Serum cortisol levels in patients with a transient global amnesia.

Transient global amnesia (TGA) can be triggered by a high level of blood cortisol. We assessed whether patients had a higher level of cortisol during the TGA than shortly after. We included 52 patients, 21 with blood collected during the TGA episode and 31 shortly thereafter. We compared these two groups after adjustment for time of blood collection. The cortisol level was significantly higher in the per-ictal group (P=0.03) and negatively correlated with the time elapsed from symptom onset (P=0.005). The results are consistent with of the hypothesis of a hyperreactivity of the hypothalamic-pituitary-adrenal axis.

Detection of adult attention deficit hyperactivity disorder with cognitive complaint: Experience of a French memory center.

INTRODUCTION: Attention deficit hyperactivity disorder (ADHD) is a frequent neurodevelopmental mental disorder. It can persist in adulthood and be expressed as a cognitive complaint. METHODS: We conducted a descriptive study in a French memory center concerning patients seen over a period of two years. All patients for whom the final diagnosis was ADHD were included. All patients benefited from standard neuropsychological tests and a psychiatric specific consultation. RESULTS: Thirteen patients were included with an average age of 50.2±19 years. Main complaints related to memory, attention, focusing and organizational functioning. These difficulties had negative social, professional and academic consequences. ADHD history in descendants was noted in 46% of patients. More than 20% of subjects had motor, verbal or mental restlessness. Neuropsychological assessment highlighted impaired performances in executive functions (38%), sustained attention (67%), divided attention (45%), working memory (46%) and information processing speed (75%). A psychiatric history or comorbidities were present in 85% of patients, mostly of the anxio-depressive type. The more prevalent presentations of ADHD were the combined (38%) and inattentive (38%) types. DISCUSSION: Adult ADHD can masquerade as a cognitive impairment, including a stable cognitive complaint from infancy to old age. Inattentive, hyperactive and impulsive symptoms change with time and become more internalized (such as concentration difficulties or mental restlessness). No neuropsychological pattern has been reported but fluctuating deficits in sustained, divided attention, working memory and information processing speed are frequently observed in adult ADHD. A specific psychiatric expertise is essential in diagnosis and care for ADHD and its commonly associated psychiatric comorbidities.

Factors associated with timing of early neurological improvement after thrombolysis for ischaemic stroke.

BACKGROUND: Early neurological improvement (ENI) after fibrinolysis for ischaemic stroke is strongly associated with recanalization and favourable outcome. However, it remains unknown why some patients recover within the first hour after treatment (very ENI, VENI) whereas others recover later within 24 h. AIM: The factors associated with the timing of ENI were assessed. METHODS: Consecutive stroke patients treated with intravenous recombinant tissue plasminogen activator (rt-PA) within 4.5 h after onset in four stroke centres of our geographical area were retrospectively studied. VENI assessed at 1 h and ENI assessed at 24 h post-treatment were defined by National Institutes of Health Stroke Scale (NIHSS) improvement by 40% from baseline. RESULTS: Of 421 patients, 65 (15%) had VENI and 110 (26%) had ENI. Patients with VENI had significantly lower serum creatinine level than patients with ENI (79 ± 19 vs. 91 ± 35 µmol/l; P = 0.01). After adjustment for age, sex, baseline NIHSS, hypertension and blood glucose level, patients with low serum creatinine level were more likely to have VENI (lowest tertile, odds ratio 3.8, 95% confidence interval 1.5-9.7; intermediate tertile, odds ratio 1.8, 95% confidence interval 0.8-4.3; P for trend <0.01). VENI patients were as likely as ENI patients to have a modified Rankin scale score ≤2 at 3 months. CONCLUSIONS: Low serum creatinine levels are associated with VENI, suggesting that swiftness of the efficacy of rt-PA or of neurological recovery may depend on renal function.

Hippocampal modifications in transient global amnesia.

