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[A case of bilateral lesions in the temporal convexity: attempt to define symptoms].

The case is reported of a boy aged 15 who suffered a bilateral lesion of the temporal convexity after a brain traumatism; he died 5 years later and the symptoms during that period are described. The clinical picture is adequately described neither under the title of dementia, nor under that of aphasia. The concept of an abolition involving the systems of cultural mediation (linguistic, technical) is introduced. The status of echolalia and echo-praxia is discussed.

Adolescent↗

Perseveration and other repetitive verbal behaviors: functional dissociations.

This article will review types of perseveration from a neurolinguistic perspective. During the course of the article, continuous, stuck-in-set, and recurrent perseveration will be placed in contradistinction to several other types of repetitive behaviors commonly associated with neurogenic communication disorders. These include echolalia in mixed transcortical aphasia; conduite d'approche and conduite d'ecart in fluent aphasias; lexical and nonlexical automatisms in nonfluent aphasias; palilalia in neuromotor disorders, such as Parkinson's disease (PD); and sound, syllable, word, and phrase repetitions in neurogenic stuttering. When differentiating these phenomena from perseveration, it is helpful to consider the salient factors that condition observed behaviors in individual patients, such as overall speech fluency, inventory of available utterances, nature of eliciting tasks, and propositionality of responses. Information such as communication disorder diagnosis, underlying etiology, and known sites of lesion from each patient's total clinical profile may also assist with differentiation.

Aphasia↗

Beyond echoplaylia: promoting language in children with autism.

The article was written to support the use of play as a medium to extend and enrich the communicative exchanges and, more specifically, the symbolic language of children on the autistic spectrum. The first argument in support of such use of play lies within the features of autistic communication, and particularly the extreme challenges encountered in imaginative play and narrative thought. The second argument on behalf of play lies within the selection of specific intervention strategies, which are discussed subsequently. Finally, the article presents a case illustration of how adult facilitated dramatic peer play led to a breakthrough in a range of symbolic behaviours in a 9-year-old girl with autism, who up to that point had not progressed beyond a semi-communicative use of echolalia, best described as 'situation association'.

Autistic Disorder↗

Jumping Frenchmen of Maine.

The "Jumping Frenchmen of Maine" were described by George Beard in 1878. They had an excessive startle response, sometimes with echolalia, echopraxia, or forced obedience. In 1885, Gilles de la Tourette concluded that "jumping" was similar to the syndrome that now bears his name. Direct observations of jumpers have been scarce. We studied eight jumpers from the Because region of Quebec. In our opinion, this phenomenon is not a neurologic disease, but can be explained in psychological terms as operant conditioned behavior. Our cases were related to specific conditions in lumber camps in the 19th and the beginning of the 20th century.

Culture↗

Elimination of echolalic responding to questions through the training of a generalized verbal response.

Echolalia, the parroting of the speech of others, is a severe communication disorder frequently associated with childhood schizophrenia and mental retardation. Two echolalic children, one schizophrenic and one retarded, were treated in a multiple-baseline design across subjects. Each child was taught to make an appropriate, non-echolalic verbal response (i.e., "I don't know") to a small set of previously echoed questions. After such training, this response generalized across a broad set of untrained questions that had formerly been echoed. The results obtained were the same irrespective of the specific experimenter who presented the questions. Further, each child discriminated appropriately between those questions that had previously been echoed and those that had not. Followup probes showed that treatment gains were maintained one month later. The procedure is economical, in that it produces a rapid and widespread cessation of echolalic responding.

Adolescent↗

Replacing maladaptive speech with verbal labeling responses: an analysis of generalized responding.

We taught three mentally handicapped students to answer questions with verbal labels and evaluated the generalized effects of this training on their maladaptive speech (e.g., echolalia) and correct responding to untrained questions. The students received cues-pause-point training on an initial question set followed by generalization assessments on a different set in another setting. Probes were conducted on novel questions in three other settings to determine the strength and spread of the generalization effect. A multiple baseline across subjects design revealed that maladaptive speech was replaced with correct labels (answers) to questions in the training and all generalization settings. These results replicate and extend previous research that suggested that cues-pause-point procedures may be useful in replacing maladaptive speech patterns by teaching students to use their verbal labeling repertoires.

