Upload
rosa-a
View
212
Download
0
Embed Size (px)
Citation preview
DEVELOPMENTAL LANGUAGE DISABILITY
SIMULATING MENTAL RETARDATION
Archie A. Silver) M.D. and Rosa A. Hagin) Ph.D.
There are a number of children, estimated at from 5 to 15 per cent ofthe American grade school population, boys outnumbering girls threeto one, who, without any demonstrable evidence of structural damageto the central or peripheral nervous systems, have difficulty in dealingwith stimuli on a symbolic level. These children are suffering fromdevelopmental language disorders (Brain, 1961; Chess, 1944), variously described as congenital language disorders or lags (Head, 1927),specific language disorders (Silver and Hagin, 1960), developmentalaphasias or dysphasias, dyslexias, dysgraphias, and dyspraxias (Bender, 1958; Money, 1962), congenital auditory imperception (Weisenberg and McBride, 1935; Worster-Drought and Allen, 1929a, b),congenitally word-deaf (Weisenberg and McBride, 1935) or wordblind children (Hallgren, 1950; Morgan, 1896), strephosymbolic(Orton, 1937) or sinlingualism (Peacher, 1950). These children havecertain characteristics in common:
1. There is a strong family history of language disturbance (Hallgren, 1950).
2. All have evidence that the neurophysiological organization, corresponding to cerebral dominance, is not fully established (Silverand Hagin, 1960).
3. All have specific perceptual problems in more than one perceptual area. These specific perceptual problems are characterized bydifficulty in orientation in space and in time, so that there are errorsin right-left orientation; disturbance in body image relative to orien-
From Department of Neurology and Psychiatry, New York University-Bellevue Medical Center. Supported in part by the Field Foundation and Carnegie Corporation ofNew York. Dr. Silver is Associate Clinical Professor of Psychiatry and Dr. Hagin is anInstructor in Clinical Psychology.
485
486 Archie A. Silver and Rosa A. Hagin
tation in space; problems with figure-background perception in visual, auditory, and sometimes tactile modalities; difficulty in orienting a Gestalt figure in space with a tendency toward primitive verticalization; inability to change direction in the reproduction of angularGestalt figures and errors in the temporal sequence of auditory stimuli (Silver and Hagin, 1960).
While these are the common characteristics unifying the developmental disorders of language, there are, of course, important individual differences. The pattern of perceptual deficit varies, so that:(a) we may group these children depending upon the area or areas ofmaximal perceptual defect. For example, those children with predominantly visual deficits will symptomatically have reading problems and those with predominantly auditory defects will have problems in spoken language; (b) even within each group of maximaldefects, an individual perceptual pattern and profile may be drawnfor each child.
The distribution of perceptual assets and deficits is important, notonly in understanding the extent of the individual child's deficits, butalso in planning remedial measures (Silver and Hagin, 1964).
This paper will focus on one aspect of the problem of developmental language disorders, namely, the child who has difficulty with spoken language. This defect in communication may so restrict the child'sfunctioning and his opportunity for the meaningful integration ofnew learnings that he will give the outward appearance of a pervasive mental retardation with all the prognostic pessimism that thistraditionally implies. The diagnosis of a developmental languagedisability implies a much more optimistic prognosis provided thatthe defect is clearly identified, the areas of perceptual deficit described,and a stimulating educational experience offered. Three patients willbe described: the first to illustrate the natural history of a child withhis major difficulty in emissive speech, the second to illustrate thechild with major difficulty in receptive speech, and the third to indicate some of the complexities in diagnosis and prognosis.
