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PubMed1991

A neurobehavioral syndrome after failure to thrive on chloride-deficient formula.

Eleven of 13 children, who demonstrated a failure-to-thrive pattern in infancy attributable to chloride-deficient Neo-Mull-Soy formula, had distinctive cognitive impairments four to nine years later. These included: a language disorder primarily involving articulation, word finding and naming; visual-motor and fine motor difficulties; and attention deficit disorder, often featuring repetitive behaviours, withdrawal and perseveration ('overfocus'). In contrast, global intellectual abilities were within the normal range in all 11 children. This residual neurobehavioral syndrome is too rare in the developmentally disabled population to reflect a chance association. It has not been associated either with protein-calorie malnutrition or chloride-deficiency diseases.

Body Height↗
PubMed1992

Operculum syndrome in childhood: a rare cause of persistent speech disturbance.

An infant is described who developed operculum syndrome during an acute encephalitic illness. Presenting symptoms were cortical pseudobulbar palsy and focal seizures of facial origin. Persistent mutism--with normal language comprehension and orofacial motor disturbance--were the main neurological sequelae. Similarities between this case and other permanent or transient causes of cortical pseudobulbar palsy are discussed, as well as the possible relationship with certain types of childhood language disorders.

Brain Damage, Chronic↗
PubMed1995

Social and biological risk factors for mild and borderline impairment of language comprehension in a cohort of five-year-old children.

Biological risk factors during intra-uterine life, delivery and the neonatal period, and measures of social adversity during pregnancy, were studied as predictors of a 'mildly impaired' (50 to 74) or 'borderline' (75 to 84) score on the Peabody Picture Vocabulary Test (PPVT) at aged five years in 3906 children. Biological risk factors in pregnancy were associated with neither PPVT outcome. Gestation of < 36 weeks, > 3 minutes to establishment of respiration and admission to intensive care were associated with a lower PPVT score indicating mild impairment, though only in the unadjusted analyses. A five minute Apgar score of < 5 and male sex were related to borderline scores, though only the latter remained significant after statistical allowance for possible confounding. In contrast, almost all measures of social adversity were related to both PPVT outcomes even after statistical adjustment for the influence of other factors.

Apgar Score↗
PubMedDate not supplied

Acquired epileptic aphasia (the Landau-Kleffner syndrome) due to neurocysticercosis.

A healthy 7-year-old boy developed a language disorder associated with clinical seizures and a paroxysmal EEG. Computed tomography and magnetic resonance imaging revealed a small cysticercus deep in the left Sylvian fissure. We postulate a cause and effect relationship between the parasitic cyst and the clinical manifestations supported by the strategic location of the cyst and the critical age at which this lesion developed. This case provides evidence that the syndrome of acquired epileptic aphasia may be explained in terms of an unilateral structural brain lesion.

Aphasia↗
PubMedDate not supplied

A case of Landau-Kleffner syndrome secondary to inflammatory demyelinating disease.

A 6-year old girl developed acquired aphasia with epilepsy and a paroxysmal EEG (Landau-Kleffner syndrome). Isoelectric CSF focusing showed oligoclonal IgG bands. Small lesions were visualized in periventricular left frontal white matter and right parietal lobe centrum semiovale with magnetic resonance imaging (MRI). After a week of ACTH therapy, the EEG paroxysmal activity disappeared; during the next few months, the language disorder improved. Further MRI examination showed a decrease in size and signal of the left frontal lesions, with localized white matter atrophy, dilatation of the subarachnoidal spaces, and disappearance of the right parietal lesion. The clinical and neuroradiologic features and the laboratory data suggest an acute disseminated encephalomyelitis.

Adrenocorticotropic Hormone↗
PubMed1996

Postictal language dysfunction in patients with right or bilateral hemispheric language localization.

