Background: Concepts and Features

#### **Chapter 1**

## Anatomy of Cerebellum

*Rajasekhar Sajja Srinivasa Siva Naga*

#### **Abstract**

The cerebellum receives inputs from spinal cord, cerebrum, brainstem, and sensory systems of the body and controls the motor system of the body. The Cerebellum harmonizes the voluntary motor activities such as maintenance of posture and equilibrium, and coordination of voluntary muscular activity including learning of the motor behaviours. Cerebellum occupies posterior cranial fossa, and it is relatively a small part of the brain. It weighs about one tenth of the total brain. Cerebellar lesions do not cause motor or cognitive impairment. However, they cause slowing of movements, tremors, lack of equilibrium/balance. Complex motor action becomes shaky and faltering.

**Keywords:** Cerebellum, Spinocerebellar ataxia, Cortex, Medulla, Peduncles, Nuclei

#### **1. Introduction**

The Cerebellum is the largest part of the hindbrain and develops from the alar plates (rhombic lips) of the metencephalon.

It lies between the temporal and occipital lobes of cerebrum and the brainstem in the posterior cranial fossa. It is attached to the posterior surface of the brainstem by three large white fibre bundles. It is attached to the midbrain by superior cerebellar peduncle, pons by middle cerebellar peduncle, and medulla by inferior cerebellar peduncle.

Cerebellum is concerned with three primary functions: a) coordination of voluntary motor functions of the body initiated by the cerebral cortex at an unconscious level, b) maintenance of balance, and posture, c) Maintenance of muscle tone. It receives and integrates the sensory inputs from the cerebrum and the spinal cord necessary for a planning and smooth coordination of the movements [1].

Cerebellar lesions result in irregular and uncoordinated, awkward intentional muscle movements. Cerebellar lesions often present as occipital headache that worsen at night, nausea, vomiting and unsteadiness. On examination the patients often have bilateral papilledema, nystagmus, slight slurred speech, irregular and ataxic movements [2].

#### **2. External features of cerebellum**

Cerebellum consists three parts; two laterally located **hemispheres** joined in the midline by the **vermis** (*worm*). Somatotopy: Vermis controls the central parts of the body (trunk) and its lesions produce truncal ataxia. And each hemisphere control ipsilateral limb, and their lesion causes ataxia of ipsilateral limbs. Superior surface of the cerebellum is separated from the occipital lobe cerebrum by tentorium cerebelli of dura mater. Superior vermis protrudes above the cerebellar hemispheres whereas the inferior vermis is buried in a deep groove present between the two bulging lateral lobes. The surface of the cerebellum features highly convoluted folds(folia) that are oriented transversely. These folds are separated by fissures of variable depths. Some of the deep fissures can be used as landmarks to anatomically divide the Cerebellum into three lobes: **anterior, posterior** and **flocculonodular lobes**. On the superior surface, a deep **primary fissure** separates the small anterior lobe from the large posterior lobe. On the inferior surface, a prominent **posterolateral fissure** isolates the flocculus of cerebellar hemisphere together with the nodule of the vermis from the rest of the cerebellum as flocculonodular lobe [2].

#### **2.1 Cerebellar lobes (Phylogenetic/Evolutionary and Functional divisions)**

	- It lies anterior to the primary fissure. It regulates the muscle tone. It receives input from muscle spindles (stretch receptor) and Golgi tendon organs (GTOs) through spinocerebellar tract.

b.Posterior lobe (Neocerebellum-Corticopontocerebellar tract)

• It lies between the primary fissure and posterolateral fissure. It regulates the voluntary motor activity. It receives enormous inputs from neocortex through cortico-pontocerebellar tract.

c.Flocculonodular lobe (Vestibulocerebellum- Vestibulocerebellar tract)

• It consists of flocculus and the nodule (vermis). It regulates the maintenance of balance and posture.

#### **2.2 Longitudinal organisation of Cerebellum**

Cerebellum consists of three functional zones that are longitudinally oriented, and these zones are connected to specific cerebellar nuclei.


#### **3. Internal structure of the cerebellum**

Like the cerebral cortex, the **Cerebellum** also consists of outer shell of **grey matter** (cerebellar cortex) and the inner core **white matter**. The white matter consists of afferents and efferent fibres that go to and from the cortex. The white *Anatomy of Cerebellum DOI: http://dx.doi.org/10.5772/intechopen.97579*

mater present underneath the grey mater resembles branches of a tree, hence named *arbor vitae cerebelli* (tree of life). The fibres reach the cortex in a characteristic branch like projections.

