SMA Motor Map - an extensive neurophysiologic protocol to assess (dys)function of the peripheral motor circuits in SMA

Recruiting

Conditions: Spinal Atrophy
Spinal Muscular Atrophy
10029299

Registration Number: NL-OMON49834

Lead Sponsor: niversitair Medisch Centrum Utrecht

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 177

Inclusion Criteria

Part One:
• SMA type 1, 2, 3 and 4, confirmed with a homozygous or heterozygous deletion
of SMN1
• *12 years and older
• Treatment-naïve concerning SMN- modulators or NMJ-modulators (including
pyri-dostigmine and oral salbutamol)
o In case of the use of pyridostigmine by indication of SMA, patients are asked
to stop treatment 1 day prior to the examination

Part Two:
• SMA type 1, 2, 3 and 4, confirmed with a homozygous or heterozygous deletion
of SMN1
• *12 years and older
• Treatment with SMN- modulator

Exclusion Criteria

Strong apprehension against the performance of EMG of any kind

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Part 1<br /><br>CMAP (APB (median nerve))<br /><br>CMAP scan (APB (median nerve))<br /><br>Excitability testing (APB (median nerve))<br /><br>H-reflex (FCR (median nerve))<br /><br>RNS (3Hz at APB (median nerve) and trapezoid (n accesorius); 50Hz at APB<br /><br>(median nerve))<br /><br>SNAP (median/radial/ulnar nerve, sural nerve)<br /><br><br /><br>Part 2<br /><br>CMAP scan (APB (median nerve))<br /><br>SNAP (median nerve, sural nerve)<br /><br>Excitability testing (APB (median nerve))</p><br>

Secondary Outcome Measures