Journal of Autism and Childhood Sch&ophrenia, Vol. 5, No. 2, 1975
On the Organic Nature of Some Forms of Schizoid or Autistic Psychopathy S. S. M n u k h i n ~ a n d D . N . I s a e v 2
Leningrad Pediatric Medical Institute Leningrad, U.S.S.R.
Editorial Comments. It is increasingly recognized that organic brain
damage plays a major etiological role in infantile autism and related disorders of communication. Facts that particularly prompt this conclusion include the frequent occurrence of behavioral disturbances resembling those of classical autism in diseases such as phenylketonuria and tuberous sclerosis; the high incidence of seizures in infantile psychosis (Rutter & Lockyer, 1967; Rutter, Greenfeld, & Lockyer, 1967); the idiosyncratic behavioral reaction shown by many psychotic children to neuroleptic drugs (Campbell, Fish, David, Shapiro, Collins, & Koh, 1972); and the often abnormal prenatal and early developmental histories of many of these children. Although the last-mentioned association is known anecdotally to pediatricians and neurologists, it has rarely been documented in detail and is a major theme of the article by Mnukhin and Isaev that follows. The authors' patients, carefully followed from childhood into late adolescence, showed an impressive frequency of abnormalities of pregnancy and delivery of a kind capable of causing cerebral hypoxia. Furthermore, the physical examination frequently indicated "dysmorphic" features hinting at underlying cerebral dysgenesis. From these observations and a review of relevant literature, the authors argue cogently that organic brain damage constitutes the basic etiology of their patients' behavioral difficulties. Their report is of additional significance since it presents what is probably the first detailed account in
' Deceased. 2Requests for reprints should be sent to Dr. Dmitry N. Isaev, Professor of Psychiatry and Chairman of the Department of Psychiatry, Leningrad Pediatric Medical Institute, 2 Litovska}a Street, Leningrad, 194100, U.S.S.R. 99 9 P l e n u m Publishing C o r p o r a t i o n , 2 2 7 West 1 7 t h Street, N e w Y o r k , N . Y . 1 0 0 1 1 . NO part o f this p u b l i c a t i o n m a y be r e p r o d u c e d , stored in a retrieval system, o r t r a n s m i t t e d , in any f o r m or bY any means, e l e c t r o n i c , m e c h a n i c a l , p h o t o c o p y i n g , m i c r o f i l m i n g , recording, or o t h e r w i s e , w i t h o u t w r i t t e n p e r m i s s i o n o f t h e p u b l i s h e r .
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the English language of clinical experience with childhood psychosis in the Soviet Union. Lorcan O'Tuama, M. D. Associate Professor o f Medicine (Neurology) and Pediatrics Chief, Section o f Pediatric Neurology School o f Medicine University o f North Carolina
REFERENCES Boullin, D. J., Coleman, M., O'Brien, R. A., & Rimland, B. Laboratory predictions of infantile autism based on 5-hydroxytryptamine efflux from blood platelets and their correlations with the Rimland E-Z score. Journal of Autism and Childhood Schizophrenia, 1971, 1, 63-71. Campbell, M., Fish, B., David, R., Shapiro, T., Collins, P., & Koh, C. Response to triiodothyronine and dextroamphetamine: A study of preschool schizophrenic children. Journal of Autism and Childhood Schizophrenia, 1972, 2, 342-358. Ornitz, E. M. Childhood autism - - A review of the clinical and experimental literature. California Medicine, 1973, 118, 21-47. Rutter, M., Greenfeld, D., & Lockyer, L. A rive- to fifteen-year followup study of infantile psychosis. II. Social and behavioral outcome. British Journal of Psychiatry, 1967, 113, 1183-1199. Rutter, M., & Lockyer, L. A rive- to fifteen-year followup study of infantile psychosis. I. Description of sample. British Journal of Psychiatry, 1967, 113, 1169-1182.
ON T H E O R G A N I C N A T U R E OF SOME FORMS OF SCHIZOID OR AUTISTIC P S Y C H O P A T H Y Representative case histories are presented to illustrate the typical clinical features o f autistic psychopathy. These patients showed several clinical features compatible with organic encephalopathy. Physical signs suggesting the presence o f an underlying cerebral clysgenesis were particularly frequent. These patients superficially resembled schizophrenics but differed in their delayed and peculiar intellectual development, the response o f their behavior to external stimuli, and their frequent and severe motoric defects. From these observations and a review o f the pertinent #terature, we conclude that the patients clescribed in this report have an organic deficiency of brain function, determined congenitally or in the early postnatal period.