The four cerebellar nuclei are distributed deeply within the white mater in each cerebellar hemisphere. The cerebellar nuclei, while connected to the cerebellar cortex, give off the outflow form the cerebellum to the other parts of the brain. The connections are primarily to brainstem nuclei and the thalamus.

#### **3.1 Cerebellar cortex**

On the surface of the cerebellum, a highly convoluted cortex forms numerous transversely oriented folium. The cerebellar cortex is filled with cerebellar neuronal cell bodies, dendrites, and various synapses. The cortex is histologically divided into three layers:

#### a.Outer, **Molecular layer** (Fibre rich)

	- a.**Molecular layer:** It is the outer most layer, present adjacent to the pia mater. *White fibres*: It contains dendritic arborisation of Purkinje cells and parallel fibres of Granule cells. *Cell bodies*: It contains **stellate** (outer) **cells** and **basket** (inner stellate) **cells**.
	- b.**Purkinjee cell layer:** It is present between the molecular and the granule cell layer. It consists of single row of cell bodies of **Purkinje cells**. The dendrites branch extensively and extend into the outer molecular layer. The dendritic branches are flattened in a single axis and are oriented at right angles to the long axis of the folium and the parallel fibres. Because of this arrangement, the branches of Purkinje fibres are perpendicularly traversed by the parallel fibres of molecular layer. The axons of Purkinje cells *form the only output* from the cerebellar cortex. Axons of the Purkinje cells end in cerebellar nuclei (dentate, emboliform, globose, fastigial) and vestibular nuclei and has an inhibitory effect (gama-aminobutyric acid, GABA) on them. Therefore, entire cerebellar output is facilitated through the inhibition of the cells of deep cerebellar nuclei.

**Excitatory input:** Parallel fibres of granule cells (Glutamate) and climbing fibres (Aspartate) excite the Purkinje cells.

**Inhibitory inputs:** Golgi cells, basket cells and stellate cells inhibit (GABA) the Purkinje cells.

c.**Granule cell layer:** It is present between Purkinje cell layer and the white mater of cerebellum and contains granule cells**.** It contains granule cells, Golgi cells, and cerebellar glomeruli. Cerebellar glomeruli are made up of granule cell dendrite, Golgi tenson axon and mossy fibre rosette. The parallel fibres of **granule cells** excite Purkinje cells, basket cells, stellate cells, Golgi cells.

**Excitatory input:** Mossy fibres excite the granule cells. **Inhibitory inputs:** Golgi cells inhibit the granule cells

### **4. White Fibres of cerebellum**

Afferents travel through cerebellar peduncles and reach the cerebellar cortical neurons to stimulate them. Based on the origin, the afferents reaching cerebellar cortex are classified as: 1) Climbing fibres, 2) Mossy fibres.

#### **4.1 Mossy fibres**

The afferent fibres (excitatory) of **spinocerebellar tract, pontocerebellar tract, and vestibulocerebellar tract** are called as mossy fibres. Mossy fibres branch and terminate in an excitatory synapse with the granule cells as mossy fibre rosette, of several folia. The axons of granule cells enter the molecular layer, through Purkinje layer and split to form two **parallel fibres** which run along the long axis of the folium. Mossy fibres excite granule cells which discharge via their parallel fibres.

Spinocerebellar tract Olivocerebellar tra + ct Mossy fibres → → +Granule cells →Parallel fibres

#### **4.2 Climbing fibres**

The afferent fibres (excitatory, aspartate) of **olivocerebellar tract** from contralateral inferior olivary nucleus of medulla are called as climbing fibres. They terminate on the dendrites of Purkinje cells and the deep cerebellar nuclei [4].

#### **5. Cerebellar nuclei**

**Four** cerebellar nuclei lie deep within the cerebellar white matter of each hemisphere. They are arranged from lateral to medial as follows:


#### **5.1 Extracerebellar afferents of cerebellar nuclei**

The collateral branches of Mossy fibers coming from: a) vestibular nuclei, b) reticular nuclei, c) pontine nuclei, d) spinocerebellar tract.

