acidosis, 173
activated microglia, 1
adaptation, 25
adult-onset, 154
advanced care planing (ACP), 18, 68, 72
Africa, 141
African American, 137
airway management, 60
ALS, 20, 68, 69, 71, 82, 134, 136, 153, 156
disease progression, 24
patients, 170
registry, 137
-CBS, 98
-FTD continuum, 30
alsin, 186
-PDC mixed case, 142
-plus, 123
FRS-R, 78, 131, 142
alternative augmentative communication, 81
alternative splicing, 199, 204
ambulatory model of care, 62
AMPA receptor, 197
AMPK, 196, 214
amyloid, 200
amyotrophic lateral sclerosis, 76, 118, 120, 122, 155, 189
anabolic steroids, 190
Anakinra, 232
anatomy, 185
androgen, 176, 190
androgen receptor, 190
anti-inflammatory macrophage (M2), 175
antisense, 15, 16, 17, 63
anti-SOD1 antibody, 184
arterial carbon dioxide pressure, 86
assessment, 102, 129
astrocyte(s), 49, 50, 189, 211, 231, 232
activation, 213
ataxin-2 (ATXN2), 156
attachment style, 104
atypical features, 134
automated imaging, 15
autonomic dysfunction, 121
autophagy, 58, 180, 200, 214
avulsion, 222
axon
integrity, 192
protection, 191
axonopathy, 2
bacterial artificial chromosome, 195
balloon-based manometry, 88
behaviour, 11
behavioural assessment, 101
behavioural changes, 12, 102
benign fasciculations, 112
bereavement, 65
biarsenical labelling, 57
bioinformatics analyses, 154
biomarker(s), 37, 38, 46, 54, 105, 106, 115, 116, 117, 127, 148, 221, 230, 235
blood
-brain barrier, 233
-spinal cord barrier, 197
BMAA, 227
body mass index (BMI), 84, 85, 182
bone marrow-derived cell, 220
brain computer interface, 96
diseases, 19
mapping, 110
branched chain amino acids (BCAA), 84
breaking bad news, 70
breathing, 89
breath-stacking, 87
Bulbar, 53, 92
function, 74
symptoms, 73
bvFTLD, 130
C. elegans, 194
C9ORF72, 8, 15, 17, 29, 33, 34, 128, 160, 162, 163, 164, 165, 166, 167, 179
calcium homeostasis, 25
channel blocker, 16
signaling, 201
caloric balance, 59
calreticulin, 206
cannabinoid receptor type 2 ligand, 32
care, 65, 71
caregiver, 66, 104
case management, 65
catalase activity, 174
CBT, 70
ceftriaxone, 229
cell(s), 48
death, 231
-penetrating peptides, 231
cellular trafficking, 169
central venous catheter, 229
cerebellar substructures, 30
cerebellum, 31
cerebrospinal fluid, 101, 124
cgrp, 188
chart review, 66, 112
chest physical therapy, 76
Chinese, 161, 162
CHMP2B, 180
CHOP, 206
choroid plexus, 19
chromatin, 43, 49
clinical, 46, 143
phenotype, 122
prediction, 131
psychology, 103
research, 131
tool, 30, 165
trial(s), 45, 46, 52, 53, 125, 127, 228
CLIP-seq, 208
cluster, 139
clusterin, 198
co-
culture, 49, 210, 216
occurrence, 134
cognition, 11, 12, 99, 100, 109, 110
cognitive, 11
dysfunction, 98
evaluation, 95
impairment, 95, 100, 101
coherence, 38
cohort study, 146
communication, 81, 132
complicated grief, 104
conflicts, 18
continuity of care, 67
control, 72
coordination, 71
coordinator, 67
COPII, 27
coping, 65, 133
corpus callosum, 99, 107, 108
cortical connectivity, 222
thickness, 106
corticospinal tract, 2
cost, 66
cough, 87, 88
creatine, 125
kinase, 120
creatinine, 37, 127
CRMP4, 156
crowd-sourcing, 131
CST, 186
C-terminals, 186
cycle ergometer, 128
cyclosporin A, 224
Cys111Tyr mutation, 153
cystatin C, 214
cytokines, 183
cytoskeleton, 157
data mining, 131
de novo mutation, 43
death receptor 6, 211
