ライフサイエンスコーパス: ALS

1 ALS is more commonly seen in men than women and the same

2 ALS-total group and patients with bvFTD had similar dise

3 tein quality control in C9, and perhaps all, ALS/FTD.

4 Furthermore, expression of an ALS-linked mutation in the pathway disrupted mitochondri

5 In our analysis, ALS led to significant metabolic pathway alterations, wh

6 s, the unification of research in ageing and ALS requires high fidelity models to better recapitulate

7 Median time between blood collection and ALS death was 8 years (range = 1-15).

8 em characterizes frontotemporal dementia and ALS in many individuals with these neurodegenerative dis

9 Hallmarks of both FTLD and ALS are the toxic cytoplasmic inclusions of the prion-li

10 led granulins (GRNs) contributes to FTLD and ALS progression, with specific GRNs exacerbating TDP-43-

11 RN) protein, are strongly linked to FTLD and ALS.

12 s reported from TDP-43 and SOD1 ALS mice and ALS patients with those from SCA2 mice.

13 ays shared with TDP-43 and SOD1 ALS mice and ALS patients.

14 erse causal relationship between smoking and ALS.

15 s) derived from healthy individuals (WT) and ALS patients harboring mutations in the superoxide dismu

16 LS and PD patient's clinical data-if all ANG-ALS patients' data were available our findings could be

17 goal of better defining the etiology of ANG-ALS, we assembled all clinical onset and disease duratio

18 bilization would benefit pre-symptomatic ANG-ALS patients.

19 augmenting ANG will benefit symptomatic ANG-ALS patients.

20 This is confirmed in another ALS mouse model, TDP43(A315T).

21 e testing (Edinburgh Cognitive and Behaviour ALS Screen (ECAS)) during life.

22 sing the Edinburgh Cognitive and Behavioural ALS Screen were included.

23 ndings support a cognitive continuum between ALS and bvFTD and shed light on the cognitive heterogene

24 e disorders are also overrepresented in both ALS and FTD, but this remains an unexplained epidemiolog

25 nd whether its phase behavior is affected by ALS-linked mutations.

26 resynaptic homeostatic plasticity induced by ALS-like motor neuron degeneration, which maintains exci

27 ost common cause of familial ALS and FTD (C9-ALS/FTD), and lead to both repeat-containing RNA and dip

28 an cells expressing GGGGCC repeats and in C9-ALS patient motor cortex.

29 lize with intracellular RAN aggregates in C9-ALS/FTD BAC mice.

30 and exacerbating proteostasis defects in C9-ALS/FTD.

31 ucleocytoplasmic transport contributes to C9-ALS/FTD, but an inventory of proteins that become redist

32 blood and brain tissue from patients with C9-ALS/FTD all show an elevated type I interferon signature

33 y, our results suggest that patients with C9-ALS/FTD have an altered immunophenotype because their re

34 o autoimmune diseases seen in people with C9-ALS/FTD.

35 gest that the expression of expanded C9orf72 ALS/FTD repeat RNA alone affects nuclear POM121 expressi

36 a promising therapeutic approach for C9orf72 ALS/FTD and other expansion diseases.

37 cordingly, SUMO activity ameliorated C9ORF72-ALS-related neurodegeneration in Drosophila.

38 work of German motor neuron disease centers (ALS/MND-NET).

39 al analysis of 27 patients with non-demented ALS who had undergone cognitive testing (Edinburgh Cogni

40 ive impairment in patients with non-demented ALS.

41 redictor of TDP-43 pathology in non-demented ALS.

42 teral Sclerosis and Frontotemporal Dementia (ALS/FTD).

43 biomarkers in ALS have been shown to detect ALS-associated pathology in vivo, although anatomical pa

44 G appears to decrease the risk of developing ALS but exacerbate ALS once in progress.

45 the link between two motor neuron diseases, ALS and spinal muscular atrophy (SMA).

46 cular changes in spinal MNs of TDP-43-driven ALS at motor symptom onset.

47 MNs at motor symptom onset in TDP-43-driven ALS models.

48 t they are general features of TDP-43-driven ALS.

