AAV, 44
actin
binding protein, 192
polymerization, 35
active zone, 195
ADAR2, 197
adeno-associated virus (AAV), 181, 197, 198
AAC, 15
advance care planning, 19, 114
adverse clinical signs, 106
age-period-cohort, 133
aggregates, 7
aggregation, 21
aging, 175
airway clearance, 103
albumin, 34
albuterol, 102
ALS8, 36
ALS COSMOS, 128, 129
ALS pathogenesis, 21
ALS registry, 130
ALSFRS-R, 42, 65, 100, 130
ALSFRSR-Lite, 122
ALS-LAUS, 59
ALSoD, 147
AMPA receptor, 197
AMP-activated protein kinase (AMPK), 174
androgen receptor, 180
anisotropy, 43
anthropometry, 98
antibodies, 22, 201
anticipatory equipment provision, 120
anticoagulant, 180
anti-ganglioside antibody, 59
anti-glutamate toxicity, 189
anti-neuroinflammatory, 189
anxiety, 115
apathy, 54
apoptosis, 163, 188
ARHGEF28 gene, 143
aspiration, 48, 62
assessment, 116, 126
assisted
dying, 31
suicide, 31
assistive
device, 119
products, 121
astrocyte, 178, 185, 202
ataxin 2(ATXN2), 135, 171
attentional control, 79
audit, 123
autonomy, 88
autophagy, 11, 12, 153, 156, 173
autopsy, 66
axon, 176, 192
axonal transport, 176
bacterial artificial chromosome (BAC), 36, 192
basal ganglia, 70
behaviour, 54, 83, 205
behavioural, 82
assessment, 54
changes, 55
impairment, 53, 86
symptomatology, 81
benign fasciculation syndrome, 75
bereavement needs, 126
beta-adrenergic agonist, 102
bioinformatics, 28, 147
biomarkers, 1, 5, 32, 33, 41, 42, 51, 61, 63, 65, 68, 69, 75, 78, 155
biostatistics, 9
bladder, 112
blink reflex, 58
BMAA, 130, 132, 173, 179
body mass index (BMI), 48
bone turnover, 132
bowel, 112
brain tissue bank, 161
brain-computer interaction, 14
bulbar function, 107
bulbar, 98, 136
burden on family, 126
buttons, 97
C5aR1, 184
C9ORF72, 2, 3, 6, 11, 16, 17, 134, 144, 145, 150, 164, 165, 184, 193
Ca2 + regulation, 154
calcium sensitivity, 100
calcium signalling, 16, 169
Canada, 137
care network, 123, 124, 146
care planning, 117
care provision, 18
care, 124, 125
caregiver
burden, 86
outcomes, 86
caregivers, 88, 89, 90, 105,126
case control, 139
case report, 141
cathepsin b, 174
causes of death, 66
CCL2/CCR2, 50
cell biology, 170
cell therapy, 163
cerebellum, 158
cerebrospinal fluid, 155
CHCHD10, 151
chemical chaperones, 44
chest x-ray, 99
chromatin remodeling, 167
classifier construction, 73
clinical features, 53, 140
clinical trial(s), 1, 9, 26, 107, 200, 207
cluster analysis, 134
co-culture, 176
co-design, 121
coenzyme Q10, 182
cognition, 53, 70, 83, 84
cognitive, 83, 116
impairment, 55, 72
profile, 81
screening, 81
testing, 83
collaborations, 210
comfort, 120
commissioning, 14
common ALS genes, 141
communication, 14, 110, 114, 159
co-morbidities, 139
complement system, 184
coping strategies, 87, 116
copper, 45
cortical hyperexcitability, 6
cortical thickness, 69
corticospinal motor neurons (CSMN), 50, 191, 198
cost, 128
Cough Assist, 103
cough, 62
cox proportional hazards regression analysis, 28
creatinine, 6, 34, 65
CSMN, 191
CX3CR1, 150
cyanobacteria, 130
cystatin c (CysC), 174
cysteine, 187
cytokines, 157
cytoskeleton, 193
datacite, 161