PURPOSE: As shown previously, when temporal lobe complex partial seizures (TLCPS) originate from the language dominant hemisphere, patients cannot read a test phrase correctly within 60 s of the end of the ictal discharge. We wished to assess whether postictal language testing results discordant with this pattern identified patients with non-left (right hemisphere or mixed) language dominance. METHODS: Since 1988, all patients undergoing video/EEG monitoring at our institution have been given a test phrase to read aloud as soon as a seizure is detected. Inclusion criteria for this study were (a) postictal language testing within 60 s of seizure end for at least one TLCPS, (b) > 90% seizure reduction after anterior temporal lobectomy with 2-year minimum follow-up, and (c) language localization by either intracarotid amobarbital test (IAT) or direct electrical stimulation of left hemisphere cortex. RESULTS: Two hundred twenty-four seizures in 64 patients were analyzed. Discordant postictal language patterns were noted in 10 of 11 patients with IAT documented non-left language dominance and in 15 of 53 with left dominance (p = 0.006; sensitivity 90.9%, specificity 71.7%). CONCLUSIONS: Postictal language testing accurately identifies patients with non-left language dominance and may be useful in selecting appropriate patients for IAT.

Adult↗
PubMed1993

Ideomotor apraxia in Alzheimer's disease.

Ideomotor apraxia, tested on verbal command and by imitation, was checked in 23 patients suffering from dementia of Alzheimer's type of different severity and in 17 age-matched controls. A significant deterioration of ideomotor praxis could be shown even in mild dementia. Correlations of ideomotor apraxia and aphasia, tested by the Token test were found to be significant.

Aged↗
PubMed2007

Heritable risk factors associated with language impairments.

There is a strong genetic contribution to children's language and literacy impairments. The aim of this study was to determine which aspects of the phenotype are familial by comparing 34 parents of probands with language/literacy impairments and 33 parents of typically developing probands. The parents responded to questionnaires regarding previous history for language/reading impairment and participated in psychometric testing. The psychometric test battery consisted of tests assessing non-verbal IQ, short-term memory, articulation, receptive grammar, reading abilities and spelling. Self-report measures demonstrated a higher prevalence of language and literacy impairments in parents of affected probands (32%) compared with parents of unaffected probands (6%). The two groups of parents differed significantly in their performance on the non-word repetition, oromotor and digit span tasks. Non-word repetition gave the best discrimination between the parent groups even when the data from the parents who actually were impaired as ascertained by direct testing or self-report were removed from the analyses. This suggests that non-word repetition serves as a marker of a family risk for language impairment. The paper concludes with a discussion of issues associated with ascertainment of specific language impairment (SLI).

Adult↗
PubMed1991

Word-finding ability and design fluency in developmental dyslexia.

Certain oral as well as visual language disorders now appear characteristic of the developmental dyslexia syndrome. Recent research has revealed word-finding difficulties in dyslexic children but has left unanswered questions about specificity and causality. Verbal and non-verbal fluency in dyslexics and non-dyslexic CA- and RA-matched controls were measured by means of cued lexical retrieval and stick design tasks. Evidence for dyslexia-related difficulties was found only at the word form level of access. At the semantic level, dyslexics' word finding was superior. Non-verbal (design) fluency was normal. Such findings implicate long-term linguistic memory processes in dyslexia and are consistent with neurocognitive models that emphasize diffuse left hemisphere dysfunction.

Attention↗
PubMed1996

Temporal processing deficits of language-learning impaired children ameliorated by training.

Children with language-based learning impairments (LLIs) have major deficits in their recognition of some rapidly successive phonetic elements and nonspeech sound stimuli. In the current study, LLI children were engaged in adaptive training exercises mounted as computer "games" designed to drive improvements in their "temporal processing" skills. With 8 to 16 hours of training during a 20-day period, LLI children improved markedly in their abilities to recognize brief and fast sequences of nonspeech and speech stimuli.

Child↗
PubMed1996

Language comprehension in language-learning impaired children improved with acoustically modified speech.

A speech processing algorithm was developed to create more salient versions of the rapidly changing elements in the acoustic waveform of speech that have been shown to be deficiently processed by language-learning impaired (LLI) children. LLI children received extensive daily training, over a 4-week period, with listening exercises in which all speech was translated into this synthetic form. They also received daily training with computer "games" designed to adaptively drive improvements in temporal processing thresholds. Significant improvements in speech discrimination and language comprehension abilities were demonstrated in two independent groups of LLI children.

Algorithms↗
PubMed1995

Association study with two markers of a human homeogene in infantile autism.

