Int Arch Otorhinolaryngol 2014; 18 - a2250
DOI: 10.1055/s-0034-1388997

Speech-Language Performance in Friedreich Ataxia: A Case Report

Rafaela Soares Rech 1, Gabriela Rodrigues da Silva 1, Jerusa dos Santos Dames 1, Lauren Medeiros Paniagua 1, Maria Cristina de Almeida Freitas Cardoso 1
  • 1Fundação Universidade Federal de Ciências da Saúde de Porto Alegre

Introduction: Friedreich ataxia is a progressive neurodegenerative disease with autosomal recessive inheritance. It has typical onset at puberty and displays various functional changes, among them dysphagia and dysarthria.

Objectives: To report the speech-language therapy in the care of a patient with AF in a hospital outpatient clinic.

Case Report: An 18-year-old male patient was referred by Neurology Department for speech therapy with a chief complaint of dysarthria and secondary swallowing. Through history of disease was performed two assessments: dysphagia and dysarthria. By the protocol Speech-Language Evaluation of Neurogenic Oral pharyngeal dysphagia, the patient had difficulty on swallowing for solid and pasty consistency, with multiple swallows, being diagnosed with mild oral pharyngeal dysphagia. By the dysarthria assessment were observed twitching movements with tongue, trembling and monotone voice and imprecise articulation. Five speech therapy sessions were conducted between September 2013 and January 2014 and organized home exercises plan. The focus of rehabilitation was swallowing, aiming for safe and efficient feed, but was also driven to speech exercises. Patient showed clinical improvement in swallowing after five therapy sessions. It was featured in examination of swallowing videofluoroscopy, which demonstrated “power within functional limits with spontaneous compensation.”

Conclusion: After therapeutic sessions the patient reported remission of choking, found in clinical and instrumental evaluation, and also showed improvement in speech. Patient was instructed to keep the speech-language therapy exercises at home as well, keeping facilitators and postural maneuvers to aid swallowing safe and efficient. Patient follows speech therapy.

Keywords: Friedreich ataxia, deglutition disorders, dysarthria.