Adolescent↗

Modifying echolalic speech in preschool children: training and generalization.

An operant-conditioning technique was used to teach three handicapped children exhibiting echolalia to respond appropriately to a series of questions. The technique included punishment of echolalic responses and reinforcement of appropriate responses that were cued by a tape recording of the appropriate response. Frequencies of correct responses to the questions increased for each child following a within-subject multiple baseline design. Echolalic responding eliminated in response to trained questions. Generalization of training to several functional responses was found in addition to increased spontaneous use of a functional phrase. Generalization of trained responses across settings and stimuli was also suggested. A 12-month follow-up of two of the subjects revealed no increase in echolalic responding and moderate maintenance of the trained responses. The subjects also showed notable response generalization to untrained questions at the time of the follow-up probe.

Autistic Disorder↗

[Isolation syndrome of the language area. Neuropsychologic and pathologic study].

A 60 year-old patient in whom spontaneous language was abolished still had a marked capacity for completing phrases and an irrepressible echolalia. From a formal point of view, linguistic ability remained at a high level. Auditory verbal comprehension was severely affected, but was not completely abolished. There was occipital blindness. Pathologic examination reveated extensive bilateral lesions in the regions supplied by the anterior and posterior cerebral arteries. Bilateral destruction of the supplementary motor areas was noted, but the perisylvian language areas were intact. These disorders constitute a syndrome of isolation of language areas which can be used to study anatomical correlations and particularly the role of the supplementary motor area in linguistic and paralinguistic processes.

Akinetic Mutism↗

Gilles de la Tourette and the discovery of Tourette syndrome. Includes a translation of his 1884 article.

In 1885, Gilles de la Tourette described 9 patients who suffered from a disorder characterized by involuntary movements, echolalia, echopraxia, coprolalia, and strange, uncontrollable sounds. In his article, Gilles de la Tourette presented some earlier descriptions of this disorder. To appreciate what first led Gilles de la Tourette to Tourette syndrome, however, it is necessary to turn to an article that he published a year earlier. In his 1884 article, Gilles de la Tourette cited several movement disorders that he thought were similar to each other, yet different from true chorea. After describing these disorders, namely, "jumping" of Maine, latah of Malaysia, and miryachit of Siberia, he briefly mentioned a boy in Charcot's ward in Paris, France, who seemed to exhibit the same condition. In an addendum, he then said that other cases were now surfacing in Paris and that he would write an additional article describing these individuals. To achieve a more thorough understanding of the events that led Gilles de la Tourette to his 1885 description of the disorder that now bears his name, we herein present an English-language translation of his 1884 article along with a commentary.

Eponyms↗

Tourette syndrome. The pediatric perspective.

I report the clinical details of Tourette syndrome in 15 children. The condition typically starts at age 6 years with eyeblinking, and the child soon develops other tics and abnormal vocalizations. Coprolalia and echolalia occure but are infrequent. The average delay in correct diagnosis in this series was four years. Treatment with haloperidol produces a good or excellent response in three quarters of the patients. Many of the children have a history of encephalopathic events, "soft signs" on neurologic examination, and problems in school. Personal and social adjustmen are generally good, however.

Child↗

Sign language tics in a prelingually deaf man.

Gilles de la Tourette syndrome is characterized by vocal and motor tics starting in childhood. Vocal tics may be either noises or words, and the vocal language tics may consist of obscenities (coprolalia) and repetitions of speech that has been heard (echolalia). We describe a prelingually deaf man who has the full array of tics seen in Gilles de la Tourette syndrome, but in whom vocal language tics are replaced by equivalent sign language tics. This is, to our knowledge, the first report of sign language tics in a person with prelingual deafness. The implications of this phenomenon for the separation between language and ideas in tics and the equivalence of sign language to spoken language is discussed.

