Functional studies of the parotid and pancreas glands in amyotrophic lateral sclerosis

Abstract

SYNOPSIS Functional studies of the pancreas and parotid glands are reported in 17 patients with amyotrophic lateral sclerosis (ALS). The exocrine function of the pancreas was studied by measuring amylase concentration after stimulation with the endogenous secretin-pancreozymine test (ESP). Under these conditions, the pancreatic amylase concentration in ALS patients was found to be markedly decreased by about 450 when compared with those of healthy control subjects.Different conclusions in the literature about a possible impairment of the exocrine pancreas in ALS patients induced us to study the function of the parotid gland, which has close structural, functional, and physiopathological relationship with the pancreas. Flow rate and bicarbonate concentration of parotid saliva were measured after indirect stimulation (intraoral citric acid) and direct stimulation (pilocarpine). After indirect stimulation, both parotid flow rate and bicarbonate concentration from ALS patients were found to be decreased by about 66% and 700 respectively, when compared with controls. On the other hand, direct stimulation with pilocarpine in ALS patients elicited normal responses in both flow rate and bicarbonate concentration of saliva. It is concluded that the pancreatic and parotid deficiencies observed in ALS patients do not indicate primary disease of these exocrine glands. This interpretation is further emphasized by the results obtained by a sweat test, plasma osmolarity, and sialographic studies. The possibility that the gland impairments observed might be due to modifications of the neuroendocrine mechanisms regulating their secretory activity is suggested.

Practice parameter: The care of the patient with amyotrophic lateral sclerosis (an evidence-based review) [RETIRED]

“…Saliva production is actually decreased in patients with ALS. 13,14 Thus, poor handling of saliva appears to be the major cause of sialorreha in ALS and in other disorders such as cerebral palsy, mental retardation, developmental disability, Down syndrome, and oropharyngeal carcinoma. In these patients sialorrhea has been managed by attempting to decrease saliva production, improve handling of secretions, or divert and remove saliva.…”

Impaired salivary gland function reveals autonomic dysfunction in amyotrophic lateral sclerosis

“…We therefore conclude that sialorrhea in ALS patients is not primarily caused by an enhanced salivary flow rate but is rather the result of dysphagia. Most likely, low 99m Tc-pertechnetate uptake reflects an alteration of neuroendocrine function of the salivary glands, which is supported by previous stimulation studies of Charchaflie [2]. We cannot exclude that differences of 99m Tc-pertechnetate uptake between bulbar ALS patients and controls may be jeopardized by bulbar symptoms.…”

“…Both sympathetic and parasympathetic dysfunction were found [3,4,9,10,12,13]. Charchaflie et al [2] described a reduced parotid flow rate and bicarbonate concentration after indirect stimulation of the glands with intraoral citric acid in 10 ALS patients. Direct stimulation of the glands with pilocarpine elicited normal responses in both flow rate and bicarbonate concentration of saliva in five ALS patients.…”

Unilateral parotid electron beam radiotherapy as palliative treatment for sialorrhea in amyotrophic lateral sclerosis

“…Saliva production is normal or reduced in ALS [3,11,17] and thus the mechanism(s) for drooling include dysphagia, weakened facial muscles and mouth closure, and poor head control. Although radiotherapy is an effective adjunctive treatment for drooling in ALS [4], the optimal approach has not been defined.…”

“…When the bulbar musculature is weak, patients experience dysphagia, especially for liquids. Impaired swallowing and inability to maintain mouth closure, coupled with normal saliva production, cause sialorrhea [3,4]. The resultant drooling decreases quality of life (QOL), causes social embarrassment, further limits their ability to speak and maintain proper respiratory function, and increases the risk for aspiration [5].…”