decision making, 88, 118, 122
decision-making capacity, 53
DeltaFS (FS), 65
demographics, 138
dendrites, 167
depression, 55, 86, 87, 110, 115
design, 120
diabetes mellitus, 25
diagnosis, 57
diagnostic delay, 5
diagnostic test, 33, 42
diaphragm pacing, 39, 97, 99, 107, 209
diaphragm, 100
dietary intervention, 187
dietician, 96
diffusion tensor imaging (DTI), 32, 43, 69, 71, 72, 73, 78, 79
digital object identifiers (DOI's), 161
digitalisation, 18
dignity therapy, 89, 90
disability, 109, 128
discrete choice experiment, 123
disease
model, 162
modifier, 29
progression, 41, 52, 61, 62, 68, 84, 210
severity, 86
status, 33
disease-modifying drugs, 1
double transgenic mice, 7
Drosophila, 166, 195, 196
drug design, 44
dying, 118
dysarthria, 109
dysphagia, 48, 62, 93, 97, 108
early initiation, 102
early use, 94
eating behaviours, 91
echomyography, 66
education, 124, 166
eHealth, 18
electromyogram, 76
electronic medical record, 122
electrophysiology, 75
elongator, 23
end of life care, 112
end of life, 39, 40, 114, 117, 118
ER stress, 154, 156, 174, 203
endoplasmic reticulum, 12
energy expenditure, 102
environmental risk factor, 130
environmental toxin, 173
EphA4, 149, 185
ephrin-B2, 185
epidemiology, 24, 125, 127, 129, 131, 137, 139, 142
equipment utilization, 119
ER-mitochondria associations, 203
ethics, 118
euthanasia, 31
event related potentials (ERPs), 72
excitotoxicity, 161, 177, 190, 204
exercise, 63, 100, 206
exome sequencing, 29, 146
experiences, 111, 122
expiratory muscle strength training (EMST), 100, 206
extracellular vesicles, 153
extrapyramidal, 60
familial ALS, 137, 141
fasciculation potential, 76
fasciculations, 75
fast skeletal troponin activator, 208
fatty acid metabolism, 187
ferritin, 65
fiber tracking, 72
fibrillation, 7
fibroblasts, 154
financial ability, 90
fitness theory, 132
flow cytometry, 180
fMRI, 77, 80, 85
forced vital capacity (FVC), 99, 103
Force-time curve analysis, 66
FOSMN, 58
fractional anisotropy, 72
frontotemporal dementia (FTD), 3, 53, 69, 70, 78, 81, 82, 83, 91
FTD-ALS, 151
functional
connectivity, 71
disability, 90
status, 87
FUS, 166, 167, 192, 195
FUS/TLS, 23, 35
gastrostomy, 47, 108
gender, 84, 205
gene, 24, 142, 143
carrier, 137
expression, 3, 28
therapy, 197, 198
genetic
association, 28
determinants, 146
mutations, 170
testing, 144
genetics, 134, 145, 146, 147, 148
genome wide association study (GWAS), 28
genomics, 29
gingerol, 203
Gle1, 148
glia, 158
glial
biology, 52
cells, 208
glucose, 6
glutamate release, 177
glutamate, 8
glutamate-mediated excitotoxicity, 188
goals for care, 114
Golgi fragmentation, 186
graph analysis, 71
Group I metabotrophic glutamate receptors, 188
Guam ALS/PDC, 132
guidelines, 117
GWAS, 143, 147, 170
haploinsufficiency, 22
haplotype, 144
head injury, 25
health
foods, 47
politics, 119
services, 123
healthcare, 122
hearing
aids, 110
loss, 110
heat shock protein(s), 183, 204
hematopoietic stem cell, 180
HFE H63D, 181
HFE polymorphism, 182
HIF1 alpha, 185
high-resolution ultrasound, 73
histone acetylation, 167
HMG-CoA reductase inhibitor, 182
