as sneezing, coughing, and even thinking, lled me with passionate curiosity
as to how this process worked . . . Any thought that would normally be only mildly
discomforting, that one usually overcomes without eort, causes me this feeling of
anxiety” (Mayer- Gross & Steiner, 1921, pp. 288, 294). e young man undertook
nothing with enthusiasm or pleasure; he felt more like a spectator than a participant
in his own life. Less frequent was a sensation of mental acceleration, similar to the
drivenness typical for children with encephalitis lethargica. e French doctor mentioned previously noted 28 years aer his acute illness that it was “as if I am under
pressure and about to burst, especially when I have to express something that interests or moves me; I speak volubly and cannot take time to think, I rush feverishly to
the end of what I have started, towards something uncertain, and am vaguely anxious that my power [to speak] might suddenly fail me” (Delater, 1948, p. 230). Some
people found that what they saw, heard, or felt— the ticking of a watch, the sound of
a passing tram, or an odor, for example— persisted for longer than was objectively
justied (for instance, Novelli & Quagliotti, 1929). Bradyphrenia shared some features with “subcortical dementia” later described in progressive supranuclear palsy
(Albert et al., 1974) and the “obsessional slowness” of obsessive– compulsive disorder (Hymas et al., 1991).
About one in three people with post- encephalitic parkinsonism experienced
bradyphrenia, usually before motor symptoms developed. Some authors nevertheless argued that it was the conscious eort invested in motor performance that
was the primary problem; as mental and motor activities once silently facilitated by
unconscious automatisms now required their full attention, nothing proceeded as
smoothly or as quickly as it once did. With time, bradyphrenia deepened to akinesia, and the blockage of thought ow could no longer be overcome by conscious
strategies.
Many authors regarded a related phenomenon, “decit of drive,” as a distinct element of chronic encephalitis lethargica and distinguished two types of volitional deficit. In the rst, a person asked to wipe their nose responded slowly, and the action
might break down at any point; in the second, even a delayed response, once begun,
would proceed smoothly. For people in the rst group, emotional indierence meant
that many stimuli had neither the emotive content nor the motivating eects they
once did; for example, an itch was still unpleasant, but the attempt to relieve it was
slow and uncoordinated, or they could recognize the beauty of a musical performance without being moved by it. e problem might be overcome by increasing
the emotional content of the stimulus; danger and the insistence of a doctor or relative were particularly eective, or even specically imagining such motivators to
enable a response. In people with the second type of drive decit, their deeper apathy
hindered conscious registration of stimuli and compromised even the initiation of
decision- making. Patients in the rst group were less oppressed by their illness than
those in the second, and they retained a remarkably rich internal life, despite the
Infectious Disease and Neurocognition
gradual “rusting” of their thought processes (Bostroem, 1930; Hauptmann, 1922;
Runge, 1928; van der Scheer, 1936; reviewed by Foley, 2012).
People with chronic encephalitis lethargica seemed emotionally detached, but
some explained they would be more expressive if their volitional and motor problems had allowed it; indeed, some wrote aectionate letters to loved ones they clearly
missed (Jakob, 1923, pp. 200– 201). Oliver Sacks similarly recorded the general serenity of his patients in the early 1970s, several decades aer the acute illness; they
were curiously unperturbed by their situation when temporarily “awakened” by LDOPA, although fully cognizant of lost time (Sacks, 1991). Irregular emotional and
petulant outbursts directed at people or property reected underlying irritability,
but these transitory eruptions (comparable with those of the behavioral syndrome
in children) oen subsided because the patient recognized they were irrational.
Depression was relatively unusual and typically related to feelings of personal inadequacy rather than despair, although many people calmly expressed sorrow
when questioned about their illness (Fleck, 1927; Geimanovich, 1927; Paulian &
Stanesco, 1932). Suicide was less common than earlier in the illness, but suicidal
thoughts were occasionally sucient to break through the akinesia of parkinsonism (Fleck, 1933).
e typical importunity or clinginess of chronic encephalitis lethargica, together
with “pathologic curiosity” and “pathologic lack of boredom,” extended to the irksomely incessant pestering of hospital sta with repeated questions; some patients
meticulously monitored everything that happened around them, whether in hospital or at home (Chodos, 1933; Jacob- Klener, 1934).
