Can phonological and semantic short-term memory be dissociated? Further evidence from landau-kleffner syndrome.

Recent studies have made a distinction between short-term storage capacities for phonological information and short-term storage capacities for lexico-semantic information (R. Martin, Lesch, & Bartha, 1999). In this multiple case study, we tried to provide further evidence for the dissociability of phonological and lexico-semantic short-term memory (STM) components, by studying verbal STM in three patients who had recovered from Landau-Kleffner syndrome. Furthermore, we explored to what extent apparent dissociations between phonological and lexico-semantic STM could be related to underlying phonological and lexico-semantic processing impairments. We found clear dissociations between phonological and lexico-semantic STM measures in patients TG, JPH, and DC, whose performance was impaired in nonword immediate serial recall and in a rhyme probe task, while performance was normal for a category probe task. These patients also presented reduced phonological effects (word length, phonological similarity, phonotactic frequency) but normal lexico-semantic effects (lexicality, word imageability, word frequency) in STM. Moreover, there were no systematic correspondencies between phonological and lexico-semantic STM and phonological and lexico-semantic processing impairments. Implications for current models of STM and language processing are discussed.

EEG criteria predictive of complicated evolution in idiopathic rolandic epilepsy.

BACKGROUND: Although so-called "benign" epilepsy with centrotemporal spikes (BECTS) always has an excellent prognosis with regard to seizure remission, behavioral problems and cognitive dysfunctions may sometimes develop in its course. To search for clinical or EEG markers allowing early detection of patients prone to such complications, the authors conducted a prospective study in a cohort of unselected patients with BECTS. METHODS: In 35 children with BECTS, academic, familial, neurologic, neuropsychological, and wake and sleep EEG evaluations were repeated every 6 to 12 months from the beginning of the seizure disorder up to complete recovery. RESULTS: In 25 of 35 patients (72%), behavioral and intellectual functioning remained unimpaired. In 10 of 35 patients (28%), educational performance and familial maladjustment occurred. These sociofamilial problems were correlated with impulsivity, learning difficulties, attention disorders, and minor (7/35 cases, 20%) or serious (3/35 cases, 8%) auditory-verbal or visual-spatial deficits. Worsening phases started 2 to 36 months after onset and persisted for 9 to 39 months. Occurrence of atypical evolutions was significantly correlated with five qualitative and one quantitative interictal EEG pattern: intermittent slow-wave focus, multiple asynchronous spike-wave foci, long spike-wave clusters, generalized 3-c/s "absence-like" spike-wave discharges, conjunction of interictal paroxysms with negative or positive myoclonia, and abundance of interictal abnormalities during wakefulness and sleep. Clinical deterioration was not linked with seizure characteristics or treatment. CONCLUSION: Different combinations of at least three of six distinctive interictal EEG patterns and their long-lasting (> or =6-month) persistence seem to be the hallmarks of patients with BECTS at risk for neuropsychological impairments.

Electrophysiological evidence of persisting unilateral auditory cortex dysfunction in the late outcome of Landau and Kleffner syndrome.

OBJECTIVES: In the late outcome of Landau and Kleffner syndrome (LKS), a childhood-acquired epileptic aphasia, most patients show after complete recovery of epilepsy a permanent one-ear extinction on dichotic listening tests contralateral to the temporal cortex previously affected by the epileptic focus. The pathophysiological significance of this dichotic extinction is not yet understood. It may be a consequence of a permanent dysfunction in the auditory system due to epileptic activity during the maturing period of the auditory system. Evoked potentials were used to check this hypothesis and to localize the level of the dysfunction along the auditory pathways. METHODS: Early, middle latency and late auditory evoked potentials were recorded in 5 right-handed children having recovered from LKS. They were compared with those of 5 control children paired for age and gender. RESULTS: In all 5 LKS patients, early and middle latency auditory evoked potentials were normal. But the amplitude of N1c (arising from associative auditory areas) was strongly reduced at temporal electrodes contralateral to the extinguished ear, whereas latency and amplitude of N1b (related to primary auditory areas) were in the normal range. CONCLUSIONS: Unilateral voltage reduction of late auditory evoked potentials over the temporal areas previously involved by epileptic discharges suggests a permanent dysfunction in the associative auditory cortex, the behavioral expression of which is the unilateral dichotic extinction.

[Cognitive consequences of Rolandic Epilepsy]. / Retentissement cognitif de l'épilpsie à pointes centro-temporales.