The purpose of this paper is to describe a symptom complex generally termed autistic psychopathy, to emphasize the role of exogenous organic
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factors in its etiology, and to distinguish it from schizophrenia. To accomplish this aim we present short extracts from case histories of some of our patients. BACKGROUND
Early workers, notably Krepelin and Birnbaum, delineated the clinical picture of abnormal personality and emphasized its characteristic disorders of behavior and thought. Ozeretski9 (1938) noted the preference of schizoid psychopaths for solitude, their tendency to reasoning, obsessional interests, motor incoordination, and impulsiveness. The terms pathologically reserved and autistic were also applied to this group (Gurevich, 1932; Giliarovski 9 1954; Sukhareva, 1959). More recently, behavioral disturbances related to but separable from classically described schizophrenia have become of intense interest to psychiatrists. Kanner's description of infantile autism (Kanner, 1942-1943) spurred interest in this group of patients. Asperger (1944) observed autistic psychopaths and stressed their tendency to show motor incoordination, marked scattering of abilities, impoverished affect, and thought disturbance; they are incapable of putting themselves into the state of mind of another person. Several authors noticed the association of this syndrome ~with organic brain disease, including epilepsy (Grunberg & Pond, 1957), postencephalitic states (Van Krevelen, 1960), various organic encephalopathies, temporal lobe pathology (Sarvis, 1960), and postnatal trauma (Ruyter, 1961). Schain and Yannet (1960) stressed the frequency of clinical features suggesting organic brain disease in autistic children. In their patients, mental retardation and speech difficulties were common and almost one-half suffered from epilepsy, Frederiks (1964), Grunes and Szyrynski (1965), and Schopler (1965) felt that childhood autism is attributable to sensory deprivation resulting from dysfunction of the reticular formation. Despite this abundance of data indicating a connection between some forms of schizoid or autistic psychopathy and early organic brain disease (Nissen, 1963; Davies, 1966; Mnukhin, Zelenetskaja, & lsaev, 1967), some contemporary workers still consider them as diseases more akin to schizophrenia (Planansky, 1966), and others (Horvai, 1966) have poirtted6oE the difficulty of distinguishing symptomatically between some cases of schizophrenia or schizoid states and the psychopathiclike state described above which is caused by organic brain disease. We have taken the position repeatedly that most cases of autistic psychopathy are due to antenatal, intranatal, or postnatal organic damage of the brain and that these are caused by serious forms of dystrophy. This point of view has been based on out 27 clinical observations, some of which w› now wish to report. J
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CASE HISTORIES N.F. N.F. is ten years of age. His mother suffered from toxemia in the first half of pregnancy and his birth weight was eight pounds. He had a serious febrile illness during his first year and was thought to be moribund. He walked at the age of one and talked at the age of three, At the age of three he had a serious form of scarlet fever which was complicated by otitis and simultaneously he had dysentery. His early mental development was quite good and he began to read at an early age. From the age of eight years behavioral aberrations appeared, such as a compulsive washing of his hands, which he attributed to their smelling of glue. He also had some " f i t s , " as he described them, with loss of consciousness sometimes accompanied by feelings of thirst and dryness in his mouth. At the age of eight he had speech therapy, which continued through the preparatory and first grades. He was hospitalized because of behavioral peculiarities and was noted to be unusually withdrawn and reserved. He spoke frequently about inventions such as "autoclocks." His behavioral peculiarities in the classroom included biting his pencil and penciling his brows with ink. He would constantly lick his fingers. He was extremely restless and was not interested in books or in television. He was noticed to have very poor skills in arithmetic and to be lacking in concepts of time and space, but he could read fluently and write grammatically. Physical Examination. He was overweight and had a poor physique with hypogenitalism. He had a slight enlargement of the head. The second, third, fourth, and fifth toes on the right foot were hypoplastic. Syndactyly of the toes of the right foot and of the third and fourth toes of the left foot were noted. He had a high palate. Neurological Examination. His cranial nerves were normal, but he was felt to have muscular hypotonia. No pathological reflexes were found. An EEG was interpreted as indicating diffuse encephalopathy. He had two seizures in the hospital, one during an operation on his finger and the other during a lesson. These seizures were accompanied by loss of consciousness with generalized tonic convulsions. Five years after this hospitalization it was learned that he was in a school for the mentally retarded, was making good academic progress, and was even tempered, well disciplined, and taking an interst in radio mechanics. He was able to help his parents at home but had difficulty with detailed work because of motor incoordination. He appeared attached to his parents, but his emotional reactions generally were blunted.
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S.M. S.M. was thirteen years o f age. He was a f i r s t b o r n child with a normal birth history. He was noted to be restless from an early age and was easily distractible with a short attention span. He tended not to play with other children and in kindergarten was noticed to be behaviorally quite different from his peers. At the age o f eight he attended a school for the mentally retarded and was noticed to have poor learning ability with particular difficulty in arithmetic. He was admitted to our hospital at the age o f twelve because o f irritability, easy excitability, and aggressive outbursts. His intellect was subnormal. He learned to read and write but could not learn how to count. He could add concrete objects only up to the number 20 and would make mistakes when recounting. He showed poor response to teaching and was unable to complete easy work in spelling Russian. He could not complete words and made several mistakes in copying. He did not know his teacher's naine, the seasons, or the months o f the year and could hOt distinguish his body parts or imitate rhythms. He recounted bizarre episodes in which he said he had seen two giants with guns and without eyes. This turned out to be related to a story that he had read not long before. His concepts o f space orientation developed slowly. His affect was generally inappropriate and fluctuated between his being whining and irritable and periods when he would be somewhat euphoric. Despite these difficulties his expressive language was good and he could use complicated phrases such as " I entreat you to discharge me from the hospital." Physical Examination. He had a poor physique and was overweight. He had marked hirsutism o f the lower extremities. His ear helixes were poorly differentiated. He had fiat feet and the first and second toes of his feet were widely spaced. His hands and feet were large and coarse. He had pigmented areas on his back and had kyphoscoliosis. Neurological Examination. He had facial asymmetry with general hypotonia and motor incoordination. He had particular difficulty with fine movements of the fingers. Five years after this hospitalization it was learned that he had received an eighth-grade education at a school for mentally retarded children and at that time was working at a medical labor center. He had great difficulty with all operations requiring motor dexterity and his general behavioral traits had not greatly changed. /./. I.I. is 14 years old. His birth history was normal. Early retardation o f development was noted and he " d r a g g e d " his right foot until the age o f
