Differences in nerve excitability properties between isolated bulbar palsy and bulbar-dominant amyotrophic lateral sclerosis

Objectives: Isolated bulbar palsy (IBP) is a rare variant that can show a benign course, while progressive bulbar palsy (PBP) has been regarded as a bulbar-dominant type of classical amyotrophic lateral sclerosis (cALS). This study aimed to identify differences in the excitability properties between them. Methods: We consecutively collected data on 22 ALS patients: 13 with cALS, 5 with PBP, and 4 with IBP. An automated nerve excitability test (NET) was applied to measure the strength-duration time constant, threshold electrotonus (TE), current-threshold relationship, and recovery cycle. The axonal excitability properties were compared between the ALS groups and 25 controls. Results: Compared to controls, the cALS group showed a greater change in the depolarizing phase of TE of 90-100 ms after depolarizing current [TEd((90-100))] (53.3 +/- 1.3 [mean +/- SEM] for cALS and 49.0 +/- 0.7 for control, P<0.01) and lower S2 accommodation (19.6 +/- 0.8 and 22.6 +/- 0.7, respectively; P=0.01). There was a nonsignificant tendency for a high TEd((90-100)) pattern to be less prominent in the IBP group than in the PBP group (51.5 +/- 4.22 and 48.8 +/- 1.5, respectively). In addition, all of the parameters of nerve excitability other than S2 accommodation in the PBP and IBP groups did not differ significantly from those in the controls. Conclusions: The excitability properties of IBP and PBP differ from those of cALS. The pattern of NET in PBP was more similar to that in cALS than that in IBP. These findings suggest that IBP is a different entity from bulbar-dominant ALS and PBP.