hnRNPs, 17
homeobox genes, 150
hospital admissions, 139
H-reflex, 67
HSP110, 183
HSPB8, 172
human adipose stem cells, 188
Huntington’s disease, 5
hypoventilation, 39, 99
illness experience, 114
illness representations, 116
immune activation, 10, 157
immune system, 184
immunohistochemistry, 2, 131
impaired communication, 106
impairment, 91
imputation, 28, 147
in vitro model, 161
incentives, 210
incidence, 133, 136
indirect calorimetry, 101
induced pluripotent stem cells (iPSCs), 162, 164, 165
inflammation, 50, 158
influencing prognostic factors, 150
information seeking, 88
infusion, 199
inhibition, 77
innate immunity, 50
in-patient mortality, 139
instability, 151
insulin dependence, 25
internal conflict, 115
internet search, 88
interneurons, 190
interviews, 89, 111
intracellular
recording, 190
targeting, 44
intracerebroventricular, 9
intrathecal baclofen (ITB), 204
intrinsic functional connectivity, 77
in-vitro
differentiation, 162
fibrillation, 7
iron, 199
Istanbul, 149
Japan, 106
K area, 131
Kii ALS/PDC, 132
Kinect, 76
Kocaeli, 149
lactate-shuttle, 175
lead, 132
lip force, 97
lipopolysaccharide, 199
literature review, 109
LMND, 59
longitudinal analysis, 32
longitudinal, 54, 70
long-term care, 104, 126
long-term mechanical ventilation (LTMV), 160
long-term–follow-up, 204
lower extremity strength, 64
lower motor neuron disease (LMND), 58
magnetic resonance imaging (MRI), 69, 70, 78
magnetoencephalography (MEG), 61
managed care, 119
matrin 3, 29, 169
MCP1, 158
MCP1/CCR2, 50
measurement, 112
mechanical insufflation/exsufflation, 103
mechanically assisted coughing (MAC), 103
medical Medicare, 128
meditation, 89
MEIM, 66
mental representation, 93
mesenchymal stem cell(s) (MSC), 157, 199
mesenchymal stromal cell(s) (MSC), 163, 200
meta-analysis, 81
metabolism, 25, 91, 175, 176, 182
metabolomics, 41
metals, 139
methylation, 143, 144
MGUS, 59
MHCI, 184
microglia, 50, 155
microRNA, 152
microsatellite expansion, 2
microtubule stability, 21
microtubules, 186
military, 24
mindfulness, 89
mirror neuron, 85
misfolded, 163, 201
mitochondria, 154, 175, 176
mitochondrial DNA instability, 151
MND-FTD continuum, 79
mobility, 64
modifier, 149
modifying gene, 150
modulation, 50
molecular chaperone, 183
molecular tests, 145
monocyte, 10
morphine usage, 112
motor neuron, 165
circuitry, 198
motor neurons, 16, 197
motorcontrol, 77
mouse model, 181
mouse, 192
mTOR, 12
multicenter, 43
multidimensional, 54
multidisciplinary
care, 113, 127
team, 124
multinational research, 111
multiple time point, 73
MUNIX, 42, 77
muscle, 41, 162
atrophy, 208
reinnervation, 37
mutation carrier, 152
mutation, 148, 165
myopathy, 136
myosin VI, 153
natural history, 140
neck collar, 120
needle EMG, 74
neural correlates, 79
neuregulin1 antagonist, 200
neurodegeneration, 169
neuroendocrine, 205
neurofilament light chain (Nfl), 32
neurofilament(s), 33, 152
neuroinflammation, 10, 174
neurological diseases, 16
neurons, 158, 191
neuropathological findings, 159, 160
neurophysiology, 61
neuroprotection, 23, 161
neuroprotective, 189