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rive. He did not speak until he was four. From a very early age he was noted to be quick tempered, given to daydreaming, distractible, and uncooperative. He had to leave school at the age of 71A because of extreme restlessness and lack of attention. At the rime of his admission he was studying sixth-grade material. He had no friends and liked to play with younger children. He was excitable and easily frustrated, often threatening those around him. His actions were quite impulsive, as when he went to visit a hospital for mental diseases and was detained there because of odd behavior. At 12 years of age he suffered from cerebral concussion and began to have seizures. During the seizures he became pale, felt fearful, and fell to the ground. These spells were accompanied by breathing difficulty and disorientation. His physical examination was remarkable only for mild facial asymmetry. At the most recent follow-up he was attending a technical training school f o r the handicapped. He has difficulty learning trade skills because of motor clumsiness. He is hOt motivated to learn at this school and dreams of working as an inspector in the militia. He is restless, irritable, and sociable with those whom he likes, but his life adjustment is poor and he cannot defend himself. V.B. V.B. is 15 years old and has a father who uses alcohol excessively. Birth history was normal except for neonatal jaundice. He walked and talked by one year, when he suffered from toxic dyspepsia and lost the ability to walk for several months thereafter. He had measles, scarlet fever, and hepatitis. He showed striking behavioral peculiarities from early childhood, playing by himself, not fraternizing with other children, and being given to daydreaming. From the age of 7 he had great difficulty with arithmetic, though he learned quickly to read and to write. At the time of admission, he was in the eighth grade. He asked unnecessary questions, read a great deal, and wrote verses. He had ruade poor academic progress, especially in mathematics. His concepts of time were developing slowly. He had frequent episodes of depression and felt that his thinking had been worsened by medication. He showed depersonalization periodicaUy and was often irritable and importunate. He did not attend school regularly. Physical examination showed marked smallness of stature. There was asymmetry of the face and of the palpebral fissures. Skull films showed epiphysial calcification. DISCUSSION These short extracts from the case histories of four patients provide a representative sample of the characteristic clinical picture presented by the
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group of children and adolescents we have studied. Their peculiar characteristics are striking, even on cursory examination, especially their motor clumsiness and highly bizarre manner of interacting with people around them. Some of them have no marked intellectual deficit but most have an unusual combination of such deficits, viz., difficulty with reading, writing, and calculation; disturbed body-image recognition; poorly developed concepts of time and space; and difficulty in performing tasks involving sequential acts. They are given to pointless verbal philosophizing and illogical reasoning and have a tendency to ask unnecessary questions and to make accusatory statements. Many have obsessive restricted interests in, for example, calendars, geographic maps, and similar objects. They are often preoccupied with fantasies which they will sometimes act out, as when they " p l a y " such scenes as "taking part in battles" or "pursuing bandits." The content of their fantasies tends to be limited and seems to be derived from books they have read or from the conversation of those around them. Despite their intellectual retardation, they can usually speak quite weU, have a good mechanical memory, and often attract attention by using elaborate sentences which vary in their relevance to the context. Their behavior lacks any clear aire and is characterized by a succession of apparently unmotivated acts which seem to arise as a response to accidental external stimuli. They give the impression of being able to perform any task, provided they are stimulated energetically and systematically. Affectively, these patients also present many peculiarities. They lack deep-seated emotions and affections and often show less than the expected degree of inhibition in unfamiliar situations. For example, at school they frequently stand up during the lesson, walk about the classroom, ask unnecessary questions, or begin to sing. Despite their behavioral difficulties, these patients sometimes make satisfactory academic progress and may achieve a seventh- or eighth-grade education in schools for the mentally retarded. This is accomplished in the great majority of cases through their parents' constant help and systematic stimulation. However, irrespective of their scholastic progress, they are generally helpless in adult life and seldom master a trade. From the foregoing description, it can be seen that the intellectual, affective, and behavioral disturbances shown by this group of patients could easily lead to their being considered as suffering from schizophrenia. Their disease would then be regarded as a psychopathic change in personality occurring after a schizophrenic deterioration in early childhood or as a manifestation of a slowly developing schizophrenic process. As they grow older their behavior would become progressively more eccentric and less adjusted to life. When admitted to adult psychiatric hospitals, they would in fact be regarded as suffering from long-standing schizophrenia and would be treated ineffectively with insulin shock and psychotropic drugs in high dosage.
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However, a consideration of the history of these patients allows us to hold with a high degree of certainty that their disorder is not caused by a schizophrenic process or by a stable postschizophrenic change in personality. In the overwhelming majority, the developmental history points to exogenous organic disturbances. These include abnormalities of pregnancy or delivery and a high incidence of the minor diseases of childhood (toxic dyspepsia and dysentery, among others). Physical examination in many cases hints at the saine conclusion and shows poor stature, signs suggestive of endocrinopathy (especially obesity and precocious puberty), and various "dysgenetic" signs (syndactyly, hypoplastic fingers, high palate, poorly differentiated helixes, and flat-footedness, for instance). Some show neurological " s o f t " signs. We feel that the epileptic seizures frequently seen in these patients should also be construed as indicating an organic neurological basis for their syndrome, and we reject the view that these seizures represent a combination of schizophrenia and epilepsy, the so-called schizoepilepsy. Several of the clinical features shown by these patients would be difficult to reconcile with the conclusion that they are schizophrenic. Their delayed and peculiar intellectual development, the response of their behavior to external stimuli, and the frequency and severity of their motoric defects would ail be unusual features of schizophrenia. While their emotional state bears some resemblance to that of schizophrenics, a careful analysis shows features which are characteristic of this group of patients and unlike those shown by schizophrenics. These features include their obsession with restricted interests, their tendency to fantasize, and their inappropriate lack of inhibition. These features can hardly be regarded as indicating emotional poverty, particularly since these children seem quite deeply attached to their parents and close relatives. These observations lead us to think that the group of patients described in the present study and generally classified as s c h i z o i d or autistic has a gross organic deficiency of brain function, either congenital or arising in the early postnatal period. The observations suggest that diencephaiic and brain stem systems rather than cortical systems are primarily affected and there may also be involvement of the reticular formation and of the limbic system. The attention defects, affective disturbance, reduced activity level, and the aimless s t i m u l u s - b o u n d behavior that we have described support this conclusion. REFERENCES
Asperger, H. Die autistischenPsychopathenim Kindesalter.Archiv far Psychiatrie und Nervenkrankheiten, 1944, 117, 76-136. Burns, C. Autopathy:FoUow-upof cases. Acta Paedopsychiatrica, 1964,31, 257-262.