CASE PRESENTATIONS
I. Larry is now almost eighteen years old, a senior in high school,with good possibility of college acceptance. We have known himsince he was four and a half years old. At age two years and ten
Developmental Language Disability 487
months, because his parents were concerned about his lack of speech,he was seen by a psychologist who found him to have a Gesell Developmental Quotient of between 60 and 70, with retardation at borderline to defective level. Perceptually, adaptively, and motorically, hewas not at a two-year level. He understood verbal commands, such aspicking up objects from the floor, and seemed to respond to picturesof a car. He pulled at his mother's hand when he wanted her to helphim with a book. His only vocalizations were grunting noises. At agethree years and eleven months, he was still considered retarded, withperceptual motor development no higher than two and a half years.His speech still showed ability to comprehend verbal stimuli, but hedid not make any words. The Seguin Form Board, however, which hecould not do at his previous examination, he now easily accomplishedat a four-and-a-half-year level.
At age four and a half, he was seen in our clinic to evaluate possibleemotional causes for his difficulty. At that time, he was a tall, thinchild with a sad, vigilant expression. He leaned against his mother atfirst, but he was able to leave her and play, at his age level, with awooden train and tracks. He could easily copy a circle and attemptedcrossed horizontal and vertical lines. On verbal command he couldpoint to parts of his body and identify objects by pointing. He alsounderstood a gesture that indicated he should write or draw on theboard. He could not, however, name objects verbally. He was ableto demonstrate how the train runs and what to do with a ball. Whenforced, he said "eee" for "please." The mother said that he understood "everything verbally" and that he had a language of his own, aconsistent vocalization (idioglossia) for specific objects. He couldclearly say "no." He used either hand in his motor activity. He didnot sustain attention and became easily frustrated; to frustration heresponded with temper tantrums. He was fearful of new situationsand of the dark. Neurological examination, other than the above, wasnormal.
For the next three years, Larry received speech therapy; and by agesix and a half, his mother wrote that his speech was markedly improved-so much so that he even was using several sentences. He attended a normal kindergarten, recognized letters of the alphabet,read and wrote his name and a few words with reversals. He couldcount to ten; he could ride his tricycle; his behavior improved; his
488 Archie A. Silver and Rosa A. Hagin
fears (except for stairs from which he had fallen at one time) hadsubsided; and he joined children in games. He still was impatient,negativistic, and suspicious of new situations. The school reported anI.Q. in the low average range on a group intelligence test. So, by theage of six and a half years, a child originally functioning as defective,was now reaching into the average range.
At age nine and a half years, he was finishing second grade of aschool with high academic standing. He could now read, write, addand subtract at a second grade level. However, visual-motor perception showed rotational and angulational problems; laterality was stillnot clearly established; and his body-image concept was impaired.His speech, except for hesitation under tension, now showed a vocabulary commensurate with his grade and age. He still had difficultysettling down to work and became easily discouraged and frustrated.
Larry was seventeen when we saw him again. He was now at aboutthe middle of his class in an excellent suburban parochial high school.He had become a well-built young man, who recognized the examinerwith appropriate affect and responded clearly and appropriately. Hisenunciation was precise, almost too precise, giving a certain "stiffness"to his voice. He functioned with a full-scale I.Q. of Ilion the Wechsler Adult Intelligence Scale (121 verbal, 96 performance). On a perceptual battery he still showed mild verticalization and angulationproblems on the Bender-Gestalt drawings. He still had difficulty withfigure-background perception as seen on the marble board. He hadnot yet established clear-cut cerebral dominance and had a concreteapproach to the Goldstein Object-Sorting Tests. On the DiagnosticReading Test his silent reading rate was at the 25th percentile andhis comprehension of contextual material was at the 33rd percentile.On the other hand, his vocabulary was at the 66th percentile and hisoral reading (Jastak Wide Range Achievement Test) placed him atcollege level. While he was thus able to read along with the averagechild of his age and grade, he did so with great labor, slowness, anddifficulty in integrating. His handwriting was immature and donewith difficulty. Psychologically he was still easily discouraged, feltinadequate, and was afraid to tackle college. He still resented authority, particularly that of his parents.
