Syndromes of "Minimal Cerebral Damage"

Syndromes of "Minimal Cerebral Damage" RICHMOND S. PAINE, M.D.(:<

As early as the 1920's a few articles in the medical literature referred to "nervous conditions affecting behavior and learning," but little attention was paid to what is now referred to as "minimal cerebral dysfunction" until the years preceding World War II. Kramer and Pollnow 18 (1932) and Kahn and Cohen 16 (1934) were among the earlier writers on the subject, and Orton25 ( 1937) wrote one of the classic papers. Other authors have considered disorders of behavior and learning after specific types of cerebral insults such as encephalitis 6 • 14 • 32 and head injuries. 5 Strauss and Lehtinen33- 35 pioneered in the remedial education of children with the combinations of various irregularities of learning, psychological function, behavior and neurologic abnormalities which were then usually described as "minimal brain damage." Werner 37 • ss emphasized the role of perceptual disabilities in the syndrome. Currently, the diagnosis of "minimal brain damage" or "minimal brain injury" or "minimal cerebral dysfunction" is being made all over the country for large numbers of children, often on little evidence and often without critical review of the characteristics of each child. Any child who is hyperactive or who fails to learn satisfactorily at school and is not obviously feeble-minded or psychotic is likely to be placed in this category arbitrarily. Some physicians, psychologists and educators leap to the conclusion that drug therapy or placement in a special class is required for all such children and that these measures will promptly solve most of their problems. Opinions regarding the validity of this diagnostic entity and its characteristics vary widely among different authorities. This article will attempt to give an overview of the problem, some possible concepts as to the mechanisms involved, and From the Departments of Neurology and Neurological Surgery, Children's Hospital of the District of Columbia, and The George Washington University School of Medicine " Neurologist, Children's Hospital of the District of Columbia; Professor of Pediatric Neurology, The George Washington University School of Medicine

Pediatric Clinics of North America-Yo!. 15, No. 3, August, 1968

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at least one person's view of what the physician can and cannot, as well as should and should not, do in regard to the large numbers of children of this type with whom we are all now confronted. For a more complete discussion, the reader is referred to the excellent book by Dr. Herbert Birch4 published in 1964.

CHARACTERISTICS OF MINIMAL CEREBRAL DYSFUNCTION

In the currently popular conventional view, minimal cerebral dysfunction refers to a heterogeneous group of children who show varying degrees and varying combinations of abnormalities in coordination and other neurologic signs, behavior, performance at school, results of psychologic tests and frequently electroencephalograms as well. The children are infrequently brought to medical attention before they reach school age or at least kindergarten. Those who are seen in the preschool years are usually brought because of a parental complaint of hyperactivity. Sometimes excessive clumsiness is noted, and there is sometimes a history of delayed walking and other developmental milestones. Most children of this type are recognized as deviant from their peers only when the pressures and expectations of the educational system come into play, and the teachers and the school authorities are usually the prime movers in obtaining a medical evaluation. From the educator's point of view, these are children who are deviant in two principal ways (often in both). They may be disturbing influences in the classroom-children who cannot sit still in their seats, who tolerate direction or frustration poorly, and who are often hyperactive and up and around the room every few minutes. Another facet of deviant behavior is the inability to learn at a rate and to a degree commensurate with the child's presumed or demonstrated intelligence. The behavioral characteristics complained of by the teacher have often been noted previously by parents. In addition to being hyperactive, the child is generally hectic and disorganized, and his behavioral responses are frequently inappropriate. He may not foresee the consequences of what he does and is socially inept and unpopular with his peers. Occasionally such children are suspected of cerebral palsy or mental retardation, but more often they are not sufficiently different from their agemates to obtain definite recognition or to get any concessions as regards what is expected of them. An overlay of emotional difficulties is present almost as a rule, and often this is erroneously suspected of being the primary problem. Irregularities in results of psychometric tests are among the most impressive findings. The irregularities usually noted are those that are accepted in psychological circles as being characteristic of persons with organic encephalopathies. The first step in evaluation of a child with a

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learning disorder or with aberrant behavior at school is often psychologic evaluation, and the suspicion of "organicity" from this is frequently what leads to referral for pediatric or neurologic evaluation. Typically, the psychologic test confirms that the child has intelligence greater than that evidenced by his performance at school. Nevertheless there is reason to believe that many of these children have full scale IQ's below those of their parents and siblings, so that some general depression of overall intelligence may be involved. However, the typical irregularities can be superimposed on an IQ in any range, from superior to dull normal. At the borderline level or below, overall retardation becomes the predominant difficulty, and such children are best thought of as primarily retarded, even though their behavioral and other characteristics may differ from children with more uniform retardation. The degree of irregularity beyond the scatter between best and poorest areas of performance to be expected with most people, will vary from one case to another, as will the individual areas of greatest difficulty. Still, certain patterns recur again and again, and the experienced physician can often predict what these will be before the test is carried out, just as he can often tell parents the behavioral patterns before they have a chance to tell him (though it may or may not be wise to do this). In most groups of children studied, the performance IQ's on the Wechsler Intelligence Scale for Children are significantly below the verbal scores, often by 20 to 30 points or more. Depending on the interests of individual centers, differing populations of patients will be attracted for referral. Thus a center particularly interested in problems of language may get patients with verbal IQ's lower than the performance ones. Certain of the subtests of the WISC are more depressed than others, and the poorest areas are likely to be coding, block design and other items dependent on perception of Gestalten and visual motor coordination or those dependent on abstraction and inferential reasoning or mathematical concepts. Tests such as the Bender for copying of geometrical designs or the Graham-Kendall test for reproduction of designs from memory are also depressed. The mental age obtained from scoring the Goodenough Draw-a-Person test is below the child's mental age on the Binet or other general psychometric tests. The last-mentioned problem in drawing the human figure may depend in some way on impaired concept of body image or concept of space, but may be largely a problem of visual motor coordination in reproducing a concept. The testing psychologist also usually comments on the child's short span of concentration, his easy distractibility, his tendency to think concretely rather than to abstract, a tendency to perseverate with difficulty in changing the line of thought and a low tolerance for fmstration. The last may result in some display of temper or more frequently in abandoning the task at hand, either refusing to go farther or turning to something else.

