Table 3

Patients with EDMD but without LMNA mutations

PatientSTA analysisAgeDistribution of myopathyContracturesCardiac involvement
PM, pacemaker.
1 FS normal35generalisedrigid spinesick sinus syndrome, PM
2 LM normal20humeroperonealelbow, ankle, rigid spinenone
3 RD normal18humeroperonealelbow, ankle, rigid spinenone
4 Asci normal38proximalrigid spinearrhythmia
5 MaM normal49proximal, distalfinger, elbow, knee, ankle, rigid spinenone
6 KM normal24proximal, distalelbow, anklenone
7 OAr normal17proximal, axialelbow, ankleconduction defect
8 Kra normal13generalisedelbow, rigid spinenone
9 ZPa normal26proximalelbow, anklenone
10 Mara normal12humeroperonealelbow, ankle, rigid spinenone
11 NC3 normal28proximal, distalelbow, ankle, rigid spinenone
12 NG7 normal15proximal, distalelbow, rigid spinenone
13 NI9 normal18proximal, distalelbow, rigid spinenone
14 NK11 normal43proximalelbow, rigid spinenone
15 NM13 normal40proximal, distalelbow, rigid spineconduction defects, PM
16 NQ17 normal17proximal, distalelbow, rigid spinenone
17 NU21 not done25humeroperonealelbow, rigid spinenone
18 NW22 normal34humeroperonealelbow, ankle, rigid spinenone
19 191 normal28humeroperonealelbow, rigid spinenone
20 263 normal24humeroperonealelbow, ankle, rigid spinenone
21 686 normal23humeroperonealelbow, ankle, rigid spinenone
22 2993 normal25humeroperonealelbow, rigid spinenone
23 DAP not done37generalisedelbow, ankle, rigid spineconduction defects