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Introduction: Eosinophilic meningitis is a rare form of meningitis with sequelae or death occurring in approximately 2-3% of cases. The most frequent etiological agent is the parasite Angiostrongylus cantonensis. The aim of this study was to characterize New Caledonian cases and to assess the extent to which of A. cantonensis was involved., Material and Methods: We performed a retrospective study of all cases of eosinophilic meningitis (EM) admitted to the Territorial Hospital of New Caledonia, from 2004 to 2019. We performed a descriptive and a multivariate analysis to identify association of variables with severe and fatal cases (or cases with sequelae)., Conclusion: Angiostrongyliasis was confirmed as being responsible for 17 of the 92 reported EM cases in New Caledonia from 2004 to 2019 with most being young adults and non-walking infants, and with two peaks of incidence one during the dry season and one during the rainy season. Considering the high incidence and regularity of cases, the potential reservoirs should be identified to target prevention campaigns., Competing Interests: The authors have declared that no competing interests exist.

Zika virus (ZIKV) infection has been associated with neurologic disorders including Guillain-Barré syndrome (GBS). In New Caledonia during the ZIKV outbreak (2014-2015), case-control and retrospective studies have been performed to assess the link between ZIKV and GBS. Among the 15 cases included, 33% had evidence of a recent ZIKV infection compared to only 3.3% in the 30 controls involved. All patients were Melanesian, had facial diplegia and similar neurophysiological pattern consistent with acute inflammatory demyelinating polyneuropathy, and recovered well. Furthermore, during the peak of ZIKV transmission, we observed a number of GBS cases higher than the calculated upper limit, emphasizing the fact that ZIKV is now a major trigger of GBS.

Amyotrophic lateral sclerosis (ALS) is a genetically heterogeneous neurodegenerative syndrome hallmarked by adult-onset loss of motor neurons. We performed exome sequencing of 252 familial ALS (fALS) and 827 control individuals. Gene-based rare variant analysis identified an exome-wide significant enrichment of eight loss-of-function (LoF) mutations in TBK1 (encoding TANK-binding kinase 1) in 13 fALS pedigrees. No enrichment of LoF mutations was observed in a targeted mutation screen of 1,010 sporadic ALS and 650 additional control individuals. Linkage analysis in four families gave an aggregate LOD score of 4.6. In vitro experiments confirmed the loss of expression of TBK1 LoF mutant alleles, or loss of interaction of the C-terminal TBK1 coiled-coil domain (CCD2) mutants with the TBK1 adaptor protein optineurin, which has been shown to be involved in ALS pathogenesis. We conclude that haploinsufficiency of TBK1 causes ALS and fronto-temporal dementia.

Previous work has shown a relationship between parietal lobe anatomy and nonnative speech sound learning. We scanned a new group of phonetic learners using structural magnetic resonance imaging and diffusion tensor imaging. Voxel-based morphometry indicated higher white matter (WM) density in left Heschl's gyrus (HG) in faster compared with slower learners, and manual segmentation of this structure confirmed that the WM volume of left HG is larger in the former compared with the latter group. This finding was replicated in a reanalysis of the original groups tested in Golestani and others (2002, Anatomical correlates of learning novel speech sounds. Neuron 35:997-1010). We also found that faster learners have a greater asymmetry (left > right) in parietal lobe volumes than slower learners and that the right insula and HG are more superiorly located in slower compared with faster learners. These results suggest that left auditory cortex WM anatomy, which likely reflects auditory processing efficiency, partly predicts individual differences in an aspect of language learning that relies on rapid temporal processing. It also appears that a global displacement of components of a right hemispheric language network, possibly reflecting individual differences in the functional anatomy and lateralization of language processing, is predictive of speech sound learning.