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Figure 1. Three-dimensional figure assembled from notched sticks of a child's building set. The figure is constructed without the child watching and he is then asked to make an identical one from a collection of assorted sticks, as a test of constructional praxis. This figure can be duplicated readily by the average normal 6-year-old. Some modem sets of this type have sticks of different colors for different lengths; these are not suitable, since part of the task is for the child to select components of the correct length.

Conventional neurologic examinations usually show no abnormalities of the standard signs such as cranial nerves and reflexes, although a certain number of patients will have extensor plantar responses or hyperreflexia. The view is often advanced that a neurologist experienced with children could frequently find "soft neurological signs." Probably the major neurological abnormality is the general behavior of the child (this is the boy who demolishes one's office in 2% minutes). General clumsiness is frequently noted and is likely to be more conspicuous for fine muscle coordination than for gross function such as running, jumping or hopping. Visual motor function is likely to be even more impaired, for such things as writing or drawing or catching a ball. Skilled acts such as tying the shoes or doing buttons may be impossible past the age at which most children have learned them, or may be clumsily or slowly executed. In the course of evaluating these functions or on asking the child to extend his upper limbs in front of him and to spread the fingers, minor degrees of choreoathetosis or tremor may be detected. This has to be distinguished from the outer limit of normal fidgeting, and it is difficult to know exactly where to draw the line, even after considerable experience. There are a few tests of constructional praxis which have been standardized for certain ages, and the type of child under discus-

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sion will do more poorly at these than at other tasks. Examples are shown in Figure 1, a three-dimensional construction of notched sticks which is to be duplicated from a collection of sticks of assorted sizes, and Figure 2, a two-dimensional plastic puzzle involving assembly of geometrical shapes (jigsaw puzzles are not nearly so useful as test materials, because the figure to be composed appears to furnish clues enabling even neurologically impaired children to assemble them). The physician may want to make use of the generally accepted standards for the ages at which a child should be able to copy certain geometrical shapes, for example, a circle at 3Y:!, a square at 4Y:!, a triangle at 5, a horizontal diamond at 6 and a vertical diamond at 7. What the child can do would of course have to be compared not with his chronological age but rather with his known or estimated mental age. The Kohs test of block designs can also be used, but in general the physician should be very hesitant to borrow tests which the child may subsequently encounter in a full-scale psychologic evaluation. Clinical psychologists administer these tests more scientifically, know the required test situations and the objective methods of scoring, and it is better for the physician not to give the child advance experience of the materials. Physicians working in areas remote from psychologic resources or with very limited numbers of psychologists available, may need to do a lot of preliminary screening in order to decide whom to refer. Under these circumstances the individual physician would be well advised to learn as much as possible about the administration and interpretation of what he is going to use, from one of his psychologic colleagues, and even then he must recognize his own limitations.

Figure 2. Octagon puzzle sold under name "Euclid" ( Tryne Games, Inc., Springfield Gardens, L.I., N.Y.) This can be assembled by an intelligent 8-year-old. (Figs. 1 and 2 from Paine, R. S. and Oppe, T. E.: Neurological Examination of Children. London, William Heinemann Medical Books, 1966. Reproduced by permission of Spastics International Medical Publications.)

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Electroencephalography is frequently requested. Most surveys indicate that a higher proportion of electroencephalographic abnormalities are recorded from children with the other characteristics of minimal cerebral dysfunction than in the general population. The abnormalities, which may be present in 50 per cent or more of cases, are less often spike-wave complexes or seizure discharges than merely lesser disorders of rate, amplitude or rhythm. There is some debate as to the potential value of routine EEG's, and the indications for making these and the potential benefits will be discussed later, when the overall plan of evaluation of individual patients is considered.

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INCIDENCE AND PREVALENCE OF MINIMAL CEREBRAL DYSFUNCTION

It is currently widely maintained that this disorder affects 5 per cent or more of the entire random child population, which would make it the commonest neurological diagnosis among children (compared with 0.5 per cent each for epilepsy and cerebral palsy and at most 3 per cent for mental retardation of mild or greater degree). Others have put the figure as high as 10 per cent. The prevalence of the problem naturally depends on the diagnostic criteria which are applied. If one views this as an educational diagnosis, it then tends to become equated with what educators call "learning disability." This is defined in a variety of ways, usually by something such as "inadequacy of academic achievement not explicable on the basis of mental retardation, sensory or physical handicap, or gross emotional maladjustment." More complete definitions are those of Barsch2 and of Kirk: 17 A child with learning disabilities is one with adequate mental ability, sensory processes, and emotional stability who has specific deficits in perceptual, integrative, or expressive processes which severely impair learning efficiency. This includes children who have central nervous system dysfunction which is expressed primarily in impaired learning efficiency (Barsch, 1967). A learning disability refers to a specific retardation or disorder in one or more of the processes of speech, language, perception, behavior, reading, spelling, writing, or arithmetic (Kirk, 1967).

It is apparent that this type of diagnostic net would catch not only the child whom the physician or psychologist might designate as having a minimal chronic brain syndrome or minimal cerebral dysfunction, but also the lazy, the nonmotivated and many other children less readily classifiable. Some of these merely have maturational lags in respect to readiness for certain types of learning, as most conspicuously in the case of reading. Here, if one uses the diagnostic criterion of being two years behind age level in reading, despite adequate intelligence, no gross sensory deficit and adequate conventional instruction, there may be

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as many as 20 per cent of the population of public schools who suffer from this, which might then better be called "reading retardation." The physician who is experienced in working with neurologically handicapped children already will have recognized that many of the psychologic and behavioral irregularities which have been described are precisely those that have been encountered in many children with spastic cerebral palsies or epilepsy. The view could be advanced that the irregularities on the psychologic test, with or without mild depression of full scale IQ, represent a borderline or minimal version of mental retardation and that the clumsiness and minor abnormalities of neurologic signs are subclinical forms of cerebral palsy. 27 Similarly, the frequent abnormal EEG's could be conceived of as indicating subclinical epilepsy.27 We know that many patients with cerebral palsy also suffer from epilepsy or mental retardation, and that among those who do not there are nevertheless a good many with corresponding minor irregularities. All of this suggests that the patients' difficulties may in many instances have an organic basis. Retrospective studies of children classified as having minimal cerebral dysfunction on various bases have usually shown an increased frequency (compared with general obstetrical experience) of such events as abnormalities during the mothers' pregnancies, difficult births, delayed resuscitation or neonatal abnormalities, and sometimes of postnatal cerebral insults such as meningitis, encephalitis or severe head injuries. Not much more than half, or possibly only a minority of the children under discussion will have such histories when studied in retrospect. This may mean that some of the children are as they are because of unrecorded or unrecognized cerebral insults, and it is well to remind oneself that roughly one third of children with cerebral palsy remain unexplained by retrospective medical histories. Others may represent "cerebral dysgenesis" or what is probably better referred to as suspected but undefined maldevelopment of the brain. Still others probably have nothing more than irregular maturation of cerebral function along different lines. The medical diagnosis of minimal cerebral dysfunction is justifiably made with less frequency than that of learning disability from an educational point of view, but if the educators and psychologists of a school system are sophisticated, and the physician equally so, the diagnostic conclusions of the two disciplines will agree far oftener than not. This has been true of as many as 87 per cent of educational referrals in one study. 28 It has already been mentioned that the clinical psychologist often instigates a medical evaluation because of a pattern of irregularity on psychometric tests similar to that encountered with organic brain syndromes. The earlier view was that the irregularity should be present without reducing the full scale IQ or "without apparent lowering of the

