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Recent research has revealed that the cerebellum plays a critical role in social reasoning and in particular in understanding false beliefs and making trait attributions. One hypothesis is that the cerebellum is responsible for the understanding of sequences of motions and actions, which may be a prerequisite for social understanding. To investigate the role of action sequencing in mentalizing, we tested patients with generalized cerebellar degenerative lesions on tests of social understanding and compared their performance with matched healthy volunteers. The tests involved understanding violations of social norms making trait and causal attributions on the basis of short behavioral sentences and generating the correct chronological order of social actions depicted in cartoons (picture sequencing task). Cerebellar patients showed clear deficits only on the picture sequencing task when generating the correct order of cartoons depicting false belief stories and showed at or close to normal performance for mechanical stories and overlearned social scripts. In addition, they performed marginally worse on trait attributions inferred from verbal behavioral descriptions. We conclude that inferring the mental state of others through understanding the correct sequences of their actions requires the support of the cerebellum., SCOPUS: ar.j, info:eu-repo/semantics/published

Objectives: To understand the subjective pain experience of patients, healthcare providers rely heavily on self-reporting. However, to quantify this unique pain experience, objective parameters are not yet available in daily clinical practice. With regard to patients with failed back surgery syndrome (FBSS) treated with spinal cord stimulation (SCS), pain therapists may recover the individual functional information about the patient's posture from the implantable pulse generator (IPG) of the stimulator. The aim of this study is to investigate whether subjective self-reporting is in correlation with the functional capacities of a patient. Materials and Methods: Thirty-nine patients with FBSS, treated with SCS were included. The accelerometer in the IPG detects positional changes and provides an objective output of seven functional positions (lying back, lying prone, lying left, lying right, transition, upright, and upright + mobile). The Oswestry Disability Index (ODI), VAS-diary, and the Pittsburgh Sleep Quality Index (PSQI) were assessed to evaluate physical functioning, pain intensities, and subjective sleep quality. Additionally, 21 patients wore a wearable actigraph device to objectify sleep quality. The agreement and Spearman correlations between objective and subjective parameters were assessed. Results: Spearman rank correlations revealed no significant correlations between the ODI (subscales walking, sitting, standing, and sleeping) and the output of the IPG (percentage upright + mobile, transition, upright, and lying, respectively). Sleep parameters measured with the Actiwatch and the PSQI were not in agreement. Conclusions: This study demonstrated that self-reporting questionnaires do not correlate with the findings of objective measurements. Therefore, we recommend using both subjective and objective parameters when determining treatment options for FBSS patients.

When we feel sad or depressed, our face invariably “drops”. Conversely, when we try to cheer someone up, we might tell them “keep your smile up”, so presupposing that modifying the configuration of their facial muscles will enhance their mood. A crucial assumption that underpins this hypothesis is that mental states are shaped by information originating from the peripheral neuromotor system — a view operationalised as the Facial Feedback Hypothesis. We used botulinum toxin (BoNT-A) injected over the frown area to temporarily paralyse muscles necessary to express anger. Using a pre-post treatment design, we presented participants with gradually changing videos of a face morphing from neutral to full-blown expressions of either anger or happiness and asked them to press a button as soon as they had detected any change in the display. Results indicate that while all participants (control and BoNT-A) improved their reaction times from pre-test to post-test, the BoNT-A group did not when detecting anger in the post-test. We surmise that frown paralysis disadvantaged participants in their ability to improve the detection of anger. Our finding suggests that facial feedback causally affects perceptual awareness of changes in emotion, as well as people’s ability to use perceptual information to learn.

This multi-study connectivity analysis explores the functional connectivity of the cerebellum with the cerebrum in social mentalizing, that is, understanding the mind of another person. The analysis covers 5 studies ( n = 92) involving abstract and complex forms of social mentalizing such as (a) person and group impression formation based on behavioral descriptions and (b) constructing personal counterfactual events (i.e., how the past could have turned out better). The results suggest that cerebellar activity during these social processes reflects a domain-specific mentalizing functionality that is strongly connected with a corresponding mentalizing network in the cerebrum. A significant pattern of connectivity was found linking the dorsal medial prefrontal cortex (mPFC) and the right temporo-parietal junction (TPJ) with the right posterior cerebellum, and linking the latter with the left TPJ. In addition, in the cerebrum, further connectivity was found through links of the bilateral TPJ with the dorsal mPFC, orbitofrontal cortex and between right and left TPJ. The discussion centers on the role of these cerebro-cerebellar connections in matching external information from the cerebrum with internal predictions generated by the cerebellum. These internal predictions might involve the sequencing of the person's behaviors.

BACKGROUND: Extraforaminal lumbar disc herniation (EFLDH) accounts for 7%-12% of all lumbar disc herniations. We report on a surgical technique for EFLDH, which requires only minimal resection of the facet joint and also allows access to the preforaminal space, if necessary. METHODS: The medical records of 61 consecutive patients treated with disc fragment herniectomy through a facet joint quadrantectomy for EFLDH at the Universitair Ziekenhuis Brussel were critically evaluated with respect to preoperative clinical signs and symptoms, surgery-related complications and outcome at 6 weeks after intervention. Patient satisfaction after the surgery was evaluated using the MacNab classification. RESULTS: The prevalence of leg pain decreased from 100% before the intervention to 19.7% at follow-up. Only 9 patients (14.8%) suffered from residual motor deficit at follow-up, compared with 37 patients (60.7%) in the preoperative situation. The postoperative improvement was highly significant for all parameters (P < 0.0001) and this evolution is also reflected in the MacNab classification showing 62.3% excellent, 23.0% good, 13.1% fair, and only 1.6% poor satisfaction. Using logistic regression analysis, only the presence of a preoperative sensory deficit was found to be an independent predictor for excellent patient satisfaction on the MacNab classification. CONCLUSIONS: Disc fragment herniectomy through a facet joint quadrantectomy for EFLDH is a safe and minimal invasive technique resulting in very satisfactory results. Preservation of facet joint stability and the possibility to convert to a classic approach to reach for preforaminal components, if necessary, are major advantages of this technique.

Apraxic agraphia (AA) is a so-called peripheral writing disorder following disruption of the skilled movement plans of writing while the central processes that subserve spelling are intact. It has been observed in a variety of etiologically heterogeneous neurological disorders typically associated with lesions located in the language dominant parietal and frontal region. The condition is characterized by a hesitant, incomplete, imprecise or even illegible graphomotor output. Letter formation cannot be attributed to sensorimotor, extrapyramidal or cerebellar dysfunction affecting the writing limb. Detailed clinical, neurocognitive, neurolinguistic and (functional) neuroimaging characteristics of three unique cases are reported that developed AA following a thalamic stroke. In marked contrast to impaired handwriting, non-handwriting skills, such as oral spelling, were hardly impaired. Quantified Tc-99m ECD SPECT consistently showed a decreased perfusion in the anatomoclinically suspected prefrontal regions. The findings suggest crucial involvement of the anterior (and medial) portion of the left thalamus within the neural network subserving the graphomotor system. Functional neuroimaging findings seem to indicate that AA after focal thalamic damage represents a diaschisis phenomenon.

