Amyotrophic lateral sclerosis is a fatal CNS neurodegenerative disease. Despite intensive
research, current management of amyotrophic lateral sclerosis remains suboptimal from …

We investigated the disease progression and survival in 230 Japanese patients with multiple
system atrophy (MSA; 131 men, 99 women; 208 probable MSA, 22 definite; mean age at …

… (F and G) Large myelinated fibre density was fairly well preserved in 16‐year‐old patient
with a Cx32 mutation (Ser26Leu) (F), while they were markedly diminished and axonal sprouts …

Background The intrathecally administered antisense oligonucleotide tofersen reduces
synthesis of the superoxide dismutase 1 (SOD1) protein and is being studied in patients with …

… , -3 and 4) were found to be compound heterozygotes for a 2254 G→T transition resulting
in a G695V (paternal) mutation, and a 2114 G→A transversion resulting in a W648X (maternal) …

… Segmental demyelination and remyelination clustered on individual fibres, and g ratios (axon
diameter total fibre diameter) in the sural nerve showed an increased scatter in some cases…

Through functional expression screening, we identified a gene, designated Humanin (HN)
cDNA, which encodes a short polypeptide and abolishes death of neuronal cells caused by …

… 1g). In cells with nuclear inclusions, CBP was again redistributed from a pan-nuclear pattern
to concentration within the ataxin-3 inclusions (Fig. 1g… (g–i) HeLa cells expressing full-length …

Background In a previous phase 3 study in patients with amyotrophic lateral sclerosis (ALS),
edaravone did not show a significant difference in the Revised ALS Functional Rating Scale …

We assessed the clinicopathological features of 92 patients with primary Sjögren's
syndrome-associated neuropathy (76 women, 16 men, 54.7 years, age at onset). The majority of …