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intellectual level" (Strauss and Lehtinen) .34 However, more recent surveys suggest that the intelligence of a group of children under discussion is somewhat depressed compared with that of their parents and siblings. 28 A difficult point to consider is whether the typical psychologic findings are sufficient in themselves to make the diagnosis. In one survey 28 the psychologic findings had a higher correlation with abnormality in the electroencephalogram and with histories of potential cerebral insult than did the neurologic signs. At present the question just posed cannot be confidently answered. The children under discussion proved to have progressive or directly treatable organic cerebral lesions only with extreme rarity. Thus it is less important to try to make a "diagnosis" than to categorize each child as to the abilities and disabilities he has, the way in which he functions and learns, and then to try to make plans for him on the basis of this knowledge. It is best not to think of minimal cerebral dysfunction as a diagnosis, nor to put it specifically in the competence of the educator or physician or psychologist, but rather to assemble the information available from each as a basis for planning.

DEFINITION OF MINIMAL CEREBRAL DYSFUNCTION

Having described some of the characteristics of an admittedly heterogeneous group of children, it is appropriate to attempt some kind of definition. A task force organized by the National Institute of Neurological Diseases and Blindness, the Division of Chronic Diseases of the U.S. Public Health Service, and the National Society for Crippled Children and Adults considered this matter at length7 and reached the following conclusion: The diagnostic and descriptive categories included in the term brain dysfunction syndrome refer to children of near average, average, or above average general intelligence with learning and/or certain behavioral abnormalities ranging from mild to severe, which are associated with subtle deviant function of the central nervous system. These may be characterized by various combinations of deficits in perception, conceptualization, language, memory, and control of attention, impulse, or motor function. Similar symptoms may or may not complicate the problems of children with cerebral palsy, epilepsy, mental retardation, blindness, or deafness. These aberrations may arise from genetic variations, biochemical irregularities, perinatal brain insults, illnesses or injuries sustained during the years critical for the development and maturation of the central nervous system or from unknown causes. The definition also allows for the possibility that early severe deprivation or trauma could result in central nervous system alterations which may be permanent. During the school years, a variety of special learning disabilities is the most prominent manifestation of the condition which can be designated by this term.

This group was composed mainly of physicians and psychologists, and a team of educators would doubtless arrive at a somewhat different categorization. Still, at least from the medical and psychologic point of view, the definition reached by the task force I referred to is probably

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the best currently available, and can serve as a starting point for further thought.

MINIMAL CEREBRAL DYSFUNCTION AND DEVELOPMENTAL APHASIAS AND DYSLEXIAS

Some children who otherwise qualify entirely as examples of minimal cerebral dysfunction also have considerable problems in functions of language (see also p. 611). These may include deficits in the understanding of what is said to them, in their own speech, or in reading or writing, or very often in more than one of these functions. A purist might define the developmental aphasias of childhood as near or total absence of spoken language, almost always in association with some disability in understanding the speech of others, not explicable on the basis of hearing loss, mental retardation, psychosis, environmental abnormality or gross brain damage. The final exclusion mentioned might be extended further to exclude children who have only borderline evidence of cerebral dysfunction. I am convinced that there is such a thing as developmental aphasia in childhood, that this nearly always includes some degree of receptive deficit as well as an expressive one, and that there is frequently a coexisting peripheral hearing loss and even more often some defect in central auditory transmission or in analysis of the coded transmission at a cerebral level. In personal experience of examining children in a school for aphasics, a substantial number, but nevertheless a minority, also showed other signs of neurologic abnormality; the proportion of children thus involved was comparable to the result of similar screening carried out with a population of deaf children. Perhaps it is best to conclude that developmental aphasia may exist as a nearly pure entity, but that many patients have other abnormalities of cerebral function and that these may include those considered under the rubric of minimal cerebral dysfunction. However, a child with nearly total absence of speech is not minimally handicapped. It is best to conceive of the two diagnostic categories as both being heterogeneous, as overlapping in some features, but mainly as a separate categorization of children according to their major manifestation of disability. Such an approach would lead to the conclusion that developmental aphasia is much less common than minimal cerebral dysfunction. The relationship between minimal cerebral dysfunction and dyslexia is even more complex. It is common experience that many or most children with minimal cerebral dysfunction have difficulty in learning to read and to write (see also p. 669). It is immediately plausible that if a child has difficulty in discriminating between different shapes he will have similar difficulty in discriminating between letters, and that if he has right-left confusion he will have difficulty in discriminating

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between d and b. Equally, if he has impaired visual-mofor coordination, his handwriting will be poor. Thus, in the broadest definition, he would have to be considered dyslexic and dysgraphic. He would also fall within the boundaries of a definition of "dyslexia" recently developed by a study group of the World Federation of Neurology: A disorder in children, who, despite conventional classroom experience, fail to attain the language-skills of reading, writing and spelling commensurate with their intellectual abilities.

This definition is really one of reading retardation and includes the lazy, the nonmotivated and the emotionally disturbed. The same study group developed a more tightly worded definition of what might be called specific developmental dyslexia: A disorder manifested by difficulty in learning to read despite conventional instruction, adequate intelligence and sociocultural opportunity. It is dependent upon fundamental cognitive disabilities which are frequently of constitutional origin.