Objective The authors compared the effectiveness of 2 intensive therapy methods: Constraint-Induced Aphasia Therapy (CIAT; Pulvermüller et al., 2001) and semantic therapy (BOX; Visch-Brink & Bajema, 2001). Method Nine patients with chronic fluent aphasia participated in a therapy program to establish behavioral treatment outcomes. Participants were randomly assigned to one of two groups (CIAT or BOX). Results Intensive therapy significantly improved verbal communication. However, BOX treatment showed a more pronounced improvement on two communication—namely, a standardized assessment for verbal communication, the Amsterdam Nijmegen Everyday Language Test (Blomert, Koster, & Kean, 1995), and a subjective rating scale, the Communicative Effectiveness Index (Lomas et al., 1989). All participants significantly improved on one (or more) subtests of the Aachen Aphasia Test (Graetz, de Bleser, & Willmes, 1992), an impairment-focused assessment. There was a treatment-specific effect. BOX treatment had a significant effect on language comprehension and semantics, whereas CIAT treatment affected language production and phonology. Conclusion The findings indicate that in patients with fluent aphasia, (a) intensive treatment has a significant effect on language and verbal communication, (b) intensive therapy results in selective treatment effects, and (c) an intensive semantic treatment shows a more striking mean improvement on verbal communication in comparison with communication-based CIAT treatment.

BackgroundAphasia is characterized by difficulties in connected speech/writing. AimsTo explore the differences between the oral and written description of a picture in individuals with chronic aphasia (IWA) and healthy controls. Descriptions were controlled for productivity, efficiency, grammatical organization, substitution behaviour and discourse organization. Methods & ProceduresFifty IWA and 50 healthy controls matched for age, gender and education provided an oral and written description of a black-and-white situational drawing from the Dutch version of the Comprehensive Aphasia Test. Between- and within-group analyses were carried out and the reliability of the test instrument was assessed. Outcomes & ResultsThe language samples of the healthy controls were more elaborate, more efficient, syntactically richer, more coherent, and consisted of fewer spoken and written language errors than the samples of the IWA. Within-group comparisons showed that connected writing is more sensitive than connected speech to capture aphasic symptoms. Conclusions & ImplicationsThe analysis of both modalities (speech and writing) at the discourse level allows one to assess simultaneously micro- and macro-linguistic skills and their potential interrelations in a given IWA. Connected writing appears to be more sensitive in discriminating IWA from healthy controls than connected speech. This method for analyzing language samples should, however, be used in conjunction with other assessment tools.

International audience; Abstract : Background: The thumb plays a critical role for manual tasks during the activities of daily life and the incidence of neurological or musculoskeletal disorders affecting the voluntary movements of the thumb is high in the elderly. There is currently no tool to assess repetitive motor sequencing of the thumb during ageing.Objectives: To report a novel procedure (the Click Test) assessing the effects of ageing on fast motor sequencing of the thumb.Methods : Healthy subjects (n = 252; mean age +/- SD: 49.76 +/- 19.97 years; range: 19-89 years; F/M: 151/101) were asked to perform fast repeated flexion/extension movements of the thumb using a mechanical counter.Results: Motor performances (assessed by the number of clicks during 3 time periods: 15, 30 and 45 sec), significantly decreased as a function of age for both the dominant (age effect; p< 0.0001 for 15, 30 and 45 sec) and the non-dominant hand (p

Until today, there is no satisfying explanation for why one language may recover worse than another in differential bilingual aphasia. One potential explanation that has been largely unexplored is that differential aphasia is the consequence of a loss of language control rather than a loss of linguistic representations. Language control is part of a general control mechanism that also manages non-linguistic cognitive control. If this system is impaired, patients with differential aphasia could still show bilingual language activation, but they may be unable to manage activation in non-target languages, so that performance in another language is hindered.To investigate whether a loss of cognitive control, rather than the loss of word representations in a particular language, might underlie differential aphasia symptoms.We compared the performance of seven bilinguals with differential and eight bilinguals with parallel aphasia with 19 control bilinguals in a lexical decision and a flanker task to assess bilingual language co-activation and non-linguistic control respectively.We found similar cognate effects in the three groups, indicating similar lexical processing across groups. Additionally, we found a larger non-linguistic control congruency effect only for the patients with differential aphasia.The present data indicate preserved language co-activation for patients with parallel as well as differential aphasia. Furthermore, the results suggest a general cognitive control dysfunction, specifically for differential aphasia. Taken together, the results of the current study provide further support for the hypothesis of impaired cognitive control abilities in patients with differential aphasia, which has both theoretical and practical implications.

Background: Evidence suggests that the concordance between amyloid-PET and cerebrospinal fluid (CSF) amyloid-beta (A beta) increases when the CSF A beta(1-42)/A beta(1-40) ratio is used as compared to CSF A beta(1-42) levels alone. Objective: In order to test this hypothesis, we set up a prospective longitudinal study comparing the concordance between different amyloid biomarkers for Alzheimer's disease (AD) in a clinical setting. Methods: Seventy-eight subjects (AD dementia (n = 17), mild cognitive impairment (MCI, n = 48), and cognitively healthy controls (n = 13)) underwent a [F-18] Florbetapir ([F-18]AV45) PET scan, [F-18] FDG PET scan, MRI scan, and an extensive neuropsychological examination. In a large subset (n = 67), a lumbar puncture was performed and AD biomarkers were analyzed (A beta(1-42), A beta(1-40), T-tau, P-tau(181)). Results: We detected an increased concordance in the visual and quantitative (standardized uptake value ratio (SUVR) and total volume of distribution (VT)) [F-18]AV45 PET measures when the CSF A beta(1-42)/A beta(1-40) was applied compared to A beta(1-42) alone. CSF biomarkers were stronger associated to [F-18]AV45 PET for SUVR values when considering the total brain white matter as reference region instead of cerebellar grey matter Conclusion: The concordance between CSF A beta and [F-18]AV45 PET increases when the CSF A beta(1-42)/A beta(1-40) ratio is applied. This finding is of most importance for the biomarker-based diagnosis of AD as well as for selection of subjects for clinical trials with potential disease-modifying therapies for AD.

Objective: Approximately 97% of the right-handers has left hemisphere language dominance. Within the language dominant hemisphere Broca's area is of crucial importance for a variety of linguistic functions. As a result, tumour resection in and around Broca's area is controversial. However, studies showed that by means of Direct Electrical Stimulation (DES) tumour resection in this region can be safely performed. We report unexpected anatomoclinical findings in a right-handed patient who underwent tumour resection in the left prefrontal lobe. Methods: Language functions in this right-handed patient were extensively examined in the pre-, intra, and postoperative phase by means of a standardised battery of neurolinguistic and neurocognitive tests. Results obtained in the pre- and postoperative phase are compared. In addition, intraoperative DES findings and postoperative functional Magnetic Resonance Imaging (fMRI) and Diffusion Tensor Imaging (DTI) results are reported. Results: Tumour resection near Broca's area was safely performed since no positive language sites were found during intraoperative DES. Since no linguistic deficits occurred in the pre-, intra-, or postoperative phase, atypical language dominance was suspected. Neuropsychological investigations, however, disclosed permanent executive dysfunction. Postoperative fMRI and DTI confirmed right cerebral language dominance as well as a crossed cerebro-cerebellar functional link with the left cerebellar hemisphere. Discussion: Atypical right hemisphere language dominance in this right-handed patient is reflected by: (1) the total absence of language problems in the pre-, intra- and postoperative phase, (2) absence of positive stimulation sites during DES, (3) a clearly more pronounced arcuate fasciculus in the right cerebral hemisphere (DTI), (4) a crossed functional connection between the right cerebrum and the left cerebellum (fMRI). Two hypothetical explanations for the pattern of crossed cerebral language dominance are put forward: (1) preoperative brain plasticity mechanisms inducing a shift of language functions to the right hemisphere or (2) right hemisphere language dominance as a maturational variant. This case with atypical cerebral language dominance shows that although DES is the 'gold standard' to identify eloquent language regions and their pathways, fMRI and DTI are important adjuncts to guide surgery, to identify language lateralisation and to study anatomoclinical correlations. (C) 2013 Elsevier B.V. All rights reserved.

