Neural correlates of verbal semantic memory in patients with temporal lobe epilepsy.

Functional imaging data suggest that the core network engaged in verbal semantic memory (SM) processing encompasses frontal and temporal lobe structures, with a strong left lateralization in normal right handers. The impact of long term temporal lobe epilepsy (TLE) on this network has only partly been elucidated. We studied verbal SM in 50 patients with chronic, intractable TLE (left TLE=26, right TLE=24) and 35 right handed normal controls using a verbal fMRI semantic decision paradigm. All patients had language lateralized to the left hemisphere, as verified by the intracarotid amobarbital procedure. Within and between group analyses showed remarkable, group-specific activation profiles. The control group activated frontal and temporal areas bilaterally, with a strong left predominance. Left TLE patients showed a shift of activations of left frontal and medial temporal areas to homologous regions in the right hemisphere. Furthermore, left TLE subjects utilized subcortical structures such as the thalamus and putamen to accomplish the verbal SM task. Contrastively, the activation pattern of right TLE patients resembled that of normal controls, but exhibited "hypofrontality" with a shift from frontal to posterior regions in the temporal, parietal and occipital lobe. Our results show that chronic epileptic activity originating from temporal seizure foci is associated with an alteration of neural circuits which support semantic language processing and that side of seizure focus has a specific impact on the resulting activation network. These findings presumably result from morphological changes and from functional reorganization which are both inherent to chronic TLE.

Language lateralization in temporal lobe epilepsy: a comparison between fMRI and the Wada Test.

PURPOSE: Recent studies have claimed that language functional magnetic resonance imaging (fMRI) can identify language lateralization in patients with temporal lobe epilepsy (TLE) and that fMRI-based findings are highly concordant with the conventional assessment procedure of speech dominance, the intracarotid amobarbital test (IAT). METHODS: To establish the power of language fMRI to detect language lateralization during presurgical assessment, we compared the findings of a semantic decision paradigm with the results of a standard IAT in 68 patients with chronic intractable right and left temporal lobe epilepsy (rTLE, n=28; lTLE, n=40) who consecutively underwent a presurgical evaluation program. The patient group also included 14 (20.6%) subjects with atypical (bilateral or right hemisphere) speech. Four raters used a visual analysis procedure to determine the laterality of speech-related activation individually for each patient. RESULTS: Overall congruence between fMRI-based laterality and the laterality quotient of the IAT was 89.3% in rTLE and 72.5% in lTLE patients. Concordance was best in rTLE patients with left speech. In lTLE patients, language fMRI identified atypical, right hemisphere speech dominance in every case, but missed left hemisphere speech dominance in 17.2%. Frontal activations had higher concordance with the IAT than did activations in temporoparietal or combined regions of interest (ROIs). Because of methodologic problems, recognition of bilateral speech was difficult. CONCLUSIONS: These data provide evidence that language fMRI as used in the present study has limited correlation with the IAT, especially in patients with lTLE and with mixed speech dominance. Further refinements regarding the paradigms and analysis procedures will be needed to improve the contribution of language fMRI for presurgical assessment.

Is math lateralised on the same side as language? Right hemisphere aphasia and mathematical abilities.

The main purpose of the present study was to learn how mathematical abilities are located and develop in the brain with respect to language. Mathematical abilities were assessed in six right-handed patients affected by aphasia following a lesion to their non-dominant hemisphere (crossed aphasia) and in two left-handed aphasics with a right-sided lesion. Acalculia, although in different degrees, was found in all cases. The type of acalculia depended on the type of aphasia, following patterns that have been previously observed in the most common aphasias resulting from left hemisphere lesions. No sign of right hemisphere or spatial acalculia (acalculia in left lateralised right-handed subjects) was detected. These results suggest that, as a rule, language and calculation share the same hemisphere. A primitive computational mechanism capable of recursion may be the precursor of both functions.

Facial recognition in primary focal dystonia.