Adult↗

Behavioral features of CHARGE syndrome (Hall-Hittner syndrome) comparison with Down syndrome, Prader-Willi syndrome, and Williams syndrome.

CHARGE syndrome, or Hall-Hitner syndrome (HHS), has been delineated as a common syndrome that includes coloboma, choanal atresia, cranial nerve dysfunction (particularly asymmetric facial palsy and neurogenic swallowing problems), characteristic ear abnormalities, deafness with hypoplasia of the cochlea and semicircular canals, genital hypoplasia, and variable heart defects, orofacial clefting, tracheo-esophageal fistula, renal anomalies, thymic/parathyroid hypoplasia, spine anomalies, short broad neck with sloping shoulders, and characteristic facial features. We conducted behavioral and personality assessments in 14 boys with HHS syndrome aged 6-21 years, and compared their characteristics with similar data from 20 age-matched boys with Down syndrome (DS), 17 boys with Prader-Willi syndrome (PWS), and 16 boys with Williams syndrome (WS). We used the Reiss Profile of Fundamental Goals and Motivation Sensitivities, the Achenbach Child Behavior Checklist (CBCL), and the Aberrant Behavior Checklist (ABC). All 14 boys with HHS were legally deaf, and 10 of the 14 were also legally blind. In comparison these other syndromes, boys with HHS had behavior that resembled autistic spectrum disorder. They were socially withdrawn, lacked interest in social contact, and manifested reduced seeking of attention from others, with hyperactivity and a need to maintain order. Though the boys with HHS showed decreased social interaction, they were not as socially impaired as in classic autism. Their language was delayed due to dual sensory impairment, cranial nerve deficits, and chronic medical problems, but their language style was not abnormal (no echolalia or jargon, no scripted phrases, and no pronoun reversal). Boys with HSS appeared frustrated, but they were not aggressive, or at risk for delinquency, manifesting few stereotypic behaviors or unusual preoccupations. They did not have a restricted repertoire of activities and interests. Their behavioral features appeared to be due to dual sensory impairment affecting hearing and vision, rather than to primary autistic spectrum disorder, but successful remediation requires similar educational interventions, which are discussed herein.

Abnormalities, Multiple↗

Developmental and behavioural disturbances in 13 boys with fragile X syndrome.

Developmental and behavioural aspects were studied in 13 boys aged 2.6-12.5 years from three families with the fragile X syndrome. The following observations were made. Moderate to severe retardation was present in all boys; non-verbal IQs ranged between 25 and 67 (mean 46 +/- 14); IQ and age were negatively correlated (P less than 0.01). Language development was grossly delayed in all boys; most had severe articulation problems. Imitative and symbolic play (e.g. doll play) were strikingly retarded as compared to abstract play (e.g. block design). Autistic features such as no use of eye contact, stereotyped movements and echolalia were found in 9/13 boys; the same number showed aggressive behaviour. General activity was reduced during the 1st year of life; most boys became very hyperactive during the second year; and short attention span and increased distractability were observed in all. Motor development was mildly delayed; all boys were clumsy and moderately hypotonic. The fragile X syndrome ought to be considered in retarded boys with a dissociated developmental pattern, in particular a striking delay in language and play development, and autistic features.

Child↗

"Echoing approval": a new speech disorder.