To this definition might well be added that the cognitive disabilities are often familial. General experience with specific dyslexics in the narrow definition indicates that there are some features which such children have in common with the considerably larger number with minimal cerebral dysfunction. For example, the digit repetition test in the WISC is below average in both (but not in all with minimal cerebral dysfunction), as is the coding subtest. Both groups have impaired right-left orientation and an increased frequency of mixed, crossed or incompletely established dominance. Both groups have a preponderance of males, but this is higher in specific dyslexia ( 4:1 to 9:1) than in minimal cerebral dysfunction ( 3: 1 or 4: 1 ) , in most reports. There are more important differences between the two groups-see Table 1. Authorities who have studied children whose disabilities appear to be chiefly dyslexia have generally noted that the ability to write and to spell is more impaired than that to read in many instances. Right-left confusion and mixed or incomplete cerebral dominance have also attracted much attention. The basis of all of this is poorly understood, but there may be several types. The relatively pure form may be based on perceptual disability, which Hermann 13 has called word blindness, a disability in perceiving a word at a glance, even though it may be possible to spell it out laboriously from the letters with some-but often incomplete-success. Other cases, particularly where there is marked disability at spelling, may depend on some defect in temporal or spatial analysis of sequences. Difficulties in understanding language and speaking may coexist to some degree, and there may be impaired auditoryvisual integrative organization. 1 In others the disability may be at some undefined locus in the sequence of grasp-retention-recall. 1 Causation is

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Table 1. Differences Between Children With Specific Developmental Dyslexia and With Minimal Celebral Dysfunction* SPECIFIC DEVELOPMENTAL DYSLEXIA

Abstract and inferential reasoning Arithmetical ability

Perceptual disabilities Attention span Hyperactivity Visual motor coordination WISC: Similarities Block design Full scale IQ Verbal vs. performance score Family history

MINIMAL CEREBRAL DYSFUNCTION

Good

Poor

Good, except cannot read printed problems and may have difficulty with mathematical symbols Chiefly for words Good Infrequent, unless driven Often good, but not always

Poor

Multiple Short Common Poor

High Average or above As good as siblings Performance higher than verbal Usually positive

Below average Below average Lower than siblings Verbal higher than performance U sua!! y neg a ti vc

*Modified from McGlannan. 1 9

equally speculative, although most studies recognize the strong familial tendency; some pedigrees appear to show inheritance in the male line and others in the female. While it seems clear that some cases are hereditary and constitutional, others may be based on damage to the central nervous system and still others on some disorganization associated with a disorder of hemispheric dominance. Thus even "specific dyslexia" appears to be heterogeneous. The prevalence would clearly depend on the diagnostic criteria accepted. Perhaps as many as 20 per cent of all school children have reading retardation, 3 but the number with specific dyslexia must be very much lower, 0.5 to 3 per cent in most surveys. Thus, while they have a number of features in common, minimal cerebral dysfunction and specific developmental dyslexia are best considered separate categories. Specific dyslexia is considerably the less common of the two. Finally, the prognosis may be different (see p. 799). More briefly, the typical child with minimal cerebral dysfunction does learn to read and by the fourth or fifth grade, reading and particularly spelling, which is mostly memory, are likely to be among his better areas of performance and mathematics almost invariably the poorest. The situation with the true (narrowly defined) dyslexic is quite different: he may be good at mathematics, but disability in reading persists despite the best remedial training in many cases. Most probably do compensate, but there are numerous examples of dyslexics who are unable to read anything more complicated than street signs, even as adults.

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WHAT TO CALL THE "SYNDROME OF MINIMAL CEREBRAL DYSFUNCTION"

A number of different terms have been applied to this problem, some of them bizarre and obviously unsuitable and by now forgotten. The terms that persist include "minimal cerebral injury" of Gesell 11 ( 1941) and "minimal brain injury" or "minor brain damage" of Strauss and Lehtinen.34 Some have denied that the condition even exists. A study group on child neurology3 sponsored biennially by the National Spastics Society at Oxford University considered "minimal brain damage" in 1962 and concluded that the term should be dropped: The group recommends that the term "minimal brain damage" should be abandoned. The concept of "minimal brain damage" may have served a purpose in bringing a number of hitherto unrecognized conditions, or children with varying conditions, to the attention of paediatric neuropsychiatric consultants. However, the term has now two connotations; one to describe a category of diseases of very varying clinical manifestations and, two, as a diagnostic label for certain individual children. In the first case the term does perhaps little positive harm. In the second instance-its use as a diagnostic label-it may lull the physician into a state of inactivity, as he feels his task is done. We do not think that the dropping of this term will lead to any neglect of investigation and treatment of children with these varying disorders. On the contrary we believe that this will lead to an intensive study of the children. In addition to these general reasons for abandoning the term "minimal brain damage", we have specific reasons for suggesting that the words "minimal" and "damage" should be avoided when possible. The word "minimal" is bad because there is no constant correlation between the symptoms and signs which are observed and the extent of brain lesions (this applies especially in infants). The term suggests that either the symptoms or signs are minimal and that the brain lesion is too, but this is frequently not the case. All syndromes may have minimal or maximal manifestations but a minimal manifestation of a syndrome does not create a new syndrome entity. The word "damage" should be abandoned. To lay people it represents the deleterious result of an injury, but for medical people it may have quite specific clinical connotations.

The unfortunate and inaccurate implications of words such as "minimal" and "damage" or "injury" are clear and they may produce other undue discouragement or else the implication of total recovery (from an injury). Pathological data are almost wholly lacking. The contention of Pasamanick and Knobloch29 that minimal cerebral dysfunction is part of a continuum of reproductive casualties ranging from spontaneous abortion to stillbirths, neonatal death, gross brain damage with cerebral palsy, mental retardation, etc., and more borderline manifestations, has some intuitive appeal on clinical grounds but no firm neuropathologic documentation. It would seem proper to view other cases as reflecting maturational lags in coordination, psychologic development and behavior, as well as in readiness for certain types of education. Whatever the confusion about the causative factors in this group of children and whatever one's distaste for some terms which may be applied to it, the children themselves are with us and in considerable numbers; they

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cannot be abolished so easily as a phrase to designate them. If this group of children really represents 5 per cent of the general school population, and if they get into increasing difficulties as the complexities and pressures of education continually increase, pediatricians, neurologists and other physicians will have to be prepared to take a part in their evaluation and management.