Transient global amnesia (TGA) is an acute and transient syndrome with a remarkably stereotypical set of signs and symptoms. It is characterized by the abrupt onset (no forewarning) of massive episodic memory impairment, both anterograde and retrograde. Ever since it was first described, TGA has fascinated neurologists and other memory experts, and in recent years, there has been a surge of neuroimaging studies seeking to pin down the brain dysfunction responsible for it. Several pathophysiological hypotheses have been put forward, including the short-lived suggestion of an epileptic mechanism. All the available data indicate that the brain modifications are reversible, and that the mechanism behind TGA is of a functional nature. However, while diffusion-weighted imaging studies have clearly identified the hippocampus and, more specifically, the CA1 area, as the locus of brain modifications associated with TGA, researchers have yet to determine whether the origin of the mechanism is vascular or neurochemical. Spectroscopy may provide a means of settling this issue once and for all.

Sleep oscillations related to memory consolidation during aromatases inhibitors for breast cancer.

Aromatase inhibitors (AIs) are associated with sleep difficulties in breast cancer (BC) patients. Sleep is known to favor memory consolidation through the occurrence of specific oscillations, i.e., slow waves (SW) and sleep spindles, allowing a dialogue between prefrontal cortex and the hippocampus. Interestingly, neuroimaging studies in BC patients have consistently shown structural and functional modifications in these two brain regions. With the aim to evaluate sleep oscillations related to memory consolidation during AIs, we collected polysomnography data in BC patients treated (AI+, n = 17) or not (AI-, n = 17) with AIs compared to healthy controls (HC, n = 21). None of the patients had received chemotherapy and radiotherapy was finished since at least 6 months, that limit the confounding effects of other treatments than AIs. Fast and slow spindles were detected during sleep stage 2 at centro-parietal and frontal electrodes respectively. SW were detected at frontal electrodes during stage 3. Here, we show lower frontal SW densities in AI + patients compared to HC. These results concord with previous reports about frontal cortical alterations in cancer following AIs administration. Moreover, AI + patients tended to have lower spindle density at C4 electrode. Regression analyses showed that, in both patient groups, spindle density at C4 electrode explained a large variance of memory performances. Slow spindle characteristics did not differ between groups and sleep oscillations characteristics of AI- patients did not differ significantly from those of both AI + patients and HC. Overall, our results add to the compelling evidence of the systemic effects of AIs previously reported in animals, with deleterious effects on cortical activity during sleep and associated memory consolidation in the current study. There is thus a need to further investigate sleep modifications during AIs administration. Longitudinal studies are needed to confirm these findings and investigation in other cancers on this topic should be conducted.

Sleep macro- and microstructure in breast cancer survivors.

Complaints of sleep disturbance are prevalent among breast cancer (BC) patients and are predictors of quality of life. Still, electrophysiological measures of sleep are missing in patients, which prevents from understanding the pathophysiological consequences of cancer and its past treatments. Using polysomnography, sleep can be investigated in terms of macro- (e.g. awakenings, sleep stages) and micro- (i.e. cortical activity) structure. We aimed to characterize sleep complaints, and macro- and microstructure in 33 BC survivors untreated by chemotherapy and that had finished radiotherapy since at least 6 months (i.e. out of the acute effects of radiotherapy) compared to 21 healthy controls (HC). Compared to HC, BC patients had a larger number of awakenings (p = 0.008); and lower Delta power (p < 0.001), related to sleep deepening and homeostasis; greater both Alpha (p = 0.002) and Beta power (p < 0.001), related to arousal during deep sleep; and lower Theta power (p = 0.004), related to emotion regulation during dream sleep. Here we show that patients have increased cortical activity related to arousal and lower activity related to sleep homeostasis compared to controls. These results give additional insights in sleep pathophysiology of BC survivors and suggest sleep homeostasis disruption in non-advanced stages of BC.

The neural substrates of musical memory revealed by fMRI and two semantic tasks.

