Article

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.
CI - Copyright (c) 2017 Elsevier Masson SAS. All rights reserved.

Langue : ANGLAIS