There is a family history of language disability. An older sister(whom we studied) has a severe reading disability, a maternal uncle
Developmental Language Disability 489
has a reading disability, and a paternal uncle was a slow talker. Hisfather, despite slow reading, is a graduate of an Ivy League college.There was no evidence of difficulty during pregnancy, labor, or Larry'sneonatal period. His first year was characterized by two abnormalities: lack of verbal expression, and toilet training punitively accomplished by eleven months.
In short, here is a boy thought at age two years and ten months tobe defective, who is now, at age eighteen years, functioning with anI.Q. of III with even higher potential. His most striking problem wasin the area of emissive speech; now he has problems in reading comprehension and rate and in handwriting. While he has matured inmany areas of perception, he has not yet effectively established clearcut cerebral dominance and still has problems in spatial orientation.In retrospect, we should have been aware of the multiple nature ofLarry's language disorder and, perhaps, offered remedial reading tohim when he was nine years old.
In contrast to Larry, who could comprehend the spoken word andwhose family was at a professional level, David could not even understand what was said. His background is bilingual and his family has alow motivation for help.
2. David was five years and seven months of age when we first sawhim-a small, olive-skinned child of Puerto-Rican, Spanish-speakingparents. He was the fourth in a family of six children. The otologistswere certain he could hear, but he did not speak in English or inSpanish. On the Wechsler Intelligence Scale for Children, his performance score was 72, but no true verbal score could be obtained. Ineffect, he functioned at a defective level. On our examination, he wasan alert, appealing child, who sat quietly on the chair, smiling whenspoken to, showing interest in and awareness of his environment. Hecould definitely hear a whispered voice from across a play table. Hecould not, however, follow simple verbal commands and could notidentify parts of his body on verbal command. He could identify pictures of a ball when shown a ball and he could understand gestures.For example, he could not draw a picture when asked without accompanying gesture. When a circle was drawn on the board, however, hesmiled and readily copied it. The examiner pointed to his eyes, andDavid drew an eye, nose, and mouth. Perceptually, he could reproduce
490 Archie A. Silver and Rosa A. Hagin
a circle. Laterality was not yet established. He could, in short, understand nonverbal material and could execute it in a nonverbal way. Hisproblem was mainly one of receptive speech, although he appearedretarded in all areas. Neurological examination, other than above,was normal.
Now at age eight years and six months, David is in second grade ina slow class. His teacher reports that he seems to enjoy school. He cantalk in Spanish to other children and, although he does not initiatespeech with her, he can answer questions in single words of English.He is well liked by his classmates, having been elected president ofhis class; the vice-president helps him if he needs to talk. Academically, he can read at the preprimer level; arithmetic is at first grade;and his handwriting is good. On our examination, the performanceI.Q. on the Wechsler Intelligence Scale is now 89 as contrasted to 72three years ago. He showed specific defects in visual and tactile perception, although again, compared to his performance three yearsbefore, his maturation in these areas has been excellent. He is awareof his difficulty on the marble board and he attempts to compensateby placing the marble between the holes to complete a diagonal. Hisdrawing of a person scores at a mental age of six years. Clear-cut dominance is not yet established. In the area of speech, he has progressed;he can understand the spoken word and can respond in short sentences. The problem here is to know just how much he has improvedand how he compares with other children his age. The tests of HenryHead (1927) and of Weisenberg and McBride (1935), althoughuseful, have been devised for adults. Kirk and McCarthy (1961) triedto devise and standardize a test of "psycholinguistic abilities" forchildren. This test attempts to tap abilities in the receptive, associative, emissive, and "automatic" functions of spoken language. Itsmaximal age level is at eight years and nine months. While the testis still in an experimental edition and its validity is not yet established, it is one of the few comprehensive standardized tests for assessing language development in children. On this test, David's difficulty with understanding and expressing the spoken word is evidentsince he scores at about three years in his comprehension, association,and use of spoken material. On the other hand, his ability to understand visual stimuli, make visual associations, and respond in a visual-motor manner scores at about seven years with maximal function-
Developmental Language Disability 491
ing on pantomime expression of language at eight years and eightmonths (i.e., show me what you should do with this).
