Macroglossia in Amyotrophic Lateral Sclerosis

Abstract

n amyotrophic lateral sclerosis (ALS), hypoglossal motor neurons degenerate. This is recognized clinically by dysarthria and difficulty manipulating food in the oropharynx to initiate swallowing. Usual findings on examination include tongue atrophy, weakness, fasciculations, pseudobulbar affect in some, and spasticity of jaw musculature. 1 In this case report, we describe 2 atypical ALS cases with enlargement of the tongue (macroglossia).

Non‐motor manifestations in ALS patients with tracheostomy and invasive ventilation

“…We have chosen 11 non‐motor manifestations or complications, and hypothesized that five of the 11 were not just complications of advanced motor disability and long‐term TIV, but also might be ALS‐related unique manifestations. Macroglossia was recently reported as a manifestation of excessive fat accumulation in the tongue, which might be associated with the extremely low energy expenditure and nutritional imbalance observed in patients with advanced ALS . Unstable blood pressure occasionally results in circulatory collapse and sudden death .…”

“…During follow‐up, the following five non‐motor manifestations and six complications were detected: macroglossia, unstable blood pressure, hypothermia, dysuria (urinary catheter placement) and hyperglycemic state as non‐motor manifestations of ALS, and otitis media, pneumonia, urinary tract infection, cholelithiasis, urinary stones, and decubitus ulcers as complications of long‐term TIV intervention. Macroglossia in patients with advanced ALS has been described elsewhere . Briefly, we defined it as a condition in which the tongue was enlarged and always protruded over the patient's dentition and the patient could not volitionally retract the tongue into the oral cavity.…”

“…Macroglossia in patients with advanced ALS has been described elsewhere. 12,13 Briefly, we defined it as a condition in which the tongue was enlarged and always protruded over the patient's dentition and the patient could not volitionally retract the tongue into the oral cavity. To observe the tongue and its position relative to the dentition, the mouth angles were extended laterally using a mouth opener.…”

“…Patients with ALS living with TIV often display non‐motor clinical manifestations over a long disease course, including macroglossia, autonomic dysfunction, glucose intolerance or hyperglycemia, infection, gall bladder or urinary stones, and decubitus ulcers . These may affect the quality of life and survival time of patients; however, their prevalence is unknown.…”

“…However, even when survival is improved with TIV, ALS follows a persistent and progressive disease course, and patients with ALS using TIV have to cope with advancing motor disabilities and communication impairments that can impact their quality of life. 11 Patients with ALS living with TIV often display non-motor clinical manifestations over a long disease course, including macroglossia, 12,13 autonomic dysfunction, [14][15][16] glucose intolerance or hyperglycemia, 17,18 infection, 8,16 gall bladder or urinary stones, 19 and decubitus ulcers. 20,21 These may affect the quality of life and survival time of patients; however, their prevalence is unknown.…”