Relatively little is known about the underlying neuropathology of dysphagia in amyotrophic lateral sclerosis (ALS); thus, effective treatments remain elusive. Tremendous progress toward understanding and treating dysphagia in ALS may be possible through the use of an animal model of dysphagia in ALS research; however, no such animal model currently exists. The most logical candidate to consider is the SOD1-G93A transgenic mouse, the most widely investigated animal model of ALS. To investigate whether this animal model develops dysphagia, oral behaviors (lick and mastication rates) of SOD1-G93A transgenic mice (n = 30) were evaluated at three time points based on hind limb motor function: asymptomatic (60 days), disease onset (approximately 110 days), and disease end-stage (approximately 140 days). Age-matched nontransgenic littermates (n = 30) served as controls. At each time point, lick and mastication rates were significantly lower (p< 0.05) for transgenic mice compared with controls. Histologic analysis of the brainstem showed marked neurodegeneration (vacuolation) of the trigeminal and hypoglossal nuclei, two key motor components involved in mastication and licking behaviors. These results demonstrate a clinicopathologic correlation of oral dysfunction in SOD1-G93A transgenic mice, thereby establishing the SOD1-G93A transgenic mouse as a bona fide animal model of oral dysphagia in ALS.

This article reviews literature relating to the central projection of primary afferent neurons of the trigeminal nerve. After a brief description of the major nuclei associated with the trigeminal nerve, the presentation reviews several early issues related to theories of trigeminal organization including modality and somatotopic representation. Recent studies directed toward further definition of central projection patterns of single nerve branches or nerves supplying specific oral and facial tissues are considered together with data from intraaxonal and intracellular studies that define the projection patterns of single fibers. A presentation of recent immunocytochemical data related to primary afferent fibers is described. Finally, several insights that recent studies shed on early theories of trigeminal input are assessed.

The aim of the present study was to investigate the effects of intramuscular injection with hypertonic saline, a well-established experimental model for muscle pain, on central processing of proprioceptive input from jaw muscle spindle afferents. Fifty-seven cells were recorded from the medial edge of the subnucleus interpolaris (Vi) and the adjacent parvicellular reticular formation from 11 adult cats. These cells were characterized as central units receiving jaw muscle spindle input based on their responses to electrical stimulation of the masseter nerve, muscle palpation and jaw stretch. Forty-five cells, which were successfully tested with 5% hypertonic saline, were categorized as either dynamic-static (DS) (n=25) or static (S) (n=20) neurons based on their responses to different speeds and amplitudes of jaw movement. Seventy-six percent of the cells tested with an ipsilateral injection of hypertonic saline showed a significant modulation of mean firing rates (MFRs) during opening and/or holding phases. The most remarkable saline-induced change was a significant reduction of MFR during the hold phase in S units (100%, 18/18 modulated). Sixty-nine percent of the DS units (11/16 modulated) also showed significant changes in MFRs limited to the hold phase. However, in the DS neurons, the MFRs increased in seven units and decreased in four units. Finally, five DS neurons showed significant changes of MFRs during both opening and holding phases. Injections of isotonic saline into the ipsilateral masseter muscle had little effect, but hypertonic saline injections made into the contralateral masseter muscle produced similar results to ipsilateral injections with hypertonic saline. These results unequivocally demonstrate that intramuscular injection with an algesic substance, sufficient to produce muscle pain, produces significant changes in the proprioceptive properties of the jaw movement-related neurons. Potential mechanisms involved in saline-induced changes in the proprioceptive signals and functional implications of the changes are discussed.

Sensory nerves that supply mechanoreceptors in the mucosal lining of the oral cavity, pharynx, and larynx provide the substrate for a variety of sensations. They are essential for the perception of complex or composite sensory experiences including oral kinesthesia and oral stereognosis. Relevant to the concerns of the oral health care delivery specialist they also contribute to initiation of reflexes and coordination and timing of patterned motor behaviors. The response of oral mechanoreceptors to natural stimuli is determined to a large degree by morphological factors such as the nature of the relationship between nerve ending and certain cellular specializations, their distribution in the mucosa, the diameter of their primary afferent nerve fibers, and the central distribution of these fibers in the brainstem. Because of morphological similarities to certain cutaneous mechanoreceptors, the mucosal lining may be considered as an internal continuation of the large "receptor sheet" for localization and detection of mechanical stimuli. In some regions of the oral, pharyngeal, and laryngeal mucosa, this analogy is appropriate whereas in others, existing data suggest a different role consistent with regionally specific demands (i.e., initiation of protective reflexes).