Idiopathic focal epilepsies and particularly rolandic epilepsy are considered to have an excellent outcome with spontaneous recovery during adolescence. The characterization of this syndrome in 1959 by Beaussart and Nayrac was a great progress for childhood epileptology: the existence of a focal epilepsy with a good prognosis and no underlying brain lesion, so-called "benign" epilepsy, was recognized. Since the first descriptions, numerous neuropsychological studies were performed showing variable results. All the studies agree with the fact that children with rolandic epilepsy keep a normal global intellectual efficiency and a good long-term outcome. Nevertheless, some children may suffer transiently during the active phase of the epilepsy from oromotor dysfunction, neuropsychological deficits, or attention deficits with learning disorders. The analysis of cognitive and neurophysiological correlations evidenced a significant correlation between the epileptic focus localization and few specific dysfunctions. We evidenced mainly a significant effect of the persistence of a prolonged slow focus and a strong activation of night EEG spike and waves on cognitive decline and attention disorders. These abnormalities are mainly observed during severe or atypical evolutions of rolandic epilepsy. Preliminary longitudinal studies show that these cognitive deficits are transient. Thus, the presence of an active epileptic focus, without underlying brain lesion, could interfere with normal maturation of cognitive function.

Semiology of typical and atypical Rolandic epilepsy: a video-EEG analysis.

Since the first descriptions of Rolandic Epilepsy or benign epilepsy with centrotemporal spikes (BECTS), typical and atypical forms have been reported. Indeed, classical focal seizures are sometimes associated with various atypical ictal symptoms and cognitive or behavioural disorders. In an effort to define early clinical and EEG criteria allowing early distinction between typical and atypical forms, we recently conducted a prospective study in a cohort of children with Rolandic Epilepsy. The results of this study have been reported elsewhere. We now discuss the semiological characteristics, and comment on the video-EEG data collected during this study. Symptoms were classified into three major categories: "classical focal seizures"; "spike and wave related symptoms"; and "paraictal symptoms". Classical focal seizures constitute the electroclinical expression of the development and the propagation of a focal cortical neuronal discharge. "Spike and wave related symptoms" are brief neurological or neuropsychological phenomena having a relatively strict temporal relation with individual components of isolated focal or generalized spikes and waves. "Paraictal symptoms" consist of acquired progressive and fluctuating motor or cognitive deficits and are not directly correlated with Todd paralysis. We present detailed video-EEG material of selected cases and discuss the usefulness of such distinctions in terminology. We suggest that variability in clinical expression probably reflects the implication of different pathophysiological mechanisms, which in turn could explain differences in sensitivity to treatment. (Published with videosequences.)

Language functional neuro-imaging changes following focal left thalamic infarction.

The involvement of the left thalamus in language function has been largely demonstrated through the effects of thalamic lesion and/or stimulation upon language. However, the pathophysiological mechanisms underlying thalamic aphasias remain a matter of debate. We report here on changes in the pattern of brain activity in auditory word processing, verb generation and visual lexical decision in a patient who sustained left thalamic infarct following the surgery of intractable mesio-temporal epilepsy with left hippocampal sclerosis. To some extent, our findings exemplify the way the left thalamus brings on line the cortical networks involved in language processing, suggested in the term 'selective engagement'.

Impulsivity and control of inhibition in Benign Focal Childhood Epilepsy (BFCE).

Childhood epilepsy represents abnormal brain functioning and may affect cognitive functions that depend on the late development of the frontal lobes. This study addresses the possible consequences of benign epilepsy on frontal functions, specifically action regulation and inhibition in the absence of explicit frontal neurological signs. Thirteen children (8 males; 6-12 years old) with benign epilepsy were matched to 13 controls. They performed tasks designed to measure impulsivity and control of inhibition: CPT, Stop Signal Paradigm, Stroop test, and Matching Familiar Figures Test (MFFT). Children with epilepsy made more errors on the MFFT (p < .02), made more errors in the interference condition on the Stroop test (p < .01), and had a longer response time to the Stop Signal (p < .05) than controls, with no differences on nonexecutive functions measures. Thus, children with benign epilepsy have a deficit on some measures of impulsivity and inhibition, which may reflect poor frontal lobe functioning.

Isotope tracer-techniques in rolandic epilepsy and its variants.

For ethical and practical reasons there are few studies on brain metabolism in rolandic epilepsy and it's variants. Most studies are performed in Landau-Kleffner syndrome or epilepsy with continuous spikes and waves during slow wave sleep (CSWS) which are considered to be included within the spectrum of rolandic epilepsy. The results of studies using isotope tracer-techniques in rolandic epilepsy and its variants are summarized.

Early dissociation of verbal and nonverbal gestural ability in an epileptic deaf child.

Studies of sign language aphasia in deaf adults have provided the evidence for two separable verbal and nonverbal manual gesture systems. We report a congenitally deaf child with a idiopathic focal epilepsy of childhood who showed specific language impairment in French sign language. The child's amazing performances in miming or sketching pictures she was unable to sign support the notion of an early dissociation of the two gestural systems.