The basal ganglia seem to be involved in emotional processing. Primary dystonia is a movement disorder considered to result from basal ganglia dysfunction, and the aim of the present study was to investigate emotion recognition in patients with primary focal dystonia. Thirty-two patients with primary cranial (n=12) and cervical (n=20) dystonia were compared to 32 healthy controls matched for age, sex, and educational level on the facially expressed emotion labeling (FEEL) test, a computer-based tool measuring a person's ability to recognize facially expressed emotions. Patients with cognitive impairment or depression were excluded. None of the patients received medication with a possible cognitive side effect profile and only those with mild to moderate dystonia were included. Patients with primary dystonia showed isolated deficits in the recognition of disgust (P=0.007), while no differences between patients and controls were found with regard to the other emotions (fear, happiness, surprise, sadness, and anger). The findings of the present study add further evidence to the conception that dystonia is not only a motor but a complex basal ganglia disorder including selective emotion recognition disturbances.

Hippocampal formation involvement in a language-activation task in patients with mesial temporal lobe epilepsy.

PURPOSE: The study aims to explore the contribution of the hippocampal formation to the retained language-comprehension network in patients with unilateral mesial temporal lobe epilepsy (TLE). METHODS: We performed a functional magnetic resonance (MRI) study based on a language comprehension paradigm in 45 right-handed patients with unilateral mesial TLE and 35 healthy control subjects. Activations in the hippocampal formations in both hemispheres were analyzed for each subject as well as for groups of left TLE, right TLE, and controls. RESULTS: In sum, 82% of TLE patients displayed hippocampal activations. A significant difference in hippocampal activation between left and right TLE was found: Right TLE patients showed increased activity in the left hippocampal formation compared with left TLE patients. In contrast, patients with left TLE did not show increased activity in the right hippocampal formation compared with right TLE patients. In comparison with a healthy control group, right TLE patients activated the left hippocampal formation to a greater extent, whereas patients with left TLE did not activate the right hippocampal formation to a greater degree. These findings point to an increased involvement of the left hippocampal formation during a language-comprehension task in right TLE patients. In contrast, left TLE in right-handed patients seems not associated with an enhanced involvement of the right hippocampal formation in retained language comprehension. CONCLUSIONS: These findings suggest that effective language comprehension in right-handed subjects with TLE depends on the involvement of the left hippocampal formation and underline the risks of postoperative language decline in patients with left TLE.

Genital automatisms: a video-EEG study in patients with medically refractory seizures.

PURPOSE: Genital automatisms (GAs) are rare clinical phenomena during or after epileptic seizures. They are defined as repeated fondling, grabbing, or scratching of the genitals. The anatomic correlates of GAs have been discussed controversially. The aim of this investigation was to assess the localizing and lateralizing value of GAs. METHODS: The authors studied 207 consecutive patients with intractable seizures referred to a University Hospital for presurgical evaluation between 1998 and 2002: 135 had temporal lobe epilepsy (TLE); 23, frontal lobe epilepsy (FLE); 29, generalized epilepsies (GEs); and 20 had extratemporal or multifocal epilepsy. RESULTS: Twenty-three (11%) of 207 patients showed GAs in 42 (3%) of 1,299 seizures. GAs occurred significantly more often in men (17 of 93, 18%) than in women (six of 114, 5%; p = 0.0037). Twenty-one (16%) of 135 patients with TLE performed GAs, one (4%) of 23 with FLE and one (3%) of 29 with GE. GAs were associated with unilateral hand automatisms in 16 (70%) of 23 and with periictal urinary urge in five (22%) of 23. All patients had amnesia for the performance of GAs. CONCLUSIONS: GAs appear in the ictal or postictal period with impaired consciousness. Men exhibit GAs significantly more often than do women. GAs do not localize or lateralize per se, but may localize seizure onset in the presence of periictal urinary urge or unilateral hand automatisms. They show a tendency to occur more often in TLE.

Linguistic deficits following left selective amygdalohippocampectomy: a prospective study.