Objective The occurrence of Twiddler's syndrome in subjects with neurostimulator devices is poorly understood and might be influenced by age, sex, BMI, use of medication or psychologic disorders. Methods Two hundred thiry-five patients who received a neuromodulator were included in this retrospective study in a period between 2008 and 2015. The subjects were divided into a group of Twiddler's syndrome patients (TS) and a group of non-Twiddler's patients (NTS). Outcome measures were gender, age at implantation, type of neuromodulation, use of antipsychotics, antidepressants and opioids, the presence of other psychologic disorders and BMI. Results Both groups differ significantly in age (p = 0.024), weight (p = 0.001) and BMI (p = 0.001). No statistical difference was found in the type of neuromodulation (p = 0.537), gender (p = 0.368), the use of antipsychotics (p = 0.071), antidepressants (p = 0.097), and opioids (p = 1). Forward stepwise logistic regression of all variables showed that age of implementation (p = 0.029), the use of antipsychotics (p = 0.022) and BMI (p = 0.001) were statistically significant for predicting Twiddler's syndrome. Conclusion Twiddler's syndrome is an uncommon complication of neuromodulation implantable devices. Younger age, use of antipsychotics, and high BMI are risk factors that can be used to facilitate rapid diagnosis and treatment.

Background/Aims: We evaluated the cerebrospinal fluid/serum albumin ratio (AR) and kinetics of matrix metalloproteinase-9 (MMP-9) in blood as markers for blood-brain barrier (BBB) disruption after acute ischemic stroke. Methods: The AR was determined in 88 patients with acute ischemic stroke or transient ischemic attack. MMP-9 was measured on admission, 24, 72 h and 7 days after stroke onset. Results: The AR was related to stroke severity, the occurrence of stroke progression and the modified Rankin Scale score at month 3. MMP-9 levels on admission were significantly elevated compared to controls and dropped in the first 72 h after stroke, except in patients with stroke progression and larger infarcts in the subacute phase. Conclusions:We demonstrate that the extent of BBB breakdown in hyperacute stroke relates to initial stroke severity, stroke evolution and long-term outcome. The kinetics of MMP-9 confirm its pivotal role in secondary brain damage after ischemic stroke.

The posterior fossa syndrome (PFS) consists of transient cerebellar mutism, cognitive symptoms, and neurobehavioral abnormalities that typically develop in children following posterior fossa (PF) tumor resection. The pathophysiological substrate of the syndrome remains unclear. We investigated eight children of whom five presented with a variety of clinically relevant non-motor language symptoms associated with cognitive and behavioral disturbances after PF tumor resection. Four children developed transient cerebellar mutism followed by dysarthric speech. Non-motor language symptoms consisted of agrammatism, anomia, impaired verbal fluency, comprehension deficits, and aspontaneous speech. Neurocognitive deficits included executive dysfunctions, concentration deficits, and visuo-spatial disorders. In addition, all children presented with behavioral and affective disturbances. Functional neuroimaging studies during the phase of mutism by means of SPECT showed perfusional deficits in the anatomo-clinically suspected supratentorial areas subserving language dynamics, syntax, naming, executive functioning, affective regulation, and behavior. A significant improvement of frontal perfusional deficits paralleled the clinical remission of mutism. These results add to the view that the PFS might represent a cerebello-cerebral diaschisis phenomenon, reflecting the metabolic impact of the cerebellar lesion on supratentorial cognitive and affective functions.

Objective: Clinical, neuropsychological, structural and functional neuroimaging results are reported in a patient who developed a unique combination of symptoms after a bi-thalamic and right putaminal stroke. The symptoms consisted of dysexecutive disturbances associated with confabulating behavior and auto-activation deficits.\ud \ud Background: Basal ganglia and thalamic lesions may result in a variety of motor, sensory, neuropsychological and behavioral syndromes. However, the combination of a dysexecutive syndrome complicated at the behavioral level with an auto-activation and confabulatory syndrome has never been reported.\ud \ud Methods: Besides clinical and neuroradiological investigations, an extensive set of standardized neuropsychological tests was carried out. \ud \ud Results: In the post-acute phase of the stroke, a dysexecutive syndrome was found in association with confabulating behavior and auto-activation deficits. MRI showed focal destruction of both thalami and the right putamen. Quantified ECD SPECT revealed bilateral hypoperfusions in the basal ganglia and thalamus but no perfusion deficits were found at the cortical level.\ud \ud Conclusion: The combination of disrupted auto-activation, dysexecutive and confabulating syndrome in a single patient following isolated subcortical damage renders this case exceptional. Although these findings do not reveal a functional disruption of the striato-ventral pallidal-thalamic-frontomesial limbic circuitry, they add to the understanding of the functional role of the basal ganglia in cognitive and behavioral syndromes.

To identify neurochemical correlates of behavioral and psychological signs and symptoms of dementia (BPSD), we set up a prospective study. Patients with probable Alzheimer's disease (AD) ( n = 181), mixed dementia (MXD) ( n = 28), frontotemporal dementia (FTD) ( n = 25) and dementia with Lewy bodies (DLB) ( n = 24) were included. At inclusion, all patients underwent lumbar puncture, neuropsychological examination and behavioral assessment (battery of behavioral assessment scales). Cerebrospinal fluid (CSF) levels of norepinephrine and of (nor)epinephrine (MHPG), serotonin (5HIAA) and dopamine (DOPAC, HVA) metabolites were determined by HPLC and electrochemical detection. Spearman Rank-Order followed by Bonferroni correction was used for calculating correlations. In FTD patients, CSF norepinephrine levels were positively correlated with dementia severity ( r = 0.539; p = 0.021). CSF DOPAC levels were correlated with BPSD in general ( r = 0.537; p = 0.007), associated caregiver burden ( r = 0.567; p = 0.004) and agitated and aggressive behavior ( r = 0.568; p = 0.004). In a subgroup of FTD patients who did not receive psychotropic pharmacological treatment, a strong correlation between CSF HVA/5HIAA ratios (reflecting serotonergic modulation of dopaminergic neurotransmission) and aggressive behavior ( r = 0.758; p = 0.009) was found. In MXD patients, (verbally) agitated behavior was positively associated with the turnover of norepinephrine ( r = 0.633; p = 0.002). No significant correlations were found in AD and DLB groups. In FTD, increased activity of dopaminergic neurotransmission and altered serotonergic modulation of dopaminergic neurotransmission is associated with agitated and aggressive behavior respectively. This study demonstrated that neurochemical mechanisms underlying the pathophysiology of BPSD are both BPSD-specific and disease-specific which might have implications for future development of new and more selective pharmacological treatments of BPSD.

