[Chronic spinal amyotrophy involving the upper limbs in young adults (O'Sullivan and McLeod syndrome). MRI study of the cervical spinal cord].
In 5 cases of sporadic spinal muscular atrophy in young adults the muscular atrophy was localized in the hands and forearms. Age at onset ranged from 7 to 20 years. The duration of the disease was less than 5 years in 3 patients and more than 15 years in 2. Electromyography showed neurogenic anomalies in all wasted muscles, denervation potentials being also present in the proximal muscles of the upper limbs and distal muscles of the lower limbs in 2 patients. Motor nerve conduction velocity and sensory nerve action potentials were normal. Advantages of a topographic classification of the distal form of chronic spinal muscular atrophy are discussed. Spinal cord MRI was performed in 4 patients with a 0.5 Tesla superconducting magnet using surface coils, using a T1-weighted spin-echo technique (ET 26 ms; RT 500 ms) and a T2-weighted spin-echo technique (ET 90, 180 ms; 2,000 ms). With the T1 technique, axial MRI sections 7 to 9 mm thick of spinal cord showed a normal image between C1 and C4, and a flattened image between C5 and T1. This was considered as a possible spinal cord segmental atrophy.
Gaio
J M
JM
Clinique Neurologique, CHU Grenoble.
Lechevalier
B
B
Hommel
M
M
Viader
F
F
Chapon
F
F
Perret
J
J
D000293:Adolescent; D000328:Adult; D001132:Arm; D002908:Chronic Disease; D004576:Electromyography; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009134:Muscular Atrophy, Spinal / Q000473:pathology* / Q000503:physiopathology; D009431:Neural Conduction; D013116:Spinal Cord / Q000473:pathology*
Amyotrophie spinale chronique des membres supérieurs de l'adulte jeune (syndrome de O'Sullivan et McLeod). Etude en IRM de la moelle cervicale.