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PubMed1977

A neurophysiological basis of developmental dyslexia.

Recent advances in the understanding of the asymmetrical functions of the cerebral hemispheres provide new insight to the mechanisms underlying the developmental language disorders. It is probable that learning to read depends in part upon the ability to establish an association between a seen object (customarily perceived within the right hemisphere) and a verbal symbol (mediated by the left). It is possible that as the reading skill develops, verbal symbol recognition may also become a left hemisphere function. A complex interaction between the two developing hemispheres is involved. A clue to the nature of this problem is that developmental dyslexia is six times more common in boys than in girls. Studies defining differences in cerebral dominance in boys and girls will be reviewed in this context.

Auditory Perception↗
PubMed1990

Reaction time measures of interhemispheric transfer time in reading disabled and normal children.

This experiment was designed to test whether reading disabled boys differ from matched controls on behavioral measures of interhemispheric transfer time (IHTT). Specifically, we proposed that language-disordered reading disabled children who had deficits in naming would show either faster or slower IHTTs compared with controls. From an initial group of 118 right-handed males, we selected a group of 25 disabled and 25 normal readers, matched on age. All subjects had to obtain a full scale IQ of 90 or above, a PIQ score of 85 or above, and a scaled score of 7 or above on the Block Design Subtest of the WISC-R. After meeting additional criteria for group assignment, manual reaction time (RT) measures of IHTT were obtained in response to simple visual and tactile stimuli during two laboratory testing sessions. Half the trials were conducted with the hands in an uncrossed orientation and half with the hands crossed in order to examine the effects of spatial compatibility on estimates of IHTT. The results revealed no overall group differences in IHTT for any of the conditions. However, correlations between IHTT measures and indices of cognitive performance indicated that faster IHTTs were significantly correlated with poorer performance on measures of reading and language function in the dyslexic group. These data are discussed within the context of a model of interhemispheric transfer deficits in disabled readers.

Child↗
PubMed1975

Control of echolalic speech in psychotic children.

Immediate echolalia, a common language disorder in psychotic children, was studied in a series of replicated single-subject designs across six schizophrenic and five normal children. In Experiment 1, each child was presented with several questions and commands, some of which set the occasion for specific, appropriate responses and some of which did not. The former were referred to as discriminative stimuli and the latter, as neutral stimuli. The psychotic children tended to echo the neutral stimuli while responding appropriately to the discriminative stimuli; the normal children, in contrast, typically echoed neither type of stimulus. In Experiment 2, three psychotic children were taught appropriate responses to each of several neutral stimuli. Following this training, the children generally responded appropriately to these stimuli without echoing. A plausible interpretation of these results is that the neutral stimuli were initially incomprehensible or meaningless to the children (whereas the discriminative stimuli were comprehensible or meaningful) and that verbal incomprehensibility may be one important determinant of immediate echolalia. Finally, the results are noteworthy in that they isolate a sufficient treatment variable (i.e., the reinforcement of alternative, nonecholalic responses) for eliminating instances of this language anomaly.

Adolescent↗
PubMed2004

Clinical, cognitive and anatomical evolution from nonfluent progressive aphasia to corticobasal syndrome: a case report.

Recent clinical and pathological studies have suggested that frontotemporal lobar degeneration (FTLD) and corticobasal syndrome (CBS) show clinical and pathological overlap. We present four years of longitudinal clinical, cognitive and anatomical data in the case of a 56-year-old woman, AS, whose clinical picture evolved from FTLD to CBS. For the first three years, AS showed a progressive speech and language disorder compatible with a diagnosis of the nonfluent aphasia variant of FTLD. At year four, 10 years after her first symptom, AS developed the classical clinical signs of CBS, including alien limb phenomenon and dystonia. Voxel-based morphometry (VBM) applied to AS's four annual scans showed progression of atrophy from the inferior posterior frontal gyrus, to the left insula and finally to the medial frontal lobe. This case demonstrates the clinical overlap between FTLD and CBS and shows that the two can appear in the same patient at different stages of the disease in relation to the progression of anatomical damage.

Aphasia, Broca↗
PubMed2003

Progressive non-fluent aphasia is associated with hypometabolism centred on the left anterior insula.