The posterior fossa syndrome (PFS) is a well-known clinical consequence of posterior fossa surgery that has only been reported in a limited number of cases with a nontumoral etiology. It consists of transient cerebellar mutism, behavioral abnormalities and personality changes. We describe a 12-year-old child who developed transient cerebellar mutism associated with behavioral and emotional symptoms following rupture of a vermis arteriovenous malformation (AVM). Following the stroke, the girl experienced a 24-hour symptom-free interval. After that, she became mute and her emotional state was characterized by severe anxiety, irritability and withdrawal. After 3 days, mutism resolved and dysarthria became apparent. Two weeks after stroke, the AVM was surgically removed and the postoperative course was uneventful. This case is the first reported in which the PFS occurred after focal nonsurgically induced cerebellar damage.

Dear Sir,We read the manuscript of Bilotta et al. (2014) entitled ‘Diagnostic work up for language testing in patients undergoing awake craniotomy for brain lesions in language areas’ with much int...

Intraoperative direct electrical stimulation (DES) is increasingly used in patients operated on for tumours in eloquent areas. Although a positive impact of DES on postoperative linguistic outcome is generally advocated, information about the neurolinguistic methods applied in awake surgery is scarce. We developed for the first time a standardised Dutch linguistic test battery (measuring phonology, semantics, syntax) to reliably identify the critical language zones in detail. A normative study was carried out in a control group of 250 native Dutch-speaking healthy adults. In addition, the clinical application of the Dutch Linguistic Intraoperative Protocol (DuLIP) was demonstrated by means of anatomo-functional models and five case studies. A set of DuLIP tests was selected for each patient depending on the tumour location and degree of linguistic impairment. DuLIP is a valid test battery for pre-, intraoperative and postoperative language testing and facilitates intraoperative mapping of eloquent language regions that are variably located.

To improve clinical, neuropsychological and behavioural characterisation of the cerebrospinal fluid (CSF) biomarkers beta-amyloid((1-42)) protein (Abeta42), protein tau (tau) and tau phosphorylated at threonine 181 (P-tau181) across diagnostic dementia categories, a prospective study was set up. Patients with probable Alzheimer's disease (AD) (n=201), AD with cerebrovascular disease (CVD) (AD+CVD) (n=33), frontotemporal dementia (FTD) (n=27), dementia with Lewy bodies (DLB) (n=22) and healthy controls (n=148) were included. All patients underwent neuropsychological examination and behavioural assessment by means of a battery of behavioural assessment scales. CSF was obtained by lumbar puncture and levels of Abeta42, tau and P-tau181 were determined with commercially available ELISA kits. Negative correlations between CSF Abeta42 levels and aggressiveness (Spearman: r=-0.223; p=0.002) and positive correlations with age at inclusion (r=0.195; p=0.006), age at onset (r=0.205; p=0.003) and MMSE scores (r=0.198; p=0.005) were found in AD. In AD+CVD, CSF Abeta42 levels were correlated with MMSE (r=0.482; p=0.006), Hierarchic Dementia Scale (r=0.503; p=0.017) and Boston Naming Test (r=0.516; p=0.012) scores. In controls, age was positively correlated with CSF tau (r=0.465; p

Bilateral vascular thalamic lesions are rare. Although a variety of neurobehavioral manifestations have been described, the literature is less documented with regard to accompanying linguistic disturbances. This article presents an in-depth neurolinguistic analysis of the language symptoms of a patient who incurred bilateral paramedian ischemic damage of the thalamus. In the post-acute phase of the stroke, a unique combination of transcortical sensory aphasia with syntactic impairment was found. Because of this atypical semiological association, additional analyses of spontaneous speech were performed. In spite of the typological affinity with the grammatic characteristic of marked simplification of syntax observed in Broca's aphasia, only a wordclass specific, lexical-semantic deficit for verbs was objectified. The hypothesis that lexical-semantic disturbances in our patient might result from a functional deafferentiation of both thalami with the frontal lobe is supported by: (1) associated neuropsychological deficits of frontal origin and (2) frontal-like behavioral disturbances.

Although insights in cerebellar neurocognition and affect are continuously growing, little is known about the role of the brainstem in cognitive and behavioural processing. In this paper, it is hypothesized that the brainstem is an inherent functional part of the cerebellocerebral network subserving cognition and affect, and that isolated brainstem damage may cause a constellation of symptoms closely resembling the cerebellar cognitive affective syndrome (CCAS) following cerebellar pathology. In order to investigate these premises, the available literature on cognitive and affective disturbances following brainstem lesions was critically reviewed starting from the pioneer descriptions in the 1950s till June 2012. Three personal cases were added to a study group of 75 cases with isolated vascular brainstem damage. In a cohort of 30 patients that allowed construction of anatomoclinical correlations in a reliable way, a range of cognitive and behavioural symptoms, typically associated with impairment of cortical or limbic areas, were identified. Executive dysfunction, attentional deficits and a decline in general intellectual capacity represent the most common cognitive findings, but memory, visuospatial skills, language and praxis may be impaired as well. Almost half of the cases presented with behavioural or affective changes. Analysis of SPECT findings indicates that functional suppression of frontal, parietal and to a lesser extent also the temporal areas are common phenomena after isolated brainstem stroke. As reflected by diaschisis affecting the cerebellocerebral network, a loss of excitatory input from the brainstem to the cerebellum and cerebrum may induce disruption of several cortical regions as well as emotional control centres resulting in and a constellation of symptoms closely resembling the CCAS. The pathophysiological mechanism underlying brainstem-induced cognitive and affective disturbances is discussed.

Objective: The aim of this paper is to report the psychiatric, neuroradiological and linguistic characteristics in a native speaker of Dutch who developed speech symptoms which strongly resemble Foreign Accent Syndrome.\ud \ud Background: Foreign Accent Syndrome is a rare speech production disorder in which the speech of a patient is perceived as foreign by speakers of the same speech community. This syndrome is generally related to focal brain damage. Only in few reported cases the Foreign Accent Syndrome is assumed to be of psychogenic and/or psychotic origin.\ud \ud Method: In addition to clinical and neuroradiological examinations, an extensive test battery of standardized neuropsychological and neurolinguistic investigations was carried out. Two samples of the patient's spontaneous speech were analysed and compared to a 500,000-words reference corpus of 160 normal native speakers of Dutch.\ud \ud Results: The patient had a prominent French accent in her pronunciation of Dutch. This accent had persisted over the past eight years and has become progressively stronger. The foreign qualities of her speech did not only relate to pronunciation, but also to the lexicon, syntax and pragmatics. Structural as well as functional neuroimaging did not reveal evidence that could account for the behavioural symptoms. By contrast psychological investigations indicated conversion disorder.\ud \ud Conclusions: To the best of our knowledge this is the first reported case of a foreign accent like syndrome in conversion disorder.

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.

The clinical study of crossed aphasia in dextrals (CAD) may shed light on the discreteness and modularity of several cognitive functions, such as language, gestures and visual spatial abilities, with respect to hemispheric lateralisation. Since 1975 over 180 cases have been described, employing, however, different criteria of assessment and classification. The purpose of this paper is to review them and to propose a set of diagnostic criteria that may be useful to single out a series of reliable CAD cases on which research can be safely carried out. A detailed analysis of such series is dealt with in terms of a number of characteristics concerning both the language disorder and the associated nonverbal cognitive impairments.

