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AbstractBackground:The cerebellum was traditionally considered to be exclusively involved in the coordination of voluntary movement, gait, posture, balance and motor speech. However, this view was challenged by recent neuroanatomical, neuroimaging and clinical findings, providing preliminary evidence of a cerebellar contribution to linguistic functioning. Aim:To discuss the role of the cerebellum in a variety of linguistic functions and to explore the underlying mechanisms. Methods:A literature search was conducted via electronic databases. Exclusion criteria were: disorders following congenital cerebellar lesions, motor speech disorders, cognitive deficits outside the language sphere, neuropsychiatric disorders and insufficient information on the cerebellar role in language. Abstracts were not included. In addition, only adult subjects were taken into consideration. Results:A variety of linguistic disorders were found to occur following acquired cerebellar lesions: (1) impaired phonological and semantic fluency; (2) agrammatism (at morphological and sentence level); (3) naming and word finding difficulties; (4) cerebellar-induced aphasia; (5) reading difficulties; (6) writing problems, and (7) higher-level language deficits, including disturbed listening comprehension, impaired language proficiency and metalinguistic ability. Several hypotheses have been suggested to explain the nature of the cerebellar contribution to language. However, findings are not univocal. Conclusion:The cerebellum appears to be involved in a variety of linguistic functions. However, the precise nature of this contribution is not clear yet. Linguistic, neuroimaging, neuroanatomical and neuropsychological studies should be combined in order to disentangle the specific contribution of the cerebellum to linguistic processing.Copyright © 2007 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

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, p<0·001) and a Verbal Fluency Task (r=−0·38, p<0·001) and were associated with increased severity and frequency of psychosis (r=0·24, p<0·01), activity disturbances (r=0·44, p<0·001) and aggressiveness (r=0·43, p<0·001). In DLB, MFS total scores were negatively correlated with MMSE scores (r=−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. [ABSTRACT FROM AUTHOR]

Background. Specific guanidino compounds have been described as uraemic toxins and their concentrations are increased in renal failure due to dimished glomerular filtration, whereas the guanidino compound creatine is used as a performance-enhancing substance in athletes. The present study investigates the effects of creatine supplementation on plasma guanidino compounds in a chronic haemodialysis population. Methods. Twenty male haemodialysis patients were included in a placebo-controlled cross-over trial. Patients were treated with creatine (2 g/day) or placebo during two treatment periods of 4 weeks, separated by a washout of 4 weeks. Plasma guanidino compounds and routine biochemical parameters were determined, as well as the prognostic inflammatory and nutritional index (PINI). Results. Upon creatine supplementation, guanidinoacetate concentrations decreased by 15%, due to inhibition of creatine synthesis. Concentrations of α-keto-δ-guanidinovaleric acid increased three-fold and argininic acid concentrations doubled. Guanidinosuccinate concentrations did not change, but correlated inversely with CRP (r = −0.736; P = 0.001), PINI-score (r = −0.716; P = 0.002) and correlated positively with plasma urea concentration (r = 0.54; P = 0.02). Conclusions. Creatine supplementation in haemodialysis patients significantly altered the concentration of specific guanidino compounds. Guanidinosuccinate correlated positively with plasma urea and negatively with inflammation markers. [ABSTRACT FROM AUTHOR]

Dementia with Lewy bodies (DLB) represents the second most frequent type of neurodegenerative dementia in the elderly. Although most patients have sporadic DLB, a limited number of DLB families have been described, suggesting that genetic factors may contribute to DLB pathogenesis. Here, we describe a three-generation Belgian family with prominent dementia and parkinsonism, consistent with a diagnosis of DLB, that was autopsy confirmed for the index patient. In a genome-wide scan and subsequent finemapping of candidate loci we obtained significant linkage to 2q35-q36 (Z = 3.01 at D2S1242). Segregation analysis defined a candidate region of 9.2 Mb between D2S433 and chr2q36.3-8, adjacent to the previously reported PARK11 locus. In addition, haplotype sharing studies in another DLB family of close geographical origin with similar clinical and neuropathological features highlighted the specificity of a 2q35-q36 haplotype harbouring a pathogenic mutation that causes DLB in the Belgian family. So far, extensive sequence analysis of five candidate genes within the 2q35-q36 region has not revealed a disease-causing mutation. Together, our data re-emphasize the genetic heterogeneity of DLB, and strongly support the existence of a gene for familial DLB on 2q35-q36. Once identified this will be the first novel causal gene for DLB and can be expected to open new avenues for biological studies of the disease process. [ABSTRACT FROM AUTHOR]