Abstract: Amyotrophic lateral sclerosis (ALS) is a neurological disease that involves both upper and lower motor neurons. Prevalence is 46 per 100,000 with mean age onset of 56 years. Clinical symptoms start in the bulbar region in about 25% of patients, manifesting as progressive weakness of the oral, lingual and pharyngeal muscles that results in dysphagia, dysarthria and difficulty swallowing saliva. Sialorrhoea is a common complaint, causing difficulties in speech and feeding, skin maceration and social embarrassment. Different strategies have been used to control sialorrhoea. Conservative measures such as postural control usually precede more aggressive measures such as pharmacotherapy and radiation. The use of botulinum toxin (BTX) has been proposed and reported in recent literature. We present a case of ALS with predominant bulbar involvement on a 52-year-old female who consulted for disabling sialorrhoea poorly controlled with previous therapies. Patient presented with severe disartria and oropharyngeal musculature weakness but preserved autonomous competent swallowing. 100 UI BTX were injected under ultrasound guidance transcutaneously, 20UI on each parotid gland and 30UI on each submandibular gland with no immediate complications. Five days after BTX injection, patient was admitted with complete aphagia, dyspnea and bronchoaspiration symptomatology. Immediate introduction of a nasogastric feeding tube was necessary, and permanent percutaneous gastroenterostomy was performed during hospital stay. We describe a serious complication of BTX treatment for sialorrhoea in a patient with bulbar ALS that led to acute deterioration of bulbar function and worsening of overall clinical situation and discuss the actual guidelines for BTX treatment in such patients.

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