Preface

Spinocerebellar ataxia (SCA) is a neurological pathology that is among the most challenging pathologies in the current medical scenario. SCA has a wide spectrum of clinical presentations, including degenerative and progressive forms with a decline in functional capacity, life-threatening characteristics, and long-standing presentation with great variability in symptoms and clinical severity. Our book addresses the background concepts, generalities, and particularities related to SCA, including clinical, neurological, genetic, and functional aspects. The book also gathers necessary information for functional classification, new approaches to medical and non-medical treatment, and rehabilitation/support, including aspects of palliative supportive care.

The book is divided into three sections. The first section, "Background: Concepts and Features" begins with Chapter 1, "Anatomy of Cerebellum." This chapter examines the functional features, main pathways, and blood supply of the cerebellum as well as clinical generalities and particularities when progressive and slow degeneration of the cerebellum occurs and certain parts of the spinal cord. Chapter 2 discusses the impact of nutrition on SCA, implementing the cyclical impact of this neurological condition on nutritional status and its corresponding impact on disease progression. It also includes recommendations and standardized guidance crucial to expanding the healthcare approach and the overall wellness of individuals with SCA.

In the second section, Chapter 3, aims to help physicians identify the activity and participation aspects of SCA, mainly around the function of mobility that can become a focus of rehabilitation intervention. Neuroplasticity through self-recognition of errors is the main physiology of recovery in SCA.

The final section deals with medical and non-medical interventions. Chapter 4 approaches the therapies of cerebellar neurons derived from human pluripotent stem cells (PSCs), from development to modeling of cerebellar ataxias. It is noteworthy that PSC technology has emerged as an important tool for the generation of different types of neurons, which are used to better understand the development and pathologies of the human nervous system. Strategies for differentiating human PSCs into cerebellar neurons are presented, followed by a perspective for their further optimization and diversification through the implementation of knowledge of cerebellar development and new cell culture approaches. The reported iPSC-derived in vitro models for cerebellar ataxias are reviewed, followed by a perspective on how to improve these models by generating and exporting cerebellar neurons. Chapter 5 presents a narrative review of rehabilitation, which is an important treatment for SCA. The lack of improvement in ataxia, motor learning deficit, and unstable balance cause incapacity for activities of daily living

and restrict participation in social activities, further resulting in a disturbance in the restoration of quality of life. Finally, Chapter 6 addresses the role of palliative medicine within SCA.

#### **Prof. Dr. Patricia Bozzetto Ambrosi**

Independent Neuroradiologist and Neurologist Consultant, Clinical Professor and Academic Researcher, Fellow of European Academy of Neurology, United Kingdom

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