Among the deep cerebellar nuclei, the dentate nucleus with its crinkled bag-like appearance is the largest and the only nucleus visible to the naked eye. The dentate nucleus receives afferent fibers from the inferior olivary nucleus of the medulla, which also looks like a crinkled bag.

#### **5.2 Intracerebellar afferents of cerebellar nuclei**

Purkinje cells of the cerebellar cortex.

#### **5.3 Efferent from Cerebellum**

The majority of the efferent fibers leaving the cerebellum originate from the deep cerebellar nuclei. The efferent fibers reach: a) reticular nuclei, b) vestibular nuclei, c) red nucleus, ventral lateral nucleus of the thalamus.

#### **6. Functional anatomy of cerebellum**

Functionally, anatomically, and phylogenetically/ evolutionarily cerebellum can be divided into Archicerebellum, Paleocerebellum, Neocerebellum.

a.**Archicerebellum** consists of the flocculonodular lobe and nucleus fastigius. It is evolutionarily the first one to develop. **Connections:** Vestibulocerebellar: vestibular receptors of labyrinths, vestibular, and reticular nuclei through inferior cerebellar peduncle, spinal cord. **Function:** Maintenance of balance (equilibrium), posture, and coordination of eye movements.

**Bilateral balance control by Archicerebellum**

Superior colliculus + Striate cortex ➔Inferior cerebellar peduncle➔ Flocculonodular lobe➔Purkinje fibres (cerebellar cortex)➔Fastigeal Nucleus➔ Inferior cerebellar peduncle➔ IPSILATERAL & CONTRALATERAL vestibular nuclei + Reticular formation ➔ Vestibulospinal tract, Reticulospinal tracts➔ Spinal cord.

b.**Paleocerebellum** consists of the vermis, paravermis, fastigial nucleus, emboliform nucleus. **Function:** Controls the tone and posture of the trunk and proximal limb muscles through the vermal-cerebellar pathway. **Connections: Spinocerebellar:** Spinal cord and red nucleus through inferior and superior cerebellar peduncle.

**Contralateral muscle tone and posture control by Paleocerebellum**

Receptors of Muscle, Joint, Skin➔ Dorsal Spinocerebellar tract ➔ Inferior cerebellar peduncle➔IPSILATERAL vermis +paravermis➔ Globose nucleus + emboliform Nucleus+ Fastigial nucleus➔ Superior cerebellar peduncle➔ CONTRALATERAL red Nucleus➔ Rubrospinal tract. Receptors of Muscle, Joint, Skin ➔ Ventral spinocerebellar tract➔ Superior cerebellar peduncle➔ IPSILATERL vermis+ paravermis➔ Globose nucleus+ Emboliform nucleus➔ Superior cerebellar peduncle➔ CONTRALATERAL Red nucleus➔ Rubrospinal tract.

c.**Neocerebellum** consists of the remaining cerebellar hemisphere (except pyramid and uvula) and dentate nucleus. **Function:** Controls the highly skilled muscle coordination and trajectory, speed, and force of movements. **Connections: Cortico-pontocerebellar:** Pontine nuclei, cerebral cortex.

#### **Coordination of movement by Neocortex**

Planning+ execution of movement➔Cerebral cortex➔ corticopontine fibres➔ Pontine nuclei➔ Pontocerebellar fibres➔ CONTRALATERAL middle cerebellar peduncle➔ Lateral parts of cerebellar hemispheres ➔ Dentate nucleus➔ Superior cerebellar peduncle➔ Contralateral Red nucleus (rubrothalamic cells) + Ventral lateral nucleus of Thalamus➔ motor cortex of frontal lobe of cerebrum➔ Corticospinal tract+ Corticobulbar tract

#### **7. Cerebellar Peduncles**

These are the white fibre bundles that join the different parts of the brain stem with the cerebellum.