Language deficits in 10 patients with medically intractable left-sided temporal lobe epilepsy prior to and following selective amygdalohippocampectomy are described. Preoperatively, a pattern of minor linguistic deficits was observed in three patients; isolated minor naming deficits were detectable in one additional patient. Three months after surgery, six patients' linguistic functions were unchanged, whereas in four patients, a significant decline in linguistic functions could be observed. All four patients revealed a very similar language syndrome characterized by reduced language comprehension and fluency, well-articulated speech, frequent word-finding difficulties, circumlocutions, and semantic paraphasias in the absence of any phonological disorder. These deficits remained stable during the 12-month follow-up period. However, magnetic resonance imaging did not show any neocortical lesions outside the resection area. Possible explanations for these findings include neuronal cell loss and deafferentiation in cortical areas, disruption of the basal temporal language area pathways, reorganization of the language network in chronic temporal lobe epilepsy, and neocortical lesions due to the surgical intervention. Furthermore, correlations between linguistic and demographic data for our patients suggest that patients older at epilepsy onset are at greater risk for developing postoperative language deficits.

Linguistic and neuropsychological deficits in crossed conduction aphasia. Report of three cases.

This study describes the linguistic and neuropsychological findings in three right-handed patients with crossed conduction aphasia. Despite the location of the lesion in the right hemisphere, all patients displayed a combination of linguistic deficits typically found in conduction aphasia following analogous damage to the left hemisphere. Associated cognitive deficits varied across the three patients. In addition, all cases showed deficits classically attributed to non-dominant hemisphere damage (visuoperceptual deficits and reduced figural memory). As a result, lesion-behaviour relationships in our study sample indicate both dominant and non-dominant qualities of the right hemisphere.

Specific order impairment in arabic number writing: A case-study.

The present study examines the transitory deficit in transcoding verbal to Arabic numbers in an aphasic patient, TM. She showed a mild syntactic impairment in syntactic comprehension of verbal numbers, with preserved performance in comprehension of Arabic numbers, in access to semantic representation, as well as in reading of Arabic numbers, but she committed 75% of errors when required to write numbers in the Arabic format to dictation. In conformity to the previous literature on transcoding deficits, the majority of her errors were syntactic (60%). However, most of them were unusual "order errors" (50%) in which lexical digits (e.g., 1 to 9) were written on the left and zeros on the right of the number, which contained in the majority of the cases the correct number of digits. A similar type of errors has been reported in only one previous case study (Delazer & Denes, 1998), but not specifically studied. We discuss hypotheses concerning its origins as stemming from a syntactic disorder within existing models of transcoding (McCloskey, Caramazza, & Basili, 1985; Power & Dal Martello, 1990). We also report kinematic assessment of the patient's handwriting before and after recovery. At time of the second examination, results show that her pattern of movement fluency parallels that of healthy subjects and supports a distinction between two types of zeros within Arabic numbers, in relation to the verbal code and the rules required to produce them. This paper thus also highlights the potential usefulness of using a digitising tablet in the study of transcoding deficits.

Medial temporal lobe activation during semantic language processing: fMRI findings in healthy left- and right-handers.

Medial temporal lobe (MTL) areas are well known to serve episodic memory functions; their contribution to semantic memory has been occasionally noticed but not studied in detail. In the present fMRI study, 35 right-handed and 35 left-handed healthy subjects performed a semantic decision paradigm during which subjects heard spoken concrete nouns designating objects and had to decide on whether these objects were available in the supermarket and cost lest then a certain amount of money. The control paradigm consisted of sequences of low and high tones where subjects had to decide whether a sequence contained two high tones. The resulting contrast activation of semantic decision versus tone decision involved neocortical temporal, parietal, and prefrontal areas. Additional significant, bilateral activations in the MTL, the hippocampal formation, and adjacent areas were found. The exact incidence and location of activation was studied in a single-subject analysis for all 70 subjects. At the chosen threshold of P<0.001, 94% of subjects showed activations in the MTL and inferior temporal lobe (ITL). Activations were found along the longitudinal axis of the MTL, including the hippocampal formation and the parahippocampal gyrus. In the ITL, parts of the fusiform and lingual gyri were activated. Activations were similar in right- and left-handers. We conclude from this study that the MTL and parts of the ITL can be added to the areas activated by semantic verbal memory processing.