decision criteria, 61
-making, 6, 72
deglutition, 82, 88, 132
delay in diagnosis, 102
dementia, 123
denervation, 189
depression, 6, 73, 102, 103
dexpramipexole, 46, 125, 126, 127, 227
dHMN, 162
diagnosis, 37, 133, 135
diagnostic biomarker, 123
criteria, 133
diaphragm, 62
fluoroscopy, 93
pacing, 62, 78, 93
dicer, 208
diet, 23, 84
diffusion tensor imaging (DTI), 29, 106, 107, 108, 109, 111
dignity therapy, 13
diptheria toxin, 191
disability, 78
disease cell model, 202
progression, 37, 52, 113, 115, 121, 124, 135, 142
distal degeneration, 191
lower motor neuron disease, 155
SMA, 135
DJ-1, 172
DNA, 163
analysis, 159
damage response, 204
methylation, 182
dorsal root ganglia, 185
double transgenic mice, 56
doubly-labeled water, 87
Drosophila, 198
drug delivery, 233
discovery, 193, 214
screening, 221
DTI, 111
dysarthria, 82
dysphagia, 82, 89, 119
dyspnea, 92
Edaravone, 228
El Escorial Criteria, 126
electric shocks, 22, 146
electroconvulsive therapy, 22
electromyography (EMG), 112, 114
electrophysiology, 54
ELISA, 116
elongator, 178
emotional functioning, 145
EMPOWER, 125, 126
end of life, 18, 71, 72, 73,
endocannabinoid system, 73, 226
endogenous neuroprotection, 2
endoplasmic reticulum (ER), 168, 206
stress, 2, 206, 230
Golgi transport, 27
endoplasmic reticulum, 168
endoscopy, 132
endosomal markers, 58
endurance, 76
energy requirements, 120
enteral, 59, 85
environmental, 138, 227
eosinophil-derived neurotoxin, 115
eosinophilic-associated diseases, 227
ependymal stem cells, 217
ephrinb2, 232
epidemiology, 5, 22, 23, 24, 138
epigenetic(s), 10, 33, 49, 182
erythropoietin, 45, 189
essential tremor, 143
ethnic groups, 141, 135
ethnicity, 137, 140
evaluation, 132
ex post facto approach, 106
excitability, 2
excitotoxicity, 2, 192, 212
executive function(ing), 97, 100
exercise, 128
therapy, 76
tolerance, 130
exome sequencing, 43
expansion, 151, 156
eye-tracking, 81, 96, 128
facial nerve, 222
FACS, 219
familial amyotrophic lateral sclerosis (FALS), 158
familial, 205
fasciculation potentials, 112
fat metabolism, 182
fatigue, 80
fatty acids, 182
FDG-PET, 183
FGGY gene, 162
fiber tracking, 107, 108
fibroblasts, 25
fibronectin, 114
FLJ10986, 162
fluency, 96
fMRI, 105
FMRP, 9
foetal neural stem cells, 47
food intake, 223
Forced Vital Capacity (FVC), 62, 90, 121
fractional anisotropy, 111
frail terminality, 71
France, 139
frontal
assessment battery, 98
lobe, 99
fronto-executive dysfunction, 101
frontotemporal (Dementia) (FTD), 11, 52, 95, 168
-MND continuum, 100
FrSBe, 101
FTLD, 163
-U, 27
function, 69
functional benefit, 45
near-infrared spectroscopy (fNIR), 97
recovery, 192
functionality, 77
decline, 61
FUS, 8, 10, 160, 193, 203, 204, 205
/TLS, 204
G - group I metabotrophic glutamate receptors, 188
G147C, 152
gastrostomy, 86
gender, 130, 185
gene mutation, 171
therapy, 21, 197, 235
-expression, 148
genetic(s), 5, 22, 52, 149, 157, 158, 159, 161, 163, 234
analysis, 151
background, 41, 178
counselling, 5, 148
genetic epidemiology, 152
mouse model, 188
testing, 5, 42
genome
-wide association study (GWAS), 42
-wide SNP analysis, 160
genomics, 35
ghrelin, 223
glia, 49
GLT-1B, 196
glutamate receptors, 16
release, 212
uptake, 235
glutamate, 50, 213
-mediated excitotoxicity, 188
gluten sensitivity, 39
glycogen, 173
grief response, 65