Progressive non-fluent aphasia (PNFA) is a syndrome in which patients lose the ability to communicate fluently in the context of relative preservation of single word comprehension and non-linguistic cognitive abilities. Neuroimaging in case studies with PNFA has failed to identify a consistent neural substrate for the language disorder. In this study of a group of patients (n=10) whose presenting complaint was progressive dysfluency, resting cerebral metabolism was measured using [18F]fluorodeoxyglucose-PET and analysed with the technique of statistical parametric mapping (SPM). Regional atrophy was assessed with voxel-based morphometry (VBM). Seven patients had a 'pure' PNFA syndrome, while the remaining three had additional features of a more pervasive dementia. Compared with controls, the patients showed hypometabolism in several regions that, most notably, included the left anterior insula/frontal opercular region. The VBM analysis revealed only one small area of atrophy in the left peri-Sylvian region. Analysis of the pure PNFA cases (n=7) relative to controls yielded qualitatively similar results to those of the whole group, suggesting that these cases were also at risk of a more generalized dementia, a finding borne out in subsequent follow-up of two cases to date. The PNFA group was then compared with a group with Alzheimer's disease (n=10) whose clinical profile did not include non-fluent aphasic features. In this analysis, the only persisting hypometabolic region was that centred over the left anterior insula. VBM did not identify any regional differences in atrophy between PNFA and Alzheimer's disease. In the light of current theories of fluent language production, the findings offer anatomical evidence that the breakdown in fluency is due to a motor articulatory planning deficit (speech apraxia) combined with a variable degree of agrammatism.

Aged↗
PubMed1999

An event-related potential study in schizophrenia using Japanese sentences.

To examine the neurophysiological and cognitive characteristics of language disorder in schizophrenia, the N400 component and late positive component (LPC) of event-related potentials (ERPs) were investigated in medicated schizophrenic patients and health comparison subjects. The subjects were required to indicate whether Japanese sentence completions were semantically congruous or incongruous. The ERPs for the range of 300-500 ms to the incongruous completions contained a more negative component (N400), followed by LPC, which was inversely more positive for the incongruous than congruous condition. The N400 effect and the mean amplitude of the LPC were reduced in the patients. The attenuated N400 effect in schizophrenics mainly originated from an enhanced negativity for the congruous completions, suggesting that the use of context is poor in schizophrenia.

Adult↗
PubMed1989

Modeling versus modeling plus evoked production training: a comparison of two language intervention methods.

The effectiveness of two language intervention procedures, modeling versus modeling plus evoked production, was compared using a single-subject alternating treatments design. Four language-disordered children (5:5-6:11) participated in the study, consisting of four baseline sessions and 10-14 training sessions. There was no marked difference in the effectiveness of the modeling procedure and the modeling plus evoked production procedure as measured by performance on generalization probes following each training session. In fact, the highest levels of accuracy attained under the two training procedures were quite comparable. There was a tendency, however, for the modeling plus evoked production treatment to result in a slightly more stable pattern of acquisition for 3 of the subjects. Although steps were taken to minimize multiple-treatment interference, the possibility of these effects must be considered, and caution should be taken in interpreting these findings to mean that these two intervention methods would produce equivalent results when administered independently.

Age Factors↗
PubMed2001

Disruption of a novel gene (IMMP2L) by a breakpoint in 7q31 associated with Tourette syndrome.

Gilles de la Tourette syndrome (GTS) is a complex neuropsychiatric disorder characterized by multiple motor and phonic tics. We identified a male patient with GTS and other anomalies. It was determined that he carried a de novo duplication of the long arm of chromosome 7 [46,XY,dup(7)(q22.1-q31.1)]. Further molecular analysis revealed that the duplication was inverted. The distal chromosomal breakpoint occurred between the two genetic markers D7S515 and D7S522, which define a region previously shown to be disrupted in a familiar case of GTS. Yeast and bacterial artificial chromosome clones spanning the breakpoints were identified by means of FISH analysis. To further characterize the distal breakpoint for a role in GTS, we performed Southern blot hybridization analysis and identified a 6.5-kb SacI junction fragment in the patient's genomic DNA. The DNA sequence of this fragment revealed two different breaks in 7q31 within a region of approximately 500 kb. IMMP2L, a novel gene coding for the apparent human homologue of the yeast mitochondrial inner membrane peptidase subunit 2, was found to be disrupted by both the breakpoint in the duplicated fragment and the insertion site in 7q31. The cDNA of the human IMMP2L gene was cloned, and analysis of the complete 1,522-bp transcript revealed that it encompassed six exons spanning 860 kb. The possible role of IMMP2L and several other candidate genes within the region of chromosomal rearrangement, including NRCAM, Leu-Rch Rep, and Reelin, is discussed. The 7q31 breakpoint interval has also been implicated in other neuropsychiatric diseases that demonstrate some clinical overlap with GTS, including autism and speech-language disorder.

