Format

Send to

Choose Destination
See comment in PubMed Commons below
Muscle Nerve. 2013 Jul;48(1):117-21. doi: 10.1002/mus.23722. Epub 2013 Apr 30.

Sensorimotor polyneuropathy in patients with SMA type-1: electroneuromyographic findings.

Author information

1
Akdeniz University Hospital, H Block, Department of Child Neurology, Antalya, Turkey. oduman@akdeniz.edu.tr

Abstract

INTRODUCTION:

Generally, spinal muscular atrophy (SMA) is believed to be a pure motor neuron disease. We retrospectively evaluated our electrodiagnostic findings in SMA type 1 patients to demonstrate co-existence of sensorimotor neuropathies.

METHODS:

Electroneuromyographic (ENMG) studies in 15 patients (11 boys, 4 girls) were reviewed independently by 2 neurophysiologists. Upper extremity findings were compared with normal right arm controls.

RESULTS:

Patient ages ranged from 1.5 to 26 months. Four SMA patients (26.7%) had decreased sensory nerve action potentials (SNAPs) or sensory nerve conduction velocities. Of them, median SNAPs could not be elicited in 3, and sural SNAPs could not be elicited in 2. Compound muscle action potential amplitudes were severely decreased in 14 (93.3%) and normal in 1.

CONCLUSIONS:

Survival motor neuron 1 (SMN1) gene analysis should be considered if clinical features are consistent with SMA, even if pathological or electrophysiological findings demonstrate peripheral sensorimotor polyneuropathies.

PMID:
23629817
DOI:
10.1002/mus.23722
[Indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Loading ...
    Support Center