Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative disorder with a fatal outcome and a mean survival time ranging from 2 to 5 years.¹ No treatment is currently available to modify the disease process of ALS. Age,^2,3 site of onset,² functional and respiratory status,^4-6 cognitive dysfunction,⁷ clinical phenotypes,^8,9 serum triglyceride and cholesterol levels,^10-12 creatinine level,^3,13 albumin level,¹³ and C9orf72 expansion¹⁴ have been proposed as possible candidates associated with the prognosis of ALS. However, there is still no consensus on which biomarkers are reliable prognostic factors in ALS.¹⁵

Given the widespread debate on the definition of the terms “Body Schema” and “Body Image”, this article presents a broad overview of the studies that have investigated the nature of these types of body representations, especially focusing on the innovative information about these two representations that could be useful for the rehabilitation of patients with different neurological disorders with motor deficits (especially those affecting the upper limbs). In particular, we analyzed (i) the different definitions and explicative models proposed, (ii) the empirical settings used to test them and (iii) the clinical and rehabilitative implications derived from the application of interventions on specific case reports. The growing number of neurological diseases with motor impairment in the general population has required the development of new rehabilitation techniques and a new phenomenological paradigm placing body schema as fundamental and intrinsic parts for action in space. In this narrative review, the focus was placed on evidence from the application of innovative rehabilitation techniques and case reports involving the upper limbs, as body parts particularly involved in finalistic voluntary actions in everyday life, discussing body representations and their functional role.