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Peripheral nerve abnormalities in pediatric patients with spinal muscular atrophy.

Abstract：

:We examined the specific nerve conduction deficits distinguishing spinal muscular atrophy (SMA) subtypes I and II. Five SMA I patients (age, 0.2-1.1 years) and 10 SMA II patients (age, 1.0-2.8 years) were examined. Patients were compared to age-matched controls for motor and sensory conduction velocity (MCV and SCV) changes, compound muscle and sensory nerve action potential amplitudes (CMAP and SNAP), and F-wave occurrence (FO). Slower MCVs were found in three of five SMA I patients; all five exhibited markedly decreased CMAP amplitudes. Tibial nerve CMAP amplitudes significantly reduced in SMA II patients (p<0.01). Slower SCVs and decreased SNAP amplitudes were observed in three of five SMA I patients but not in SMA II patients. Although FOs were reduced in both extremities of SMA I patients, the reduction was prominent in the tibial nerve of SMA II patients (p=0.031). Loss of motor units may be widespread in the early stage of SMA I, while specific to the legs in young SMA II patients. SMA I showed sensory nerve degeneration, especially of large myelinated fibers. SMA II showed no sensory nerve abnormalities.

journal_name

Brain Dev

journal_title

Brain & development

authors

Yonekawa T,Komaki H,Saito Y,Sugai K,Sasaki M

doi

10.1016/j.braindev.2012.03.009

subject

Has Abstract

pub_date

2013-02-01 00:00:00

pages

165-71

issue

2

eissn

0387-7604

issn

1872-7131

pii

S0387-7604(12)00074-5

journal_volume

35

pub_type

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