Recognizing a musical excerpt without necessarily retrieving its title typically reflects the existence of a memory system dedicated to the retrieval of musical knowledge. The functional distinction between musical and verbal semantic memory has seldom been investigated. In this fMRI study, we directly compared the musical and verbal memory of 20 nonmusicians, using a congruence task involving automatic semantic retrieval and a familiarity task requiring more thorough semantic retrieval. In the former, participants had to access their semantic store to retrieve musical or verbal representations of melodies or expressions they heard, in order to decide whether these were then given the right ending or not. In the latter, they had to judge the level of familiarity of musical excerpts and expressions. Both tasks revealed activation of the left inferior frontal and posterior middle temporal cortices, suggesting that executive and selection processes are common to both verbal and musical retrievals. Distinct patterns of activation were observed within the left temporal cortex, with musical material mainly activating the superior temporal gyrus and verbal material the middle and inferior gyri. This cortical organization of musical and verbal semantic representations could explain clinical dissociations featuring selective disturbances for musical or verbal material.

Musical and verbal semantic memory: two distinct neural networks?

Semantic memory has been investigated in numerous neuroimaging and clinical studies, most of which have used verbal or visual, but only very seldom, musical material. Clinical studies have suggested that there is a relative neural independence between verbal and musical semantic memory. In the present study, "musical semantic memory" is defined as memory for "well-known" melodies without any knowledge of the spatial or temporal circumstances of learning, while "verbal semantic memory" corresponds to general knowledge about concepts, again without any knowledge of the spatial or temporal circumstances of learning. Our aim was to compare the neural substrates of musical and verbal semantic memory by administering the same type of task in each modality. We used high-resolution PET H(2)O(15) to observe 11 young subjects performing two main tasks: (1) a musical semantic memory task, where the subjects heard the first part of familiar melodies and had to decide whether the second part they heard matched the first, and (2) a verbal semantic memory task with the same design, but where the material consisted of well-known expressions or proverbs. The musical semantic memory condition activated the superior temporal area and inferior and middle frontal areas in the left hemisphere and the inferior frontal area in the right hemisphere. The verbal semantic memory condition activated the middle temporal region in the left hemisphere and the cerebellum in the right hemisphere. We found that the verbal and musical semantic processes activated a common network extending throughout the left temporal neocortex. In addition, there was a material-dependent topographical preference within this network, with predominantly anterior activation during musical tasks and predominantly posterior activation during semantic verbal tasks.

Direct voxel-based comparison between grey matter hypometabolism and atrophy in Alzheimer's disease.

Although the patterns of structural and metabolic brain alterations in Alzheimer's disease are being refined and discrepancies between them are being underlined, the exact relationships between atrophy and hypometabolism are still unclear. In this study, we aimed to provide a direct comparison between grey matter atrophy and hypometabolism in a sample of patients with clinically probable Alzheimer's disease, using a voxel-based method specially designed to statistically compare the two imaging modalities. Eighteen patients with probable Alzheimer's disease of mild severity and 15 healthy aged controls underwent both high-resolution T1 MRI and resting-state (18)FDG-PET. The MRI data sets were handled using optimized VBM. The PET data were coregistered to their corresponding MRI, corrected voxel-wise for partial volume averaging and spatially normalized using the same parameters as those of their corresponding MRI volume. A differential smoothing was applied on the MRI and PET data sets to equalize their effective smoothness and resolution. For each patient, Z-score maps of atrophy and hypometabolism were created by comparing to the controls data set, respectively averaged to provide the profile of hypometabolism and atrophy, and entered in a voxel-by-voxel SPM analysis to assess the statistical differences between hypometabolism and atrophy. The observed patterns of hypometabolism and atrophy were consistent with previous studies. However, the direct comparison revealed marked regional variability in the relationship between hypometabolism and atrophy. Thus, the hypometabolism significantly exceeded atrophy in most altered structures, particularly in the posterior cingulate-precuneus, orbitofrontal, inferior temporo-parietal, parahippocampal, angular and fusiform areas. In contrast, a few hypometabolic structures among which the hippocampus exhibited similar degrees of atrophy and hypometabolism, a profile that significantly differed from the posterior cingulate. Excessive hypometabolism relative to atrophy suggests the intervention of additional hypometabolism-inducing factors, such as disconnection and amyloid deposition, resulting in genuine functional perturbation ahead of actual atrophy and perhaps of pathology as well. Conversely, in the hippocampus, where disconnection processes are also likely to occur, relative synaptic compensatory mechanisms may be taking place, maintaining neuronal activity in the face of structural alterations.

