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. 2019 Apr 1;10(2):278–292. doi: 10.14336/AD.2018.0917

Table 1.

General and clinical characteristics of the study subjects in the muscle biopsy study cohort.

Type of cohort (N) Muscle biopsy cohort* (N = 87)
ALS patients ONP patients Healthy controls
Patients' characteristics (n =49) (n = 14) (n = 24)
Gender (n) Female 15 8 19
Male 34 6 5
Age at illness onset (mean ± SD) 57.02 ± 12.42 43.79 ± 16.02
Age at biopsy (mean ± SD) 58.65 ± 12.52 42.34 ± 25.89 55.07 ± 26.07
Disease duration, months (mean ± SD) 19.54 ± 25.15
Clinical Phenotype (n) ALS, spinal 42
ALS, bulbar 6
ALS + FTD 1
OMD 5
SMA 5
CIDP 1
KD 1
NMD 1
AD (Early) 1
ALSFRS-r at biopsy (mean ± SD) 40.24 ± 6.20
El Escorial criteria at onset (n) Unavailable 33
Defined 1
Probable 6
Possible 5
Suspected 4
Genetic Diagnosis (n) SALS 26
FALS 6
SALS + FTD 2
MND-FTD 1
PLS-SALS 1
PMA-SALS 1
Unavailable 12
*

All subjects were of caucasian ethic group.

ALS, amyotrophic lateral sclerosis; SALS, sporadic ALS; FALS, familial ALS; ALSFRS-r, the revised ALS Functional Rating Scale; ONP, other neuropathies; FTD, frontal temporal dementia; OPMD, oculopharyngeal muscular dystrophy; SMA, spinal muscular atrophy; CIDP, chronic inflammatory demyelinating polyneuropathy; KD, Kennedy Disease; MND, motor neuron disease (cervical myelopathy); AD, Alzheimer Disease (early); PLS, Primary Lateral Sclerosis; PMA, Primary Muscular Atrophy.