Benign focal amyotrophy

Abstract

Within lower motor neuron diseases, benign focal amyotrophy is a rare syndrome characterized by insidious neurogenic asymmetric atrophy restricted to upper or lower limbs with a good prognosis over time. Described under several terms, the nosology is probably heterogeneous. In juvenile distal upper-limbs forms, specific MRI signs with in particular a compression of the spinal cord by forward displacement of dura, lead to evoke a mechanical process. In other forms, occurring later in the life, affecting proximal part of upper limbs or lower limbs, the physiopathology is still unknown and a focal spinal muscular atrophy is suspected. In this review, we will discuss the clinical, electrophysiological and radiological features of each presentation.

Keywords: Benign; Focal spinal muscular amyotrophy; Hirayama disease; MRI; Motor neuron.