Three-dimensional surface mapping of hippocampal atrophy progression from MCI to AD and over normal aging as assessed using voxel-based morphometry.

The hippocampus is the brain structure of highest and earliest structural alteration in Alzheimer's disease (AD). New developments in neuroimaging methods recently made it possible to assess the respective involvement of the different hippocampal subfields by mapping atrophy on a 3D hippocampal surface view. In this longitudinal study on patients with mild cognitive impairment (MCI), we used such an approach to map the profile of hippocampal atrophy and its progression over an 18-month follow-up period in rapid converters to AD and "non-converters" compared to age-matched controls. For the sake of comparison, we also assessed the profile of hippocampal atrophy associated with AD and with increasing age in a healthy control population ranging from young adult to elderly. We found major involvement of the lateral part of the superior hippocampus mainly corresponding to the CA1 subfield in MCI and AD while increasing age was mainly associated with subiculum atrophy in the healthy population. Moreover, the CA1 subfield also showed highest atrophy rates during follow-up, in both rapid converters and "non-converters" although increased effects were observed in the former group. This study emphasizes the differences between normal aging and AD processes leading to hippocampal atrophy, pointing to a specific AD-related CA1 involvement while subiculum atrophy would represent a normal aging process. Our findings also suggest that the degree of hippocampal atrophy, more than its spatial localization, predicts rapid conversion to AD in patients with MCI.

[The evolution of adult clinical neuropsychology in Paris from 1957 to 2000]. / L'évolution de la neuropsychologie clinique de l'adulte à Paris de 1957 à 2000.

This sketchy account of the history of French-speaking clinical neuropsychology from 1957 to 2000 first recalls the Salpêtrière school leaded by Alajouanine and his disciples, and second the Ste Anne psychiatric hospital, with on one hand the Chair of Mental and Brain Diseases, and on the other hand a number of research teams gathered around disciples of Dejerine such as Jean Lhermitte. Because of the large scope of this topic, the authors could not elude the need for a bibliographic selection that may seem questionable.

Voxel-based mapping of grey matter volume and glucose metabolism profiles in amyotrophic lateral sclerosis.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive disease of the nervous system involving both upper and lower motor neurons. The patterns of structural and metabolic brain alterations are still unclear. Several studies using anatomical MRI yielded a number of discrepancies in their results, and a few PET studies investigated the effect of ALS on cerebral glucose metabolism. The aim of this study was threefold: to highlight the patterns of grey matter (GM) atrophy, hypometabolism and hypermetabolism in patients with ALS, then to understand the neurobehavioral significance of hypermetabolism and, finally, to investigate the regional differences between the morphologic and functional changes in ALS patients, using a specially designed voxel-based method. Thirty-seven patients with ALS and 37 age- and sex-matched healthy individuals underwent both structural MRI and 18[F]-fluorodeoxyglucose (FDG) PET examinations. PET data were corrected for partial volume effects. Structural and metabolic abnormalities were examined in ALS patients compared with control subjects using two-sample t tests in statistical parametric mapping (SPM). Then, we extracted the metabolic values of clusters presenting hypermetabolism to correlate with selected cognitive scores. Finally, GM atrophy and hypometabolism patterns were directly compared with a one-paired t test in SPM. RESULTS: We found GM atrophy as well as hypometabolism in motor and extra motor regions and hypermetabolism in medial temporal lobe and cerebellum. We observed negative correlations between the metabolism of the right and left parahippocampal gyri and episodic memory and between the metabolism of right temporal pole and cognitive theory of mind. GM atrophy predominated in the temporal pole, left hippocampus and right thalamus, while hypometabolism predominated in a single cluster in the left frontal superior medial cortex. CONCLUSIONS: Our findings provide direct evidence of regional variations in the hierarchy and relationships between GM atrophy and hypometabolism in ALS. Moreover, the 18FDG-PET investigation suggests that cerebral hypermetabolism is deleterious to cognitive function in ALS.