growth factors, 216
hormone, 26
Guam, 142
guidelines, 64
hand function, 83
handedness, 122
HCA, 221
heat factor-1, 229
proteins, 16
hematology, 227
hematopoietic stem cell, 219
hemochromatosis, 23
hereditary motor neuropathy, 154
hereditary spastic paraplegia (HSP), 150
heritability, 42
heterogeneity, 152
heterogeneous nuclear ribonucleic acids, 58
high content screening, 214
fat diet, 39, 214
penetrance, 43
-resolution ultrasound, 113
histone post-translational modifications, 182
historical placebo, 127
hnRNP, 28
hnRNPA, 158
hnRNP K, 8
hnRNPA3, 8
home care, 71, 73
ventilation service, 90
homozygosity, 149
HRT, 130
Hsp70, 214
HSPB1, 162
human antibodies, 20
induced pluripotent stemcells, 15
urine, 116
wild-type SOD1, 56
human, 235
hydration, 85
hyperalimentation, 84
hypnosis, 13
hypothesis, 124
identity-by-descent, 160
IL-1 receptor antagonist, 232
imaging, 111
immune activation, 175
immunofluorescence, 194
immunohistochemistry, 173
immunomodulation, 19, 175
immunotherapy, 20, 184
impact, 86
in vitro pharmacology, 214
in vivo, 225
incidence, 137, 138, 139
inclusion, 153, 208
(bodies), 168
individual level analysis, 107
infection, 229
inflammation, 189, 216, 224
initial dosage, 79
innate immunity, 19
insulin resistance, 26
interactome, 199
interneurons, 3
intractable neurological disease, 67
intravenous immunoglobulin, 233
iPSC(s), 16, 170, 171, 201, 219
irisin, 117
iron, 190, 224
metabolism, 23, 116
juvenile ALS, 144
Kii ALS/PDC, 228
language, 12
late-onset, 162
L-BMAA, 139
leucine, 84
lifestyle, 140
limb dominance, 122
lipids, 39, 181
lithium, 223
locked-in, 81
longitudinal study, 75
loss, 72
of function, 179
lower motor neuron, 135, 154
L-Serine, 227
lymphoblasts, 163
lymphocyte, 36
magnetic fields, 22, 146
magnetic resonance imaging (MRI), 4, 29, 113
mainland of China, 41
malnutrition, 59, 87
MAMs, 209
management, 59
MAPT mutations, 155
mechanical in-exsufflator, 87
mesenchymal, 48, 175, 216, 218
meta-analysis, 42, 95
metabolic equivalent, 130
metabolism, 26, 84, 172, 173, 181
metabolomics, 38, 124, 210
methylation, 33, 178
MGF, 222
mice, 55
microarray, 148, 194
microglia, 149, 189, 220
microRNA(s), 10, 123, 149, 157, 208, 217
microtubule(s), 2, 27
migration, 105
mindfulness, 103
MIP, 121
misdiagnosis, 134
misfolded, 225
mitochondria, 26, 172
dysfunction, 171, 172
function, 25
model, 9
modifiers, 41, 178
modulator, 178
molecular basis, 159
modeling, 161
monocytes, 19
morphine, 68, 79
mosaicism, 156
motor neuron(s), 21, 34, 49, 170, 209, 216
culture, 215
motor nerve biopsy, 4
death, 50
disease, 140
disorders, 124
phenotype, 52
protection, 192
unit number, 117
mouse (model), 41, 55, 178, 179, 180, 195, 224
MRI, 29, 113
mRNA, 170
mtDNA, 25
multidisciplinary, 6, 66, 69, 74
multifocal motor neuropathy, 233
multinational research, 69
multiple sclerosis, 153
multisystem proteinopathy, 159
muscle biopsy, 36
progenitor cells, 171, 172, 176, 200, 220
mutation analysis, 158, 161
mutation, 43, 151, 152, 154, 155, 161
myelin, 195
myocardal infarction, 121
myosin VI, 169
NADPH oxidase, 187, 228
nanobody, 20
national registry, 137
near infrared spectroscopy, 110
neck weakness, 146
Neulasta, 219
neural stem cell (NSC), 215, 218
neuregulin, 186
neuro degeneration, 117, 177, 179, 207
degenerative disease, 25
