Table 3

MisSOD1 immunohistochemical results

ANumber of patients with misSOD1 inclusions in spinal cord motor neurons
SOD1 fALSC9orf72HRE expansionOther ALS mutationsControls, neurodegenerativeControls, non-neurological
Cervical spinal cord17 (17)17 (18)11 (11)1 (10)0 (14)
Thoracic spinal cord16 (16)15 (15)11 (11)1 (3)0 (18)
Lumbar spinal cord16 (16)15 (16)11 (11)4 (10)2 (19)
BProportion of motor neurons with misSOD1 staining
SOD1fALSC9orf72HRE expansionsOther ALS mutationsControls, neurodegenerativeControls, non-neurological
Cervical spinal cord+ (+ – ++)++ (0 – +++)++ (+ – ++)0 (0 – ++)0 (0)
Thoracic spinal cord+ (+ – ++)++ (+ – +++)++ (+ – ++)0, 0, ++*0 (0)
Lumbar spinal cord+ (+ – ++)++ (0 – +++)++ (+ – ++)0 (0 – +)0 (0 – +)
  • Table of findings from sections stained with the131–153 Ra-ab and 57–72 Ra-ab. Data for number of patients with misSOD1WT inclusions show the total number of patients in parenthesis. Data for proportion of misSOD1WT inclusions with staining are shown as median (range) referring to a four-tiered semiquantitative scale (0=no glial or motor neuron with staining; + ≤25% of the motor neurons with staining; ++=25%–75% of the motor neurons with staining; +++ ≥75% of the motor neurons with staining). The total number of patients in each group was as follows: 17 SOD1, 18 C9orf72HRE, 11 other ALS mutations (2 with FUS, 1 ALSIN+KIF5 A, 1 VAPB, 1 NEK1, 6 KIF5A); 10 Controls neurodegenerative, 20 Controls non-neurological. Sections from all levels were not available from all patients. In total, of the 20 non-neurological controls, only two had some staining. *Since thoracic spinal cord only was available from three patients from the neurodegenerative control group each result is presented individually.

  • ALS, amyotrophic lateral sclerosis; fALS, familial ALS.