Thomas Benke

Riluzole in Huntington's disease (HD): an open label study with one year follow up.

Abstract In an open label study, we administered riluzole (50 mg twice a day) to nine patients with genetically confirmed Huntingtons disease (HD) (clinical stages 1–3; mean age 46.4 (SD 9.3) years; mean disease duration 8 (SD 3.3) years). The study was designed to evaluate (1) safety and tolerability of riluzole and (2) effects of riluzole on motor impairment, functional disability, cognitive impairment, and behavioral abnormalities using the Unified HD Rating Scale. Patients were evaluated at baseline and after three and twelve months of riluzole therapy. Laboratory tests (hematology and liver enzymes) were repeated monthly. All adverse experiences, reported spontaneously or observed directly by the investigator, were recorded. Riluzole was well tolerated. No increase of serum liver enzymes was seen throughout the study in all but one patient showing a mild elevation. At three months, mean total motor scale (TMS), mean TMS chorea subscore, and mean total functional capacity scale were significantly improved compared with baseline. At twelve months, however, this beneficial effect on motor status and overall function was not sustained. In contrast, severity and frequency of behavioral dysfunction as well as psychomotor speed assessed by the symbol digit modalities test were improved compared with baseline. Our data suggest that there are transient antichoreatic effects and more sustained effects of riluzole on psychomotor speed and behavior in patients with HD. A double-blind, placebo-controlled trial appears highly warranted to establish definitely the symptomatic versus neuroprotective actions of riluzole in HD.

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.

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.

Sex equality in intrahemispheric language organization

Sex differences in inter- and intrahemispheric cerebral organization, found in previous studies, have been used to explain sex differences in cognitive abilities. This study highlights data to contradict such widely held beliefs. Intrahemispheric language organization was examined by determining the location of lesions causing aphasia by computed tomography. No sex differences in the incidence of anterior, posterior, or central lesions was found. The distribution of left-hemisphere lesions in stroke patients, with or without aphasia, was also equal among the sexes. This study does not support the postulated gender difference in intrahemispheric cerebral organization.

Palilalia and Repetitive Speech: Two Case Studies

Palilalia, a disorder of speech characterized by compulsive repetitions of utterances has been found in various neurological and psychiatric disorders. It has commonly been interpreted as a defect of motor speech. This article describes palilalia and other variants of verbal repetitive behavior, such as monosyllabic iterations and conduite dapproche. The clinical features of palilalia, its prevalence in different language tasks, and the individual patterns of verbal repetitive behavior are illustrated in two patients with a long-standing cerebrovascular disease. An attempt is made to locate the origin of different forms of verbal repetitions in a standard model of speech production (Butterworth, 1980a; Garrett, 1980; Levelt, 1989) by analysis of their morphology and correlation with impairments of lexical or phonological processes. From these observations it is suggested that palilalia results from control malfunctions at the level of the Articulator, whereas other variants of pathological verbal iterations result from an impairment of the Formulator or from malfunctions of both the Articulator and the Formulator.

Hemispheric mechanisms of motor speech

Abstract A perceptual rating scale was used to evaluate the articulatory impairment of patients with suprabulbar, vascular lesions of the right (RHD, 35 patients) or the left (LHD, 35 patients) hemisphere. Both groups were matched for age, time elapsed since onset, lesion volume and lesion location. The amount and type of motor speech impairment differed significantly, resulting in hemispheric profiles of articulation. Slurring and rate reduction were the prominent features of left hemisphere dysarthria and dysprosody of the right. Verbal apraxia was found in six patients of the LHD group. Intrahemispheric correlations between dysarthria, lesion volume, and several hemispheric variables (orofacial apraxia, aphasia severity and visuospatial functions) did not exceed the 0–6 level. Most lesions causing hemispheric dysarthria were located in the basal ganglia, the cortical sensorimotor and opercular region. The observed lesion distribution and speech patterns implicate distinct hemispheric mechanisms of moto...