neuropsychiatric symptoms, 90
neuropsychiatry, 134
neuropsychology, 53, 91
neurotransmission, 195
neurotrophic factor(s), 199, 208
new clinical subtype, 58
newly diagnosed, 87
next generation sequencing, 141
NIPPV, 104
NMDAR, 8
NMR, 171
non-invasive ventilation (NIV), 38, 101, 102, 104
non-linear progression, 73
novel gene mutation, 146
novel mutation, 143
NRF2, 16, 202
nuclear body, 167
nucleolar stress, 17
nursing, 124
nutrition, 47, 96, 98
nutritional assessment, 98
nutritional factor, 131
observational studies, 128
oligodendrocytes, 164, 182
ONO1301, 185
optineurin (OPTN), 141, 153
optogenetics, 37
oral secretions, 38
orexin, 186
outcome measure, 76
oxidation, 187
oxidative stress, 16, 33, 47, 63, 155, 176, 181
p.A382T mutation, 142
p62 protein, 143
p62, 156
p62/SQSTM1, 11
p75NTR, 198
pain, 19, 64, 110, 111
palliative care, 39, 90, 118, 205
palmomental reflex (PMR), 75
paraneoplastic, 59
parental survival, 132
partial least squares regression, 133
pathology, 158, 190
pathophysiology, 168
patient counselling, 61
patient empowerment, 19
patient views, 209
patient-derived fibroblast lines, 163
patients stratification, 210
patients’ perspective, 114
patient-ventilator asynchrony, 104
PBL, 124
PBR28, 51
peak cough expiratory flow (PCEF), 103
percutaneous endoscopic gastrostomy (PEG), 93, 94, 95, 96, 97
peripheral immune activation, 68
peripheral immune cells, 184
PET, 51
PGC-1alpha, 175
pharmacodynamics, 207
phenotype, 24, 83, 135
PHQ9, 55
phrenic, 98
physical therapy, 113
physician practice, 64
pluripotency, 16
PML, 167
population based registry, 135, 207
population-based study, 135
preclinical, 6
predict scale, 115
prediction, 41
preferences, 123
preferential vulnerability, 152
premanifest, 5
pre-symptomatic, 5, 68, 73, 137
prevalence, 136, 138
primary lateral sclerosis (PLS), 83
PRO-ACT, 133, 210
proactive care, 119
product innovation, 121
profilin, 171
profilin1, 35, 192
prognosis, 26, 34, 48, 61, 112, 210
prognostic and treatment response, 32
prognostic factors, 135
predictors, 140
progression, 26, 200
progressive muscular atrophy (PMA), 80
proliferation, 50
proteasome, 173
protein
aggregation, 11, 23, 155, 170
disulphide isomerase, 156
homeostasis, 170
misfolding, 7, 45, 153, 173
processing, 52
quality control, 172
translation, 165
proteomics, 169
protocol, 94
psychological, 205
psychosis, 79
psychotherapeutic, 205
Pur-alpha, 165, 166
Q121G, 148
QOL-DyS, 109
qualitative, 112
study, 117
quality improvement, 122
quality of life, 19, 55, 89, 93, 108, 109, 110, 111, 116, 117, 119, 120, 125, 127
quantitative analysis, 76
Rab1, 12
RAN translation, 2, 22
RBM45, 16
reachable workspace, 76
recruitment, 128
rectus abdominis, 74
referral pathway, 57
referral, 57
registry, 160
regression, 133
regulatory T lymphocyte, 157
relative risk, 134
reliability, 83
repeat expansion, 164, 145
reporter line, 195
respiratory, 39, 98
failure, 38, 99
function test, 38, 62
function, 10, 100
management, 107, 206
muscle strength test, 62
support, 232
resting state, 77
resting-state fMRI, 71, 78
retinal degeneration, 166
