Year: 2024
Type: article
Study design: Case Report
Abstract: Amyotrophic lateral sclerosis (ALS) is a rare, fatal, and progressive neurodegenerative disorder disease characterized by varied clinical presentations, with early motor symptoms being the most prevalent.However, the early phases of the disease may present primarily with bulbar symptoms.Speech and swallowing problems are hallmark features of bulbar onset amyotrophic lateral sclerosis (B-ALS).Patient with B-ALS tend to have a poorer prognosis and show more pronounced neuroimaging changes.This case illustrates the clinical presentation, diagnostic approach, and complications in a 50-year-old female patient presenting with isolated lower motor neuron disease features across multiple body regions, ultimately diagnosed with bulbar-onset amyotrophic lateral sclerosis (ALS) based on the Gold Coast Criteria.Case presentation: A 50 years old female who presented with a progressively worsening difficulty of swallowing, cough with choking and slow speech that had nasal quality with decreased tone for a duration of two years.After structural and local causes were ruled out, a diagnosis of ALS was made with the Gold Coast diagnostic criteria.Conclusion: ALS with bulbar onset can have a grave prognosis and hence requires early diagnosis and a multidisciplinary approach toward effective treatment.Clinicians should be aware of the possibility of ALS presenting as an isolated lower motor neuron disease when it involves at least two body regions in the absence of alternative explanation.
Source: Mathews Journal of Neurology
DOI: 10.30654/mjn.10029
Authors: Malegna Temesgen, Eskindir Kebede, Abraham Kibret, Zelalem Getahun, Tariku Bahiru
Institution: St. Paul's Hospital Millennium Medical College
Language: English
Subfield: Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4406251827