#### **7.1 Superior cerebellar peduncle (Midbrain**➔ **Cerebellum)**

#### **Afferent fibers from**:


#### **Efferent fibers to**:


#### **7.2 Middle cerebellar peduncle (Pons** ➔ **Cerebellum)**

#### **Afferent fibers from**:


#### **Efferent fibers to: No efferents.**

#### **7.3 Inferior cerebellar peduncle**

#### **Afferent fibers from:**


#### **Efferent fibers to**:


#### **7.4 Major pathways**

Cerebellum-Cerebrum-Cerebellum circuit Purkinje cells ➔ Dentate nucleus ➔ superior cerebellar peduncle➔ dentatothalamic tract➔ Contralateral ventral lateral nucleus of Thalamus ➔ primary motor cortex of precentral gyrus (Brodmann's area 4, motor strip)➔ corticopontine tract➔ pontine nuclei➔ pontocerebellar tract➔ contralateral cerebellar cortex➔ mossy fibers.

### **8. Blood supply of Cerebellum**

The cerebellum is supplied by posterior circulation originated from vertebral arteries. The vertebral artery gives rise to the posterior inferior cerebellar artery (PICA), which supplies the posterior part of the inferior surface of the cerebellum. The basilar artery gives rise to the anterior inferior cerebellar artery (AICA), which supplies the anterior part of the inferior surface. The superior cerebellar artery (SCA) supplies the superior surface of the cerebellum [1].

#### **9. In the clinic**

#### **9.1 Cerebellar disorders**


#### **9.2 Midline lesions**

The midline lesions of the cerebellum (vermis) cause loss of control of trunk posture resulting in truncal ataxia. Patients present with the inability to sit or stand, as there would be involuntary swinging of the body back and forth to stabilize around the center of gravity.

#### **9.3 Unilateral cerebellar lesions**

Cerebellar tracts **do not decussate** like the cerebrum. The symptoms (limb ataxia) produced by the lesions of cerebellar hemispheres are ipsilateral. Unilateral lesions of cerebellar hemispheres cause ipsilateral loss of arm or leg coordination resulting in an unsteady gait (No motor or sensory loss). Limb ataxia can be tested by asking the patient to do a "**heel to shin**" test. When patients with limb ataxia try to walk, the body has difficulty coordinating muscle movements, leading to shifting **the center of gravity**. When there is a fall due to a significant shift in the center of gravity, the fall is usually towards the same **side of the lesion**. The patient often compensates for this by lowering their center of gravity by **wide stepped gait** [5]**.**

#### **9.4 Bilateral cerebellar dysfunction**

Bilateral cerebellar dysfunction causes the following symptoms:

a.**Dysarthria**: slurring of speech


Diseases in which cerebellum is affected bilaterally: a) hypothyroidism, b) alcoholic intoxication, c) multiple sclerosis, d) degenerative diseases, e) metabolic disorders).

**Charcot's triad:** A characteristic combination of nystagmus, dysarthria, and intentional tremor are observed in multiple sclerosis.

#### **9.5 Other causes of cerebellar dysfunction**

Tumours (Astrocytoma, Medulloblastoma, Ependymomas), Hypertensive hemorrhage, cerebellar infarctions.

#### **9.6 Cerebellar dysfunction + Hydrocephalus**

Cerebellar infarctions (edema), cerebellar tumors compressing IVth ventricle.

#### **9.7 Points to ponder**

Anterior vermis syndrome, Posterior vermis syndrome, Hemispheric syndrome [5].

### **10. Conclusion**

Cerebellum consists of median vermis and prominent lateral hemispheres. It forms the roof of the fourth ventricle behind the brain stem. It is attached to the parts of brainstem by cerebellar peduncles which are large white fibre bundles carrying afferent and efferent fibres of cerebellum. Afferent systems of cerebellum include climbing fibres and mossy fibres. It also receives fibres from brainstem reticular formation. Climbing fibres are connected to the contralateral inferior olivary nucleus of medulla oblongata, at one end, and the proximal dendrites of a single Purkinjee cell in the cerebellar cortex, at the other end. Mossy fibres are connected to spinal cord, brain stem, at one end and multiple Purkinjee cells of cerebellar cortex at another end.

#### **11. Cerebellum and Spinocerebellar ataxia**

**Case study:** 55 old male presents with a history of poor hand coordination, slurred speech, rapid eye movements, reduced intellectual function. Physical examination reveals cerebellar ataxia, spasticity, negative Babinski sign. Brain CT scan showed mild cerebral and marked cerebellar atrophy.

