Ricerc@Sapienza

Amyotrophic lateral sclerosis is a progressive neurodegenerative disease involving upper and lower motor neurons. Mean serum creatine kinase (CK) is moderately elevated in ALS. The reason for CK increase is not understood yet: it could be an epiphenomenon of increased motor neuron firing rate or of an increased muscular metabolism. If the second hypothesis is confirmed, this would be another evidence of a central role of muscle metabolism in ALS progression. Moreover, no significance of CK levels for prognosis is already known.
The aim of this study is to evaluate CK levels in ALS patients over time, related to the rate of disease progression, and differences with other neuropathies such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with secondary axonal damage.
ALS patients will be recruited in the center of neuromuscular disease and divided in two groups, i.e. fast or slow disease progressors, according to the average monthly reduction on ALS functional rating scale (ALSFRS) score; CK levels will be assayed for 16 months with a mean interval of 4 months. Relationship with site of onset and nerve compound motor action potential (cMAP) will be analyzed.
CIDP patients with secondary axonal damage will undergo CK measurements at baseline. This control population shares with ALS a similar axonal motor damage and increased motor neuron firing rate in survived motor axons, but no changes in muscular metabolism.
Any difference in CK levels between the two population will confirm a key role of muscle in ALS. Morever, CK could be associated to disease progression and prognosis il ALS patients.