Pathophysiology of the behavioral variant of frontotemporal lobar degeneration: A study combining MRI and FDG-PET.

Gray matter (GM) lobar atrophy and glucose hypometabolism are well-described hallmarks of frontotemporal lobar degeneration (FTLD), but the relationships between them are still poorly understood. In this study, we aimed to show the patterns of GM atrophy and hypometabolism in a sample of 15 patients with the behavioral variant of FTLD (bv-FTD), compared to 15 healthy controls, then to provide a direct comparison between GM atrophy and hypometabolism, using a voxel-based method specially designed to statistically compare the two imaging modalities. The participants underwent structural magnetic resonance imaging and 18F-fluorodeoxyglucose (FDG) positron emission tomography examinations. First, between-group comparisons of GM volume and metabolism were performed. Then, in the patient group, correlations between regional alterations and direct between-modality voxelwise comparison were performed. Finally, we examined individual patterns of brain abnormalities for each imaging modality and each patient. The observed patterns of GM atrophy and hypometabolism were consistent with previous studies. We found significant voxelwise correlations between changes in GM and FDG uptake, mainly in the frontal cortex, corresponding to the typical profile of alterations in bv-FTD. The direct comparison revealed regional variability in the relationship between hypometabolism and atrophy. This analysis revealed greater atrophy than hypometabolism in the right putamen and amygdala, and left insula and superior temporal gyrus, whereas hypometabolism was more severe than GM atrophy in the left caudate nucleus and anterior cingulate cortex. Finally, GM atrophy affected the right amygdala/hippocampus and left insula in 95 % of the patients. These findings provide evidence for regional variations in the hierarchy of hypometabolism and GM atrophy and the relationships between them, and enhance our understanding of the pathophysiology of bv-FTD.

Prophylactic cranial irradiation for patients with small-cell lung cancer in complete remission.

BACKGROUND: Prophylactic cranial irradiation in patients with small-cell lung cancer decreases the overall rate of brain metastases without an effect on overall survival. It has been suggested that this treatment may increase neuropsychological syndromes and brain abnormalities indicated by computed tomography scans. However, other retrospective data suggested a beneficial effect on overall survival for patients in complete remission. PURPOSE: Our purpose was to evaluate the effects of prophylactic cranial irradiation on brain metastasis, overall survival, and late-occurring toxic effects in patients with small-cell lung cancer in complete remission. METHODS: We conducted a prospective study of 300 patients who had small-cell lung cancer that was in complete remission. The patients were randomly assigned to receive either prophylactic cranial irradiation delivering 24 Gy in eight fractions during 12 days (treatment group) or no prophylactic cranial irradiation (control group). A neuropsychological examination and a computed tomography scan of the brain were performed at the time of random assignment and repeatedly assessed at 6, 18, 30, and 48 months. Patterns of failure were analyzed according to total event rates and also according to an isolated first site of relapse, using a competing-risk approach. RESULTS: Two hundred ninety-four patients who did not have brain metastases at the time of random assignment were analyzed. The 2-year cumulative rate of brain metastasis as an isolated first site of relapse was 45% in the control group and 19% in the treatment group (P < 10(-6)). The total 2-year rate of brain metastasis was 67% and 40%, respectively (relative risk = 0.35; P < 10(-13)). The 2-year overall survival rate was 21.5% in the control group and 29% in the treatment group (relative risk = 0.83; P = .14). There were no significant differences between the two groups in terms of neuropsychological function or abnormalities indicated by computed tomography brain scans. CONCLUSIONS: Prophylactic cranial irradiation given to patients with small-cell lung cancer in complete remission decreases the risk of brain metastasis threefold without a significant increase in complications. A possible beneficial effect on overall survival should be tested with a higher statistical power. IMPLICATIONS: The results of the trial favor, at present, the indication of prophylactic cranial irradiation for patients who are in complete remission. A longer follow-up and confirmatory trials are needed to fully assess late-occurring toxic effects. The possible effect on overall survival needs to be evaluated with a larger number of patients in complete remission, and a meta-analysis of similar trials is recommended.