filament heavy chain, 115
inflammation, 1, 46, 115, 183, 187, 188, 225, 228, 232, 233
muscular junction, 191
muscular remodeling, 177
pathology, 57, 167
peptide(s), 183, 188, 235
protection, 218, 225, 226
neuroprotective compounds, 215
psychiatric symptoms, 12, 102
psychiatry, 99
psychological assessment/tests, 96, 100, 128
trophic factors, 213, 220
neutron activation analysis, 140
new susceptibility loci, 42
NFkB, 211
nocturnal pulse oximetry, 93
non invasive ventilation, 61, 62
-cell autonomous, 49, 50, 211
-human primates, 21
-invasive ventilation (NIV), 61, 92
-pathogenic, 43
NIV, 92
nonsense-mediated mRNA decay, 203
normative data, 96
nosological significance, 142
NRF2, 207, 235
Nuclear Magnetic Resonance, 124
nuclear stress bodies, 209
nutritional, 85
nutrition, 83
occupation, 138
occupational exposures, 146
ocular motor apraxia, 123
oligodendrocytes, 49
oligogenic, 152
optineurin, 169
OPTN, 160
oral secretions, 60
outcome measure(s), 53, 131
oxidative stress, 23, 24, 26, 174, 190, 206, 207, 210
P2X7 receptor, 149, 187
P62, 168
p62/SQSTM1, 180
pain, 129
palatal plate, 82
palliative care, 18, 68, 79
partners, 70
pathological lesion selectivity, 229
pathology, 3, 35
pathophysiology, 170
patient data, 52
decision-making, 6
services, 133
-reported outcome measure, 145
patients’/perspective, 65
PDI, 230
percutaneous endoscopic gastrostomy, 86
periodic limb movements, 94
peripheral nerve, 4
peripherin, 196
personality, 99
PFN1, 157
pgc-1a, 117
pharmacology, 230
phase 1 clinical trial, 47
phenotype(s), 106, 134
phenotyping, 180
phosphorylated Smad2, 201
phrenic nerve conduction, 93
motor response, 91
physical activity, 144, 145
actvities, 130
function, 129
impairment, 73, 145
therapy, 76
PINK1, 172
place of death, 73
planning, 72
plasma, 114, 123
PLS, 113
polyadenylation, 203
polyglutamine, 41
polyglutamine-induced motor neuron disease, 229
polyunsaturated fatty acids, 23
pooling of data, 106
population-based, 138, 142
Positron Emission Tomography (PET), 31, 32, 95
power wheelchair, 80
PP2A, 196
presynaptic mGlu1/mGlu5 receptors, 212
prevalence, 137
primary lateral sclerosis, 109, 112, 133
primitive reflexes, 98
Pringle criteria, 133
prion, 55
-like, 55, 158, 159
PROACT database, 127
profilin, 157, 158
prognosis, 39, 120, 135, 136, 143
prognostic factor(s), 120, 146
biomarker, 36
prognostication, 114
progranulin, 101, 202
progression, 6
progressive muscular atrophy (PMA), 233
proinflammatory macrophage (M1), 175
propagated protein misfolding, 55
propagation, 198
protein aggregation, 57, 170
degradation, 168
misfolding, 200, 213
stability, 56
psychological intervention(s), 13, 102, 103
well-being, 7
factors, 74
support, 13
q-ball imaging, 108
quality of life (QoL), 6, 7, 13, 64, 69, 73, 74, 75, 76, 77, 78, 80, 91, 103, 219
quantitative PCR (qPCR), 166, 219
RAN translation, 8
rare variant discovery, 160
RBM45, 209
RBPs, 207
recessive variant, 149
referral, 90
register, 139
rem sleep behaviour disorder, 94
remote clinics, 64
repeat expansion(s), 33, 162, 164
respiration, 114
respiratory aspects, 77
function, 91, 93
impairment, 91
Resting Energy Expenditure (REE), 90
resting state fMRI, 29, 109, 110, 113
retrograde transport, 220
retrospective, 66
RIPK1, 211
risk, 144
factor(s), 5, 22, 156
RNA binding proteins, 207, 209