49 to refine clinical and electrophysiological ALS diagnostic criteria and complement prospective clini

50 To estimate ALS risk, we applied conditional logistic regression mod

51 se the risk of developing ALS but exacerbate ALS once in progress.

52 the inclusion formation in cells expressing ALS mutation P525L.

53 crossed with mice of both sexes, expressing ALS-linked gene mutants for TAR DNA-binding protein (TDP

54 02Q in S1R has been reported in few familial ALS cases.

55 d RNA-binding protein implicated in familial ALS and frontotemporal dementia (FTD).

56 n, TDP-43 inclusions are evident in familial ALS phenotypes linked to multiple gene mutations includi

57 More interestingly, familial ALS patient fibroblasts showed higher levels of FUS K510

58 2 gene are the most common cause of familial ALS and FTD (C9-ALS/FTD), and lead to both repeat-contai

59 are the second most common cause of familial ALS, and considerable evidence suggests that these mutat

60 pan in the SOD1-G93A mouse model of familial ALS.

61 95% confidence interval (CI), 0.77-0.93; for ALS: adjusted OR, 0.28; 95% CI, 0.14-0.49); for PD: adju

62 se-modifying therapy currently available for ALS patients and a substantial failure in bench to bedsi

63 thods, to test whether smoking is causal for ALS.

64 Our results may provide new insights for ALS pathogenesis, and the proposed research methodology

65 ase, offering a novel treatment paradigm for ALS, SBMA, SMA, and related disorders.

66 this model as a human-relevant platform for ALS research and drug discovery studies.

67 ed metabolomics was performed on plasma from ALS participants (n=125) around 6.8 months after diagnos

68 n prospectively collected blood samples from ALS patients and controls, to explore whether metals are

69 HI3L1-positive cells in tissue sections from ALS, DCs and non-neurological DCs.

70 FTD-ALS causing mutations are known to be involved in endoso

71 23 bvFTD, 20 FTD-ALS and 30 controls participated.

72 al dysfunction to RV de-repression in an FTD-ALS model without TDP-43 pathology.

73 creen to identify mutations affecting an FTD-ALS-related phenotype in Drosophila caused by CHMP2BIntr

74 bvFTD and FTD-ALS groups performed similarly, although with slightly g

75 ognition and behaviour between bvFTD and FTD-ALS, and patients carrying the C9ORF72 repeat expansion.

76 Behaviour in FTD-ALS was dominated by apathy.

77 Patients with bvFTD or FTD-ALS and healthy controls underwent neuropsychological te

78 tion and behaviour between patients with FTD-ALS and bvFTD alone.

79 ng whole-genome sequence data from 2,442 FTD/ALS patients, 2,599 Lewy body dementia (LBD) patients, a

80 gs in an independent collection of 3,674 FTD/ALS patients.

81 onship between HTT repeat expansions and FTD/ALS syndromes and indicate that genetic screening of FTD

82 s and indicate that genetic screening of FTD/ALS patients for HTT repeat expansions should be conside

83 vealed the classical TDP-43 pathology of FTD/ALS, as well as huntingtin-positive, ubiquitin-positive

84 We find that FUS-ALS mutations induce a widespread loss of function on ex

85 d may represent important targets for future ALS therapeutics.

86 markedly extends survival in the SOD1(G93A) ALS mouse model, providing evidence for gliosis as a pot

87 the Kyoto Encyclopedia of Genes and Genomes ALS pathway, as well as new targets of potential relevan

88 Here we show that mice bearing human ALS-associated TBK1 missense loss-of-function mutations,

89 As in human ALS, expression of mutant dog SOD1 was associated with s

90 og SOD1 would recapitulate features of human ALS (ie, SOD1 protein aggregation, reduced cell viabilit

91 A714 and (11)C-JNJ717 was performed on human ALS brain sections in comparison to immunofluorescence w

92 detect CB2 upregulation on postmortem human ALS spinal cord tissues.

93 de that loss of TBK1 kinase activity impacts ALS disease progression through distinct pathways in dif

94 to previous discoveries of RV activation in ALS affected patients.