Adolescent↗
PubMed2001

Lexical representation and processing of morphologically complex words: evidence from the reading performance of an Italian agrammatic patient.

The study of patients with acquired language disorders has provided crucial evidence for contemporary theories on mental lexical representation. This is particularly true for the representation of morphologically complex words. In this paper we analyzed the performance of a patient (M.B.) affected by agrammatism and dyslexia. M.B. was required to read aloud simple and morphologically complex words. The patient's pattern of errors was interpreted as the result of a predominant use of the lexical routine (phonological dyslexia). Three reading tasks were developed which allowed us to test M.B.'s ability to read morphologically complex words (reading of regular and irregular plurals; reading of high- and low-frequency singular and plural nouns; reading of evaluative suffixes). Errors were determined by frequency effect rather than by type of suffix (i.e., inflectional or derivational). High-frequency morphologically complex items seemed to meet stored representations, thus avoiding the parsing procedures that are required for less frequent items. These results are in keeping with dual route models of lexical representation of morphologically complex words.

Adult↗
PubMedDate not supplied

[Psychology, audition and language in different disorders of childhood. Communication and neuropsychological aspects].

INTRODUCTION: A great number of language problems in children are related to neurological lesions in the central nervus systems. DEVELOPMENT: Cerebral palsy is one of the lesions more frequent. In many occasions the neurological alteration is accompanied by transmission hypoacusies, language delay an dysarthric problems in speech and voice. Moreover some of these cases have difficulties in attention, slow in speed of processing information, and different executive capacity depending on neurological and context factors. This work focuses into the aspects to consider the observation, evolution and treatment of these patients.

Brain↗
PubMed1980

Imitating gestures. A quantitative approach to ideomotor apraxia.

The ability to carry out movements on imitation was assessed with a 24-item test in uniterally hemisphere-damaged patients. On the basis of a cutoff score derived from the performances of 100 control patients, 20% of the right brain-damaged patients were calssified as apraxic. Most right brain-damaged patients were only mildly defective, but a few showed a striking impairment. In left brain-damaged patients apraxia was not only more frequent, but also much more severe and was nearly always associated with aphasia. However, the correlation between the motor and the language disorder was not particularly high, and the link between the two symptoms was thought to be dependent on the contiguity of the underlying nervous structures.

Aphasia↗
PubMed1981

Acquired reversible autistic syndrome in acute encephalopathic illness in children.

In seeking the neurologic substrate of the autistic syndrome of childhood, previous studies have implicated the medial temporal lobe or the ring of mesolimbic cortex located in the mesial frontal and temporal lobes. During an acute encephalopathic illness, a clinical picture developed in three children that was consistent with infantile autism. This development was reversible. It was differentiated from acquired epileptic aphasia, and the language disorder was differentiated aphasia. One child has rises in serum herpes simplex titers, and a computerized tomographic (CT) scan revealed an extensive lesion of the temporal lobes, predominantly on the left. The other two, with similar clinical syndromes, had normal CT scans, and no etiologic agent was defined. These cases are examples of an acquired and reversible autistic syndrome in childhood, emphasizing the clinical similarities to bilateral medial temporal lobe disease as described in man, including the Klüver-Bucy syndrome seen in postencephalitic as well as postsurgical states.

Acute Disease↗
PubMed1995

Long-term prognosis of poststroke aphasia studied with positron emission tomography.

OBJECTIVE: To evaluate positron emission tomography with the use of fludeoxyglucose F 18 as a predictor of the long-term outcome of poststroke aphasia. BACKGROUND: Positron emission tomography shows functional deficits after stroke even in morphologically intact brain regions. The regional metabolic impairment can be related to the clinical deficit. Little is known about whether regional hypometabolism early after stroke predicts the long-term prognosis of stroke sequelae. PATIENTS AND METHODS: Twenty-two patients with language disturbance caused by a single lesion in the territory of the left middle cerebral artery were studied with fludeoxyglucose positron emission tomography and with a neuropsychological test battery that included a test of receptive language (Token Test) and a test of word fluency (/f/, /a/, /s/ test). The neuropsychological test was readministered about 2 years after the initial test. RESULTS: Regional cerebral metabolic rates of glucose measured early after stroke showed a highly significant correlation with the results of the 2-year follow-up test. The receptive language disorder best correlated with cerebral metabolic rates of glucose in the left superior temporal cortex, and word fluency correlated with cerebral metabolic rates of glucose in the left prefrontal cortex. CONCLUSION: Cerebral metabolic rates of glucose in speech-relevant brain regions measured early after stroke are a predictor of the eventual outcome of aphasia.