**Diagnosis:** Spinocerebellar ataxia.

#### **12. Spinocerebellar ataxia**

**Introduction:** Spinocerebellar ataxias (SCA), are a group of hereditary ataxias transmitted by autosomal dominant inheritance, in which there is a progressive and slow degeneration of cerebellum and certain parts of spinal cord. Among the many types of SCAs, they are classified based on the gene mutation responsible for a specific type of SCA. The types are described as SCA1 through SCA40.

**Symptoms:** The signs and symptoms across the different types generally include abnormal speech (dysarthria), uncoordinated walk (gait), poor handeye coordination, vision problems and difficulty in processing, learning and remembering information. The main symptom include ataxia, where smooth coordination of voluntary motor functions is lost, and there is also nystagmus where the vestibulo-cerebellar fibres and vestibulo-cerebellum are involved. Owing to the degenerative nature of the disease, not only dorsal and ventral spinocerebellar fibres carrying proprioceptive fibres from skeletal muscles and joints, almost all the functions of the cerebellum are affected in Spinocerebellar ataxias.

**Etiology:** Certain types of SCA are caused due to mutation called trinucleotide repeat expansion, where a particular segment of DNA is repeated number of times beyond the tolerable limit. Such nucleotide repeats are unstable and alter their length while passing through generations and often lead to early age onset of the disease. The risk of transmission of the disease from the affected generation to the next is 50%.

**Diagnosis:** If the disease-causing mutation is known then the carrier testing for at-risk relatives and prenatal testing can be done to diagnose the disease.

**Treatment:** There is no specific treatment for SCA. For ataxia, physiotherapy to strengthen the muscles can be done. Physical aids such as crutches and walkers can be used to assist daily activity of the patient [2].

*Spinocerebellar Ataxia - Concepts, Particularities and Generalities*

#### **Author details**

Rajasekhar Sajja Srinivasa Siva Naga Department of Anatomy, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

\*Address all correspondence to: drsrinivas83@gmail.com

© 2021 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

*Anatomy of Cerebellum DOI: http://dx.doi.org/10.5772/intechopen.97579*

#### **References**

[1] Gray's Anatomy. 41st edition. London: Elsevier; 2016:331-348.

[2] Grays Clinical Neuroanatomy. Philadelphia: Elsevier; 2011: 229-244

[3] Snells Clinical Neuroanatomy. Philadelphia: Wolters Kluwer;2019:229-48.

[4] Fitzgerald's Clinical Neuroanatomy and Neuroscience. 7th Edition. New York: Elsevier;2016: 38-39.

[5] Klockgether T, Mariotti C, Paulson HL. Spinocerebellar ataxia. Nat Rev Dis Primer*s*. 2019;5:24. https://doi. org/10.1038/s41572-019-0074-3

#### **Chapter 2**

## Impact of Nutrition in Spinocerebellar Ataxia

*Donnette Alicia Wright and Kadiann Peta-Gay Hewitt-Thompson*

#### **Abstract**

This chapter explores the link between the health outcomes of spinocerebellar ataxia and diet and nutrition as well as overall quality of life and well-being that is achieved as a result of nutritional support and nutritional profile. Spinocerebellar ataxia is a hereditary condition characterized by degenerative changes to parts of the brain, extending to the spinal cord, that affects mobility and voluntary actions. Due to the deteriorating impact of this neurological disorder, the management of health and wellness of the individual is imperative in stemming physiological decline and morbidity. The connections between dietary intake, quality of life and well-being are important components of the health response in providing optimum health outcomes for clients diagnosed with spinocerebellar ataxia. Consequently, an examination of factors that impede, promote and generally affect dietary intake, nutritional status and profile is essential towards improving disease related quality of life and morbidity and mortality risk. The cyclical impact of the neurological condition on nutritional status and its corresponding impact on disease progression is an important exploratory point. Finally, recommendations and standardized guidance are crucial to expanding the health care approach and the overall wellness of individuals with spinocerebellar ataxia.

**Keywords:** nutritional support, quality of life, muscle strength, dysphagia, weight control and wellness

#### **1. Introduction**

#### **1.1 Methodology and literature review**

A content analysis of the literature, especially produced over the last decade, was carried out exploring the condition spinocerebellar ataxia and the possible impact that diet and nutrition may have on the outcome of the condition, utilizing the keywords as a guide. The paper was presented in sections concerning diet and wellness, analyzing each concept and the relationship between diet, nutrition, and spinocerebellar ataxia especially in light of quality of life and wellness.

