Objective: To highlight the role of self-initiated coping strategies in symptoms management of patients with neurodegenerative ataxia.

Background: Ataxia is a neurological condition that manifests itself with lack of coordination. The differential diagnosis is broad, but in the most cases this isn’t possible to treat the underlying cause of the condition. As the disease progresses, ataxia frequently led to speech disorders and consequently impact on communication participation and psychosocial wellbeing. Thus, knowing to what extent communication is affected allows professionals to provide adequate rehabilitation, having a shared decision-making with improvement of patient outcomes.

Method: Case report with accompanying video and literature review.

Results: A 68-year-old man presented with 4-year history of progressive impairment of speech and swallowing. Two years later, his symptoms gradually worsened and he noted loss of dexterity and gait disturbance. He denied diplopia, hearing loss, sensory disturbances or urinary and fecal disturbances. [IC1] He had diabetes for 20 years and history of smoking. The patient was born from nonconsanguineous parents and had no family history of neurological disease. Neurological examination revealed dysarthria, horizontal gaze-evoked nystagmus, limb ataxia on finger-to-nose and heel-to-shin tests, difficulty in tandem walking and a wide-base gait. Normal muscle bulk and strength. No signs of bradykinesia or tremor. Blood tests and brain magnetic resonance imaging (MRI) were unremarkable. The genetic panel for recessive ataxia was negative. Patient was diagnosed with neurodegenerative ataxia of unknown etiology and referred for occupational and speech therapy. During last year, the patient developed depressed mood and anhedonia and was referred for psychiatric evaluation and subsequently medicated. At the last appointment, he presented with a portable voice amplifier, which enabled him to communicate. This self-initiated coping strategy leaded to better compliance in occupational therapy and reduction in antidepressant medication.

Conclusion: This case demonstrates the benefit of self-initiated coping strategies, as reinventing old-fashion devices, in symptom management and patient’s quality of life. Despite suffering from a degenerative disease, assessing the impact of speech disorders is important to determine appropriate symptom approach.

References: Salman MS. Epidemiology of Cerebellar Diseases and Therapeutic Approaches. Cerebellum. 2018 Feb;17(1):4-11;
Ashizawa T, Xia G. Ataxia. Continuum (Minneap Minn). 2016 Aug;22(4 Movement Disorders):1208-26;
Hafiz S, De Jesus O. Ataxia. [Updated 2022 Apr 5]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/speaking-aids-in-neurodegenerative-ataxia-how-to-deal-with-own-disability/