**1. Introduction**

The global prevalence of spinocerebellar ataxia is 1 to 5 per 100,000 but higher in Asian populations while lower in European countries [1]. Ataxia is the loss of neural control over muscular tone and function affecting gait, coordination, eye movement, and speech; swallowing and other voluntary activities are also affected. Spinocerebellar ataxia (SCA) is a large and complex group of hereditary (genetic, familial), progressive, neurodegenerative, and heterogeneous disease that mainly affects the cerebellum. SCA is a subgroup of inherited cerebellar ataxia and is a rare disease. Currently, in excess of 40 distinctive genetic SCAs have been identified. The genetic contributions and impact of SCA is very complex to understand but is critical to diagnosing and classifying the disease which determine treatment approaches [1, 2]. SCA presents clinically with several symptoms related to the neuromuscular systems and is characterized by cerebellar gait and limb ataxia (with dysmetria, dysdiadochokinesia, intention tremor, dysarthria, and nystagmus), which may be

present together with extracerebellar signs, such as ophthalmoplegia, pyramidal signs, movement disorders (including parkinsonism, dystonia, myoclonus, and chorea), dementia, epilepsy, visual disorders (including pigmentary retinopathy), and peripheral neuropathy. These symptoms contribute to significant physiological impact which influences Disability Adjusted Life Years (DALY). One DALY is the equivalent of one healthy life-year lost and contributes to the global disease burden [3, 4]. Individuals diagnosed with SCA may present clinically with self care deficits and experience increased nutrient requirements coupled with limitations in dietary intake. The progressive nature of the disease makes it critical for early nutritional intervention and management to correct, resolve and limit the negative health and nutrition outcomes associated with the disease. Consequently, health care practitioners must collaborate to create the best therapeutic outcome for the client diagnosed with SCA.
