Abstract
Recent studies have shown the involvement of the sensory nervous system in patients with amyotrophic lateral sclerosis (ALS). The aim of our study was to investigate the correlation between the laryngeal sensitivity deficit and the type of ALS onset (bulbar or spinal) in a large series of 114 consecutive ALS patients. Participants were subdivided into two groups, bulbar and spinal ALS, according to the clinical onset of disease and submitted to a clinical and instrumental evaluation of swallowing, including a fiber-optic endoscopic evaluation of swallowing with sensory testing. Dysphagia severity was scored using the Penetration–Aspiration Scale (PAS) and the Pooling score (P-score). In addition, three patients with laryngeal sensitivity deficit were submitted to a laryngeal biopsy to assess the status of the sensory innervation. All patients showed a normal glottal closure during phonation and volitional cough. Fifty-six subjects (49%), 14 spinal- and 42 bulbar-onset ALS, showed dysphagia at the first clinical observation (PAS score >1; P-score >5). Dysphagia resulted more frequently in bulbar-onset ALS (P < 0.01). Thirty-eight (33%) patients had a sensory deficit of the larynx. The sensory deficit of the larynx was significantly more frequent in bulbar-onset ALS (P < 0.01). The sensory deficit of the larynx among dysphagic patients was also significantly more frequent in bulbar-onset ALS (P = 0.02). Several abnormalities were found in all three subjects who underwent a laryngeal biopsy: in one patient, no intraepidermal fiber was found; in the other two, the fibers showed morphological changes. Our observations are important to consider for assessment and management of dysphagia in patients with ALS.
Highlights
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease causing progressive physical disabilities due to deficits of the motor nervous system
A sensory deficit of the larynx was found in 33% of ALS patients, by means of the sensitivity test performed during
All the patients of our sample showed a normal glottal closure during phonation and volitional cough, assuring us that the failure in laryngeal adduction reflex (LAR) triggering was not related to a motor deficit
Summary
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease causing progressive physical disabilities due to deficits of the motor nervous system. Immunohistochemistry, by means of a selective pan neuronal marker used to highlight peripheral nerve tissue, the pan-axonal anti-protein-gene-product 9.5 (PGP 9.5), has emerged as a valuable tool to analyze sensory and autonomic innervation [4,5,6]. Using this immunohistochemical technique, a significant reduction was found in epidermal nerve fiber density in the distal calf of 28 patients with ALS, supporting the concept of distal axonopathy in ALS [1]. The upper and lower motor neuron contribution to various respiratory muscles can be assessed neurophysiologically using magnetic stimulation of the motor cortex and spinal roots [10]
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