BACKGROUND AND AIM: Although upper limb movements in the vertical plane are very commonly used during the activities of daily life, there is still a lack of a reliable and easy standardized procedure to quantify them. In particular, ageing is associated with a decline in performances of coordinated movements, but a tool to quantify this decline is missing. METHODS: We created a novel portable test called counting arm movement test (CAM test). Participants were asked to perform fast and accurate successive pointing movements towards two fixed targets (mechanical counters) located in a vertical plane in the parasagittal axis during three different time periods (15, 30, 45 s). Each upper limb was assessed separately. The test was evaluated in a group of 63 healthy subjects (mean age ± SD 49.1 ± 19.8 years; F/M 33/30; range 18-87 years). RESULTS: Motor performances (number of clicks) significantly decreased as a function of age for both the dominant side (age effect; linear regression; p < 0.0001 for 15, 30 and45 s) and the non-dominant side (linear regression; p < 0.0001 for 15, 30 and 45 s). Performances on the dominant and non-dominant side were linearly correlated with the time periods (p < 0.0001 on both sides). The symmetry index (ratio of performance on the dominant side divided by performance on the non-dominant side) was correlated linearly and positively with the duration of the test (y = 0.002x + 1.053; p = 0.0056). We also found a linear relationship between upper limb length and motor performance on the non-dominant side for 15 s (p = 0.023) and 45 s (p = 0.041). The test was characterized by a very high correlation between the results obtained by two investigators during two successive sessions in a subgroup of 7 subjects (Pearson product moment correlation: 0.989 for the dominant side and 0.988 for the non-dominant side). CONCLUSION: The CAM test appears as a robust and low cost tool to quantify upper limb pointing movements. In particular, the test strongly discriminates the effects of age upon motor performances in upper limbs. Future studies are now required to establish the sensitivity, specificity and reliability of this procedure in selected neuromuscular or skeletal diseases affecting the elderly.

Empathic interaction involves the capacity to recognize emotions experienced by another human being. Impairment may underlie many of the emotional deficits commonly associated with a range of neuropsychiatric and neurological conditions. The prefrontal cortex (PFC) has long been implicated in these processes, but the specific contribution of subregions of the PFC remain unclear. Studies regarding the role of subregions of the prefrontal cortex - such as the ventromedial prefrontal cortex (vmPFC) - in facial emotion recognition have yielded inconsistent results. The present study aims to investigate the capacity to recognize nonverbal emotional facial expressions in a group of patients with: 1) perfusion deficits in the vmPFC (vmPFC group; N = 13), 2) hypoperfusions sparing the vmPFC (non-vmPFC group; N = 12), and in 3) a control group of healthy volunteers (control group; N = 17). Regions of hypoperfusion were identified by means of Single Photon Emission Computed Tomography (SPECT). Participants were asked to recognize facial expressions of the seven basic emotions (happiness, fear, surprise, anger, disgust, sadness or neutral). Detection of facial expressions of fear, disgust and surprise was affected after functional disruption of the vmPFC. The present study confirms the role of the vmPFC in recognizing emotional facial expressions.

Objective: Poststroke depression (PSD) is commonly observed in stroke patients and has a negative impact on functional outcome and quality of life. Therefore, a prospective, longitudinal epidemiological study was conducted aiming to determine prevalence and risk factors for PSD at 1, 3, 6, 12 and 18 months poststroke.Methods: A total of 222 patients were included in the study and 201 patients entered data analysis. Demographic data, vascular risk factors, stroke characteristics, functional and neurocognitive outcome measures and psychosocial factors were considered as potential risk factors for PSD. Clinically significant signs and symptoms of PSD were quantified by means of the Cornell Scale for Depression (CSD) and the Montgomery and angstrom sberg Depression Rating Scale (MADRS).Results: PSD was present at 1, 3, 6, 12 and 18 months poststroke in 24.5%, 27.1%, 28.3%, 19.8% and 26.3% of the patients respectively. Univariate regression analyses revealed that PSD was significantly associated with stroke severity, physical disability, cognitive impairment and stroke outcome during the 18 months time frame of the study. Reduced social activities and the presence of apraxia were consistently associated with PSD whereas aphasia was only significantly associated in the first 6 months after stroke. Patients with relational problems had a 3 times greater risk of becoming depressed at 18 months poststroke than patients without relational problems (OR = 3.09; 95% CI = 131-726).Conclusions: Risk factors for PSD seem variable indicating the need for clinicians to consider the dynamic and multifactorial nature of PSD emphasizing the importance of a rigorous and long-term monitoring and support of stroke patients and their caregivers. (C) 2014 Elsevier B.V. All rights reserved.

In contrast to the estimated low incidence of crossed aphasia in dextral adults (among 1%), crossed aphasia in children has been considered a common finding for almost a century. However, reviewing the literature on crossed aphasia in dextrals (CAD) and its related topics from 1975 onward, we encountered only 5 children in a corpus of 180 cases (2.7%). Critical analysis rendered three of the reported cases ambiguous and hence not suitable to draw potentially relevant conclusions. In this review, the neurobehavioral manifestations of the two representative childhood CAD cases are analyzed and compared with adult CAD and acquired childhood aphasia (ACA). In the light of our findings, which support the position of innate cerebral specialization for language, the long-standing controversy as to whether lateralized hemispheric specialization for language is innate or develops progressively during maturation is briefly discussed.

INTRODUCTION: Although spinal cord stimulation (SCS) is a widely used treatment for chronic neuropathic pain secondary to spinal surgery, little is known about the underlying physiological mechanisms. METHODS: The primary aim of this study is to investigate the neural substrate underlying short-term SCS by means of (1)H MR spectroscopy with short echo time, in 20 patients with failed back surgery syndrome. RESULTS: Marked increase of γ-aminobutyric acid (GABA) and decrease in glucose in the ipsilateral thalamus were found between baseline situation without SCS and after 9' of SCS, indicating the key role of the ipsilateral thalamus as a mediator of chronic neuropathic pain. In addition, this study also showed a progressive decrease in glucose in the ipsilateral thalamus over time, which is in line with the findings of previous studies reporting deactivation in the ipsilateral thalamic region. CONCLUSIONS: The observation of GABA increase and glucose decrease over time in the ipsilateral thalamus may be thecausal mechanism of the pain relief due to SCS or an epiphenomenon.

The aim of the current study is to investigate structural changes on brain MRI using voxel based morphometry (VBM) related to an eight-week mindfulness based intervention (MBI) in Parkinson's Disease (PD).A total of 27 out of 30 PD patients completed a randomized controlled longitudinal trial. Fourteen patients participated in a structured eight-week program of MBI. Thirteen patients received usual care (UC) alone. MRI data sets of the brain were obtained at baseline and after eight weeks follow-up. VBM analysis was performed using DARTEL from the SPM8 software. The resulting difference maps were statistically compared to examine gray matter density (GMD) differences. Results were reported at p0.001, uncorrected for multiple comparisons.Increased GMD was found in the MBI compared to the UC group in the region of interest (ROI) analysis in the right amygdala, and bilaterally in the hippocampus. Whole brain analysis showed increased GMD in the left and right caudate nucleus, the left occipital lobe at the lingual gyrus and cuneus, the left thalamus, and bilaterally in the temporo-parietal junction. In contrast, GMD differences were found in the UC group in the left anterior lobe and dentate nucleus of the cerebellum.To the best of our knowledge this is the first quantitative analysis of neurobiological effects of MBI in PD. Increased GMD was found in the MBI group in the neural networks that have been postulated to play an important role in PD. These areas have also been implicated in the functional networks mediating the benefits of meditation.