Acute conduction aphasia: an analysis of 20 cases.

In this study, the linguistic performance of 20 patients with acute conduction aphasia (CA) is described. CA presented as a relatively homogeneous aphasic syndrome characterized by a severe impairment of repetition and fluent expressive language functions with frequent phonemic paraphasias, repetitive self-corrections, word-finding difficulties, and paraphrasing. Language comprehension as assessed by tests of auditory and reading comprehension was only mildly impaired, whereas most patients performed poorly on the Token Test. Verbal-auditory short-term memory was reduced in all patients except one and seems to play a role in associated cognitive deficits, such as impaired syntactic comprehension or reduced mental arithmetics. A follow-up examination of 12 patients showed that CA often resulted in a chronic language deficit. Lesion locations were the posterior temporal and inferior parietal lobe.

Basal ganglia lesions and the theory of fronto-subcortical loops: neuropsychological findings in two patients with left caudate lesions.

Basal ganglia lesions have a high prevalence for associated behavioural impairments. However, the exact pattern of cognitive impairments and its relationship to individual basal ganglia lesion have rarely been investigated by means of a detailed neuropsychological and lesion study. Furthermore, different mechanisms have been proposed as relevant for the observed cognitive deficits; among these, the hypothesis of fronto-subcortical loops (Alexander et al., 1986) has made predictions regarding the relationship between the damage of particular striato-frontal circuits and the resulting behavioural impairment which await clinical confirmation. We present a study of two subjects who suffered a MRI-documented focal left basal ganglia hematoma. The two patients differed in their lesions; in one patient (PJ) large parts of the caudate nucleus were destroyed whereas in the other (AS) mainly the pallidum and putamen were lesioned and the caudate suffered only minor damage. In the acute phase, the behavioural and neuropsychological abnormalities were similar in both cases and included mainly abulia, an impairment of executive and attentional functions, and a severe amnestic syndrome. After several months many functions were restored in AS, whereas PJ's abilities remained largely defective. Based on these data and on previous case studies several conclusions are drawn. Left caudate lesions induce marked and long-lasting behavioural and neuropsychological impairments comprising predominantly drive, executive control, attention, and memory. The extent of lesion in the head of the caudate nucleus is the critical factor regarding the severity and the outcome of the syndrome, whereas damage to the putamen and pallidum is less crucial for cognitive functions. A subset of behavioural alterations, among them abulia, attentional and frontal-executive dysfunctions, can well be attributed to lesions of the anterior cingulate circuit and the dorsolateral-frontal circuit at the basal ganglia level. Other impairments, most importantly the prominent amnestic syndrome, are more difficult to interpret on the grounds of this hypothesis and may be related to other pathomechanisms.

Persistent cognitive deficits associated with lithium intoxication: a neuropsychological case description.

We present the case of a 51-year-old patient with an acute lithium intoxication associated with several cognitive deficits. During the acute phase of intoxication the patient displayed general psychomotor slowing, dysarthric speech, mood changes, and incoherent discourse. Neuropsychological assessment revealed ideomotor apraxia, profound deficits of visuospatial processing, an impairment of memory and of frontal-executive functions. Other cognitive abilities, such as orientation, spontaneous speech, comprehension, naming, reading, writing, and working memory remained intact. An electroencephalogram revealed diffuse slowing with rhythmic trains, whereas MRI showed no cerebral abnormality. Follow-up examinations at 4 and 14 weeks with lithium levels in the normal range showed substantial recovery of memory abilities and executive functions, whereas praxis and visuoperceptual functions remained impaired, despite the fact that lithium was immediately withdrawn after the intoxication became manifest. We conclude that lithium intoxication may be associated with variable behavioural and cognitive impairments, some of them potentially persistent. Different from other case studies our findings suggest that lithium intoxication may cause a combined, multifocal functional impairment of subcortical and cortical neural mechanisms in both hemispheres.