EVALUATION OF THE CHILD SUSPECTED OF "MINIMAL CEREBRAL DYSFUNCTION"

It has already been mentioned that cases of this group of conditions come to attention mainly because of hyperactivity at home or at school or because of educational failure not otherwise explicable. The pediatrician may suspect that a hyperactive or clumsy child falls into this category, at one of his routine examinations. Far more often, difficulties in learning or behavior at school will lead to the child's mother asking the pediatrician to evaluate him. It is very difficult to know how far one ought to proceed, and it is probably impossible and unwise to attempt to establish a rigid schedule of diagnostic study for all cases or suspects. Because of the complexities and the time and money involved, it is well to ask oneself in the beginning whether the child's difficulties at home and at school are really of sufficient severity to warrant a detailed assessment, and also to ask whether a child may be simply not very industrious or not very bright. Once serious suspicions are aroused, however, more detailed study is usually required. A complete medical history and pediatric examination of the usual type is appropriat~ and contributory physical factors may be revealed. The treatment of a chronic anemia or an appropriate tonsillectomy may alleviate the problem. Assessment of educational levels by various achievement tests usually will have been carried out at the school already. Psychologic evaluation may also have been done through the school system, but the pediatrician must arrange for this if it has not, or if properly qualified psychologists are not on the staff of the school. A trained psychologist will be able to decide for himself what tests are appropriate. These will usually include the Wechsler Intelligence Scale for Children (or else the Binet), the Bender test of design copying, and other tests where appropriate, but the physician should not attempt to dictate the psychologist's choice of these. In appropriate instances evaluation by an audiologist or a speech pathologist, or a psychiatric consultation, may be indicated. If there is strabismus or suspected diminution of visual acuity, an ophthalmologist would properly be called in. A more difficult question is whether a full-scale neurologic work-up is necessary. The neurologist who has been trained with adults and confines himself to the conventional neurologic examination will often be of no help. The

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pediatric neurologist or the neurologist who has had some experience with children may come up with much more information. Even here, his findings are of limited practical value and correlate less with historical brain insults than do psychologic findings or EEG's. 28 The right neurologist may be a good person to coordinate all the specialists' reports, but a knowledgeable pediatrician is just as good or perhaps a better person to do this, particularly if he has long-standing knowledge of the child or rapport with the parents. The number of pediatric neurologists in the country is nowhere near the number that would be required to evaluate all the suspected children in the categories under discussion, even if most of them were not involved in academic medicine, teaching and research, to the severe limitation of the number of patients they can see, as a matter of service. 27 It seems realistic to say that pediatricians will inevitably have to do most of the neurologic evaluation and most of the coordination and interpretation, and further that a certain proportion of practicing pediatricians will have to become more knowledgeable about all aspects of the problem and that pediatricians who lack the time or aptitude or interest for this will have to refer such children elsewhere. Perhaps the main indication for a full neurologic work-up would be the suspicion of some progressive deteriorating disease, but this question seldom comes up in clinical practice, and it is usually abundantly clear that the child has a long-standing nonprogressive condition even if the manifestations change with age and with educational demands and expectations. Whether to obtain an electroencephalogram is again a difficult question. There is no doubt that such tracings are abnormal in a higher than random percentage of children with the characteristic behavioral and psychologic irregularities described. On the other hand, the abnormalities in the EEG are usually minor ones and more often than not do not contribute in any way to the practical management of the child. The possibility of unrecognized seizures must be considered, and there is the further question of conceivable benefit from giving anticonvulsant drugs to patients with EEG's showing seizure discharges even if there have been no clinical fits. I should say that an EEG may well be appropriate for all patients in a research study, but is not otherwise required routinely, and I should put the indications for electroencephalography as follows: 1. Suspicion of petit mal epilepsy or even merely frequent short episodes of apparent daydreaming or loss of contact. Whether the patient can be "snapped out" of these is not a criterion, inasmuch as petit mal fits are so brief as frequently to lead to this erroneous impression. 2. Paroxysmal and stereotyped episodes of abnormal behavior, even if consciousness is not lost. Temporal lobe or psychomotor seizures are less complex in children than in adults and may be hard to recognize as

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such. A sleep tracing should be obtained if an EEG is made under these circumstances, since abnormality may be missing in the waking record. 3. Any suspicion of a progressive or degenerative condition. 4. Abnormal neurologic signs on examination which suggest a focal lesion, or other neurologic indications from the history or examination. 5. While not a strictly medical indication, it may occasionally be wise to have an EEG made for the sake of everyone's peace of mind, if some physician or other specialist involved is pushing hard for it or has planted the idea firmly in the parents' minds.

SCREENING TESTS

At present most children with minimal cerebral dysfunction come to medical attention only when they are brought to light by the demands of the educational system. It is reasonable to think that their school careers would be made easier and their emotional health protected by earlier recognition. There is considerable interest at present in various screening tests which could be administered by physicians without much specialized training or even by nonmedical personnel. Most of these aim at examining the kindergarten or prekindergarten child, and suggestions are made that preregistration of children for kindergarten a year ahead of time would present an opportunity for this type of assessment. At present these tests are in developmental stages (Twitchell, 36 Ozer26 ). It would be unwise to apply them on a mass scale until the children screened can be followed long enough to establish the predictive value of the tests for educational performance 2 years later. Such a study of correlations at follow-up would also make it possible to simplify the tests by eliminating less predictive items. Another proposal currently being advanced is the establishment of high risk registers. The idea is to establish lists of children born after abnormal pregnancies or with abnormal births or neonatal periods, who would be considered especially "at risk" with respect to neurological sequelae later on. These registers have been required by law as a trial in some areas of England and serve the purpose of bringing registered children to the care of pediatric specialists in a country where pediatricians are far less numerous than in the United States and where most well child care is provided by general practitioners. Even there, the experience with high risk registers has been disenchanting. Many children with obvious cerebral palsy or mental retardation are not picked up (it is to be remembered that past medical histories provide no clue as to causation in 30 per cent or more), and children with minimal cerebral dysfunction would be still more likely to escape being registered. More importantly, the high risk register system results in the expenditure of large amounts of time and money on the follow-up of

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large numbers of children who, for the most part, prove to be normal. One must not overlook the possible psychological harm to the confidence of mothers and to their mother-child relationships from a system which tells 10 or 20 mothers that they have ''high risk children" but whose children eventually prove normal, in order to pick up one child who could probably be identified by visiting public health nurses or in other ways.