The main aim of this experiment was to investigate the perception of Foreign Accent Syndrome in comparison to speakers with an authentic foreign accent.Three groups of listeners attributed accents to conversational speech samples of 5 FAS speakers which were embedded amongst those of 5 speakers with a real foreign accent and 5 native speaker controls. The listening panels differed in their familiarity with foreign accented speech and speech pathology.The findings indicate that listeners' perceptual responses to the three groups of speakers were essentially different at all levels of analysis. The native speaker controls were unequivocally recognized as native speakers of Dutch while the speakers with a real foreign accent were very reliably assessed as non-native speakers. The speakers with Foreign Accent Syndrome, however, were in some sense perceived as foreign and in some sense as native by listeners, but not as foreign as speakers with a real foreign accent nor as native as real native speakers. These results are accounted for in terms of a misinterpretation of markers of speech pathology as markers regional affiliation.The findings of the experiment are consistent with the idea that the very nature of the foreign accent is different in both groups of speakers, although it cannot be fully excluded that the foreign impression in the two groups is one of degree.Readers are able to: (1) define Foreign Accent Syndrome as a motor speech disorder and identify the different subtypes of FAS, (2) describe the most important differences in listeners' perceptual reactions to FAS and real foreign accents, and (3) discuss the findings of the present study in relation to other studies investigating accent attribution in FAS.

Mild cognitive impairment (MCI) is a clinical concept that categorizes subjects who are in an intermediate cognitive state between normal aging and dementia. The aims of this study are to determine the prevalence of significant depressive symptoms in MCI and Alzheimer's disease (AD) patients and to characterize the behavior associated with significant depressive symptoms in MCI and AD patients.A cross-sectional analysis of baseline data from a prospective, longitudinal study on behavioral symptoms of dementia and MCI was performed. The study population consisted of 270 MCI and 402 AD patients. Behavioral assessment was performed by means of Middelheim Frontality Score, Behavioral Pathology in Alzheimer's Disease Rating Scale (Behave-AD) and Cohen-Mansfield Agitation Inventory. The presence of significant depressive symptoms was defined as a Cornell Scale for Depression in Dementia total score7.The prevalence of significant depressive symptoms in AD patients (25%) was higher compared with MCI patients (16%) (p = 0.005). Patients with significant depressive symptoms showed an increased severity of frontal lobe symptoms, behavioral symptoms and agitation (Middelheim Frontality Score, Behave-AD and Cohen-Mansfield Agitation Inventory total scores; p 0.001). Also, most of the individual frontal lobe and behavioral symptoms were more prevalent and severe, resulting in higher Behave-AD global scores. Mild cognitive impairment patients with depressive symptoms showed more severe behavioral symptoms and more severe verbally agitated behavior than AD patients without depressive symptoms (p 0.001).Frontal lobe and behavioral symptoms are more prevalent and severe in MCI and AD patients with significant depressive symptoms as compared with patients without depressive symptoms.

This paper for the first time reports detailed neurolinguistic findings in a patient with Neuro-Sweet syndrome. In this patient the presenting symptoms of central nervous system (CNS) involvement primarily consisted of a selective grammar deficit restricted to spontaneous speech. On MRI a left prefrontal ischemic stroke (superior part BA 6) and two small subcortical left parietal infarctions were found. Neurolinguistic analyses, however, did not reveal a profile consistent with any observations of agrammatism caused by structural damage to the language areas critically involved in grammatical processing. It is hypothesized that selectively distorted grammar might reflect disruption of the frontosubcortical network involved in language processing. Prefrontal neurobehavioral abnormalities associated with functional disruption of the inferior medial frontal regions as demonstrated by SPECT, additionally suggest that agrammatic symptoms may be linked to a higher-level cognitive disorder following encephalopathic CNS involvement.

Mutism and Subsequent Dysarthria (MSD) and the Posterior Fossa Syndrome (PFS) have become well-recognized clinical entities which may develop after resection of cerebellar tumours. However, speech characteristics following a period of mutism have not been documented in much detail. This study carried out a perceptual speech analysis in 24 children and adolescents (of whom 12 became mute in the immediate postoperative phase) 1-12.2 years after cerebellar tumour resection. The most prominent speech deficits in this study were distorted vowels, slow rate, voice tremor, and monopitch. Factors influencing long-term speech disturbances are presence or absence of postoperative PFS, the localisation of the surgical lesion and the type of adjuvant treatment. Long-term speech deficits may be present up to 12 years post-surgery. The speech deficits found in children and adolescents with cerebellar lesions following cerebellar tumour surgery do not necessarily resemble adult speech characteristics of ataxic dysarthria. (c) 2012 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Mild cognitive impairment (MCI) is a clinical concept that categorizes subjects who are in an intermediate cognitive state between normal aging and dementia. The aim of this study is to characterize behavior in MCI compared with Alzheimer's disease (AD) and healthy older patients.A cross-sectional analysis of baseline data from a prospective, longitudinal study on behavioral symptoms of dementia and MCI was performed. The study population consisted of 270 MCI, 402 AD patients, and 108 healthy controls. Behavioral assessment was performed by means of Middelheim Frontality Score, Behavioral Pathology in Alzheimer's Disease Rating Scale, Cohen-Mansfield Agitation Inventory, and Cornell Scale for Depression in Dementia.Moderate-to-severe behavioral symptoms were present in 13% of MCI patients, as compared with 39% in AD patients and 3% in controls (p 0.001). The general severity of behavioral symptoms was intermediate between controls and AD patients. The three most frequent symptoms in MCI patients were aggressiveness (49%), affective disturbance (45%), and anxiety (38%); in AD patients, the most frequent symptoms were aggressiveness (60%), activity disturbances (54%), and psychosis (40%). The prevalence and severity of frontal lobe symptoms, aggressiveness, activity disturbances, and delusions was intermediate between normal aging and AD. In addition, the severity of physically non-aggressive and verbally agitated behavior and the severity of depressive symptoms were also intermediate.The behavioral profile of MCI patients is characterized as an intermediate state between normal aging and AD for the prevalence and severity of certain behavioral symptoms. Follow-up is ongoing to test the hypothesis that behavioral disturbances in MCI predict progression to dementia.

Apraxic agraphia is a writing disorder due to a loss or lack of access to motor engrams that program the movements necessary to produce letters. Clinical and functional neuroimaging studies have demonstrated that the neural network responsible for writing includes the superior parietal region and the dorsolateral and medial premotor cortex. Recent studies of two cases with atypical lesion localisations in the left thalamus and the right cerebellum support the hypothesis that the written language network is larger than previously assumed. The aim of this study is twofold: (1) to provide a survey of cases of apraxic agraphia published between 1973 and June 2010, and (2) to provide further evidence for a role of the cerebellum in writing via three additional cases who presented with apraxic agraphia after ischemic damage in the cerebellum. Functional neuroimaging studies by means of brain perfusion SPECT showed perfusional deficits in the anatomoclinically suspected supratentorial areas, subserving language dynamics, syntax, naming, writing and executive functioning.

