Table 1.
ALS autopsy cases (N=76) included in this study; data is shown as median and interquartile range (in parentheses).
Site of onset | ALS (all) | BLMN | BUMN | CLMN | LLMN | LUMN |
---|---|---|---|---|---|---|
N | 76 | 16 | 6 | 25 | 19 | 10 |
Gender [f/m] | 30/46 | 5/11 | 3/3 | 9/16 | 9/10 | 4/6 |
Duration [mo] | 33 (24-60) | 30 (23-39) | 24 (23-33) | 30 (22-46) | 34 (30-62) | 58 (28-96)* |
Age at death [yrs] | 63 (56-70) | 66 (60-71) | 64 (46-72) | 62 (55-71) | 61 (58-66) | 58 (56-74) |
ALSFRS-R | 18 (14-23) | 24 (14-26) | 24 (19-30) | 18 (15-22) | 17 (14-23) | 21 (13-27) |
MMSE | 29 (26-30) | 29 (27-30) | 25 (24-28) | 29 (28-30) | 28 (27-29) | 29 (28-30) |
Brain weight [g] | 1353 (1231- 1427) |
1359 (1333- 1453) |
1352 (11258- 1473) |
1365 (1222- 1456) |
1323 (1252- 1420) |
1233 (1162- 1388) |
Statistical significance across ALS syndromes in Kruskal-Wallis One Way Analysis of Variance on Ranks, p = 0.02. Abbreviations: ALSFRS-R – ALS functional rating scale, revised version, BLMN – bulbar lower motor neuron onset of disease, BUMN – bulbar upper motor neuron onset of disease, CLMN – cervical lower motor neuron onset of disease, duration – duration of disease, f/m – female/male, g – grams, LLMN – lumbar lower motor neuron onset of disease, LUMN – lumbar upper motor neuron onset of disease, mo – months, yrs – years.