#### **2. Overview of spinocerebellar ataxia and diet and nutrition**

Spinocerebellar Ataxia is a heterogeneous group of neurodegenerative ataxic disorders with autosomal dominant inheritance. It is an inherited progressive disorder

with clinical features including loss of balance and coordination accompanied by slurred speech. The clinical outcome of this disease is usually manifested in adulthood [1, 2]. This condition is similar to many non-communicable diseases insomuch as it is not transmissible from personal contact and has significant impact on quality of life and wellness. Considering the deteriorating neurological features of the condition, health and wellness maintenance is paramount. Diet and nutrition is featured heavily in its effect on clinical outcomes of this condition. Spinocerebellar Ataxia may have important health impact and nutritional risk profile effect due challenges with swallowing, dysphagia, dependence in meal preparations, muscle and coordination challenges. These physiological changes that are characteristic of spinocerebellar ataxia impact dietary intake and negatively affect lean body mass [3, 4]. Importantly weight loss is a predictor of morbidity risk where sepsis and concomitant illnesses were measured outcome criteria [5]. As a consequence of these physical changes and nutritional impact, the healthcare team must be responsive to the special needs of this cohort of individuals to stem possible negative outcomes. A focus on diet and wellness may accrue to significant health benefits in this population.

Diet and wellbeing are pervasive concepts impacting sociology, psychology, medicine, and human thought. Their relationship to health and happiness are significant to human development and livelihood. This chapter explores the connection between diet and wellbeing especially as they contribute to or impact health generally and particularly that of clients diagnosed with spinocerebellar ataxia. There are important factors that create the link between diet and wellness and include dietary intake, quality of life and wellbeing. The bridge between these concepts will contribute to a better understanding of the impact of diet and wellness and provide summative recommendations for the maintenance of health especially in individuals with neurological disorders such as spinocerebellar ataxia.

#### **2.1 Dietary intake**

Dietary intake is the food and nutrient consumed by an individual daily to maintain life, health, and functionality. Dietary intake is guided by set of recommendations/standards for the daily intake of nutrients and other food components based on the recommended daily allowances. These measurements are used to assess or track food, nutrient or any non-nutritional intake by individuals. The main purpose for assessing an individual's dietary intake is for nutritional screening and surveillance, which can be used to guide research. In individuals with neurological disorders, resting energy expenditure due to hypermetabolism is increased [6]. Resting energy expenditure is the caloric requirement of an individual needed to maintain life and the function of essential organs and systems at rest. It is an important feature of health management in people with neurological illness. Moreover, it is imperative that caregivers and the health care team manage caloric needs in this population, since under supply of energy increases health risks in this vulnerable group of individuals.

#### **2.2 Quality of life**

The World Health Organization defines "Quality of Life as an individual's perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards and concerns" [7]. The quality of life can be affected by an individual's physical health, psychological state, their personal beliefs and social relationships in relation to significant features of their environment. The Centers for Disease Control and Prevention views quality of life as a concept that can be applied to various disciplines to evaluate important aspects of individuals' lives. The concept is usually defined differently for each

#### *Impact of Nutrition in Spinocerebellar Ataxia DOI: http://dx.doi.org/10.5772/intechopen.96904*

discipline. The concept of health-related quality of life has been explored since the 1900s. It evaluates a person's perception of their physical and mental health in relation to their health status and risks, social support and their socioeconomic status. Health- related quality of life also includes the community by identifying the resources, practices and policies of the community that may have an influence on the population's health perceptions and how well they function [8]. Importantly, in spinocerebellar ataxia many variables help to determine quality of life such as the capacity to carry out activities of living, the level of pain or discomfort and the independence to manage self care, Furthermore, the evidence considers disease related quality of life as an important element in neurological disorders and spinocerebellar ataxia in particular. This element of quality of life examines the progression of the disease relative the activities and competencies that the client retains [9]. The greater the physical independence, self care independence, the fewer the neurological deficit along with higher levels of comfort the higher the levels of general quality of life and disease related quality of life [9]. Nutrition and diet are inextricable to quality of life in these patients, so much as well balanced diets, lower health risks, meets energy needs and is associated with reductions in the progression of the disease. These concepts are explored in greater detail in the remainder of the chapter.