Comment, Letter, SCOPUS: le.j, info:eu-repo/semantics/published

Although the posterior fossa syndrome (PFS) can be considered as an aetiologically heterogeneous condition affecting children and adults, it most often occurs in paediatric patients after cerebellar tumour surgery. In patients with a tumoural aetiology, the syndrome is typically characterised by a short symptom-free postoperative period followed by mutism of variable duration and behavioural and affective changes. More than 200 paediatric cases have been described but reports of adult patients are extremely rare. This paper discusses PFS in adults on the basis of a comprehensive literature survey and describes the pre- and postoperative findings in a new adult patient. In the preoperative phase, cognitive, behavioural and affective abnormalities were identified, matching a diagnosis of cerebellar cognitive affective syndrome (CCAS) (Schmahmann and Sherman, 1998; Schmahmann, 2004). The immediate postoperative course was characterised by prefrontal-like behavioural and affective abnormalities, peduncular hallucinations and confusion evolving to psychosis. Akinetic mutism subsequently developed, lasted for 12 days and then alternated with episodes of diminished responsiveness in which pathological laughing and crying (PLC) occurred. Akinetic mutism resolved after treatment with a non-ergoline dopamine-agonist but CCAS persisted during longitudinal follow-up. From a semiological point of view "relapsing-remitting akinetic mutism" and PLC in our patient might add relevant information to current insights in the clinical expression of the PFS. As evidenced by a close parallelism between single photon emission computed tomography (SPECT) and clinical findings, CCAS as well as PFS seem to reflect functional disruption of the cerebello-cerebral network involved in cognitive, behavioural and affective functions. These findings may indicate that both syndromes share overt semiological resemblances and a common pathophysiological substrate. Consequently, CCAS and PFS may both be regarded as cerebellar-induced clinical conditions showing different aspects of a spectrum that range in degree of severity and symptom duration.

Background: Ischemic injury to the central nervous system causes cellular activation and disintegration, leading to release of cell-type–specific proteins into the cerebrospinal fluid (CSF). We investigated CSF concentrations of myelin basic protein (MBP), glial fibrillary astrocytic protein (GFAP), the calcium-binding protein S100B, and neuron-specific enolase (NSE) in acute ischemic stroke patients and their relation to initial stroke severity, stroke location, and long-term stroke outcome. Methods: CSF concentrations of MBP, GFAP, S100B, and NSE were assessed in 89 stroke patients on admission (mean 8.7 h after stroke onset) and in 35 controls. We evaluated the relation between CSF concentrations and (a) stroke severity (NIH Stroke Scale [NIHSS] score on admission, infarct volume), (b) stroke location, and (c) stroke outcome (modified Rankin Scale [mRS] score at month 3). Results: MBP concentration was significantly higher in subcortical than in cortical infarcts (median MBP, 1.18 vs 0.66 μg/L, P < 0.001). GFAP and S100B concentrations correlated with the NIHSS score on admission (GFAP, R = 0.35, P = 0.001; S100B, R = 0.29, P = 0.006), infarct volume (GFAP, R = 0.34, P = 0.001; S100B, R = 0.28, P = 0.008), and mRS score at month 3 (R = 0.42, P < 0.001 and R = 0.28, P = 0.007). Concentrations of NSE did not correlate with stroke characteristics. Conclusions: MBP, GFAP, S100B, and NSE display relevant differences in cellular and subcellular origins, which are reflected in their relation to stroke characteristics. MBP is a marker for infarct location. GFAP and S100B correlate with stroke severity and outcome.

The traditional view on cerebellar functioning has recently been challenged by results fromneuroanatomical, neuroimaging and clinical studies. In this contribution, eighteen patients with primary cerebellar lesions (vascular: n ¼ 13; neoplastic: n ¼ 5) were systematically investigated by means of an extensive neuropsychological test battery. Fifteen patients (83%) presented with a broad variety of cognitive and linguistic deficits following cerebellar damage. Disturbances of attention (72%), executive functioning (50%) and memory (50%) were most commonly found. Analyses of our results tend to support the hypothesis of a lateralization of cognitive modulation within the cerebellum, the right cerebellar hemisphere being associated with logical reasoning and language processing and the left cerebellum mediating right-hemispheric functions including attentional and visuo-spatial skills. In addition, nine patients (50%) presented with frontal-like behavioural and affective alterations. In an attempt to determine the working-mechanism underlying cerebellarinduced cognitive and affective disturbances, all patients were investigated by means of quantified Tc-99m-ethylenecysteine dimer (ECD) single photon emission computerized tomography (SPECT) studies. From a semiological point of view, damage to the cerebellum can cause a broad spectrum of clinically significant cognitive and affective disturbances. From a pathophysiological point of view, quantified SPECT data, reflecting the phenomenon of cerebello-cerebral diaschisis, support the functional impact of the cerebellar lesion on cortical functioning through disruption of cerebello-cerebral connections.

Developmental dyslexia is the most common learning disability in school-aged children with an estimated incidence of five to ten percent. The cause and pathophysiological substrate of this developmental disorder is unclear. Recently, a possible involvement of the cerebellum in the pathogenesis of dyslexia has been postulated. In this study, 15 dyslexic children and 7 age-matched control subjects were investigated by means of functional neuroimaging (fMRI) using a noun-verb association paradigm. Comparison of activation patterns between dyslexic and control subjects revealed distinct and significant differences in cerebral and cerebellar activation. Control subjects showed bilaterally well-defined and focal activation patterns in the frontal and parietal lobes and the posterior regions of the cerebellar hemispheres. The dyslexic children, however, presented widespread and diffuse activations on the cerebral and cerebellar level. Cerebral activations were found in frontal, parietal, temporal and occipital regions. Activations in the cerebellum were found predominantly in the cerebellar cortex, including Crus I, Crus II, hemispheric lobule VI, VII and vermal lobules I, II, III, IV and VII. This preliminary study is the first to reveal a significant difference in cerebellar functioning between dyslexic children and controls during a semantic association task. As a result, we propose a new hypothesis regarding the pathophysiological mechanisms of developmental dyslexia. Given the sites of activation in the cerebellum in the dyslexic group, a defect of the intra-cerebellar distribution of activity is suspected, suggesting a disorder of the processing or transfer of information within the cerebellar cortex.

Background Accumulation of lactate in ischemic regions has been documented in acute stroke. We evaluated the relation between lactate levels in blood and cerebrospinal fluid (CSF) and ischemic stroke evolution and outcome. Methods Lactate was measured in blood of 187 acute ischemic stroke and TIA patients at admission, 24 h, 72 h and 7 days after stroke onset. In a subpopulation of 85 stroke patients and in 51 controls, lactate was measured in CSF. Stroke evolution was evaluated by change in the NIHSS score within the first 72 h and by occurrence of progressing stroke. At 3 months after stroke, outcome was assessed on the basis of mortality rate and the modified Rankin Scale. Results We found no relation between lactate levels in blood and stroke evolution or outcome. Lactate in CSF was higher in stroke patients than in controls and correlated with stroke evolution and outcome. Multivariate regression analyses showed that CSF lactate levels, age and stroke severity are independent predictors for stroke evolution and outcome. Conclusions Lactate levels in CSF, but not in blood, are a reliable marker for metabolic crisis in acute ischemic stroke and correlate with the stroke evolution in the subacute phase and with long-term outcome.

Introduction Foreign Accent Syndrome (FAS) is a relatively rare motor speech disorder in which the pronunciation of a patient is perceived by listeners of the same language community as distinctly foreign. FAS has been well documented in adult patients with etiologically heterogeneous, though mostly vascular brain lesions affecting the motor speech network of the language dominant hemisphere. In addition, reports exist of adult patients in whom FAS was due to a psychiatric illness. Although FAS has been reported in children, such accounts are rare and have remained largely anecdotal in that there have been no formally documented cases of FAS as a developmental motor speech disorder. Methods and results For the first time, we describe the clinical, cognitive and neurolinguistic findings in two patients who in the absence of a history of psychiatric illness or acquired brain damage already presented with FAS at an early stage of speech and language development. In the first patient “developmental FAS” was associated with a dysharmonic distribution of neurocognitive test results indicating slight underdevelopment of visuo-spatial skills and visual memory. The second patient presented with “developmental FAS” associated with specific language impairment (SLI). Independent support for a diagnosis of FAS in both patients was obtained in an accent attribution experiment in which groups of native speakers of (Belgian) Dutch assessed the type of foreign accent of a sample of the patients’ conversational speech. Both patients were judged as non-native speakers of Dutch by the majority of participants who predominantly identified the accent as French. Conclusion This paper for the first time documents two patients who presented with FAS on a developmental basis. The finding that FAS does not only occur in the context of acquired brain damage or psychogenic illness but also exists as developmental motor speech impairment requires a re-definition of FAS as a clinical syndrome.