THE TREATMENT OF MINIMAL CEREBRAL DYSFUNCTION

The physician's first role, as with any medical problem, is to reach a diagnosis, or rather in this case a mixed diagnostic category, and then to consider what the causation is likely to be. Children with minimal chronic brain syndromes must be considered in the same context as those with more obvious ones, as to whether the picture could reflect a progressive condition or a disease or lesion amenable to direct medical or surgical treatment. Such is rarely the case with cerebral palsies and still more rarely with borderline manifestations. Still, the physician must consider these possibilities and plan appropriate studies in selected cases when indicated. His most important role after this is to coordinate the educational, psychologic and other specialists' reports into a unified plan. The pediatrician is usually the best person to interpret all this material to parents,24 and he is faced with the task of explaining an obscure and complicated medical situation in understandable terms even though he has considerable confusion about it himself. It should be emphasized that in most cases the condition is a persistent but nonprogressive one, that direct treatment will give only limited improvement, but that considerable improvement can generally be expected with age, that the educational situation can be managed and that the long-range prognosis is relatively favorable. One should also explain that the nature of the child's difficulties, his assets and liabilities, will change from year to year and that periodic reassessment and revision of educational plans, particularly, are required. Parents of children of the type discussed have usually been aware for some time that the child is atypical or deviant, and the air is often cleared when a careful explanation is given and it is made evident that not all the child's problems or difficult behavior is his own fault. Drug therapy has a definite but limited place. It is well known that children of the type considered frequently react in a paradoxical fashion to barbiturates, so as to become hyperexcitable or "jazzed up," and conversely that certain drugs which are normally stimulants may have a calming effect. Amphetamines and methylphenidate (Ritalin) are the preferred examples. The dosage schedule has to be worked out individually for each child. Methylphenidate can be given as one or two 10 mg.

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tablets in the morning, this dose to be repeated at midday or even in the late afternoon if it appears to be dennitely useful but to wear off. Dextroamphetamine can be given in doses of 5 or 10 mg. on the same schedule, or a time disintegration capsule of 10 or 15 mg. may be given as a single dose with breakfast. It is difficult to evaluate the effect of any of these medications because of their possible action as a placebo and because of the improvement in behavior which usually takes place concurrently with starting drugs after general evaluation and interpretation to parents and school. Reports from school teachers, if these can be obtained, may provide objective documentation of effects of therapy. Personal experience has been that amphetamines or methylphenidate will prove of signincant benent to perhaps 50 per cent of children and that in general one will be effective if the other is, or neither if the nrst one tried fails. (Nonetheless, it is usually wise to try the other if the nrst is unsuccessful.) Methylphenidate has less action as an appetite depressant than amphetamines and thus may be the nrst choice. In some instances it will be found necessary to give the medication only on schooldays and to omit it on weekends, to avoid habituation taking place, with lessening of the therapeutic response. In other cases, withdrawal for a week or two from time to time, followed by resumption of the previous dose, will restore effectiveness. Why these apparent stimulants should have a paradoxical calming effect is obscure. Possibly there is some cerebral mechanism for inhibition, that is to say, for selective responsiveness or unresponsiveness to stimuli on the basis of past experience, which is defective or late in maturing. It would then be possible that sedatives such as barbiturates would further depress this denciency and that stimulants might activate it. Many other medications can also be tried and have been found of benefl.t in some cases. These include diphenhydramine ( Benadryl) and other antihistamines, chlorpromazine (Thorazine) and other phenothiazines, and other types of tranquilizers. Chloral hydrate or other hypnotics at bedtime may be useful for the child whose need for sleep is slight and whose habits are poor about going to bed, but this must be combined with efforts at retraining of these habits and should be regarded as a measure to make retraining easier and more bearable rather than as a cure in itself. Finally, the physician has some responsibility to discuss intelligently with parents various controversial or unproved approaches to treatment. Blanket prejudged condemnation is seldom helpful to parents. It is better for physicians to present the evidence pro and con concerning such approaches as patterning, reversion to crawling and eye exercises. There is available some published information10 and some statements by the American Academy of Pediatrics, the American Academy of Neurology and by a few ophthalmologists (whose view is generally that the

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disordered ocular movements are the result of the disability in reading and not vice versa).

EDUCATIONAL MANAGEMENT

A considerable variety of special class situations exist around the country. In general, the classes are small with 10 to 15 pupils and are taught by teachers who have had some training in special education. The ability and force of personality of the teacher may be the predominant factor in determining success or failure, but various special situations have been set up. These range from small classes with widely and irregularly placed seats and desks, and a generally structured environment, to various types of cubicles in which the child sits with his back to the teacher and screened from other children so as to reduce distractibility. Objective evaluation of the benefits of, and indications for, different types of special educational management are difficult to obtain and are in the province of the educator rather than the physician. As a matter of logistics, most children with minimal cerebral dysfunction will have to be managed in regular classes with some modification of the program so as to reduce long periods of sitting still and to provide concessions about slow and messy handwriting. In some cases, drug therapy will make the difference between keeping a child in a normal class and having him placed elsewhere or even excluded from school if no special class is available. The physician's report is one bit of evidence for the pupil placement services or special educators to take into account in planning for a child's schooling, but at this point the educator becomes the key person and the physician must remember this once he has interpreted the total picture to the parents. He should remember that he himself resents it when parents come to him with a note from the school saying "Please give this boy a neurological examination and be sure to make an EEG." Equally, the educator resents it when he receives a medical report concluding with "I believe that this boy needs special education and should be placed in a small special class with cubicles." The need for physicians, psychologists and educators to communicate with one another is an obvious one. We need to learn to speak one another's languages and to communicate more effectively, including feedback as to changes in the child's behavior and learning, as well as cooperation and periodic revision of plans. A modified plan of management at home is also appropriate. The disorganized behavior of children with minimal organic brain syndromes is usually worsened in a highly permissive atmosphere. Such a child needs controls and limits even more than the average boy or girl and does much better in a structured environment. A simple daily routine, much the same from one day to another, without too many variations or

