Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease involving nerve cells that control voluntary muscle movement (Rowland LP, Shneider NA, N Engl J Med 344(22):1688-1700, 2001). The aim of this study were to determine the pattern of neurodegenerative change in (1) isometric tongue strength (ITS) and spontaneous saliva swallow (SSS) pressure, (2) saliva weight, and (3) forced vital capacity (FVC) in patients with ALS who present with primary spinal versus primary bulbar symptoms. Twenty-three consecutive patients (age=48-80years, mean=59.5years) were enrolled. Data were collected over three visits (12-week interval) for each group: 9 patients with bulbar symptoms and 14 with spinal symptoms. A significant difference was noted in SSS and ITS in the group with bulbar symptoms from Trial 1 to II and from Trial II to III. SSS and ITS showed a significant difference when comparing Trial I to III but not when comparing Trial I to II for the spinal symptom group, indicating that this group experienced a slower decline in SSS. Saliva production did not show a significant change in the bulbar symptom group but did in the spinal group. FVC was significantly different when comparing Trial I to III and Trial II to III for both groups. FVC, SSS, and ITS may be complimentary measures used as a gauge of an ALS patient's ability to efficiently take oral nutrition and to support required alterations in diet consistency.

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