Cognitive development in benign focal epilepsies of childhood.

Benign focal epilepsy of childhood (BFEC) is the most common form of epilepsy, in children from 3 to 12 years. Its prognosis is always favourable as far as the epilepsy is concerned. Nevertheless, recent clinical data suggest that children affected by BFEC are more likely to show learning difficulties and behavioural disturbances than their peers. We report here the preliminary findings of a prospective study of 22 children affected with BFEC. Electroclinical and neuropsychological changes observed during the first 18 months of the follow-up strengthen the conclusion of recent neuropsychological studies stressing the correlation between epilepsy and cognitive performances. The cognitive deficits affecting mainly non-verbal functions were significantly correlated with the frequency of seizures and spike-wave discharges and to the lateralization of the epileptic focus in the right hemisphere, whereas frontal functions like attention control, response organization and fine motor speed, were impaired in the presence of active BFEC independently of the lateralization of the epileptic focus. Our results indicate that maturing cognitive functions subserved by a cortical area distant from the epileptic focus are susceptible to interference with epilepsy.

Idiopathic rolandic epilepsy with "interictal" facial myoclonia and oromotor deficit: a longitudinal EEG and PET study.

PURPOSE: The prognosis of benign epilepsy with centrotemporal spikes (BECTS) is always favorable as far as the epilepsy is concerned. However, some data suggest that affected children may be at risk for minor cognitive impairment. We report here the longitudinal study of a young girl demonstrating that BECTS also may be associated with severe motor disturbances. METHODS: BECTS (rare left oromotor seizures, right rolandic spike-waves activated during sleep) started at the age of 3 years 6 months in a girl with normal initial psychomotor development. Her clinical, neuropsychological, and EEG status was assessed every 3-6 months. Regional cerebral glucose metabolism was measured by using the [18F]fluorodeoxyglucose-positron emission tomography (FDG-PET) method. RESULTS: Between the age of 5 and 6 years, the girl had (a) increased seizure frequency; (b) brief perioral and palpebral myoclonic jerks, concomitant with the spike component of interictal spike-waves, and (c) persistent but fluctuating oromotor deficits (drooling, dysarthria, dysphagia). The EEG showed a marked increase in abundance and amplitude of wake and sleep interictal abnormalities, which became bilateral. Awake FDG-PET revealed a bilateral increase of glucose metabolism in opercular regions. A complete and definitive EEG and clinical remission occurred at age 5 years 11 months and has persisted since (present age, 7 years 9 months). CONCLUSIONS: This case confirms that during BECTS, epileptiform dysfunctions within rolandic areas may induce "interictal" positive or negative oromotor symptoms, independent of classic seizures.

Cerebral correlates of hemispheric lateralization during a pitch discrimination task: an ERP study in dichotic situation.

OBJECTIVE: Electrophysiological correlates of perceptual asymmetry for dichotic pitch discrimination were investigated in 12 right-handed volunteers, whose dichotic listening performances attested the classical 'right ear advantage' in a verbal discrimination task. METHODS: Event related potentials (ERPs), elicited by dichotic and binaural pairs of tones applied in a classical oddball paradigm including right ear targets, left ear targets and binaural targets (5% occurrence each) were recorded from medial and lateral scalp locations. Latencies and baseline to peak amplitudes were measured for P1, N1, P2, N2 and P3 components. RESULTS: ERPs recorded in response to dichotic (compared with binaural) target pairs, exhibited delayed latencies for N2 and P3, correlated with prolonged RTs, probably linked to greater difficulty in identification of the target. They also displayed enhanced N1 and P2 voltages, which may reflect the simultaneous activation of two different populations of neurons in the auditory cortical areas. We observed specific lateralization effects for pitch discrimination with a left ear advantage on latency of early components. CONCLUSIONS: Together with amplitude asymmetries in the N2 component, the findings bring strong electrophysiological support to Kimura's structural model for dichotic perceptions with a right hemisphere prevalence in a pitch discrimination task.

Diversity of patterns of improvement in confrontation naming rehabilitation: some tentative hypotheses.

A previous group analysis of the effects of a computerized written naming rehabilitation program revealed global improvement with generalization of benefits to untrained items and to untreated oral naming (Deloche et al., 1992). The present multiple single-case analysis of the data indicates a variety of patterns of improvement and of generalization effects among individual patients. Patterns of relationships between written and oral naming behaviors help to explain the type of improvement that was observed.

Picture confrontation oral naming: performance differences between aphasics and normals.

