Dysfunction of bulbar central pattern generator in ALS patients with dysphagia during sequential deglutition
The corticobulbar control of swallowing is insufficient in ALS, and the swallowing CPG cannot work very well to produce segmental muscle activation and sequential swallowing. CPG dysfunction can result in irregular and arhythmical sequential swallowing in ALS patients with bulbar plus pseudobulbar types.
The arhythmical SWS pattern can be considered as a kind of dysfunction of CPG in human ALS cases with dysphagia.
This review examines the evidence regarding the clinical and neurophysiological differences between voluntary and spontaneous swallows. From the clinical point of view, voluntary swallow (VS) occurs when a human has a desire to eat or drink during the awake and aware state. Spontaneous swallow (SS) is the result of accumulated saliva and/or food remnants in the mouth. It occurs without awareness while awake and also during sleep. VS is a part of eating behavior, while SS is a type of protective reflex action. In VS, there is harmonized and orderly activation of perioral, lingual, and submental striated muscles in the oral phase. In SS, the oral phase is bypassed in most cases, although there may be partial excitation. Following the oral phase, both VS and SS have a pharyngeal phase, which is a reflex phenomenon that protects the upper airway from any escape of food and direct the swallowed material into the esophagus. This reflexive phase of swallowing should not be confused with SS. VS and SS are similar regarding their dependence on the swallowing Central Pattern Generator (CPG) at the brainstem, which receives sensory feedback from the oropharynx. There are differences in the role of the corticobulbar input between VS and SS.
Abstract The phases of swallowing are controlled by central pattern-generating circuitry of the brain stem and peripheral reflexes. The oral, pharyngeal, and esophageal phases of swallowing are independent of each other. Although central pattern generators of the brain stem control the timing of these phases, the peripheral manifestation of these phases depends on sensory feedback through reflexes of the pharynx and esophagus. The dependence of the esophageal phase of swallowing on peripheral feedback explains its absence during failed swallows. Reflexes that initiate the pharyngeal phase of swallowing also inhibit the esophageal phase which ensures the appropriate timing of its occurrence to provide efficient bolus transport and which prevents the occurrence of multiple esophageal peristaltic events. These inhibitory reflexes are probably partly responsible for deglutitive inhibition. Three separate sets of brain stem nuclei mediate the oral, pharyngeal, and esophageal phases of swallowing. The trigeminal nucleus and reticular formation probably contain the oral phase pattern-generating neural circuitry. The nucleus tractus solitarius (NTS) probably contains the second-order sensory neurons as well as the pattern-generating circuitry of both the pharyngeal and esophageal phases of swallowing, whereas the nucleus ambiguus and dorsal motor nucleus contain the motor neurons of the pharyngeal and esophageal phases of swallowing. The ventromedial nucleus of the NTS may govern the coupling of the pharyngeal phase to the esophageal phase of swallowing.