95 over, the three main proteins aggregating in ALS/FTD, including in sporadic cases, are also targeted

96 plex neurobiological disease processes as in ALS and posit that the proposed methodology is not restr

97 Neuroimaging-based biomarkers in ALS have been shown to detect ALS-associated pathology i

98 onset and progression of MN degeneration in ALS, and in modulating Treg recruitment and microglia ph

99 Review, we synthesize these developments in ALS and discuss the further developments and refinements

100 ons are involved preferentially and early in ALS.

101 on to MN is an early but reversible event in ALS.

102 ion, contractility and metabolic function in ALS.

103 meostasis and the key pathways implicated in ALS pathophysiology.

104 -1 and CHI3L1 are significantly increased in ALS, and CSF Chit-1 and CHI3L1 levels correlate to the r

105 43 NBs, whose dysfunction may be involved in ALS pathogenesis.

106 the literature of microglial involvement in ALS and discuss the evidence for the neurotoxic and neur

107 lls support their fundamental involvement in ALS/FTD.

108 genic role for alternative TDP43 isoforms in ALS, and implicate sTDP43 as a key contributor to the su

109 CSF Chit-1 exhibited increased levels in ALS as compared with DCs and HCs.

110 mplicated as a key pathological mechanism in ALS, and each lower motor unit cell type vulnerable to i

111 Individual differential metabolites in ALS cases versus controls were assessed by Wilcoxon rank

112 nset and progression of neurodegeneration in ALS/FTD.

113 cation is ubiquitously altered at the NMJ in ALS.

114 dapted response to denervation at the NMJ in ALS.SIGNIFICANCE STATEMENT Understanding how the complex

115 %) in primary motor cortices was observed in ALS subjects, as measured by both V(T) and SUVR(40-60) a

116 gation levels mimic true disease patterns in ALS patients.

117 the use of staging systems as end points in ALS clinical trials and to understand the timing of bene

118 approach for slowing disease progression in ALS.

119 tion that may reflect disease progression in ALS.

120 t cadmium, lead, and zinc may play a role in ALS etiology.

121 and has potential for PET imaging studies in ALS patients and possibly other brain disorders.

122 utonomous effect on motor neuron survival in ALS.

123 gregation-prone RNA-binding proteins that in ALS can mislocalize to the cytoplasm of affected motor n

124 Therefore, in ALS, standardized analysis across these 2 tracers enable

125 stem in AD, mediating motoneuron toxicity in ALS, and stimulating peripheral leukocyte migration into

126 novel P2X7R tracer, in vitro and in vivo in ALS.

127 sms underlying sex-specific vulnerability in ALS-FTD.

128 pendent, thus reflecting MU vulnerability in ALS.

129 sis of neurodegenerative diseases, including ALS caused by a C9orf72 hexanucleotide repeat expansion.

130 expansions in the long normal range increase ALS risk.

131 hese changes in spinal MNs of an independent ALS mouse model caused by a different patient mutant all

132 y and ATP levels of flies expressing the key ALS protein, TDP43.

133 alterations, which had correlations to known ALS pathomechanisms in the basic and clinical literature

134 Caenorhabditis elegans model of SOD1-linked ALS identified the USP7 ortholog as a suppressor of prot

135 or devising therapies to treat UBQLN2-linked ALS/FTD.

136 parallel-group, double-blinded trial (LIPCAL-ALS study) was conducted between February 2015 and Septe

137 trating the applicability of NIR-HSI and MCR-ALS in the identification of fiber in pasta.

138 ve resolution-alternating least squares (MCR-ALS) algorithm for multiset analysis based on the incorp

139 resolution by alternating least-squares (MCR-ALS) enhanced with signal shape constraints based on par

140 ve resolution-alternating least-squares (MCR-ALS) is the model of choice when dealing with matrix dat

141 ve resolution alternating least squares, MCR-ALS, followed by principal component analysis, PCA, and

142 hysiological process leading to FUS-mediated ALS/FTD.

143 d as a potential treatment for SOD1-mediated ALS.

144 port the presence of NK cells in post-mortem ALS motor cortex and spinal cord tissues, and the expres

145 ations, supporting the concept that multiple ALS genes interact in a regulatory network.