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demands for nonroutine decisions is usually beneficial. The rules should be simpler and fewer than for other children of the same age, but they should be the same from one day to another and the same for the mother as for the father. Discipline can be maintained as with other children or, better, as for those of a slightly younger age. It is usually best to be firm but above all consistent about important issues, and it is far wiser to overlook or not make a point of the hundred or more lesser actions which occur each day and are usually thought naughty by adult standards. The understanding that some of these offenses are not intentional or even are unavoidable will ease the tension in the home. A roomful of unfamiliar objects in bright colors notoriously stimulates, as in the average doctor's office; reduction of the number of exposed toys and other objects, and painting the child's own room in quiet restful colors, tend to reduce hyperactivity. Such a child is much less frustrated by inanimate objects than by people, and it is preferable to put a hook on the closet door high enough so that the child cannot reach it, rather than constantly to tell him to stay out of the closet. A young child may find it relaxing (so may his mother) if he spends an hour once or twice a day in his own room, which has been stripped for action so that he does not have to continually hear the words "Don't touch." Even though one may not like the idea of confining the child, it may be far from a bad idea to put a screen door or a Dutch half-door between his room and the rest of the house. This permits him to see and hear and to be seen and be heard, but is some measure of restraint even if he is able to get it open. The same effect may be achieved outdoors by fencing in the yard or a part of it. All sorts of modified programs are possible. A resourceful psychologist, or the psychiatrist if he is involved, can give advice about this type of planning, but in most cases it will be the lot of the pediatrician to do so. Similar modifications are possible at school. Some child has to be the one to get up to open the window, turn on the lights or erase the blackboard, and to be chosen for this can be a great comfort to a boy who suffers intensely from enforced long periods of sitting in his seat. Slow and messy handwriting is usually inevitable, and concessions about this are appropriate. In the case of written tests it might be fairer to give these by spoken question and answer if feasible, or at least to allow the child extra time to finish or to grade him on what he can produce in the allotted time, without marking him down for lack of neatness. In later school years, reduction of assignments involving English composition, will avoid the child's having to spend 2 or 3 hours producing something which his classmates produce in less than 1. Past the age of 11 or 12, it may be feasible to teach the child to type, and this may prove a quicker or at least a more legible way of getting his thoughts down on paper. When the attention span is short, it is obvious that three teaching sessions of 20 minutes each with a break between

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are preferable to a solid hour. The extent to which these modifications can be made in the average classroom naturally varies with circumstances, including the general philosophy of the school and the personality and flexibility of the teacher, and depends heavily on the size of the class. Even in the most fortunate areas, special classes and specially trained teachers are so limited that most children with minimal cerebral dysfunction inevitably have to be managed in regular classes. The consequence is that the classroom teacher will have to work out the best modified program he can, and will need the encouragement of the principal or headmaster in doing this. Parents often ask whether a private school would be preferable. In a few large cities and in a few suburbs there are private schools planned especially and directly for children of the type now discussed. Children with minimal cerebral dysfunction may be able to fit into schools aimed at the mildly backward or at those who are behind educationally because of circumstances such as having lived in various parts of the world where educational facilities are limited. It is sometimes possible to find a private school with small classes and uncritical admission policies in which such a child will fit well if the curriculum is not too rigid or demanding. More and more there is a tendency for private schools to upgrade themselves because of the abundance of applicants. This raising of standards may not be undesirable in itself, but it makes it more difficult to find a place for a child with a learning disorder or with any other special problem. All too often one finds that a community offers, on one side, schools for the mentally retarded and, on the other side, private schools aimed at preparing a child for Harvard right from the first grade, and nothing in between. In general, the special education classes or modified programs in regular classes in the local public schools will come closer to providing what the child needs than will the available private schools, but this is of course an individual matter from one community to another. Parents who are Roman Catholics often ask about parochial schools. Again, speaking in general, Catholic schools usually have larger classes, a higher pupil-teacher ratio, and a less flexible program. Thus for the most part they are a poorer choice than public schools, although in some instances the firmly structured program may be beneficial. It is apparent that the choice among types of school, even if this is financially possible, depends on circumstances which are different in every community. The physician must either be familiar with these circumstances and with the available resources or must turn to an educational consultant or social worker or psycholo_sist who has this first-hand knowledge. In many jurisdictions the public school authorities will reimburse the parents for part of the tuition for a private school if this is recommended medically and educationally, and provided the public schools have no placement to offer which is deemed suitable by the

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school authorities. This is usually handled by a department of special education or a pupil placement or pupil personnel service. Such a unit is also usually the best source of advice as to resources outside the public school system, and the physician is well advised to make contact with it and to use it.

PROGNOSIS

Despite the length of time that what we are now calling minimal cerebral dysfunction has been recognized, there is very little information available about follow-up. A review of the early cases at the Cove School in Racine, Wisconsin (Strauss and Lehtinen), would be of the highest interest, but has not yet been carried out. Some limited studies are discouraging, such as that of Menkes,2° in which most of the children have turned out badly. However, these were patients who had come to the attention of the psychiatric clinic at the Johns Hopkins Hospital and were thus a selected group. Another follow-up study now in progress, as yet unpublished, is based on cases known to the Department of Psychiatry at the Children's Hospital in Washington, D.C. My personal impression from having done the neurologic reassessment of some of these patients is that the abnormal physical neurologic signs have generally disappeared and that the prognosis for a satisfactory adjustment in life is not as bad as in the Menkes series. Obviously, no definitive comment can be made until more surveys are completed. It is a common "clinical impression" that the hyperactivity and distractibility which plague the teachers of the early primary grades tend to subside by the time the children reach the fifth or sixth grade, perhaps in the majority of cases. This may seem small comfort to the parents of a hyperactive 5- or 6-year-old, but it should be explained and does offer some hope for the future. Clumsiness also tends to improve, just as coordination improves in normal children with increasing age. The irregularity in different areas of performance on psychologic tests and in learning at school also tends to even out to some extent, but usually not completely so. Much depends on the prompt recognition, adequate study, and interpretation and optimal planning for management. An important point is that children with minimal cerebral dysfunction are not obviously enough different from their agemates to be accorded the protection from competition and the concessions which are given to the cerebral palsied or the mentally retarded. Some overlay of emotional problems exists in almost every case; depending on the child's personality and constitution and the way he is handled, the emotional problems may be paramount. One sees children of this type with disproportionate frequency in psychiatrists' offices, in child guidance clinics, in juvenile courts and among school dropouts. Among adults, the number who are

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in marginal employment, reduced socioeconomic circumstances or in psychiatric or correctional institutions is probably greater than in the general population. Nevertheless one suspects that he meets every day on the street many patients who formerly fell into the category of minimal cerebral dysfunction, but who are not much different from everyone else once the demands of educational pressures are over. They may be slightly awkward adults, they may be socially ill at ease or less successful than their siblings, but nevertheless must have made some reasonably satisfactory adjustment. Achieving this depends in no small part on acceptance by the patient and by his parents of his irregularities and limitations, and of educational and occupational aims which may be less than hoped for, or less than those of his brothers and sisters.