retrograde transport, 173
RGNEF protein, 143
riluzole, 207, 208
risk factors, 24
RNA, 2, 3
binding proteins, 152
dysregulation, 168
Foci, 2
granules, 23, 172
metabolism, 35, 154
processing, 148
sequencing, 202
toxicity, 22
RNAi, 44
RP-PCR, 144
rs62063857, 149
safety, 94
saitohin (STH), 149
sALS, 149
SAM domain, 23
Sardinia, 142
SBMA, 180, 183
scopolamine, 108
score, 26
screening protocol, 107
screening, 48, 54, 83
sedation, 94
self-assessments, 113
self-report assessments, 107
sequencing, 147
SERCA function, 203
serum, 152
service development, 123
sialorrhoea, 108
SIRT1, 186
sit to stand (STS), 64
skeletal muscle, 183
activation, 207
activator, 10
biomarkers, 154
skin, 131
sleep
disorder, 63
quality, 104
sleep/wake disturbance, 186
slow vital capacity, 10
SMA, 208
Smads, 41
small compounds, 45
snake eyes appearance, 58
social support, 125
social-affective, 91
socioeconomic, 129
SOD1 aggregation, 7
SOD1, 7, 11, 12, 44, 45, 139, 148, 153, 156, 162, 163, 176, 178, 187, 188, 201, 204
SOD1G85R, 183
SOD1G93A, 162, 174, 177, 179, 180, 181, 182, 187, 188
solid cancer, 59
southern blot, 145
Southwest of China, 144
spastic gait disorders, 74
spasticity, 112, 204
Spatial epidemiology, 134
specialists’ perception, 115
speech, 15
spinal
motoneuron, 190
muscle atrophy, 75
splicing, 3
split hand phenomenon, 77
split index, 77
SQSTM1, 143, 156
staging, 133
standards of care, 206
standards, 125
STARD criteria, 42
stem cell-derived motor neuron, 37
stem cells, 16, 200
stress granule(s), 166, 171, 172
structure, 171
support, 87
survey, 112
survival, 26, 55, 65, 66, 95, 102, 130, 132, 133, 135, 136, 207
sustained attention, 72
symptom management, 19, 64
synapse, 194
synapses, 188
synaptic proteins, 177
TARDBP, 142, 179
targeted sequencing, 146
targeted, 198
tau, 25
TDP-43, 7, 8, 21, 25, 35, 36, 149, 164, 166, 171, 172, 193, 195, 196, 203
TDP-43 mutation, 194
TDP-pathology, 60
technology, 14
TGF-b1, 178
theory of mind, 85
therapeutic target, 200
therapeutic, 202
therapy, 45, 206
totally locked-in state, 159, 160
toxicity, 193
TPPV, 126
tracheostomy invasive ventilation (TIV), 104, 105, 106
training, 117
transcranial magnetic stimulation, 6, 42, 74
transcutaneous carbon dioxide, 38
transgenic mice, 36
transgenic mouse model, 7, 35, 36, 50, 184, 192
translating mRNA molecules, 168
translation, 21, 172
transplantation, 199
treatment, 9, 19, 24, 206
trigeminal nuclei, 58
tumor necrosis factor-a (TNF- a), 131, 155
Turkey, 149
Turkish population, 139
ubiquilin 2, 181
ubiquitin, 170
UCHL1, 191
UMN-dominant, 60
United States, 138
upper airway maintenance, 38
upper motor
neuron disorders, 74
neuron dysfunction, 63
neuron signs, 67
neurons, 191
valosin-containing protein (VCP), 136
VAPB, 36
VAPB-PTPIP51 interaction, 203
varicosities, 188
vasodilation therapy, 185
VEGF, 9
ventilation withdrawal, 39, 40
ventilation, 74
verbal fluency, 80
vesicle trafficking, 186
vibration, 67
Voicebank, 15
voxel-based morphometry, 71
weight loss, 96, 97
weight, 48
wheelchairs, 120
yawn, 63
yeast, 169
YFP, 194
young carers, 125
zebrafish, 179