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Davies, G. L. Autism--Schizophrenic syndrome of childhood. Medical Officer, 1966, 115, 225-227. Frederiks, J. A. Sensore deprivatie. Nederlands Tijdschrift voor Geneeskunde, 1964, 108, 749-757. Gil{” ~, V. A. Psikhiatriia [Psychiatry]. Moscow, 1954. Grunberg, F., & Pond, D. A. Conduct disorders in epileptic children. Journal of Neurology, Neurosurgery, and Psychiatry, 1957, 20, 65-68. Grunes, W., & Szyrynski, V. Secondary pseudoautism caused by physiological isolation. Journal of Consulting Psycho~gy, 1965, 29, 455-459. Gurevich, M. O. Psikhopatologiia detskogo vozrasta [Childhood psychopathology]. Moscow, 1932. Horvai, I. Einige Probleme der Differentialdiagnose zwischen Schizophrenie und schizoider Psychopathie. Wiener Zeitschrift fur Nervenheilkunde und Deren Grenzgebiete, 1966, 24, 60-63. Isaev, D. N. Kliniko-eksperimental'nye korreliatsii pri nekotorykh formakh oligofrenii u detei. Materialy 2-ogo Vseross. s'ezda nevropatologov ipsikhiatrov (Clinico-experimental correlations in certain forms of oligophrenia in children. Proceedings of the second All-Russian congress of neuropathologists and psychiatrists.) Moscow, 1967. Pp. 238-240. Kanner, L. Autistic disturbances of affective contact. Nervous Child, 1942-1943, 2, 217-250. Kraft, T. B. Is there a familial degenerative (sc.hizoid) psychopathy? Schweizer Archiv/~9 Neurologie, Neurochirurgie und Psychiatrie, 1959, 84, 110-121. Mnukhin, S. S. O nevro- i psikhopaticheskikhizmenenil'” lichnosti na pochve t 8 form alimentarnogo istoshcheniia u detei. Zh. Nevropatologii i psikhiatrii (On neuroand psychopathological changes in the personality due to severe forms of undernourishment in children. Journal of Neuropathology and Psychiatry) 1947, 16, 75-77. Mnukhln, S. S. Ob osobom tlpe neravnomernogo pslkhlcheskogo nedorazvltna u detel. Materialy 3-ogo Vsesoiuznogo s"ezda nevropatologov i psikhiatrov (On a special type of irregular mental retardation in children. Proceedings of the Third Ail-Union Congress of Neuropathologists and Psychiatrists). M,_oscow, 1948. Mnukhin, S. S. O roli rannikh ostrykhdispepsii v proiskhozhdenii nervnopsikhicheskikh rasstroistv u detei. Voprosy detskoipsikhonevrologii (On the role of early acute dyspepsia in the genesis of neuropsychic disorders in children. Problerns of child psychoneurology). Moscow, 1958, pp. 38-44. Mnukhin, S. S. O kliniko-fiziologichesko 9 sostoianii obshchego psikhicheskogo nedorazvitiia u dete9 Trudy instituta ira. 1I. M. Bextereva (On the clinicophysiological classification of conditions of general mental retardation in children. Works of the II. M. Bexterev Institute) (Vol. 25). Leningrad, 1961, pp. 67-79. Mnukhin, S. S. O sochetanii shizoformnykh i š prolavlenn u detei. Zh. Nevropatologii i psikhiatrii ira. Korsakova (On the combination of schiz0id and epileptic symptoms in children. Korsakov Journal of Neuropathology and Psychiatry), 1963, 63, .
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1047 - 1052.
Mnukhin, S: Si O sovremennom sostoi– i puti– dal'neish›239 problemy "detskikh rezidual'nikh š Vseross. nauchn, konfer, po psikhonevrologii detskogo vozrasta (On the present state of the problem of "residual encephalopathy in children" and the directions of future research. AIl-Russian scientific conference on childhood psychoneurology). Leningrad, 1965, pp. 103-108. Mnukhin, S. S., Zelenetskaja, A. E., & Isaev, D. N. O sindrome "rannego detskogo autizma" iii sindrome Kannera u dete9 Zh. Nevropatologii i psikhiatrii ira. Korsakova (On the syndrome of "early childhood autism" or Kanner's syndrome in children. Korsakov Journal of Neuropathology and Psychiatry), 1967, 67, 1501-1506. Nissen, G. Zum fruhkind lichen Autismus. Kasuistische Mitterlung. Archiv fur Psychiatrie und Nervenkrankheiten, 1963, 204, 531-536. Ozeretskil~ N. I. Psikhopatologiy226 detskogo vozrasta (Childhood psychopathology). /Vloscow, 1938.
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Planansky, K. Conceptual boundaries of schizoidness: Suggestions for epidemiological and genetic research. Journal of Nervous and Mental Disease, 1966, 142, 318-331, Prokopova, E. D. Ob osobo 9forme neravnomernogo psikhicheskogo nedorazvitif” u dete 9(On a special form of irregular mental retardation in children). Dissertation, Leningrad, 1953. Ruyter, Th.H. de. De psychische ontwikkeling van kinderen met lichte hersenbeschadiging. Nederlands Tijdschrift voor Geneeskunde, 1961, 105, 745-748. Sarvis, M. A. Psychiatric implications of temporal lobe damage. Psyehoanalytic Study of the Child, 1960, 15, 454-481. Schain, R., & Yannet, H. Infantile autism: An analysis of 50 cases and a consideration of certain relevant neurophysiologic concepts. Journal of Pediatrics, 1960, 57, 560-567. Schopler, E. Early infantile autism and receptor processes. Archives of General Psychiatry, 1965, 13, 327-335. spiel, w . Zur Problematik sogenannter schizoid-autischer Zustandsbilder. Wiener Zeitschrift /9 Nervenheilkinde und Deren Grenzgebiete, 1966, 24, 26-30. Sukhareva, G. E. Klinicheskie lektsii po psikhiatrii detskogo vozrasta (Clinical lectures on childhood psychiatry) (Vol. 2). Moscow, 1959. Takahashi, A. On psychotic symptoms (especially the autistic tendency) of mentally retarded children. Japanese Journal of Child Psychiatry, 1960, 1, 50-57. Van Krevelen, D. A. Autismus infantum (Infantile autism). Aeta Paedopsychiatrica, 1960, 27, 97-108. Van Krevelen, D. A. The psychopathology of autistic psychopathy. Acta Paedopsychiatrica, 1962, 29, 22-31. Van Krevelen, D. A. On the relationship between early infantile autisrn and autistic psychopathy. Acta Paedopsychiatrica, 1963, 30, 303-323.
