LGMD ID
25
Autosomal recessive LGMDs
2A
Incidence Score
100.00
Age of Onset LessThan5
2.00
Age of Onset 5to12
6.00
Age of Onset 13to25
10.00
Age of Onset MoreThans25
2.00
Age of Onset UN
5.00
CK LessThan200
0.20
CK 200 500
0.20
CK 500 2000
0.30
CK 2000 7000
0.22
CK MoreThan7000
0.08
CK UN
0.20
Distal Weakness Primary
0.08
Distal Weakness Significant
0.25
Distal Weakness LNone
0.67
Distal Weakness UN
0.33
Facial Weakness Yes
0.10
Facial Weakness No
0.90
Facial Weakness UN
0.50
Breathing Diff Yes
0.15
Breathing Diff No
0.85
Breathing Diff UN
0.50
Cardiomyopathy Yes
0.09
Cardiomyopathy No
0.91
Cardiomyopathy UN
0.50
EYES AFF
0.03
EYES UNAFF
0.97
EYES UN
0.50
Intellect CNS Affected
0.03
Intellect CNS Normal
0.97
Intellect CNS UN
0.50
Scapular Winging Pronounced
0.40
Scapular Winging Mild
0.30
Scapular Winging No
0.30
Scapular Winging UN
0.33
Calf Hypertrophy Pronounced
0.20
Calf Hypertrophy Mild
0.30
Calf Hypertrophy None
0.50
Calf Hypertrophy UN
0.33
Pain Cramps CEI
0.15
Pain Cramps PB
0.05
Pain Cramps Noone
0.80
Pain Cramps UN
0.33
Joint Contractures Pronounced
0.50
Joint Contractures Mild
0.30
Joint Contractures None
0.20
Joint Contractures UN
0.33
Assym Pronounced
0.10
Assym Mild
0.25
Assym LNone
0.65
Assym UN
0.33
Rate Progress Rapid
0.35
Rate Progress Moderate
0.40
Rate Progress Slow
0.25
Rate Progress UN
0.33
Biopsy M Yes
0.90
Biopsy M No
0.10
Biopsy M UN
0.50
Biopsy Inf Yes
0.25
Biopsy Inf No
0.75
Biopsy Inf UN
0.50
Biopsy VIB Yes
0.10
Biopsy VIB No
0.90
Biopsy VIB UN
0.50
Desmin Yes
0.05
Desmin No
0.95
Desmin UN
0.50
ToeWalk Yes
0.60
ToeWalk No
0.40
ToeWalk UN
0.50
FootDrop Yes
0.25
FootDrop No
0.75
FootDrop UN
0.50
ToeStand Yes
0.15
ToeStand No
0.85
ToeStand UN
0.50
Quad Yes
0.10
Quad Comp
0.30
Quad Spared
0.60
Quad UN
0.33
RipplingMuscle Yes
0.08
RipplingMuscle No
0.92
RipplingMuscle UN
0.50
NasalSpeech Yes
0.10
NasalSpeech No
0.90
NasalSpeech UN
0.50
ElbowContract Yes
0.30
ElbowContract No
0.70
ElbowContract UN
0.50
CardiacCond Yes
0.10
CardiacCond No
0.90
CardiacCond UN
0.50
DilatedMyo Yes
0.03
DilatedMyo No
0.97
DilatedMyo UN
0.50
MacroGlos Yes
0.10
MacroGlos No
0.90
MacroGlos UN
0.50
Skin Affected Yes
0.05
Skin Affected No
0.95
Skin Affected UN
0.50
Head SM
0.05
Head LRG
0.05
Head NML
0.90
Head UN
0.50
Brain Abnormal Yes
0.05
Brain Abnormal No
0.95
Flexible Joints Yes
0.20
Flexible Joints No
0.80
Flexible Joints UN
0.50
Neck Weak Yes
0.15
Neck Weak No
0.85
Neck Weak UN
0.50
More Affected Legs
0.65
More Affected Arms
0.12
More Affected Comp
0.23
More Affected UN
0.33
Finger Cont Yes
0.20
Finger Cont No
0.80
Finger Cont UN
0.50