Abstract Details

Title Evaluation of quality of life of patients with rare neuromuscular diseases in a University Hospital in Rio de Janeiro / Brazil

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) Neuromuscular and Clinical Neurophysiology (EMG) Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 139

Objective Evaluate the relationship between the risk of dysphagia, depression and fatigue and the quality of life of patients with rare neuromuscular diseases.

Background The rare diseases in Neurology are chronic and degenerative neuromuscular injuries with progressive motor and/or bulbar deficit that can compromising the quality of life of patients and their families.

Design/Methods We use the EAT-10, PHQ9, Fatigue Severity Scale questionnaires and it´s relation to quality of life measured by SF 36. Demographic data was also assessed. Patients with spinal muscular atrophy (15,7%), amyotrophic lateral sclerosis (33,3%), spinocerebellar ataxia (2%), chronic inflammatory demyelinating polyneuropathy (2%), Charcot–Marie–Tooth disease (3,9%), limb-girdle muscular dystrophies (9,8%), myotonic dystrophy (3,9%), Krabbe disease (2%), Fabry disease (3,9%), Neurofibromatosis (9,8%), inflammatory myopathies (2%), Pompe disease (5,9%), and porphyria (5,9%) were interviewed. Statistical analysis was performed using SPSS 22.0 version.

Results 51 patients (21 males, 30 females) were aged between 15 and 75 years (42.9 ± 16.9). The highest mean SF 36 subdomain score belonged to mental health (52,471 ± 25.8) and the lowest score to functional capacity (23.5 ± 28.3). The mean EAT-10, PHQ9 and fatigue score were 10.5 ± 13.4; 13.6 ± 8.0 and 43.4 ± 16.1 respectively. There was a negative and statistically significant correlation between EAT-10 and functional capacity and physical aspects (Pearson correlation: - 0.207, p value 0.145; - 0.335, p value 0.16 respectively). Then, as the risk for dysphagia increases, the functional capacity and physical aspects scores decreases (poorer Qol). Additionally, there was a positive correlation between fatigue and depression (Spearman´s rho: 0.566, P value 0.00) and between risk for dysphagia and depression (rho: 0.316, p value 0.024).

Conclusions There is a relationship between the risk of dysphagia and the worst quality of life. Depressed patients tend to present more risk for fatigue and dysphagia, worsening their quality of life.

Authors/Disclosures
Karina L. Pires, MD (Universidade Federal do Estado do Rio de Janeiro) PRESENTER	Dr. Pires has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Elisa G. Gouvea	Miss Gouvea has nothing to disclose.
	No disclosure on file
	No disclosure on file

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Abstract Details