146 nalyses of human brain samples from the NYGC ALS cohort, truncated STMN2 RNA was confined to tissues

147 Approximately 10% of ALS cases are familial and 90% are sporadic.

148 MNs is a key pathological feature of >95% of ALS cases.

149 expansion, the most common genetic cause of ALS and FTD, produces sense- and antisense-expansion RNA

150 RNA-binding protein TDP-43, are one cause of ALS, and TDP-43 mislocalization in MNs is a key patholog

151 the mitochondrial defects characteristic of ALS/FTD and contributes to the FUS toxic gain of functio

152 d survival times, reinforcing the concept of ALS as a disorder with extensive systemic pro-inflammato

153 g of how C9 fits into the broader context of ALS/FTD pathology has remained lacking.

154 lutes provides a very good discrimination of ALS from healthy controls which is comparable to that ob

155 and TRAF6 may be relevant to the etiology of ALS.

156 Lipid dysregulation is a feature of ALS; however, it is unclear whether disrupted lipid home

157 neurodegeneration, and clinical features of ALS.

158 ve this phenomenon in other genetic forms of ALS, including those caused by TDP-43, VCP and SOD1 muta

159 ulation of sTDP43 within neurons and glia of ALS patients.

160 thesis, was reduced in the spinal cord GM of ALS patients.

161 mation of TDP-43-positive SGs, a hallmark of ALS.

162 shed light on the cognitive heterogeneity of ALS, expanding its possible neuropsychological profiles.

163 However, the impact of ALS-causative mutations on splicing has not been fully c

164 this study charts the proteomic landscape of ALS-related Ubqln2 mutants and identifies candidate clie

165 sis occurs in the spinal cord gray matter of ALS patients.

166 nit dynamics in a B6.SOD1G93A mouse model of ALS for several weeks.

167 In the mSOD1 model of ALS, the excitability of motoneurons is poorly controlle

168 beneficial in the SOD1(G93A) mouse model of ALS.

169 rotective effects in the SOD1(G93A) model of ALS.

170 P and DR6 is confirmed in vitro in models of ALS.

171 Using brain networks of ALS patients (n = 208) and matched controls across longi

172 protein which forms aggregates in neurons of ALS and FTD patients as well as in a subset of patients

173 However, as the pathomechansims of ALS are increasingly recognized to involve a multitude o

174 potentially disparate pathogenic pathways of ALS-linked FUS mutants.

175 role of microglia in the 'cellular phase' of ALS is crucial in the development of mechanistically rat

176 trated behavioural phenotypes reminiscent of ALS-FTD in males.

177 lysis demonstrated an over-representation of ALS relevant pathways, including calcium ion dependent e

178 tory power in the clinical stratification of ALS compared to healthy controls.

179 A genome-wide association study of ALS (20 806 cases; 59 804 controls) was used as the outc

180 nding protein FUS causes certain subtypes of ALS/FTD by largely unknown mechanisms.

181 to be dysregulated in the nervous systems of ALS patients.

182 Treatment of ALS-derived motor neurons with AdoMet also suppresses th

183 To this end, phenotypic ALS skeletal muscle models were developed from induced p

184 SC) line derived from a C9orf72-HRE positive ALS/frontotemporal dementia patient using CRISPR/Cas9 ge

185 1-positive cells were observed in postmortem ALS motor cortex as compared with controls, and these ce

186 roviding evidence for gliosis as a potential ALS therapeutic target.

187 Current findings align with previous ALS patient biopsy studies and suggest an active contrib

188 rms for neurologists to consistently publish ALS and PD patient's clinical data-if all ANG-ALS patien

189 netic evidence that mutant S1R recapitulates ALS pathology in vivo while increasing S1R confers neuro

190 es, including amyotrophic lateral sclerosis (ALS) and Alzheimer's disease (AD).

191 C9orf72 cause amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) and lead to the p

192 Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are neurodegenera

193 Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are overlapping n

194 Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two related n

195 disease (AD), amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) as well as in the

196 Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) constitute aggres

197 etic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) is a hexanucleoti

198 sociated with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) susceptibility, a

199 nown cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), yet a clear unde

200 The amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)-linked RNA-bindin

201 signature of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).

202 etic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).

203 tic cause for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).