Purpose: To investigate the diffusion tensor properties of the human optic nerve in vivo using a non-Carr-Purcell-Meiboom-Gill (CPMG) fast spin echo (FSE) sequence., Materials and Methods: This non-CPMG FSE sequence, which is based on a quadratic phase modulation of the refocusing pulses, allows diffusion measures to be acquired with full signal and without artifacts from geometric distortions due to magnetic field inhomogeneities, which are among the main problems encountered in the orbital area., Results: Good-quality images were obtained at a resolution of 0.94 x 0.94 x 3 mm. The mean diffusivity (MD) and fractional anisotropy (FA) were respectively 1.1 +/- 0.2 x 10(-3) mm(2)/second and 0.49 +/- 0.06, reflecting the optic nerve anisotropy., Conclusion: This non-CPMG-FSE sequence provides reliable diffusion-weighted images of the human optic nerve. This approach could potentially improve the diagnosis and management of optic nerve diseases or compression, such as optic neuritis, orbit tumors, and muscle hypertrophy., ((c) 2005 Wiley-Liss, Inc.)

Recent studies in human neuroimaging, primate neurophysiology, and developmental neuropsychology indicate that the human ability for arithmetic has a tangible cerebral substrate. The human intraparietal sulcus is systematically activated in all number tasks and could host a central amodal representation of quantity. Areas of the precentral and inferior prefrontal cortex also activate when subjects engage in mental calculation. A monkey analogue of these parieto-frontal regions has recently been identified, and a neuronal population code for number has been characterized. Finally, pathologies of this system, leading to acalculia in adults or to developmental dyscalculia in children, are beginning to be understood, thus paving the way for brain-oriented intervention studies.

Current theories of number processing postulate that the human abilities for arithmetic are based on cerebral circuits that are partially laid down under genetic control and later modified by schooling and education. This view predicts the existence of genetic diseases that interfere specifically with components of the number system. Here, we investigate whether Turner syndrome (TS) corresponds to this definition. TS is a genetic disorder which affects one woman in 2500 and is characterized by partial or complete absence of one X chromosome. In addition to well-characterized physical and hormonal dysfunction, TS patients exhibit cognitive deficits including dyscalculia. We tested 12 women with Turner syndrome and 13 control subjects on a cognitive battery including arithmetical tests (addition, subtraction, multiplication, division) as well as tests of the understanding of numerosity and quantity (cognitive estimation, estimation, comparison, bisection, subitizing/counting). Impairments were observed in cognitive estimation, subitizing, and calculation. We examine whether these deficits can be attributed to a single source, and discuss the possible implications of hormonal and genetic factors in the neuropsychological profile of TS patients.

Cognitive theories of numerical representation suggest that understanding of numerical quantities is driven by a magnitude representation associated with the intraparietal sulcus and possibly under genetic control. The aim of this study was to investigate, using fMRI and structural imaging, the interaction between the abnormal development of numerical representation in an X-linked condition, Turner syndrome (TS), and the development of the intraparietal sulcus. fMRI during exact and approximate calculation in TS showed an abnormal modulation of intraparietal activations as a function of number size. Morphological analysis revealed an abnormal length, depth, and sulcal geometry of the right intraparietal sulcus, suggesting an important disorganization of this region in TS. Thus, a genetic form of developmental dyscalculia can be related to both functional and structural anomalies of the right intraparietal sulcus, suggesting a crucial role of this region in the development of arithmetic abilities.

Background and Purpose: In cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a large increase in water diffusion has been found both inside and outside the cerebral lesions as detected on conventional MRI. The aim of the present study was to assess the sensitivity of diffusion tensor imaging for monitoring the progression of cerebral tissue damage during the course of CADASIL., Methods: With the use of diffusion tensor imaging, whole brain trace of the diffusion tensor [Trace(D)] histograms were obtained in 22 CADASIL patients and 12 age-matched controls at baseline, in 14 patients after a mean delay of 21 months, and in 5 controls after a mean delay of 29 months. Parameters derived from these histograms (mean value, peak height, and peak location) were analyzed at baseline and during the follow-up., Results: At baseline, all the histogram parameters differed between patients and controls and were found to be significantly correlated with both the Mini-Mental State Examination score and Rankin Scale score in the patient group. The follow-up study showed a decrease in the peak height associated with an increase in the mean value of whole brain Trace(D) histograms in the 14 CADASIL patients scanned twice. The diffusion changes appeared larger in the patients whose Rankin score increased during the study period., Conclusions: These results suggest that the measurement of water diffusion over time is a sensitive marker for the progression of tissue damage in the brain. Thus, quantitative diffusion MRI can be used to monitor disease progression in CADASIL and possibly in other types of small-vessel brain disorders.