Epidemiological data and family studies in autism show that there is a genetic susceptibility factor in the aetiology of this syndrome. We carried out an association study in infantile autism. Two markers of the homeogene EN2 involved in cerebellar development were tested in a population of 100 autistic children and in a population of 100 control children. With the MP4 probe showing a PvuII polymorphism, significant differences in the allele frequencies between the two populations were found (chi 2 = 7.99, df = 1, p < 0.01). With the MP5 probe showing an SstI polymorphism, no difference appeared (chi 2 = 1.17, not significant). Several clinical examinations allowed us to characterise the autistic children. Most of them had high scores for autistic behaviour and language disorders but low scores for neurological syndromes. Two children had a significant family history and six children had confirmed syndromes or diseases of genetic origin. Discriminant analysis between clinical and molecular data did not give significant results. These preliminary results must be supported by further analyses of this gene and by studies of its potential involvement in the pathophysiology of the autistic syndrome.

Adolescent↗
PubMed2005

Interictal language functions in temporal lobe epilepsy.

OBJECTIVE: To evaluate interictal language functions in patients with medically intractable left and right sided mesial temporal lobe epilepsy (TLE). METHODS: Spontaneous speech, language comprehension, confrontation naming, repetition, reading, writing, and word fluency were examined in 12 patients with left sided TLE and 11 patients with right sided TLE. RESULTS: Four patients out of 23 displayed language deficits in more than one language domain. Three further patients exhibited isolated language deficits. Linguistic deficits were observed in both left TLE and right TLE. In quantitative analyses left and right TLE only differed in spontaneous speech (p = 0.02); no difference was found in other language functions, laterality quotient of Wada test, or overall IQ. Qualitative error analysis of object naming, however, showed typical errors associated only with left TLE. Patients with linguistic deficits were older at testing compared to patients without linguistic deficits (p = 0.003), whereas other factors including side of TLE, handedness, educational level, age at epilepsy onset, and duration of epilepsy did not differ between groups. CONCLUSIONS: Possible explanations for these findings include neuronal cell loss and deafferentiation in cortical areas, and disruption of the basal temporal language area pathways. Our study suggests that some patients with chronic mesial TLE exhibit linguistic deficits when specifically tested, and underlines the need to routinely investigate linguistic functions in TLE.

Adult↗
PubMed1992

Subcortical aphasia from a thalamic abscess.

A patient is reported who was treated successfully for a left thalamic abscess that resulted in subcortical aphasia. A SPECT scan showed large areas of hypoperfusion in the cortex of the left hemisphere. At follow up after seven months there was marked improvement in the language disorder and the cortical hypoperfusion. It is suggested that aphasia in patients with subcortical lesions results from secondary cortical dysfunctions. The evidence is confined to patients with stroke lesions. The possible implications of this case on current theories of pathophysiological mechanisms, in particular the ischaemic penumbra theory and the cortical diaschisis theory, are briefly discussed. Cortical diaschisis may be the appropriate explanation in this patient.

Adult↗
PubMed1994

Initial letter and semantic category fluency in Alzheimer's disease, Huntington's disease, and progressive supranuclear palsy.

Ten patients with dementia of Alzheimer's type, 10 patients with progressive supranuclear palsy, and 10 patients with Huntington's disease were compared on two types of verbal fluency task--namely, initial letter fluency and category (semantic) fluency. The groups were carefully matched for overall level of dementia on the dementia rating scale, and were compared with 25 age matched normal controls. The controls found letter fluency more difficult than category fluency, and this relative pattern of performance was repeated in the progressive supranuclear palsy and Huntington's disease groups, although both groups were significantly impaired on both tasks. By contrast, patients with Alzheimer's disease performed just as poorly as the progressive supranuclear palsy and Huntington's disease groups on the category tasks, but were significantly less impaired at letter fluency, performing at near normal levels on this task. From these results, it is suggested that the performances of patients with progressive supranuclear palsy and Huntington's disease relate largely to initiation and retrieval problems secondary to disruption of frontostriatal circuits, whereas in Alzheimer's disease, the poorer performance on category fluency is due principally to the breakdown of semantic knowledge, which probably reflects temporal neocortical involvement.

Adult↗