David demonstrates a severe problem in auditory speech comprehension, complicated by a bilingual home in which the general intelligence level is dull-normal to borderline at best, and where themotivation for learning is low. Yet, even in this setting David has, inthree years, matured at least three years in speech, and his emotionaladjustment seems good. As expected, specific perceptual problems, inaddition to the auditory ones, remain, and lateral dominance is notyet clearly established. It is anticipated that our goals here must bemodest.
Larry and David, both initially retarded in all areas but especiallyin language, illustrate the maturation which can occur in these developmental problems. The question is frequently asked, however,how do we know there are no structural defects of the central nervoussystem to account for the deficits found. Classical neurological signsand electroencephalogram are normal: behavioral syndromes associated with structural disease of the central nervous system and histories that are suggestive of possible organic damage are absent in thechildren described above.
In a certain percentage of children with developmental languagedisorder, perhaps in three out of ten, some abnormality in neurological examination, EEG, behavior or history suggest structural damage.Whether this is superimposed upon a developmental lag or whetherit is directly etiological cannot always be determined. Yet, the recognition of structural defects is most important, since prognosis is guarded and treatment more difficult in those children with "organic" signs.Abie illustrates this complex.
3. Abie is now ten years and eleven months old, a tall, sturdy boyof Puerto-Rican, English-speaking parents. He is 62 inches tall,weighs l25Ibs., and his head circumference is 20~ inches. His eyes areslanted and his classmates call him "chink"; his parents also haveslanted eyes. Abie gives the impression of being fat with a girdle obesity and a full-moon face. He appears to have an endocrine problemand, although when he was four, his carpal age was considered oneyear retarded, now, aside from deposition of fat, the pediatricianfinds no endocrine abnormalities. Abie first came to us when he was
492 Archie A. Silver and Rosa A. Hagin
eight years and ten months of age because of unmanageable behaviorat school. He was pleasant, friendly, and affable, and mildly hyperkinetic. He had no difficulty in hearing and in understanding whatwas said to him; hearing tests revealed no selective hearing loss. Butit was apparent that he had difficulty in naming objects. When asked,for example, to point to his nose, he readily did so with an expressionimplying, "how can you ask such a silly thingl" Yet, he could notname what he had just pointed to. He said "n-n-n" (Is it a nose?).There was no hesitation. He said "yes" and he could write "nose,"but he could say "nos" nly after repetition of the word by the ex-aminer. He could erbally give the names of some common ob-jects, but he so es could spell them, sometimes approximatedthem, som . e them, or pantomimed their use. His problemwas sso iati and expressive verbal language. He had theoth acteri tics e expect in developmental language lags: lack
ear-cut cere ral ominance, the spatial disorientation with rightleft confusion, v ti alization, angulation difficulties in primitive Gestalt drawings, tac . e and visual figure-background abnormality. Hedrew a clock face in great detail, but could not tell the time. He couldnot deal with abstractions, even on a visual level.
Repeat examinations, at age nine years and eleven months and atten years and eleven months, show a significant spurt in maturation,in visual and tactile perception, and in associative aspects of speech.In this area, he had, in one year, matured two and a half years. Reading also has shown a spurt. The Illinois Test of Psycholinguistic Abilities reveals graphically the relatively intact receptive auditory language ability and the problems in associative and emissive speech.Abie, like Larry, understands the spoken word, but is limited in hisverbal expression. His word-finding difficulty shows up in occasionalneologisms, confusion of word pairs within a class (e.g., "hard" usedinstead of "soft" when describing a cushion), and verbal detours (e.g.,he called the picture of a painter a "painting man"; he called a buttonhole a "jacket-hole").
Again, we see the pervasiveness of perceptual deficit through allperceptual modalities and the tenacity with which these deficits linger.