Oral confrontation naming was compared in 108 normal subjects controlled for education, age, and gender and in 18 aphasic patients for the same set of 115 pictures. Demographic variables influenced both normals' and aphasics' performance. However, the nature of aphasics' misnamings on the one hand and the differential effects of characteristics of pictures and words on normals' and aphasics' responses on the other indicated specific deficits in patients. The classical hypothesis that aphasics' misnamings and the production of word associations by normals should rely on similar mechanisms (Rinnert & Whitaker, 1973) is questioned. Nondominant responses observed in normals accounted for a larger proportion of verbal errors than associates to target words.

Regional cerebral glucose metabolism in children with deterioration of one or more cognitive functions and continuous spike-and-wave discharges during sleep.

The Landau-Kleffner syndrome (LKS) and the syndrome of continuous spike-and-wave discharges during slow sleep (CSWS) were originally described, and are still considered, separately. The former combines an acquired aphasia with spike-and-wave discharges that are activated by slow wave sleep, behavioural disturbances, and sometimes epileptic seizures. The latter is characterized by continuous spike-and-wave discharges during slow wave sleep, usually combined with global intellectual deterioration and epileptic seizures. These two syndromes share many common features: (i) onset during childhood; (ii) deterioration of cognitive functions that were previously normally acquired; (iii) seizure type; (iv) EEG pattern; (v) pharmacological reactivity; (vi) regression of the neuropsychological symptoms, of the EEG abnormalities and of the seizures before the end of adolescence; (vii) absence of obvious structural lesion detected by CT or MRI scan. Therefore, we postulated that these patients might, in fact, be presenting several facets of a single process associating the deterioration of cognitive functions and continuous spike-and-wave discharges during slow wave sleep. The pathogenesis of this syndrome remains unknown. Seven patients, presenting CSWS associated with neuropsychological deterioration (isolated aphasia, three cases; language disturbances with more widespread cognitive deterioration, three cases; isolated apraxia, one case) were studied using PET with [18F]fluorodeoxyglucose (FDG). We hoped to find metabolic arguments in favour of a unifying hypothesis, and to reveal clues as to pathogenesis. We present the retrospective analysis of 21 studies performed between 1986 and 1993, 12 of which were done during sleep. For three of these patients, follow-up studies were obtained until recovery. The metabolic patterns were very variable from one patient to another and in the same patient over time. Among the six patients studied during the active phase of the affection, our results showed unilateral, focal or regional increase in glucose metabolism of the cortex in five patients. This hypermetabolism was observed during sleep with continuous spike-and-wave discharges, but also persisted during wakefulness. In the last patient, the metabolic pattern was different: decreased regional glucose metabolism was observed during wakefulness, whereas during sleep, the metabolic pattern in the temporal areas varied during the course of the affection. After recovery, the metabolic pattern in four children (including the seventh patient) was either normal or showed focal or regional, uni- or bilateral decrease in cortical glucose metabolism. Despite this apparent disparity, four basic metabolic characteristics formed a common pattern in all patients, in line with our unifying postulate: (i) the metabolism of the cortical mantle was higher than in the subcortical structures, especially in the thalamic nuclei. This metabolic pattern is characteristic of an immature brain. (ii) The metabolic abnormalities involved focal or regional areas of the cortex. This finding is in good agreement with recent neurophysiological data suggesting a focal origin of the spike-and-wave discharges. (iii) The metabolic disturbances predominantly involved associative cortices. The pattern of neuropsychological deterioration is in good agreement with the topography of the disturbances of cortical glucose metabolism. (iv) The thalamic nuclei remained symmetrical despite significant cortical asymmetries, suggesting either that cortico-thalamic neurons do not participate in the generation of spike-and-wave discharges or that they are inhibited by the pathologic mechanisms. We hypothesize that the acquired deterioration of cognitive function with CSWS is caused by an alteration of the maturation of one or several associative cortices, primarily involving local interneurons and cortico-cortical associative neurons.

[Event-related potentials and language processing]. / Potentiels cognitifs et traitement du langage.

The contribution of cognitive event-related potentials (ERPs) and particularly of the N400 component to the study of language processing is considered. After a general presentation of the various components of cognitive evoked potentials which are differentiated from early potentials (sensitive to perceptive parameters), the neurophysiological and psychological correlates of ERPs are discussed. Before the presentation of the main studies concerned with the correlation between specific late components and linguistic processes, it is proposed an introduction to some theoretical models of language processing which serve as conceptual framework supporting most of the experimental approaches in this field. The results of EPs studies devoted to functional asymetries in language processing are briefly mentioned. Then a number of studies are analysed, these demonstrate the sensitivity of event-related potential measures to linguistic manipulations. Finally, the studies concerned with the N400 are reviewed, this potential appears to be the more specifically language-related ERP component, even if its connection with specific linguistic processes or its differentiation from N200 are still under debate. Nevertheless language-related electrophysiological investigations may become in the near future a useful complementary approach to our understanding of language processing and language disorders.