Benign focal amyotrophy

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-...

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Bibliographic Details
Published in:Revue neurologique 2017-05, Vol.173 (5), p.338-344
Main Author: Cintas, P.
Format: Article
Language:English
Subjects:
Benign
Brachial Plexus Neuritis - complications
Brachial Plexus Neuritis - diagnosis
Brachial Plexus Neuritis - physiopathology
Brachial Plexus Neuritis - therapy
Extremities - physiopathology
Focal spinal muscular amyotrophy
Hirayama disease
Humans
Magnetic Resonance Imaging
Motor neuron
MRI
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Summary: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.
ISSN:0035-3787
DOI:10.1016/j.neurol.2017.03.016