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See detailLimbic hyperconnectivity in the vegetative state
Di Perri, C; Bastianello, S; Bartsch, AJ et al

in Neurology (2013), Vol 81

Objective: To investigate functional connectivity between the default mode network (DMN) and other networks in disorders of consciousness. Methods: We analyzedMRI data from 11 patients in a vegetative ... [more ▼]

Objective: To investigate functional connectivity between the default mode network (DMN) and other networks in disorders of consciousness. Methods: We analyzedMRI data from 11 patients in a vegetative state and 7 patients in a minimally conscious state along with age- And sex-matched healthy control subjects. MRI data analysis included nonlinear spatial normalization to compensate for disease-related anatomical distortions. We studied brain connectivity data from resting-state MRI temporal series, combining noninferential (independent component analysis) and inferential (seed-based general linear model) methods. Results: In DMN hypoconnectivity conditions, a patient's DMN functional connectivity shifts and paradoxically increases in limbic structures, including the orbitofrontal cortex, insula, hypothalamus, and the ventral tegmental area. Conclusions: Concurrently with DMN hypoconnectivity, we report limbic hyperconnectivity in patients in vegetative and minimally conscious states. This hyperconnectivity may reflect the persistent engagement of residual neural activity in self-reinforcing neural loops, which, in turn, could disrupt normal patterns of connectivity. [less ▲]

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See detailMigraine prevention with a supraorbital transcutaneous stimulator. A randomized controlled trial.
Schoenen, Jean ULg; Vandersmissen, Bart; Jeangette, Sandrine et al

in Neurology (2013), 80

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See detailThe earlier the smaller the better for natalizumab-associated PML: in MRI vigilance veritas ?
PHAN BA, Remy ULg; Belachew, Shibeshih ULg; Outteryck, Olivier et al

in Neurology (2012), 79

Natalizumab-associated progressive multifocal leukoencephalopathy (N-PML) in multiple sclerosis (MS) is due to CNS infection by the opportunistic JC virus (JCV). As of december 2011, 193 confirmed cases ... [more ▼]

Natalizumab-associated progressive multifocal leukoencephalopathy (N-PML) in multiple sclerosis (MS) is due to CNS infection by the opportunistic JC virus (JCV). As of december 2011, 193 confirmed cases of N-PML have been observed, giving rise to an overall risk of approximately 0,202%. N-PML pathogenesis remains partially elusive although risk factors have now been clearly delineated. In patients with prior JCV infection detected by serum anti-JCV antibodies, duration of therapy and prior use of immunosuppressants (IS) increase the risk of N-PML. The clinical outcome of MS patients who developed N-PML was highly variable, ranging from asymptomatic case to varying degrees of neurological disability or even death. It was also observed in real life setting that the earlier N-PML was diagnosed and treated, the better was the clinical outcome. Clinical vigilance is now considered as the established cornerstone of PML risk-management algorithm. Here we present early MRI features of 4 out of 8 N-PML cases, which were observed in Wallonia-Brussels and Northern France in more than 4 years of post-marketing utilization of natalizumab for both regions. We are not aware of the specific context and outcome of the 4 other N-PML cases, which were diagnosed and treated in other centers. The reported cases emphasize that (i) N-PML can have a long presymptomatic course while still being clearly detectable with MR imaging, (ii) N-PML can have a benign outcome provided it is diagnosed and treated early, (iii) a clinically symptomatic N-PML may be a further advanced infection with a poorer prognosis, and (iv) periodic brain MR scans, particularly in high risk situations, are likely to provide earlier detection of N-PML and better outcomes. [less ▲]

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See detailImmune Reconstitution Inflammatory Syndrome in Natalizumab-Associated PML
Phan-Ba, Rémy ULg; LOMMERS, Emilie ULg; Moonen, Gustave ULg et al

in Neurology (2012), 78(1), 73

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See detailLarge-scale replication and heterogeneity in Parkinson disease genetic loci.
Sharma, Manu; Ioannidis, John P. A.; Aasly, Jan O. et al

in Neurology (2012), 79(7), 659-67

OBJECTIVE: Eleven genetic loci have reached genome-wide significance in a recent meta-analysis of genome-wide association studies in Parkinson disease (PD) based on populations of Caucasian descent. The ... [more ▼]