204 patients with amyotrophic lateral sclerosis (ALS) and in approximately 50% of patients dying of front

205 with familial amyotrophic lateral sclerosis (ALS) and mutations in the gene encoding superoxide dismu

206 persons with amyotrophic lateral sclerosis (ALS) are not known.

207 tia (FTD) and amyotrophic lateral sclerosis (ALS) by analyzing whole-genome sequence data from 2,442

208 Amyotrophic lateral sclerosis (ALS) can overlap genetically, pathologically and clinica

209 cal trials in amyotrophic lateral sclerosis (ALS) continue to rely on survival or functional scales a

210 patients with amyotrophic lateral sclerosis (ALS) exhibit mild cognitive deficits in executive functi

211 Amyotrophic lateral sclerosis (ALS) is a fatal disease involving motor neuron degenerat

212 Amyotrophic lateral sclerosis (ALS) is a fatal disease, characterized by the selective

213 Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting moto

214 Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder caused by the

215 Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disease characterized by m

216 Amyotrophic lateral sclerosis (ALS) is a multifactorial, multisystem pro-inflammatory n

217 Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the

218 tia (FTD) and amyotrophic lateral sclerosis (ALS) is incompletely understood.

219 iagnosed with amyotrophic lateral sclerosis (ALS) today face the same historically intransigent probl

220 ein FUS cause amyotrophic lateral sclerosis (ALS), a devastating neurodegenerative disease.

221 ing a role in amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by exten

222 ases, such as amyotrophic lateral sclerosis (ALS), a relentlessly progressive and universally fatal d

223 noses of OAG, amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD), Parkinson's disease (PD)

224 on's disease, Amyotrophic lateral sclerosis (ALS), and other neuroinflammatory conditions, EVs releas

225 is altered in amyotrophic lateral sclerosis (ALS), as early as childhood, suggesting these pathways a

226 generation in amyotrophic lateral sclerosis (ALS), but actual proof of hyperexcitation in vivo is mis

227 ty factor for amyotrophic lateral sclerosis (ALS), but results are conflicting and at risk of confoun

228 In amyotrophic lateral sclerosis (ALS), immune cells and glia contribute to motor neuron (

229 ed a model of amyotrophic lateral sclerosis (ALS), in which astrocytes expressing mutant superoxide d

230 epilepsy, and amyotrophic lateral sclerosis (ALS), mantis-ml achieved an average area under curve (AU

231 eases such as amyotrophic lateral sclerosis (ALS), multiple sclerosis, and Parkinson's disease, perip

232 tion (NIV) in amyotrophic lateral sclerosis (ALS), the question of enteral nutrition is increasingly

233 thogenesis of amyotrophic lateral sclerosis (ALS), we generated transgenic mice with neuron-specific

234 oteomics with amyotrophic lateral sclerosis (ALS)-associated C9ORF72 dipeptides uncovered attenuated

235 ression of an amyotrophic lateral sclerosis (ALS)-associated superoxide dismutase 1 (SOD1) mutant pro

236 on (FTLD) and amyotrophic lateral sclerosis (ALS).

237 important in amyotrophic lateral sclerosis (ALS).

238 with familial amyotrophic lateral sclerosis (ALS).

239 es, including amyotrophic lateral sclerosis (ALS).

240 o the risk of amyotrophic lateral sclerosis (ALS).

241 thogenesis of amyotrophic lateral sclerosis (ALS).

242 al changes in amyotrophic lateral sclerosis (ALS).

243 to comprehend amyotrophic lateral sclerosis (ALS).

244 e early-onset amyotrophic lateral sclerosis (ALS).

245 tic target in amyotrophic lateral sclerosis (ALS).

246 e of familial amyotrophic lateral sclerosis (ALS).

247 d features in amyotrophic lateral sclerosis (ALS).

248 with sporadic amyotrophic lateral sclerosis (ALS).

249 patients with amyotrophic lateral sclerosis (ALS)/Frontotemporal dementia (FTD), the (G4C2)-RNA repea

250 etic cause of amyotrophic lateral sclerosis (ALS)/frontotemporal dementia (FTD).

251 transcriptomes reported from TDP-43 and SOD1 ALS mice and ALS patients with those from SCA2 mice.

