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Abstract
Monomelic amyotrophy (MA) is a variant of motor neuron disease (MND), characterized by muscle weakness and atrophy restricted to one limb. We describe the case of a 56-year-old Italian patient who developed a segmental muscular atrophy limited to the lower left limb. After 11 years of clinical stability he developed progressive spread of the disease to all limbs and to bulbar and respiratory muscles. The patient died from respiratory failure 15 years after disease onset. This case demonstrates that monomelic amyotrophy may rarely evolve to a diffuse fatal MND, even after more than a decade of clinical stability. Our findings support the idea that MA is part of the clinical continuum of MND.
Declaration of interest: A. Chiò serves on the editorial advisory board of Amyotrophic Lateral Sclerosis and has received research support from Ministero della Salute (Progetti Finalizzati), Regione Piemonte (Progetti Finalizzati), Ministero dell’ Università e della Ricerca (PRIN 2007), Università di Torino (fondi ex 60%), Fondazione Vialli and Mauro for ALS Research, ONLUS, and Federazione Italiana Giuoco Calcio. A. Calvo has received research support from Regione Piemonte (Progetti Finalizzati).