On the Organic Nature of Some Forms of Schizoid or Autistic Psychopathy S. S. M n u k h i n ~ a n d D . N . I s a e v 2
Leningrad Pediatric Medical Institute Leningrad, U.S.S.R.
Editorial Comments. It is increasingly recognized that organic brain
damage plays a major etiological role in infantile autism and related disorders of communication. Facts that particularly prompt this conclusion include the frequent occurrence of behavioral disturbances resembling those of classical autism in diseases such as phenylketonuria and tuberous sclerosis; the high incidence of seizures in infantile psychosis (Rutter & Lockyer, 1967; Rutter, Greenfeld, & Lockyer, 1967); the idiosyncratic behavioral reaction shown by many psychotic children to neuroleptic drugs (Campbell, Fish, David, Shapiro, Collins, & Koh, 1972); and the often abnormal prenatal and early developmental histories of many of these children. Although the last-mentioned association is known anecdotally to pediatricians and neurologists, it has rarely been documented in detail and is a major theme of the article by Mnukhin and Isaev that follows. The authors' patients, carefully followed from childhood into late adolescence, showed an impressive frequency of abnormalities of pregnancy and delivery of a kind capable of causing cerebral hypoxia. Furthermore, the physical examination frequently indicated "dysmorphic" features hinting at underlying cerebral dysgenesis. From these observations and a review of relevant literature, the authors argue cogently that organic brain damage constitutes the basic etiology of their patients' behavioral difficulties. Their report is of additional significance since it presents what is probably the first detailed account in
' Deceased. 2Requests for reprints should be sent to Dr. Dmitry N. Isaev, Professor of Psychiatry and Chairman of the Department of Psychiatry, Leningrad Pediatric Medical Institute, 2 Litovska}a Street, Leningrad, 194100, U.S.S.R. 99 9 P l e n u m Publishing C o r p o r a t i o n , 2 2 7 West 1 7 t h Street, N e w Y o r k , N . Y . 1 0 0 1 1 . NO part o f this p u b l i c a t i o n m a y be r e p r o d u c e d , stored in a retrieval system, o r t r a n s m i t t e d , in any f o r m or bY any means, e l e c t r o n i c , m e c h a n i c a l , p h o t o c o p y i n g , m i c r o f i l m i n g , recording, or o t h e r w i s e , w i t h o u t w r i t t e n p e r m i s s i o n o f t h e p u b l i s h e r .
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the English language of clinical experience with childhood psychosis in the Soviet Union. Lorcan O'Tuama, M. D. Associate Professor o f Medicine (Neurology) and Pediatrics Chief, Section o f Pediatric Neurology School o f Medicine University o f North Carolina
REFERENCES Boullin, D. J., Coleman, M., O'Brien, R. A., & Rimland, B. Laboratory predictions of infantile autism based on 5-hydroxytryptamine efflux from blood platelets and their correlations with the Rimland E-Z score. Journal of Autism and Childhood Schizophrenia, 1971, 1, 63-71. Campbell, M., Fish, B., David, R., Shapiro, T., Collins, P., & Koh, C. Response to triiodothyronine and dextroamphetamine: A study of preschool schizophrenic children. Journal of Autism and Childhood Schizophrenia, 1972, 2, 342-358. Ornitz, E. M. Childhood autism - - A review of the clinical and experimental literature. California Medicine, 1973, 118, 21-47. Rutter, M., Greenfeld, D., & Lockyer, L. A rive- to fifteen-year followup study of infantile psychosis. II. Social and behavioral outcome. British Journal of Psychiatry, 1967, 113, 1183-1199. Rutter, M., & Lockyer, L. A rive- to fifteen-year followup study of infantile psychosis. I. Description of sample. British Journal of Psychiatry, 1967, 113, 1169-1182.
ON T H E O R G A N I C N A T U R E OF SOME FORMS OF SCHIZOID OR AUTISTIC P S Y C H O P A T H Y Representative case histories are presented to illustrate the typical clinical features o f autistic psychopathy. These patients showed several clinical features compatible with organic encephalopathy. Physical signs suggesting the presence o f an underlying cerebral clysgenesis were particularly frequent. These patients superficially resembled schizophrenics but differed in their delayed and peculiar intellectual development, the response o f their behavior to external stimuli, and their frequent and severe motoric defects. From these observations and a review o f the pertinent #terature, we conclude that the patients clescribed in this report have an organic deficiency of brain function, determined congenitally or in the early postnatal period.