Transient cerebellar mutism is a well-known clinical entity which may develop after surgery to the cerebellum. As the period of mutism is followed by motor speech deficits, the condition has also been termed the syndrome of (cerebellar) Mutism and Subsequent Dysarthria (MSD). In children, its incidence is estimated between 8% and 31%. Unfortunately, the literature provides contradictory information regarding motor speech production post-mutism. We therefore critically reviewed data on 283 childhood cases to chart the mode of recovery of motor speech production after the mute period. After applying stringent exclusion criteria, we found that 98.8% of the children displayed motor speech deficits. This percentage is much higher than commonly reported in the literature. In addition, recovery of speech appeared to be less favourable than previously ascertained. Future studies should investigate more carefully the patients' speech characteristics in order to be able to offer children an adequate and complete rehabilitation program.

Background. In order to characterize frontal lobe features and their behavioural and cognitive correlates across diagnostic categories, we performed a cross-sectional analysis of behavioural and neuropsychological data from a large, prospective Belgian study on behavioural and psychological signs and symptoms of dementia (BPSD).Method. Patients with probable Alzheimer's disease (AD) (n=170), frontotemporal dementia (FTD) (n=28), mixed dementia (MXD) (n=29) and dementia with Lewy bodies (DLB) (n=21) were included and underwent neuropsychological and behavioural assessment by means of a battery of tests and scales. Frontal lobe symptoms were quantified by means of the Middelheim Frontality Score (MFS).Results. In AD (and to a lesser extent in MXD), MFS total scores were negatively correlated with scores on MMSE (Spearman: r=−0·36, pr=−0·38, pr=0·24, pr=0·44, pr=0·43, pr=−0·50, p=0·020). No associations were found in FTD patients.Conclusions. A cross-sectional analysis of frontal lobe features, behavioural characteristics and neuropsychological data demonstrated that, in AD (and to a lesser extent in MXD) patients, frontal lobe symptoms were associated with more pronounced cognitive deficits (of frontal origin), with increased severity and frequency of agitated and aggressive behaviour, and with increased severity of psychosis and depressive symptoms. Given the small sample sizes of the DLB and FTD patient groups, negative findings in these patient groups should be interpreted cautiously.

To determine whether apolipoprotein alleles (APOE) influence behavioral and psychological signs and symptoms of dementia (BPSD), we initiated a prospective, longitudinal study. Patients with Alzheimer's disease (AD) (N = 186), frontotemporal dementia (FTD) (N=29), mixed dementia (MXD) (N= 28), dementia with Lewy bodies (DLB) (N= 11) and Parkinson's disease dementia (PDD) (N = 7) were included. Blood was collected for DNA extraction and APOE genotyping. Behavioral assessments were performed at baseline and semi-annually thereafter, using behavioral assessment scales (Middelheim frontality score, behavioral pathology in Alzheimer's disease rating scale (Behave-AD)). In FTD patients, we identified close dependent effects of APOE epsilon 4 on the Behave-AD total and cluster aggressiveness scores. APOE epsilon 2 was associated with a higher score on the Behave-AD cluster delusions in PDD/DLB patients. No APOE effects on frequency or severity of BPSD in AD and MXD Patients Were found. In Conclusion, APOE has disease-specific effects on BPSD in FTD and PDD/DLB patients, given the reported associations of APOE epsilon 4 with aggression (FTD) and of APOE epsilon 2 with delusions (PDD/DLB). (C) 2005 Elsevier Inc. All rights reserved.

Although previous studies of Gillespie syndrome have systematically reported a generalized delay of cognitive development (mental retardation or oligophrenia), psychometric data to substantiate this view are strikingly absent. In the present study two first degree relatives (mother and daughter) with Gillespie syndrome were neuropsychologically investigated. Aside from a marked asymmetry in the Wechsler-IQ profile, consisting of significantly better results on the verbal [Verbal IQ (VIQ)] than on the nonverbal part [Performance IQ (PIQ)] of the test, cognitive and behavioral assessments revealed a pattern of abnormalities that closely resembles the ''cerebellar cognitive and affective syndrome'' (CeCAS) (Schmahmann and Sherman, 1998). Aside from prefrontal dysexecutive dysfunctions such as disturbed cognitive planning and set-shifting, parietal lobe involvement was reflected by impaired visuo-spatial memory and visuo-spatial disorganization in constructional tasks. Within the linguistic domain involvement of the prefrontal and temporal language regions was indicated by impaired letter fluency, incidences of agrammatism, apraxia of speech and disrupted language dynamics. With regard to mood and behavior, a number of personality and affective characteristics were found that are typically associated with prefrontal lobe damage and dysfunction of limbic related regions in the cingulate and parahippocampal gyri. Disinhibited symptoms characterized behavior and affect of the mother while the daughter displayed a variety of inhibited symptoms. As a result, behavioral and cognitive findings in these patients do not support the prevailing view of a global mental retardation as a cardinal feature of Gillespie syndrome but primarily reflect cerebellar induced neurobehavioral dysfunctions following disruption of the cerebrocerebellar anatomical circuitry.

Objective Given the rather limited knowledge on profiles of neuropsychiatric symptoms (behavioural and psychological signs and symptoms of dementia, BPSD) in several degenerative dementias, we designed a prospective study of which we here present the baseline data. Methods Diagnosed according to strictly applied clinical diagnostic criteria, patients with probable Alzheimer's disease (AD) (n = 205), frontotemporal dementia (FTD) (n = 29), mixed dementia (MXD) (n = 39) and dementia with Lewy bodies (DLB) (n = 23) were included. All patients underwent a neuropsychological examination and behavioural assessment by means of a battery of scales (Middelheim Frontality Score (MFS), Behave-AD, Cohen-Mansfield Agitation Inventory, Cornell Scale for Depression in Dementia). Results In AD and MXD, activity disturbances and aggressiveness occurred in more than 80% of the patients. With a prevalence of 70%, apathy was very common whereas delusions and hallucinations were rare in FTD patients. Frequently used behavioural assessment scales like the Behave-AD systematically underestimated BPSD in FTD whereas the MFS displayed high sensitivity for frontal lobe symptoms. Hallucinations discriminated DLB patients from other dementias. A high prevalence of disinhibition (65%) in DLB pointed to frontal lobe involvement. Conclusions Behavioural assessment may help differentiating between different forms of dementia, further stressing the need for the development of new and more sensitive behavioural assessment scales. By means of the MFS, frontal lobe involvement was frequently observed in DLB. As 70% of FTD patients displayed apathy, prevalence was about two times higher compared to the other disease groups, meanwhile indicating that apathy is frequently observed in dementia, irrespective of its etiology. Copyright © 2005 John Wiley & Sons, Ltd.