252 notated pathways shared with TDP-43 and SOD1 ALS mice and ALS patients.

253 National Laboratory's Advanced Light Source (ALS) has been used in conjunction with a jet stirred rea

254 tions between mutations in UGT8 and sporadic ALS, and between ST6GAL1 mutations and conversion of mil

255 een observed to correlate in C9 and sporadic ALS/FTD patients alike, suggesting that changes along th

256 ted to be involved in C9 as well as sporadic ALS/FTD etiology, including the proteasomal and autophag

257 -fold in the spinal cord GM of male sporadic ALS patients.

258 arly cell type-specific features of sporadic ALS.

259 motor neurons to serially passaged sporadic ALS post-mortem tissue (spALS) extracts.

260 pared with samples from people with sporadic ALS/FTD; this increased interferon response can be suppr

261 vo portion, 3 male patients with early-stage ALS (59.3 +/- 7.2 y old) and 6 healthy volunteers (48.2

262 Conclusion: In symptomatic ALS patients, (18)F-DPA714 showed increased signal where

263 on through mTOR-dependent signaling and that ALS-linked FUS mutants can cause a toxic gain of functio

264 Here, we demonstrate that ALS/FTLD-linked FUS mutations in glycine (G) strikingly

265 The ALS community has made remarkable strides over three dec

266 Our data helps explain the ALS-associated cellular phenotype of dogs expressing the

267 102Q and the beneficial impact of S1R in the ALS context remain to be demonstrated in vivo.

268 light on the pathological mechanisms in the ALS/FTD spectrum and conclude that they have important c

269 y variants at these positions, including the ALS-associated G335D.

270 reported to be dysfunctional in part of the ALS/FTD spectrum.

271 ating that distinct mechanisms contribute to ALS onset and progression and propose that ANG replaceme

272 ial contribution of gene loss-of-function to ALS.

273 n TANK-binding kinase 1 (TBK1) are linked to ALS-FTD.

274 Over 70 mutations in Fus are linked to ALS/FTLD.

275 ageing might in fact prove a prerequisite to ALS, rendering the cells susceptible to disease-specific

276 ell as new targets of potential relevance to ALS pathophysiology.

277 may help to understand female resistance to ALS-FTD and thereby identify routes to therapy.

278 Similar to ALS patients, a compensatory downregulation of cholester

279 intermediates on the pathway from smoking to ALS.

280 1g1 binds more strongly to WT UBQLN2 than to ALS/FTD mutant UBQLN2 proteins.

281 f microendoscopy for diagnosing and tracking ALS, we monitored motor unit dynamics in a B6.SOD1G93A m

282 ing of the biological processes that trigger ALS and promote disease progression.

283 but not by any of the five different UBQLN2 ALS/FTD mutants tested.

284 known loss-of-function mechanisms underlying ALS, potential consequences of lowering levels of gene p

285 ts disparate molecular mechanisms underlying ALS/FTLD pathogenesis and differing recovery potential d

286 utrition is increasingly raised in NIV users ALS patients.

287 In adults with ALS due to SOD1 mutations, CSF SOD1 concentrations decre

288 ose trial evaluating tofersen in adults with ALS due to SOD1 mutations.

289 these pathways significantly associate with ALS.

290 o explore whether metals are associated with ALS mortality.

291 n the CNS of normal mice was associated with ALS-like lipid pathology, astrogliosis, neurodegeneratio

292 nalysis was conducted with the patients with ALS divided into two subgroups: patients without cogniti

293 e pattern of muscle paresis in patients with ALS using the UK Medical Research Council (MRC) scoring

294 A subcohort of 428 patients with ALS was used to study cognitive and behavioral profiles,

295 We included 2,216 patients with ALS without a C9orf72 mutation to identify clinical char

296 slightly greater difficulty in patients with ALS-FTD on category fluency and a sentence-ordering task

297 imaging phenotype of UNC13A in patients with ALS.

298 A total of 177 persons with ALS were screened for eligibility, and 137 were randomly

299 phenylbutyrate-taurursodiol in persons with ALS.

300 vioural variant FTD (bvFTD) with and without ALS are rare.