We report the cases of two patients presenting a peculiar speech disorder, which we have named "echoing approval", in which the patients echo, in replying to questions in a dialogue with short phrases, the positive or negative syntactical construction of a question, or its positive or negative intonation, but without any repetition of whole or part of sentences. When asked about their symptoms, the patients replied 80% of the time with "yes, yes", "that's right", or "exactly" to positive questions and "no, no" or "absolutely not" to negative questions, regardless of their actual symptoms and oblivious to self-contradiction. In addition, when the examining doctor was speaking to a medical colleague in the patient's presence and using medical terminology that the patient did not understand, he/she agreed or disagreed with any sentence and technical word uttered in a way entirely dependent on the syntax or intonation used. To distinguish this speech disorder from echolalia or verbal perseverations, with which it may be superficially confused, we suggest that it be called "echoing approval", as it may be part one of the manifestations of the environment-dependency syndrome. This clinical picture was found to be associated with features of transcortical motor aphasia and frontal lobe signs. One patient had a bilateral callosofrontal malignant glioma and the other a probable multiple system atrophy with global deterioration, pre-eminent frontal release signs, diffuse leukoencephalopathy and multiple lacunes. On the basis of these clinical deficits and neuroimaging features, we are unable to delineate the common, or minimal, lesioned network required for this symptomatology to occur, especially in the absence of a series of patients, and with such a difference in both the location and causes of the lesions. However, bilateral frontosubcortical dysfunction was pre-eminent in the clinical picture in both patients, even though more diffuse brain pathology was seen in one, and it might be speculated that dysfunction of the bilateral orbitofrontal and frontomesial motor frontosubcortical circuits might be involved in the aetiology of this peculiar speech disorder.

Aged↗

A comparison of language characteristics of mentally retarded adults with fragile X syndrome and those with nonspecific mental retardation and autism.

Fragile X syndrome is a recently identified form of mental retardation that is associated with a chromosomal abnormality and inherited in an X-linked manner. Previous studies have suggested that distinctive speech and language characteristics are associated with the syndrome. Twelve adult male residents of an institution for the retarded (aged 23 to 51 years) were compared on a series of speech and language measures to 12 adult males with nonspecific forms of MR who were residents of the same institution and were matched on age and IQ. A second contrast group consisted of similarly matched autistic men. Results revealed that there were no significant differences among the groups' performance, with the exception of increased rates of echolalia in the autistic group. A nonsignificant trend toward poorer performance on expressive measures on the part of the fragile X group was noted. The implications of these findings for further research on the syndrome are discussed.

Adult↗

Autism, mental retardation, and chromosomal abnormalities.

There are reports of sex chromosomal abnormalities including XXY, XYY, and fragile X karyotypes in autistic individuals, but structural autosomal defects have rarely been reported. This paper presents four patients with autism, mental retardation, minor dysmorphic features, and structural autosomal defects. These patients shared autistic features including fascination with inanimate objects, catastrophic reactions to changes in their environment or their daily routine, echolalia, and poor relatedness; IQ scores indicate mild to severe retardation. Their autosomal abnormalities included inversion/duplications of 3p and 16q, 5p+, and 17p-. Parental chromosomes were all normal. Chromosomal analysis should be performed on mentally retarded, autistic individuals, especially those with minor physical anomalies and no specific etiology for their retardation.

Adolescent↗

Cognitive processing and linguistic reference in autistic children.

Mutism or echolalia and failure to analyze sentences for their deep structures are characteristics of the language behavior of autistic children. The experimental literature indicates that cognitive processes which underlie the construction of a lexicon or the learning of ordering rules may be disturbed in these children. The inability to coordinate visual and aural stimulation inhibits lexical development, while difficulties in sequencing and use of inappropriate sequencing codes inhibits the growth of a productive grammar. It is suggested that remedial programs which include training in these processes may prove beneficial, and that substitute modes of information processing which the autistic child uses may be exploited to teach language forms.

Association Learning↗

Objectively defined linguistic parameters in children with autism and other developmental disabilities.

The language of children with autism and other developmental disabilities was examined systematically according to a set of objectively defined linguistic parameters. These criteria were drawn from clinical observations reported in the literature and from developmental norms of language acquisition. Data analysis identified sets of parameters that were correlated with psychiatrists' clinical diagnoses but failed to isolate individual parameters (such as echolalia or noncommunicativeness) that have been suggested to be pathonomic.

Autistic Disorder↗