Creatine Kinase and Progression Rate in Amyotrophic Lateral Sclerosis
Marco Ceccanti,
Valeria Pozzilli,
Chiara Cambieri,
Laura Libonati,
Emanuela Onesti,
Vittorio Frasca,
Ilenia Fiorini,
Antonio Petrucci,
Matteo Garibaldi,
Eleonora Palma,
Caterina Bendotti,
Paola Fabbrizio,
Maria Chiara Trolese,
Giovanni Nardo,
Maurizio Inghilleri
Affiliations
Marco Ceccanti
Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy
Valeria Pozzilli
Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy
Chiara Cambieri
Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy
Laura Libonati
Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy
Emanuela Onesti
Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy
Vittorio Frasca
Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy
Ilenia Fiorini
Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy
Antonio Petrucci
Centre for Neuromuscular and Neurological Rare Diseases, San Camillo Forlanini Hospital, 00152 Rome, Italy
Matteo Garibaldi
Neuromuscular Disease Centre, Department of Neurology, Mental Health and Sensory Organs (NESMOS), Sant’Andrea Hospital, Sapienza University of Rome, 00189 Rome, Italy
Eleonora Palma
Department of Physiology and Pharmacology, Laboratory Affiliated to Istituto Pasteur Italia, Sapienza University of Rome, 00185 Rome, Italy
Caterina Bendotti
Laboratory Molecular Neurobiology, Department of Neuroscience, Istituto di Ricerche Famacologiche Mario Negri-IRCCS, 20156 Milan, Italy
Paola Fabbrizio
Laboratory Molecular Neurobiology, Department of Neuroscience, Istituto di Ricerche Famacologiche Mario Negri-IRCCS, 20156 Milan, Italy
Maria Chiara Trolese
Laboratory Molecular Neurobiology, Department of Neuroscience, Istituto di Ricerche Famacologiche Mario Negri-IRCCS, 20156 Milan, Italy
Giovanni Nardo
Laboratory Molecular Neurobiology, Department of Neuroscience, Istituto di Ricerche Famacologiche Mario Negri-IRCCS, 20156 Milan, Italy
Maurizio Inghilleri
Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with no recognized clinical prognostic factor. Creatinine kinase (CK) increase in these patients is already described with conflicting results on prognosis and survival. In 126 ALS patients who were fast or slow disease progressors, CK levels were assayed for 16 months every 4 months in an observational case-control cohort study with prospective data collection conducted in Italy. CK was also measured at baseline in 88 CIDP patients with secondary axonal damage and in two mouse strains (129SvHSD and C57-BL) carrying the same SOD1G93A transgene expression but showing a fast (129Sv-SOD1G93A) and slow (C57-SOD1G93A) ALS progression rate. Higher CK was found in ALS slow progressors compared to fast progressors in T1, T2, T3, and T4, with a correlation with Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) scores. Higher CK was found in spinal compared to bulbar-onset patients. Transgenic and non-transgenic C57BL mice showed higher CK levels compared to 129SvHSD strain. At baseline mean CK was higher in ALS compared to CIDP. CK can predict the disease progression, with slow progressors associated with higher levels and fast progressors to lower levels, in both ALS patients and mice. CK is higher in ALS patients compared to patients with CIDP with secondary axonal damage; the higher levels of CK in slow progressors patients, but also in C57BL transgenic and non-transgenic mice designs CK as a predisposing factor for disease rate progression.