OBJECTIVE: Eleven genetic loci have reached genome-wide significance in a recent meta-analysis of genome-wide association studies in Parkinson disease (PD) based on populations of Caucasian descent. The extent to which these genetic effects are consistent across different populations is unknown. METHODS: Investigators from the Genetic Epidemiology of Parkinson's Disease Consortium were invited to participate in the study. A total of 11 SNPs were genotyped in 8,750 cases and 8,955 controls. Fixed as well as random effects models were used to provide the summary risk estimates for these variants. We evaluated between-study heterogeneity and heterogeneity between populations of different ancestry. RESULTS: In the overall analysis, single nucleotide polymorphisms (SNPs) in 9 loci showed significant associations with protective per-allele odds ratios of 0.78-0.87 (LAMP3, BST1, and MAPT) and susceptibility per-allele odds ratios of 1.14-1.43 (STK39, GAK, SNCA, LRRK2, SYT11, and HIP1R). For 5 of the 9 replicated SNPs there was nominally significant between-site heterogeneity in the effect sizes (I(2) estimates ranged from 39% to 48%). Subgroup analysis by ethnicity showed significantly stronger effects for the BST1 (rs11724635) in Asian vs Caucasian populations and similar effects for SNCA, LRRK2, LAMP3, HIP1R, and STK39 in Asian and Caucasian populations, while MAPT rs2942168 and SYT11 rs34372695 were monomorphic in the Asian population, highlighting the role of population-specific heterogeneity in PD. CONCLUSION: Our study allows insight to understand the distribution of newly identified genetic factors contributing to PD and shows that large-scale evaluation in diverse populations is important to understand the role of population-specific heterogeneity. [less ▲]

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See detailBrain perfusion patterns in familial frontotemporal lobar degeneration.
Seelaar, H.; Papma, J. M.; Garraux, Gaëtan ULg et al

in Neurology (2011), 77(4), 384-92

OBJECTIVE: Frontotemporal lobar degeneration (FTLD) is a clinically, genetically, and pathologically heterogeneous disorder. The aim of this study was to compare clinical features and perfusion patterns ... [more ▼]

OBJECTIVE: Frontotemporal lobar degeneration (FTLD) is a clinically, genetically, and pathologically heterogeneous disorder. The aim of this study was to compare clinical features and perfusion patterns on SPECT of patients with familial FTLD-TAR DNA binding protein 43 kDa (TDP) and MAPT mutations. METHODS: Patients were included if they had MAPT or GRN mutations, positive family history with pathologically proven FTLD in the patient or first-degree relative, or were part of FTD-MND families. All patients and 10 age- and gender-matched controls underwent measurement of brain perfusion using (99m)Tc-HMPAO SPECT. We used SPM8 to perform image processing and voxel-based group analyses (p < 0.001). Gender and age were included as nuisance variables in the design matrices. RESULTS: Of the 29 patients with familial FTLD, 19 had familial FTLD-TDP (GRN mutations in 6), and 10 had MAPT mutations. At clinical presentation, familial FTLD-TDP patients were older at onset (p = 0.030) and had more memory deficits (p = 0.011), whereas patients with MAPT had more naming deficits (p < 0.001) and obsessive-compulsive behavior (p = 0.001). The between-groups SPECT analyses revealed significantly less perfusion in the right frontal lobe, precuneus, cuneus, and inferior parietal lobule in familial FTLD-TDP, whereas significantly less perfusion was found in the left temporal and inferior frontal gyri in MAPT. Post hoc analysis of familial FTLD-TDP with unknown genetic defect vs MAPT revealed less perfusion in the right frontal and parietal lobe. CONCLUSION: Familial FTLD-TDP shows relatively more posterior hypoperfusion, including the precuneus and inferior parietal lobule, possibly related to significant memory impairment. Patients with MAPT were characterized by impaired perfusion of the temporal regions and naming deficits. [less ▲]

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See detailReport of the task force on designing clinical trials in early (predementia) AD
Aisen, P.; Andrieu, S.; Sampaio, C. et al

in Neurology (2011), 76

BACKGROUND: A large number of promising candidate disease-modifying treatments for Alzheimer disease (AD) continue to advance into phase II and phase III testing. However, most completed trials have ... [more ▼]

BACKGROUND: A large number of promising candidate disease-modifying treatments for Alzheimer disease (AD) continue to advance into phase II and phase III testing. However, most completed trials have failed to demonstrate efficacy, and there is growing concern that methodologic difficulties may contribute to these clinical trial failures. The optimal time to intervene with such treatments is probably in the years prior to the onset of dementia, before the neuropathology has progressed to the advanced stage corresponding to clinical dementia. METHOD: An international task force of individuals from academia, industry, nonprofit foundations, and regulatory agencies was convened to discuss optimal trial design in early (predementia) AD. RESULTS: General consensus was reached on key principles involving the scope of the AD diagnosis, the selection of subjects for trials, outcome measures, and analytical methods. CONCLUSION: A consensus has been achieved in support of the testing of candidate treatments in the early (predementia) AD population. [less ▲]