[Quantitative relationships between ADC and perfusion changes in acute ischemic stroke using combined diffusion-weighted imaging and perfusion MR (DWI/PMR)]. / Relation quantitative des modifications de la diffusion et de la perfusion au sein du parenchyme cerebral au cours de l'accident ischémique aigu.

UNLABELLED: MR-based diffusion- and perfusion-weighted imaging (DWI/PWI) has become the standard imaging technique to assess the individual brain pathophysiological status in acute ischemic stroke. The finding of a "mismatch" with larger PWI than DWI abnormality is thought to reflect the presence of tissue at-risk of infarction, i.e., penumbra. However, there has been no detailed study of the quantitative relationships between perfusion and diffusion changes in stroke patients. According to the experimental concept of penumbra, the ADC would be expected to remain unchanged despite decreasing perfusion until a critical threshold is reached. We have tested this hypothesis directly in man. METHODS: DWI/PWI was performed in 7 patients with MCA territory stroke within 4-10 hrs from onset. Mismatch was defined on diffusion and rMTT maps, and circular ROIs were positioned within the ADC lesion (D), the mismatch area (M), and the normal appearing cortex (N); mirror ROIs were also obtained, and affected/unaffected ratios for ADC and rCBF were computed for each ROI. RESULTS: The mean (+/-1 SD) ADC ratios were 0.60 +/- 0.09, 0.95 +/- 0.10 and 1.02 +/- 0.04 in L, M and N, respectively; the corresponding rCBF ratios were 0.32 +/- 0.12, 0.75 +/- 0.14 and 0.97 +/- 0.09, respectively. The relationship was non-linear, with the rCBF but not the ADC ratio for M being significantly lower (p < 0.01) than that for N. A threshold for decline in ADC was apparent around 0.50 rCBF ratio. COMMENT: These results directly document in man that the ADC declines only after hypoperfusion has reached a certain degree (about 50%), consistent with the concept of the ischaemic penumbra.

Nitrous oxide anesthesia-associated myelopathy.

BACKGROUND: The role of nitrous oxide exposure in neurologic complications of subclinical cobalamin deficiency has been reported, but few cases are well documented. OBSERVATION: Two weeks after surgery for prosthetic adenoma, a 69-year-old man developed ascending paresthesia of the limbs, severe ataxia of gait, tactile sensory loss on the 4 limbs and trunk, and absent tendon reflexes. After a second surgical intervention, the patient became confused. Four months after onset, the patient had paraplegia, severe weakness of the upper limbs, cutaneous anesthesia sparing the head, and confusion. Moderate macrocytosis, low serum B12 levels, and a positive Schilling test result led to the diagnosis of pernicious anemia. Results of electrophysiologic examinations showed a diffuse demyelinating neuropathy. Magnetic resonance imaging of the spinal cord disclosed hyperintensities of the dorsal columns on T2-weighted images. CONCLUSIONS: Pernicious anemia can result in severe neurologic symptoms with only mild hematologic changes. The role of nitrous oxide anesthesia in revealing subclinical B12 deficiency must be emphazised. Magnetic resonance imaging of the spinal cord might be helpful in making the diagnosis.