The purpose of this paper is to describe a symptom complex generally termed autistic psychopathy, to emphasize the role of exogenous organic
On the Organic Nature of Some Forms of Schizoid or Autistic Psychopathy
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factors in its etiology, and to distinguish it from schizophrenia. To accomplish this aim we present short extracts from case histories of some of our patients. BACKGROUND
Early workers, notably Krepelin and Birnbaum, delineated the clinical picture of abnormal personality and emphasized its characteristic disorders of behavior and thought. Ozeretski9 (1938) noted the preference of schizoid psychopaths for solitude, their tendency to reasoning, obsessional interests, motor incoordination, and impulsiveness. The terms pathologically reserved and autistic were also applied to this group (Gurevich, 1932; Giliarovski 9 1954; Sukhareva, 1959). More recently, behavioral disturbances related to but separable from classically described schizophrenia have become of intense interest to psychiatrists. Kanner's description of infantile autism (Kanner, 1942-1943) spurred interest in this group of patients. Asperger (1944) observed autistic psychopaths and stressed their tendency to show motor incoordination, marked scattering of abilities, impoverished affect, and thought disturbance; they are incapable of putting themselves into the state of mind of another person. Several authors noticed the association of this syndrome ~with organic brain disease, including epilepsy (Grunberg & Pond, 1957), postencephalitic states (Van Krevelen, 1960), various organic encephalopathies, temporal lobe pathology (Sarvis, 1960), and postnatal trauma (Ruyter, 1961). Schain and Yannet (1960) stressed the frequency of clinical features suggesting organic brain disease in autistic children. In their patients, mental retardation and speech difficulties were common and almost one-half suffered from epilepsy, Frederiks (1964), Grunes and Szyrynski (1965), and Schopler (1965) felt that childhood autism is attributable to sensory deprivation resulting from dysfunction of the reticular formation. Despite this abundance of data indicating a connection between some forms of schizoid or autistic psychopathy and early organic brain disease (Nissen, 1963; Davies, 1966; Mnukhin, Zelenetskaja, & lsaev, 1967), some contemporary workers still consider them as diseases more akin to schizophrenia (Planansky, 1966), and others (Horvai, 1966) have poirtted6oE the difficulty of distinguishing symptomatically between some cases of schizophrenia or schizoid states and the psychopathiclike state described above which is caused by organic brain disease. We have taken the position repeatedly that most cases of autistic psychopathy are due to antenatal, intranatal, or postnatal organic damage of the brain and that these are caused by serious forms of dystrophy. This point of view has been based on out 27 clinical observations, some of which w› now wish to report. J
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CASE HISTORIES N.F. N.F. is ten years of age. His mother suffered from toxemia in the first half of pregnancy and his birth weight was eight pounds. He had a serious febrile illness during his first year and was thought to be moribund. He walked at the age of one and talked at the age of three, At the age of three he had a serious form of scarlet fever which was complicated by otitis and simultaneously he had dysentery. His early mental development was quite good and he began to read at an early age. From the age of eight years behavioral aberrations appeared, such as a compulsive washing of his hands, which he attributed to their smelling of glue. He also had some " f i t s , " as he described them, with loss of consciousness sometimes accompanied by feelings of thirst and dryness in his mouth. At the age of eight he had speech therapy, which continued through the preparatory and first grades. He was hospitalized because of behavioral peculiarities and was noted to be unusually withdrawn and reserved. He spoke frequently about inventions such as "autoclocks." His behavioral peculiarities in the classroom included biting his pencil and penciling his brows with ink. He would constantly lick his fingers. He was extremely restless and was not interested in books or in television. He was noticed to have very poor skills in arithmetic and to be lacking in concepts of time and space, but he could read fluently and write grammatically. Physical Examination. He was overweight and had a poor physique with hypogenitalism. He had a slight enlargement of the head. The second, third, fourth, and fifth toes on the right foot were hypoplastic. Syndactyly of the toes of the right foot and of the third and fourth toes of the left foot were noted. He had a high palate. Neurological Examination. His cranial nerves were normal, but he was felt to have muscular hypotonia. No pathological reflexes were found. An EEG was interpreted as indicating diffuse encephalopathy. He had two seizures in the hospital, one during an operation on his finger and the other during a lesson. These seizures were accompanied by loss of consciousness with generalized tonic convulsions. Five years after this hospitalization it was learned that he was in a school for the mentally retarded, was making good academic progress, and was even tempered, well disciplined, and taking an interst in radio mechanics. He was able to help his parents at home but had difficulty with detailed work because of motor incoordination. He appeared attached to his parents, but his emotional reactions generally were blunted.
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S.M. S.M. was thirteen years o f age. He was a f i r s t b o r n child with a normal birth history. He was noted to be restless from an early age and was easily distractible with a short attention span. He tended not to play with other children and in kindergarten was noticed to be behaviorally quite different from his peers. At the age o f eight he attended a school for the mentally retarded and was noticed to have poor learning ability with particular difficulty in arithmetic. He was admitted to our hospital at the age o f twelve because o f irritability, easy excitability, and aggressive outbursts. His intellect was subnormal. He learned to read and write but could not learn how to count. He could add concrete objects only up to the number 20 and would make mistakes when recounting. He showed poor response to teaching and was unable to complete easy work in spelling Russian. He could not complete words and made several mistakes in copying. He did not know his teacher's naine, the seasons, or the months o f the year and could hOt distinguish his body parts or imitate rhythms. He recounted bizarre episodes in which he said he had seen two giants with guns and without eyes. This turned out to be related to a story that he had read not long before. His concepts o f space orientation developed slowly. His affect was generally inappropriate and fluctuated between his being whining and irritable and periods when he would be somewhat euphoric. Despite these difficulties his expressive language was good and he could use complicated phrases such as " I entreat you to discharge me from the hospital." Physical Examination. He had a poor physique and was overweight. He had marked hirsutism o f the lower extremities. His ear helixes were poorly differentiated. He had fiat feet and the first and second toes of his feet were widely spaced. His hands and feet were large and coarse. He had pigmented areas on his back and had kyphoscoliosis. Neurological Examination. He had facial asymmetry with general hypotonia and motor incoordination. He had particular difficulty with fine movements of the fingers. Five years after this hospitalization it was learned that he had received an eighth-grade education at a school for mentally retarded children and at that time was working at a medical labor center. He had great difficulty with all operations requiring motor dexterity and his general behavioral traits had not greatly changed. /./. I.I. is 14 years old. His birth history was normal. Early retardation o f development was noted and he " d r a g g e d " his right foot until the age o f