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See detailMitochondrial DNA haplogroups influence the therapeutic response to riboflavin in migraineurs.
Di Lorenzo, C.; Pierelli, F.; Coppola, G. et al

in Neurology (2009), 72(18), 1588-94

OBJECTIVES: In migraine, an interictal reduction of mitochondrial energy metabolism and a preventive effect of high-dose riboflavin were reported. To explore the relation between the two, we tested if the ... [more ▼]

OBJECTIVES: In migraine, an interictal reduction of mitochondrial energy metabolism and a preventive effect of high-dose riboflavin were reported. To explore the relation between the two, we tested if the therapeutic response to riboflavin is associated with specific mitochondrial DNA (mtDNA) haplogroups. We focused our attention on haplogroup H, which is known to differ from others in terms of energy metabolism. METHODS: Sixty-four migraineurs completed a 4-month open trial with riboflavin (400 mg QD) and were genotyped blindly for mtDNA haplogroups. RESULTS: Forty patients responded to riboflavin treatment and 24 were nonresponders. The mtDNA haplogroup H was found in 29 subjects (20 migraine without aura, 9 migraine with aura). Riboflavin responders were more numerous in the non-H group (67.5%). Conversely, nonresponders were mostly H (66.7%). The difference between the two groups was significant (chi(2) = 7.07; p = 0.01). The presence of aura had no influence on riboflavin's effectiveness (chi(2) = 0.113; p = 0.74) and was not associated with a particular haplogroup (chi(2) = 0.55; p = 0.46). CONCLUSIONS: In this pharmacogenetic study, riboflavin appears to be more effective in patients with migraine with non-H mitochondrial DNA haplotypes. The underlying mechanisms are unknown, but could be related to the association of haplogroup H with increased activity in complex I, which is a major target for riboflavin. Our results may have ethnic implications, since haplogroup H is chiefly found in the European population. [less ▲]

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See detailMitochondrial diseases associated with cerebral folate deficiency
Garcia-Cazorla, A.; Quadros, E. V.; Nascimento, A. et al

in Neurology (2008), 70(16), 1360-1362

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See detailBlink to visual threat does not herald consciousness in the vegetative state.
Vanhaudenhuyse, Audrey ULg; Giacino, J.; Schnakers, Caroline ULg et al

in Neurology (2008), 71(17), 1374-5

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See detailCobblestone-like brain dysgenesis and altered glycosylation in congenital cutis laxa. Debre type
Van Maldergem, Lionel ULg; Yuksel-Apak, M.; Kayserili, H. et al

in Neurology (2008), 71(20), 1602-1608

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See detailReduced brain choline in homocystinuria due to remethylation defects
Debray, François-Guillaume ULg; Boulanger, Y.; Khiat, A. et al

in Neurology (2008), 71(1), 44-49

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See detailAre we equal in death?
Laureys, Steven ULg; Fins, Joseph J.

in Neurology (2008), 70

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See detailVoluntary brain processing in disorders of consciousness
Schnakers, Caroline ULg; Perrin, F.; Schabus, M. et al

in Neurology (2008), 71(20), 1614-1620

Background: Disentangling the vegetative state from the minimally conscious state is often difficult when relying only on behavioral observation. In this study, we explored a new active evoked related ... [more ▼]

Background: Disentangling the vegetative state from the minimally conscious state is often difficult when relying only on behavioral observation. In this study, we explored a new active evoked related potentials paradigm as an alternative method for the detection of voluntary brain activity. Methods: The participants were 22 right-handed patients (10 traumatic) diagnosed as being in a vegetative state (VS) (n 8) or in a minimally conscious state (MCS) (n 14). They were presented sequences of names containing the patient’s own name or other names, in both passive and active conditions. In the active condition, the patients were instructed to count her or his own name or to count another target name. Results: Like controls, MCS patients presented a larger P3 to the patient’s own name, in the passive and in the active conditions. Moreover, the P3 to target stimuli was higher in the active than in the passive condition, suggesting voluntary compliance to task instructions like controls. These responses were even observed in patients with low behavioral responses (e.g., visual fixationand pursuit). In contrast, no P3 differences between passive and active conditions were observed for VS patients. Conclusions: The present results suggest that active evoked-related potentials paradigms may permit detection of voluntary brain function in patients with severe brain damage who present with a disorder of consciousness, even when the patient may present with very limited to questionablyany signs of awareness. [less ▲]