But with Abie, two complications arise: (I) his birth was via Caesarean section after a trial at labor in a breech presentation (his
Developmental Language Disability 493
mother was described as "pre-eclamptic"); and (2) his dysplasticappearance prompted chromosome studies (through the courtesyand cooperation of Dr. Kurt Hirschhorn) where four types of abnormality were found. From the buccal mucosa, there was an increasein percentage of cells containing sex chromatin. From the peripheralblood after culture and preparation, cells with the XXV configuration, cells with the triple #21 chromosome and normal cells werefound. The Karyo-type was interpreted as Kleinfelter-mosaic, mongolmosaic pattern. Although neurological examination, other than theabove, was normal, EEG revealed photic drive on the left. Skull Xrays were normal and the usual phenotypic abnormalities of mongolism were not found.
So here we have superimposed upon what appeared to be a developmental defect, evidence of brain damage from the history, behavior, and EEG, and proven genetic abnormality. How many children,normal in appearance, are masking genetic abnormality usually associated with retarded mental development? What is the relationshipbetween mild structural damage to the central nervous system andthe developmental language lags? Obviously, this case raises morequestions than we can answer at this time. He illustrates the complexinteraction of factors which must be considered in diagnosis and inmanagement.
SUMMARY
The child with developmental retardation in spoken language,with his relatively good prognosis. may give the appearance of generalized retardation with its relatively poor prognosis. Three patients,whom we have followed longitudinally, have been presented to illustrate (1) the varying pattern of perceptual assets and deficits; (2)that maturation does occur in these children with developmental language disability; and (3) that the associated perceptual problemsare found in more than one modality and tend to leave their telltalemark even when some degree of maturation has occurred.
REFERENCES
B ENDER, L. (1958). Problems in conceptualization and communication in children withdevelopmental alexia. In: Psychopathology of Communication, ed. P. Hoch &: J.Zubin. New York : Grune &: Stratton.
494 Archie A. Silver and Rosa A. Hagin
BRAIN, R. (1961). Speech Disorders. Washington, D.C.: Butterworth.CHESS, S. (1944). Developmental language disability as a factor in personality distor
tion in childhood. Amer. I. Orthopsychiat., 14:483·490.DE HIRSCH, K. (1952). Specific dyslexia or strephosymbolia. Folia Phoniatrica, 4:2!11-248.HALLGREN, B. (1950). Specific dyslexia (congenital word-blindness). Acta Psychiat, ir
Neurol., 65:224·2!12.HEAD, H. (1927), Aphasia and Kindred Disorders of Speech, 2 Vols. Cambridge: Uni
versi ty Press.KIRK, S. A. Be MCCARTHY, J. J. (1961), The Illinois Test of Psycholinguistic Abilities:
Experimental Edition. Urbana. Ill.: Institute for Research on Exceptional Chilodren, Univ. of Ill.
MONEY, J. (1962). Reading Disability: Progress and Research Needs in Dyslexia. Baltimore: Johns Hopkins Press.
MORGAN, W. P. (1896), A case of congenital word-blindness. Brit. Med. I., 2:1378.ORTON, S. T. (1937), Reading, Writing and Speech Problems in Children. New York:
Norton.PEACHER, W. E. (1950). Neurological observations in delayed speech. Quart. I. Child
Behau., 2:194·216.SILVER, A. A. Be HAGIN, R. A. (1960). Specific reading disability: delineation of the syn·
--- drome and relationship to cerebal dominance. Comprehen. Psychiat., 1:126·134.-- -- (1964), Specific reading disability: follow-up studies. Amer. I. Orthopsy.
chiat., !I4:95·102.WEISENBERG, T. H. Be McBRIDE. K. E. (1935), Aphasia: A Clinical and Psychological
Study. New York: Commonwealth Fund.WORSTER·DROUGHT, C. Be Au.EN, I. M. (1929a). Congenital auditory imperception (con
genital word- deafness) with report of a case. I. Neurol, ir Psychopathol., 9:193·208.-- --(1929b). Congenital auditory imperception: investigation of a case by Head's
method. I. Neural. .,. Psychopathol., 9:289·!I19.