Differential motor neuron involvement in progressive muscular atrophy: a comparative study with amyotrophic lateral sclerosis.

ABSTRACT:

Objective

Progressive muscular atrophy (PMA) is a clinical diagnosis characterised by progressive lower motor neuron (LMN) symptoms/signs with sporadic adult onset. It is unclear whether PMA is simply a clinical phenotype of amyotrophic lateral sclerosis (ALS) in which upper motor neuron (UMN) signs are undetectable. To elucidate the clinicopathological features of patients with clinically diagnosed PMA, we studied consecutive autopsied cases.

Design

Retrospective, observational.

Setting

Autopsied patients.

Participants

We compared clinicopathological profiles of clinically diagnosed PMA and ALS using 107 consecutive autopsied patients. For clinical analysis, 14 and 103 patients were included in clinical PMA and ALS groups, respectively. For neuropathological e

SUBMITTER: Riku Y 

PROVIDER: S-EPMC4025414 | biostudies-literature | 2014 May

REPOSITORIES: biostudies-literature