Aged↗
PubMed1997

A magnetic resonance imaging study of planum temporale asymmetry in men with developmental dyslexia.

BACKGROUND: Imaging studies have suggested anomalous anatomical asymmetries in language-related regions of the temporal and parietal lobes in individuals with developmental dyslexia. Autopsy studies have reported unusual symmetry of the planum temporale (PT) in patients with dyslexia. Methodological limitations characterize much of this literature, however. OBJECTIVE: To examine the size and asymmetry of the PT and its extension into the parietal lobe (planum parietale [PP]) in men with well-characterized, persistent dyslexia by using magnetic resonance imaging and 3-dimensional surface rendering techniques. METHODS: The brains of 16 right-handed dyslexic men aged 18 to 40 years and 14 matched control subjects were studied with magnetic resonance imaging. Most of these subjects were previously studied with positron emission tomography, which demonstrated functional abnormalities in temporal and parietal brain regions in the dyslexic group. The area of the PT was determined with the aid of 3-dimensional surface-rendering techniques. The size of the PP was estimated by measuring the length of the posterior ascending ramus on 3 parasagittal slices. RESULTS: Approximately 70% to 80% of both groups showed equivalent leftward (left > right) asymmetries of the PT; approximately 50% to 60% showed equivalent rightward (right > left) asymmetries of the PP. These asymmetries showed equivalent moderate inverse correlations with each other in both groups. CONCLUSIONS: These results challenge the notion that anomalous asymmetry of the PT is strongly associated with developmental dyslexia. Given the heterogeneity of the dyslexic population, some subgroup of dyslexic individuals (i.e., those with developmental language disorders) may show unusual symmetry or reversed asymmetry in this region. However, anomalous asymmetry of the planum did not contribute to functional abnormalities demonstrated in these patients by positron emission tomography.

Adolescent↗
PubMed2007

Progranulin mutations in primary progressive aphasia: the PPA1 and PPA3 families.

BACKGROUND: Primary progressive aphasia (PPA) is a language-based dementia characterized by fluent or nonfluent language disorder as its principal feature. OBJECTIVE: To describe progranulin gene mutations in 2 families with PPA. DESIGN: Report of affected families. SETTING: Academic research. PATIENTS: Two families, PPA1 and PPA3, were studied. Genomic DNA was isolated from 3 of 4 siblings in PPA1, from all 3 siblings in PPA3, and from more than 200 control subjects. MAIN OUTCOME MEASURES: All 12 coding exons of the progranulin gene and the 5" and 3" untranslated regions were amplified by polymerase chain reaction and were sequenced in both directions using relevant primers. RESULTS: Both affected members of PPA1 for whom DNA was available and both affected sisters of PPA3 had a progranulin gene mutation not found in the unaffected siblings or in the controls. The mutations likely cause a null allele and a reduction in the level of functional progranulin protein. Both affected members of PPA1 with autopsies had frontotemporal lobar degeneration with tau-negative ubiquinated inclusions. CONCLUSIONS: To our knowledge, these are the only known families in which affected members display phenotypical homogeneity for PPA in the initial stages of the disease. In both families, the disease segregated with progranulin gene mutations. Whether progranulin dysfunction also extends to sporadic PPA and how it affects the initial anatomical specificity of neurodegeneration remain to be determined.

Aged↗
PubMed1984

PPVT IQ validity in adults: a measure of vocabulary, not of intelligence.

Used partial correlation and multiple regression procedures to test the hypothesis that the PPVT IQ assesses more than vocabulary in adults. Ss were 84 inpatients from psychiatry and neurology wards. The relationships between the PPVT IQ and other measures of vocabulary, intelligence, memory, visual-motor speed and education were examined in the statistical analyses. Although these variables have significant simple correlations with the PPVT IQ, the multivariate analyses show that the Vocabulary Subtest of the WAIS-R is the only important variable. No other variable or variable combinations are able to account for further PPVT IQ variance after the effect of the Vocabulary Subtest is removed. The results support the use of the PPVT as a test of adult vocabulary, but not adult intelligence, and suggest that the PPVT may be useful as part of language assessment in patients with expressive language disorders.

Adult↗