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rive. He did not speak until he was four. From a very early age he was noted to be quick tempered, given to daydreaming, distractible, and uncooperative. He had to leave school at the age of 71A because of extreme restlessness and lack of attention. At the rime of his admission he was studying sixth-grade material. He had no friends and liked to play with younger children. He was excitable and easily frustrated, often threatening those around him. His actions were quite impulsive, as when he went to visit a hospital for mental diseases and was detained there because of odd behavior. At 12 years of age he suffered from cerebral concussion and began to have seizures. During the seizures he became pale, felt fearful, and fell to the ground. These spells were accompanied by breathing difficulty and disorientation. His physical examination was remarkable only for mild facial asymmetry. At the most recent follow-up he was attending a technical training school f o r the handicapped. He has difficulty learning trade skills because of motor clumsiness. He is hOt motivated to learn at this school and dreams of working as an inspector in the militia. He is restless, irritable, and sociable with those whom he likes, but his life adjustment is poor and he cannot defend himself. V.B. V.B. is 15 years old and has a father who uses alcohol excessively. Birth history was normal except for neonatal jaundice. He walked and talked by one year, when he suffered from toxic dyspepsia and lost the ability to walk for several months thereafter. He had measles, scarlet fever, and hepatitis. He showed striking behavioral peculiarities from early childhood, playing by himself, not fraternizing with other children, and being given to daydreaming. From the age of 7 he had great difficulty with arithmetic, though he learned quickly to read and to write. At the time of admission, he was in the eighth grade. He asked unnecessary questions, read a great deal, and wrote verses. He had ruade poor academic progress, especially in mathematics. His concepts of time were developing slowly. He had frequent episodes of depression and felt that his thinking had been worsened by medication. He showed depersonalization periodicaUy and was often irritable and importunate. He did not attend school regularly. Physical examination showed marked smallness of stature. There was asymmetry of the face and of the palpebral fissures. Skull films showed epiphysial calcification. DISCUSSION These short extracts from the case histories of four patients provide a representative sample of the characteristic clinical picture presented by the
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group of children and adolescents we have studied. Their peculiar characteristics are striking, even on cursory examination, especially their motor clumsiness and highly bizarre manner of interacting with people around them. Some of them have no marked intellectual deficit but most have an unusual combination of such deficits, viz., difficulty with reading, writing, and calculation; disturbed body-image recognition; poorly developed concepts of time and space; and difficulty in performing tasks involving sequential acts. They are given to pointless verbal philosophizing and illogical reasoning and have a tendency to ask unnecessary questions and to make accusatory statements. Many have obsessive restricted interests in, for example, calendars, geographic maps, and similar objects. They are often preoccupied with fantasies which they will sometimes act out, as when they " p l a y " such scenes as "taking part in battles" or "pursuing bandits." The content of their fantasies tends to be limited and seems to be derived from books they have read or from the conversation of those around them. Despite their intellectual retardation, they can usually speak quite weU, have a good mechanical memory, and often attract attention by using elaborate sentences which vary in their relevance to the context. Their behavior lacks any clear aire and is characterized by a succession of apparently unmotivated acts which seem to arise as a response to accidental external stimuli. They give the impression of being able to perform any task, provided they are stimulated energetically and systematically. Affectively, these patients also present many peculiarities. They lack deep-seated emotions and affections and often show less than the expected degree of inhibition in unfamiliar situations. For example, at school they frequently stand up during the lesson, walk about the classroom, ask unnecessary questions, or begin to sing. Despite their behavioral difficulties, these patients sometimes make satisfactory academic progress and may achieve a seventh- or eighth-grade education in schools for the mentally retarded. This is accomplished in the great majority of cases through their parents' constant help and systematic stimulation. However, irrespective of their scholastic progress, they are generally helpless in adult life and seldom master a trade. From the foregoing description, it can be seen that the intellectual, affective, and behavioral disturbances shown by this group of patients could easily lead to their being considered as suffering from schizophrenia. Their disease would then be regarded as a psychopathic change in personality occurring after a schizophrenic deterioration in early childhood or as a manifestation of a slowly developing schizophrenic process. As they grow older their behavior would become progressively more eccentric and less adjusted to life. When admitted to adult psychiatric hospitals, they would in fact be regarded as suffering from long-standing schizophrenia and would be treated ineffectively with insulin shock and psychotropic drugs in high dosage.