Adults, including people with advanced parkinsonism, could also experience
the impulsive motor activity and akathisia seen in children aer acute encephalitis
lethargica. Compulsive thoughts, usually associated with compulsive motor phenomena, were oen rhythmic in nature (melodies, short repetitive phrases) and
less meaningful than in other psychiatric disorders, but some were more disturbing,
including incitements to socially inappropriate or dangerous behavior. As in children, the thoughts were perceived as intrusive and foreign, but not usually as voices
(Bostroem, 1930; Chłopicki, 1931; Kehrer, 1938; Runge, 1928; Stern, 1928). Speechrelated compulsions included palilalia (including the repetition of heard speech or
silent lm card text), screaming or shouting (klazomania), and compulsive singing,
whistling, and humming (Benedek, 1925; von urzó & Katona, 1927).
Oculogyric crises
Oculogyric crises were a complex, spectacular neuropsychiatric phenomenon that
aicted 15– 20 percent of people with post- encephalitic parkinsonism but were
otherwise unknown and clearly dierent from recognized ocular palsies. Every few
days or weeks, foreshadowed by a short period of vague apprehension or depression, both eyes of an aected person rolled up until only the lower iris was visible;
Encephalitis Lethargica 17
their neck arched backwards (opisthotonos), their mouth gaped open, and their
extrapyramidal and vegetative symptoms, including hypersalivation, intensied.
During the attack, a person could force their eyes down for only a few seconds.
e episodes were initially brief but could later last several hours, usually starting
in the late aernoon or evening and ending only when the person fell asleep naturally (i.e., without pharmacological aid) (Delbeke & van Bogaert, 1928; Ewald,
1925; Jellie, 1932; McCowan & Cook, 1928b; Onuaguluchi, 1961; Stern, 1927;
Walusinski, 2022).
An integral part of the attack was the alteration of mental state that commenced
before the ocular manifestations. Compulsive, oppressive thoughts caused deep anxiety and a frightening “sensation of annihilation,” seizing control of consciousness in
a state described by one man as “something between speaking and thinking” (Stern,
1927, p. 528): his head was lled with senseless words and sentence fragments, his
thought processes xed to the spot. e thoughts were typically nonsensical and repetitive (e.g., counting the vowels in conversations not otherwise heeded) and oen
rhythmic (e.g., children’s songs, mathematical sequences). Some people developed
strategies to temporarily reassert control; for instance, one man compelled to recite “how, why, what for, what” could escape by thinking “Depart from this place,
Satan, for Jesus is mine,” but “nding” this thought could itself be dicult (Stern,
1927, p. 530). Others were overwhelmed by a ood of oppressively banal memories.
Whatever thoughts engulfed them, voluntary concentration and cognition was impossible throughout the attack, and they might consequently respond with irritation
to anyone who asked them questions or made demands.
Altered perception of time or space, local or generalized anesthesia or paresthesias, visual (less oen auditory or tactile) hallucinations recognized as delusions, and distortions of body perception frequently accompanied the attacks,
as did a sense of heightened consciousness similar to the aura of epilepsy (and
also reported by some people outside of oculogyric crises). Oculogyric crises
could be “contagious” on a hospital ward (in the sense that yawning can be “contagious”), but only among people who already experienced such attacks (Delbeke
& van Bogaert, 1928; Marinesco et al., 1925; Stern, 1928, p. 166; van Bogaert &
Delbeke, 1926).
Contemporary authors could not satisfactorily explain the genesis of oculogyric
crises but suspected the involvement of dissociated sleep phenomena or disturbed
vestibular function (or both). Comparisons were drawn with “subcortical attacks”—
characterized by an aura, motor, sensory, vegetative, and mental symptoms, and paroxysmal features such as cataplexy and narcolepsy— thought to reect the liberation
of behavioral automatisms and elementary basal ganglia and brainstem motor patterns from higher control (Zingerle, 1936). Oculogyric crises have since been observed in patients treated with various psychoactive medications, chiey dopamine
D2 receptor antagonists, and occasionally in other neurological conditions (Slow &
Lang, 2017). eir pathogenesis probably involves dopamine receptor hypersensitivity, but the phenomenon is still only incompletely understood.