Identification and discrimination disorders in auditory perception: a report on two cases.

Auditory perception was investigated in two brain-damaged subjects. The first patient had a left temporoparietal ischaemic lesion. He presented a right-ear extinction in dichotic tasks, as well as difficulties in understanding and repeating verbal material and impaired identification of melodies. All discrimination tests were well performed. The second patient had a right capsulolenticular and frontal ischaemic lesion. He presented a left ear dichotic extinction and severe difficulties in discrimination of environmental sounds and melodies but no major difficulty in naming and identification. From these results, it is hypothesized that identification and discrimination involve distinct mechanisms within the processing of auditory stimuli, and that they may be selectively disrupted in brain-damaged subjects.

Characteristics and evolution of writing impairment in Alzheimer's disease.

Rapcsak et al. (Archs Neurol. 46, 65-67, 1989) proposed a hypothesis describing the evolution of agraphic impairments in dementia of the Alzheimer type (DAT): lexico-semantic disturbances at the beginning of the disease, impairments becoming more and more phonological as the dementia becomes more severe. Our study was conducted in an attempt to prove this hypothesis on the basis of an analysis of the changes observed in the agraphia impairment of patients with DAT. A writing test from dictation was proposed to 22 patients twice, with an interval of 9-12 months between the tests. The results show that within 1 year there was little change in the errors made by the patients in the writing test. The changes observed however were all found to develop within the same logical progression (as demonstrated by Correspondence Analysis). These findings made it possible to develop a general hypothesis indicating that the agraphic impairment evolves through three phases in patients with DAT. The first one is a phase of mild impairment (with a few possible phonologically plausible errors). In the second phase non-phonological spelling errors predominate, phonologically plausible errors are fewer and the errors mostly involve irregular words and non-words. The last phase involves more extreme disorders that affect all types of words. We observe many alterations due to impaired graphic motor capacity. This work would tend to confirm the hypothesis proposed by Rapcsak et al. concerning the development of agraphia, and would emphasize the importance of peripheral impairments, especially grapho-motor impairments which come in addition to the lexical and phonological impairments.

Right motor neglect associated with dynamic aphasia, loss of drive and amnesia: case report and cerebral blood flow study.

A 30-year-old right-handed man had right motor neglect, amnesia, aphasia and loss of drive following bilateral thalamic and subthalamic infarctions. Serial resting cerebral blood flow (CBF) measurements with either Xenon 133 inhalation or positron emission tomography at 1, 8 and 10 months post-onset showed a widespread and long-lasting low CBF in the cortex. An additional CBF measurement, during motor tasks, showed a marked interhemispheric asymmetry in the pattern of activation: whereas left hand movement resulted in a CBF increase in contralateral superior rolandic and prerolandic areas, no significant regional CBF changes were seen during right hand movement, despite recovery from motor neglect. This loss of CBF increase in cortical motor and premotor areas during voluntary movement of the previously neglected side points to a disruption of cortico-subcortical pathways subserving motor activation. The pathophysiology of aphasia, loss of drive and amnesia as well as their relationships to motor neglect, may also be discussed on the basis of thalamo-cortical disconnections.

Inclusions in familial cytoplasmic body myopathy are stained by anti-dystrophin antibodies.

We report here for the first time positive anti-dystrophin labelling of inclusions in three cases belonging to the same family affected by familial cytoplasmic body myopathy (CBM). Inclusions are also stained, as reported previously, by anti-actin antibodies. The anti-desmin reaction was negative in the centre of cytoplasmic bodies (CB) but showed an enhancement of staining in the peripheral part. Abnormal sarcoplasmic staining of fibres with CB was also observed with that antibody. Anti-vimentin antibody labelling was negative. At present, the significance of this labelling by anti-dystrophin antibodies is unknown, but will open new fields for further investigations in an attempt to understand CB pathogenesis.