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However, a consideration of the history of these patients allows us to hold with a high degree of certainty that their disorder is not caused by a schizophrenic process or by a stable postschizophrenic change in personality. In the overwhelming majority, the developmental history points to exogenous organic disturbances. These include abnormalities of pregnancy or delivery and a high incidence of the minor diseases of childhood (toxic dyspepsia and dysentery, among others). Physical examination in many cases hints at the saine conclusion and shows poor stature, signs suggestive of endocrinopathy (especially obesity and precocious puberty), and various "dysgenetic" signs (syndactyly, hypoplastic fingers, high palate, poorly differentiated helixes, and flat-footedness, for instance). Some show neurological " s o f t " signs. We feel that the epileptic seizures frequently seen in these patients should also be construed as indicating an organic neurological basis for their syndrome, and we reject the view that these seizures represent a combination of schizophrenia and epilepsy, the so-called schizoepilepsy. Several of the clinical features shown by these patients would be difficult to reconcile with the conclusion that they are schizophrenic. Their delayed and peculiar intellectual development, the response of their behavior to external stimuli, and the frequency and severity of their motoric defects would ail be unusual features of schizophrenia. While their emotional state bears some resemblance to that of schizophrenics, a careful analysis shows features which are characteristic of this group of patients and unlike those shown by schizophrenics. These features include their obsession with restricted interests, their tendency to fantasize, and their inappropriate lack of inhibition. These features can hardly be regarded as indicating emotional poverty, particularly since these children seem quite deeply attached to their parents and close relatives. These observations lead us to think that the group of patients described in the present study and generally classified as s c h i z o i d or autistic has a gross organic deficiency of brain function, either congenital or arising in the early postnatal period. The observations suggest that diencephaiic and brain stem systems rather than cortical systems are primarily affected and there may also be involvement of the reticular formation and of the limbic system. The attention defects, affective disturbance, reduced activity level, and the aimless s t i m u l u s - b o u n d behavior that we have described support this conclusion. REFERENCES
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Davies, G. L. Autism--Schizophrenic syndrome of childhood. Medical Officer, 1966, 115, 225-227. Frederiks, J. A. Sensore deprivatie. Nederlands Tijdschrift voor Geneeskunde, 1964, 108, 749-757. Gil{” ~, V. A. Psikhiatriia [Psychiatry]. Moscow, 1954. Grunberg, F., & Pond, D. A. Conduct disorders in epileptic children. Journal of Neurology, Neurosurgery, and Psychiatry, 1957, 20, 65-68. Grunes, W., & Szyrynski, V. Secondary pseudoautism caused by physiological isolation. Journal of Consulting Psycho~gy, 1965, 29, 455-459. Gurevich, M. O. Psikhopatologiia detskogo vozrasta [Childhood psychopathology]. Moscow, 1932. Horvai, I. Einige Probleme der Differentialdiagnose zwischen Schizophrenie und schizoider Psychopathie. Wiener Zeitschrift fur Nervenheilkunde und Deren Grenzgebiete, 1966, 24, 60-63. Isaev, D. N. Kliniko-eksperimental'nye korreliatsii pri nekotorykh formakh oligofrenii u detei. Materialy 2-ogo Vseross. s'ezda nevropatologov ipsikhiatrov (Clinico-experimental correlations in certain forms of oligophrenia in children. Proceedings of the second All-Russian congress of neuropathologists and psychiatrists.) Moscow, 1967. Pp. 238-240. Kanner, L. Autistic disturbances of affective contact. Nervous Child, 1942-1943, 2, 217-250. Kraft, T. B. Is there a familial degenerative (sc.hizoid) psychopathy? Schweizer Archiv/~9 Neurologie, Neurochirurgie und Psychiatrie, 1959, 84, 110-121. Mnukhin, S. S. O nevro- i psikhopaticheskikhizmenenil'” lichnosti na pochve t 8 form alimentarnogo istoshcheniia u detei. Zh. Nevropatologii i psikhiatrii (On neuroand psychopathological changes in the personality due to severe forms of undernourishment in children. Journal of Neuropathology and Psychiatry) 1947, 16, 75-77. Mnukhln, S. S. Ob osobom tlpe neravnomernogo pslkhlcheskogo nedorazvltna u detel. Materialy 3-ogo Vsesoiuznogo s"ezda nevropatologov i psikhiatrov (On a special type of irregular mental retardation in children. Proceedings of the Third Ail-Union Congress of Neuropathologists and Psychiatrists). M,_oscow, 1948. Mnukhin, S. S. O roli rannikh ostrykhdispepsii v proiskhozhdenii nervnopsikhicheskikh rasstroistv u detei. Voprosy detskoipsikhonevrologii (On the role of early acute dyspepsia in the genesis of neuropsychic disorders in children. Problerns of child psychoneurology). Moscow, 1958, pp. 38-44. Mnukhin, S. S. O kliniko-fiziologichesko 9 sostoianii obshchego psikhicheskogo nedorazvitiia u dete9 Trudy instituta ira. 1I. M. Bextereva (On the clinicophysiological classification of conditions of general mental retardation in children. Works of the II. M. Bexterev Institute) (Vol. 25). Leningrad, 1961, pp. 67-79. Mnukhin, S. S. O sochetanii shizoformnykh i š prolavlenn u detei. Zh. Nevropatologii i psikhiatrii ira. Korsakova (On the combination of schiz0id and epileptic symptoms in children. Korsakov Journal of Neuropathology and Psychiatry), 1963, 63, .
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1047 - 1052.
Mnukhin, S: Si O sovremennom sostoi– i puti– dal'neish›239 problemy "detskikh rezidual'nikh š Vseross. nauchn, konfer, po psikhonevrologii detskogo vozrasta (On the present state of the problem of "residual encephalopathy in children" and the directions of future research. AIl-Russian scientific conference on childhood psychoneurology). Leningrad, 1965, pp. 103-108. Mnukhin, S. S., Zelenetskaja, A. E., & Isaev, D. N. O sindrome "rannego detskogo autizma" iii sindrome Kannera u dete9 Zh. Nevropatologii i psikhiatrii ira. Korsakova (On the syndrome of "early childhood autism" or Kanner's syndrome in children. Korsakov Journal of Neuropathology and Psychiatry), 1967, 67, 1501-1506. Nissen, G. Zum fruhkind lichen Autismus. Kasuistische Mitterlung. Archiv fur Psychiatrie und Nervenkrankheiten, 1963, 204, 531-536. Ozeretskil~ N. I. Psikhopatologiy226 detskogo vozrasta (Childhood psychopathology). /Vloscow, 1938.
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Planansky, K. Conceptual boundaries of schizoidness: Suggestions for epidemiological and genetic research. Journal of Nervous and Mental Disease, 1966, 142, 318-331, Prokopova, E. D. Ob osobo 9forme neravnomernogo psikhicheskogo nedorazvitif” u dete 9(On a special form of irregular mental retardation in children). Dissertation, Leningrad, 1953. Ruyter, Th.H. de. De psychische ontwikkeling van kinderen met lichte hersenbeschadiging. Nederlands Tijdschrift voor Geneeskunde, 1961, 105, 745-748. Sarvis, M. A. Psychiatric implications of temporal lobe damage. Psyehoanalytic Study of the Child, 1960, 15, 454-481. Schain, R., & Yannet, H. Infantile autism: An analysis of 50 cases and a consideration of certain relevant neurophysiologic concepts. Journal of Pediatrics, 1960, 57, 560-567. Schopler, E. Early infantile autism and receptor processes. Archives of General Psychiatry, 1965, 13, 327-335. spiel, w . Zur Problematik sogenannter schizoid-autischer Zustandsbilder. Wiener Zeitschrift /9 Nervenheilkinde und Deren Grenzgebiete, 1966, 24, 26-30. Sukhareva, G. E. Klinicheskie lektsii po psikhiatrii detskogo vozrasta (Clinical lectures on childhood psychiatry) (Vol. 2). Moscow, 1959. Takahashi, A. On psychotic symptoms (especially the autistic tendency) of mentally retarded children. Japanese Journal of Child Psychiatry, 1960, 1, 50-57. Van Krevelen, D. A. Autismus infantum (Infantile autism). Aeta Paedopsychiatrica, 1960, 27, 97-108. Van Krevelen, D. A. The psychopathology of autistic psychopathy. Acta Paedopsychiatrica, 1962, 29, 22-31. Van Krevelen, D. A. On the relationship between early infantile autisrn and autistic psychopathy. Acta Paedopsychiatrica, 1963, 30, 303-323.
