Cortical Thinning of Motor and Non-Motor Brain Regions Enables Diagnosis of Amyotrophic Lateral Sclerosis and Supports Distinction between Upper- and Lower-Motoneuron Phenotypes.

Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder clinically characterized by muscle atrophy and progressive paralysis. In addition to the classical ALS affecting both the upper and lower motoneurons (UMN and LMN), other subtypes with the predominant (or even exclusive) affection of the UMN or LMN have been identified. This work sought to detect specific patterns of cortical brain atrophy in the UMN and LMN phenotypes to distinguish these two forms from the healthy state.

Excitability in somatosensory cortex correlates with motoric impairment in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative motoneuron disease. As previous studies reported alterations in motor cortex excitability, we evaluate excitability changes in somatosensory system. We examined 15 ALS patients and 15 healthy controls.

Muscle imaging data in late-onset Pompe disease reveal a correlation between the pre-existing degree of lipomatous muscle alterations and the efficacy of long-term enzyme replacement therapy.

Background: Late-onset Pompe disease (LOPD) is a metabolic myopathy caused by mutations in GAA and characterized by proximal muscle weakness and respiratory insufficiency. There is evidence from clinical studies that enzyme replacement therapy (ERT) with human recombinant alpha-glucosidase improves motor performance and respiratory function in LOPD.

Objective: We analyzed quantitative muscle MRI data of lower limbs to evaluate the effects of long-term ERT on muscle parameters.

VE-cadherin and ACE: markers for sepsis in post mortem examination?

Altered expression of endothelial markers - especially adhesion molecules - is diagnostically helpful for diagnosis of ante mortal undiagnosed sepsis. Up to now it is unclear whether (1) expression of Angiotensin converting enzyme (ACE) and/or VE-cadherin (VEC) plays a comparable role, (2) whether expression intensity correlates with post mortem interval. Fifty-nine lung specimens (20 lung specimens with regular morphology from tumour lobectomies, 39 from patients who died of septic ARDS due to microbiologically proven Gram-negative sepsis) were stained with an antibody against ACE (1:80) resp.