Diagnosis of Unusual Case of Dysphagia with Macroglossia and Rigid Tongue

Excerpt

A 61-year-old male presented with a progressive dysphagia with macroglossia and rigid tongue of 2-year duration. His prior history included carotid artery occlusion disease and hypothyroidism. He had visited an otolaryngologist for tongue pain 4 years previously when he was diagnosed with oral ulcer and given conservative management with observation. He had undergone neck CT then, but no significant abnormality was found. Subsequently, he was lost to follow up until 2 years ago. Back then, he revisited the otolaryngologist for swelling and rigid movement of tongue, neck CT was done again suggesting tongue cellulitis, and oropharyngeal MRI showed no abnormality except benign reactive lymph node. He was given conservative management again. However, his symptoms had not subsided, rather he had developed progressive dysphasia since then. He revisited our hospital for dysphagia, which was accompanied with dysarthria. He had undergone brain CT for the evaluation of dysphagia just before this presentation that showed chronic basal ganglia lacunar infarction (~ 0.5 cm), which did not suggest severe dysphagia with dysarthria. At our dysphagia clinic, physical examination showed that the patient had macroglossia and rigid tongue (his tongue rarely moved), which resulted in a highly restricted range of motion. He had been on a puree diet and showed coughing sign of aspiration during liquid swallowing. Other systemic examinations were normal. On investigation, his chest X-ray showed no specific abnormality, but a videofluoroscopic swallowing study (VFSS) revealed oral phase dysfunction. Tongue motions for searching and mixing food were diminished for liquid and solid foods. He showed drooling with liquid (Fig. 1). Swallowing reflex was normal, but during the pharyngeal phase, decreased elevation and closure of larynx were observed. Food remnants were observed in the vallecular recess and pyriform sinus after swallowing. Liquid aspiration and semiliquid and semisolid penetration were evident (Fig. 1). Liquid aspiration corresponded to a Penetration Aspiration Score (PAS) of 8, representing aspiration with no coughing. Aspiration happened during and after swallowing phase due to reduced laryngeal and epiglottis movements and remnants in the vallecular recess and pyriform sinus. Pharyngeal peristalsis was also decreased. Laboratory examination showed the presence of anemia and hypercalcemia; hemoglobin was 7.1 g/Dl (normal range (NL): 13.1–17.5 g/dL), calcium was 14.6 mg/dL (NL: 8.6–10.7 mg/dL), and ionized calcium was 1.87 mmol/L (NL: 1.05–1.35 mmol/L). What is the diagnosis and management of this patient?

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