REFERENCES 1. de Ajuriaguerra, J., Antonini, P., Besson, A., et al.: Problems posed by dyslexia Journal of Learning Disabilities, 1:158, 1968. 2. Barsch, R. H.: Memorandum from Ray H. Barsch to Advisory Council Members, Officers, and Committee Chairman of the C.E.C. Division for Children with Learning Disabilities, 1967. 3. Bax, M., and :t-.facKeith, R., eels.: Minimal Cerebral Dysfunction. Little Club Clinics No. 10. London, William Heinemann, Medical Books, 1963. 4. Birch, H. G.: Brain Damage in Children-The Biological and Social Aspects. Baltimore, Williams and Wilkins, 1964. 5. Blau, A.: Mental changes following head trauma in children. Arch. Neurol. Psychiat., 35:123, 1937. 6. Bond, E.: Postencephalitic, ordinary and extraordinary children. J. Pediat., 1:310, 1932. 7. Clements, S. D.: Minimal Brain Dysfunction in Children. NINDB Monograph No. 3, Washington, U.S. Government Printing Office, 1966. 8. Critchley, M.: Developmental Dyslexia. Springfield, Ill., Charles C Thomas, 1964. 9. Davidoff, R. A., and Johnson, L. C.: Paroxysmal EEG activity and cognitive-motor performance. Electroenceph. Clin. Neurophysiol., 16:343, 1964. 10. Freeman, D. D.: Controversy over "patterning" as a treatment for brain damage in children. ].A.M.A., 202:385, 1967. ll. Gesell, A., and Amatruda, C.: Developmental Diagnosis. New York, Paul B. Roeber, Inc., 1941. 12. Halgren, B.: Specific dyslexia (congenital word-blindness): A clinical and genetic study. Acta Psychiat. Neurol. Scand., Suppl. 65, 1950. 13. Hermann, K.: Reading Disability: A Medical Study of Word-Blindness and Related Handicaps. Springfield, Ill., Charles C Thomas, 1959. 14. Hohman, L.: Post-encephalitic behavior disorders in children. Johns Hopkins Hosp. Bull., 33:372, 1922. 15. Hutt, S. J., Lee, D., and Ounsted, C.: Digit memory and evoked discharges in four light-sensitiYe epileptic children. Develop. Med. Child Neurol., 5:559, 1963. 16. Kahn, E., and Cohen, L.: Organic drivenness-A brainstem syndrome and an experience. New Eng. J. Med., 210:148, 1934. 17. Kirk, S. A.: The ITPA: Its origins and implications. 1n Hellmuth,]., ed.: Learning Disorders. Seattle, Special Child Publications, 1967, Vol. 3. 18. Kramer, F., and Pollnow, H.: tJber eine hyperkinetische Erkrankung im Kindesalter. Monatschr. f. Psychiat. u. Neuro., 82:1, 1932. 19. Me Glannan, F. K.: Familial characteristics of genetic dyslexia: Preliminary report from a pilot study. Journal of Learning Disabilities, 1:185, 1968.

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20. Menkes, M. M., and Menkes, J. H.: Minimal brain damage: 25 year follow-up, Pediatrics, 89:393, 1967. 21. Money, J., and Schiffman, G., eels.: The Disabled Reader. Baltimore, Johns Hopkins University Press, 1966. 22. Money, J., ed.: Reading Disability: Progress and Research Needs in Dyslexia. Baltimore, Johns Hopkins University Press, 1962. 23. Myklebust, H. R.: Progress in Learning Disabilities. New York, Grune & Stratton, 1968, Vol. 1. 24. Ong, B. H.: The pediatrician's role in learning disabilities. In Myklebust. 23 25. Orton, S.: Reading, Writing, and Speech Problems in Children. New York, W. \V. Norton & Co., 1937. 26. Ozer, M. N.: The neurological evaluation of school-age children. Journal of Learning Disabilities, 1:87, 1968. 27. Paine, R. S.: Organic neurological factors related to learning disorders. In Hellmuth, J., ed.: Learning Disorders. Seattle, Special Child Publications, 1965, Vol. 1. 28. Paine, R. S., Werry, J. S., and Quay, H. C.: A study of "minimal cerebral dysfunction." Develop. Med. Child Neurol. (in press). 29. Pasamanick, B., and Knobloch, H.: Brain damage and reproductive casualty. Amer. J. Orthopsychiat., 80:299, 1960. 30. Pollin, W., Stabenau, J. R., and Tupin, J.: Family studies with identical twins discordant for schizophrenia. Psychiatry, 28:60, 1965. 31. Shedd, C. L.: Dyslexia and its clinical management. Journal of Learning Disabilities, 1:171, 1968. 32. Sherman, M., and Beverly, B.: The factor of deterioration in children showing behavior difficulties after epidemic encephalitis. Arch. Neuro. Psychiat., 10:329, 1923. 33. Strauss, A.: Ways of thinking in brain-crippled deficient children. Amer. J. Psychiat., 100:639, 1944. 34. Strauss, A., and Lehtinen, L.: Psychopathology and Education of the Brain Injured Child. New York, Grune & Stratton, 1947. 35. Strauss, A., and Werner, H.: Disorders of conceptual thinking in the brain-injured child. J. Nerv. Ment. Dis., 96:153, 1942. 36. Twitchell, T. E., Lecours, A. R., Rudel, R. G., and Teuber, H. L.: Minimal cerebral dysfunction in children: Motor tests. Am. Neurol. Assn., 91:353, 1966. 37. Werner, H., and Strauss, A.: Pathology of figure ground relation in the child. J. Abnorm. Soc. Psychol., 86:58, 1941. 38. \Verner, H., and Thuma, B., A deficiency in the perception of apparent motion in children with brain injury. Amer. J. Psycho!., 55:58, 1942. 2125 13th Street, N.W. Washington, D.C. 20009