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See detailCerebral response to patient's own name in the vegetative and minimally conscious states
Di, H. B.; Yu, S. M.; Weng, X. C. et al

in Neurology (2007), 68(12), 895-899

Background: A challenge in the management of severely brain- damaged patients with altered states of consciousness is the differential diagnosis between the vegetative state ( VS) and the minimally ... [more ▼]

Background: A challenge in the management of severely brain- damaged patients with altered states of consciousness is the differential diagnosis between the vegetative state ( VS) and the minimally conscious state ( MCS), especially for the gray zone separating these clinical entities. Objective: To evaluate the differences in brain activation in response to presentation of the patient's own name spoken by a familiar voice ( SON- FV) in patients with VS and MCS. Methods: By using fMRI, we prospectively studied residual cerebral activation to SON- FV in seven patients with VS and four with MCS. Behavioral evaluation was performed by means of standardized testing up to 3 months post- fMRI. Results: Two patients with VS failed to show any significant cerebral activation. Three patients with VS showed SON- FV induced activation within the primary auditory cortex. Finally, two patients with VS and all four patients with MCS not only showed activation in primary auditory cortex but also in hierarchically higher order associative temporal areas. These two patients with VS showing the most widespread activation subsequently showed clinical improvement to MCS observed 3 months after their fMRI scan. Conclusion: The cerebral responses to patient's own name spoken by a familiar voice as measured by fMRI might be a useful tool to preclinically distinguish minimally conscious state - like cognitive processing in some patients behaviorally classified as vegetative. [less ▲]

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See detailrTMS of the occipital cortex abolishes Braille reading and repetition priming in blind subjects
Kupers, R.; Pappens, M.; MAERTENS DE NOORDHOUT, Alain ULg et al

in Neurology (2007), 68(9), 691-693

To study the functional involvement of the visual cortex in Braille reading, we applied repetitive transcranial magnetic stimulation (rTMS) over midoccipital (MOC) and primary somatosensory (SI) cortex in ... [more ▼]

To study the functional involvement of the visual cortex in Braille reading, we applied repetitive transcranial magnetic stimulation (rTMS) over midoccipital (MOC) and primary somatosensory (SI) cortex in blind subjects. After rTMS of MOC, but not SI, subjects made significantly more errors and showed an abolishment of the improvement in reading speed following repetitive presentation of the same word list, suggesting a role of the visual cortex in repetition priming in the blind. [less ▲]

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See detailNeuroimaging of neuronal circuits involved in tic generation in patients with Tourette syndrome.
Lerner, Alicja; Bagic, Anto; Boudreau, Elis et al

in Neurology (2007), 68(23), 1979-87

OBJECTIVE: To identify brain regions generating tics in patients with Tourette syndrome using sleep as a baseline. METHODS: We used [15O]H2O PET to study nine patients with Tourette syndrome and nine ... [more ▼]

OBJECTIVE: To identify brain regions generating tics in patients with Tourette syndrome using sleep as a baseline. METHODS: We used [15O]H2O PET to study nine patients with Tourette syndrome and nine matched control subjects. For patients, conditions included tic release states and sleep stage 2; and for control subjects, rest states and sleep stage 2. RESULTS: Our study showed robust activation of cerebellum, insula, thalamus, and putamen during tic release. CONCLUSION: The network of structures involved in tics includes the activated regions and motor cortex. The prominent involvement of cerebellum and insula suggest their involvement in tic initiation and execution. [less ▲]

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See detailMigraine with Urticaria
Fumal, Arnaud ULg; Cremers, Julien ULg; Ambrosini, A. et al

in Neurology (2006), 67(4), 682

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See detailFamilial basilar migraine associated with a new mutation in the ATP1A2 gene
Ambrosini, A.; D'Onofrio, M.; Grieco, G. S. et al

in Neurology (2005), 65(11), 1826-1828

Basilar migraine (BM), familial hemiplegic migraine (FHM), and sporadic hemiplegic migraine (SHM) are phenotypically similar subtypes of migraine with aura, differentiated only by motor symptoms, which ... [more ▼]

Basilar migraine (BM), familial hemiplegic migraine (FHM), and sporadic hemiplegic migraine (SHM) are phenotypically similar subtypes of migraine with aura, differentiated only by motor symptoms, which are absent in BM. Mutations in CACNA1A and ATP1A2 have been found in FHM. The authors detected a novel mutation in the ATP1A2 gene (R548H) in members of a family with BM, suggesting that BM and FHM may be allelic disorders. [less ▲]

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