dysregulation, 9
foci, 17
processing, 204
toxicity, 9, 15, 167, 208
binding protein, 10, 194, 208
rodents, 232
ROS, 228
rs6690993, 162
rs6700125, 162
safety, 125
saliva, 59
satisfaction, 81
Sativex, 226
SBMA, 119, 176
SCD-1, 181
Schwann cells, 191
screening, 193
segmental trunk Bioelectric Impedance Analysis (stBIA), 90
selective vulnerability, 222
semantic, 100
sensitivity, 31
sensory nervous system, 185
sequestration, 167
serum ferritin, 116
serum lipid, 118
SETX gene, 143
SETX, 195
shoulder pain, 77
Sigma-1 receptor, 209, 225
single nucleotide polymorphism (SNP), 162
size dependent axon loss, 2
skeletal muscle activator, 45
skin, 26, 173
sleep, 77, 94
slow progression, 137
spinal muscular atrophy (SMA), 134, 177, 186, 223, 234
SMN, 27, 166, 177, 204, 205
SMNdelta7 mouse, 223
snRNA, 166
soccer, 145
social support, 6
SOD1 (superoxide dismutase 1), 1, 55, 214
aggregates, 56
G93A, 224
gene, 161
misfolding, 184
mouse, 20, 28, 37, 56, 152, 153, 154, 160, 172, 179, 180, 183, 184, 185, 191, 213, 223, 224, 225, 230, 234
/H67D, 190
somatic instability, 164
southern blot analysis, 164
sp. coeliac, 39
specificity, 31
spectroscopy, 38
speech intelligibility, 80
subsystems, 53
synthesis, 79, 12, 80
SPG27, 150
SPIOn, 105
splicing, 34, 166, 203
sport, 145
spreading of pathology, 57, 220
stem cell(s)
therapy, 105, 219, 221
derived motor neurons, 48, 216, 234
steroid injection, 77
strength, 53
stress, 205
granules, 10
superoxide dismutase 1, 55, 214
support for staff, 103
supportive care, 70
suprapubic tract, 78
survey, 5, 68, 80
survival analysis, 120
prognosis, 86
time, 12, 53, 61, 62, 69, 85, 92, 93, 118, 122, 140, 215
susceptibility, 162
swallowing, 88, 89
symptom(s), 59, 112
synaptic proteins, 212
T lymphocyte, 1, 175, 184,
tailor-made patient care, 70
Tako-Tsubo, 121
tamoxifen, 125
TARDBP, 160
tauopathy, 155
TDP 8, 9, 10, 27, 28, 43, 49, 50, 56, 57, 167, 193, 194, 195, 196, 197, 198, 199, 200, 201, 202, 203, 215
knockout mice, 197
mislocalization, 195
telehealth, 66
therapeutic(s), 15, 46, 193, 234
therapy, 230
threshold-tracking transcranial magnetic stimulation, 108
thymus, 184
thyroid hormone, 122
timing, 86
TONiC study, 7, 73, 74, 75
total energy expenditure, 87
toxicity, 55, 200
tractography, 107, 109
transcranial magnetic stimulation, 110
transcriptome, 34, 35, 164
transcript-specific, 166
transcutaneous capnography, 61
transforming growth factor-beta, 201
transgenic(s), 3, 234
transglutaminases, 39
transitional metals, 140
translational profiling, 34, 194
transplantation, 47, 218, 221
TRAP, 34
treatment, 45, 132
trophic factor, 218, 221
tropics, 141
truncation, 205
tube feeding, 83
Turkey, 159
UBQLN2, 144, 160, 206
uchl1, 185
UCP3, 171
ulnar nerve, 113
ultrastructure, 197
United States, 138
upper motor neuron(s) (UMN), 2, 31, 38, 54, 186, 222
uric acid, 136
urinary tract, 78
vaccination, 19
valocin-containing protein (VCP), 155, 160, 173
VAPB, 143, 206
ventilation, 90, 91
VFI, 96
videoendoscopic evaluation of swallowing, 82
videofluorography, 119
Visual Rating Scale, 30, 165
vitamin D, 215
voice
output communication aids, 79
reconstruction, 79
voxel-based morphometry (VBM), 30, 105, 108, 110
water turnover, 85
weaning, 91
whole-brain MRS, 31
XBP-1, 206
young onset ALS, 143
zebrafish, 179, 193