#### **2.3 Wellbeing**

The concept of well-being is usually viewed in a positive way for individuals as it indicates that they perceive that their lives are functioning well. An individual that has good living conditions such as proper housing and are employed is essential to their well-being [8]. Well-being is also recognized state where an individual experiences good health, happiness and prosperity, which includes their good mental health, having the ability to manage stress, satisfaction with their lives and the feeling that they have a meaning and purpose in life. Most people aim to achieve well-being because it represents positivity and general health [10].

The quality of life and wellness of an individual with a neurological disorder are affected by a cyclical relationship between nutrition and the disease state. Where poor nutrition is thought to exacerbate the clinical features of the disease and worsened diseases states negatively impact on nutritional risk profile. Specifically, chewing and swallowing difficulties affecting nutrient intake where suboptimal calorie intake is achieved. Furthermore, biochemical and physiology factors of neurological diseases in general and spinocerebellar ataxia in particular affect nutritional status through limited nutrient absorption and utilization, and physiological process affecting gross dietary intake including infection, depression, muscle atrophy, rigidity, tremor, dyskinesia, reluctance to feed, and dysphagia [6]. The combination of these factors may lead to undesired weight loss with worsened risk of infection, and sepsis. Furthermore, poorer nutritional states are associated with worsened ataxia, dyskinesia and tremors. Therefore, it is imperative to evaluate nutritional intake and specifically, caloric intake and expenditure of individuals properly in order to improve the quality of life and enhance health outcomes in clients with neurological diseases.

#### **2.4 Factors that affect dietary intake**

There are many factors that can have a positive or negative impact on an individual's dietary intake. The amount and quality of dietary intake is directly linked to nutritional profile of individuals. Considering the need for appropriate quantities of micronutrients and macronutrients to support nutritional wellbeing, the factors underpinning nutritional intake is essential for review. Some of the most

significant factors that impact dietary intake are appetite, importation, physical condition, built environment and health conditions. These factors will be explored in detail with a view to understanding how positive health outcomes can be accrued. Furthermore, these factors will be examined more closely in the context of neurological diseases and spinocerebellar ataxia.

The factors impacting dietary intake are expansive and are summarized in **Figure 1**.

In many countries, especially developing countries, meals are heavily dependent on importation. However, during a pandemic importation is unreliable and unstable. As a result, these countries are at a higher risk of food shortage [12], which will impact national dietary intake. This means that there may be acute or chronic impact on individual access to food primarily resulting in reduced dietary intake. A pandemic also impacts wages, whether through job loss, reduced working hours. The household purchasing power may be diminished creating impaired food access and decreased dietary intake based on the financial challenges brought on by the pandemic [12]. While importation is not controlled at the individual level, it has important personal impact. Spinocerebellar ataxia has been significantly associated with weight loss and BMI decline due to increases in metabolic demand. The risk of weight loss worsens with disease progression [13]. Importantly, weight loss and BMI decline are treated with regularly adequate intake of energy at the estimated levels to meet total daily energy expenditure. This means that efforts must be made by health care workers, families and government to facilitate safe, and adequate access to food so that energy supply may be appropriate in this population. Importation therefore has a tangential impact on the quality of life of individuals with spinocerebellar ataxia. This exists where importation affects food access, access affects dietary intake, which affects energy balance and body weight. If weight is suboptimal, individuals with ataxia are at greater health risk and the reverse is true when weight is ideal.

**Figure 1.** *Factors affecting dietary intake [11], p. 6.*

#### *Impact of Nutrition in Spinocerebellar Ataxia DOI: http://dx.doi.org/10.5772/intechopen.96904*

There are some health conditions that affect an individual's dietary intake; that may range from challenges with digestion and absorption of nutrients or side effects from prescribed medications that interfere with absorption and utilization of nutrients and dietary adjustments may be needed in medical nutrition therapy for specified health conditions. As part of the treatment of some conditions, clients may be required to eat smaller portions, restrict some food or nutrients, or limit the amount they can tolerate. Clients with spinocerebellar ataxia are similarly afflicted. The condition is reported to impact swallowing capacity, digestion, and nutrient storage, especially fat ([6]; Ko, Qu, Black, & Tso, 2020). Some micronutrients including niacin, thiamine and tocopherol have high risk of deficiency in clients with spinocerebellar ataxia [14–16]. Consequently, nutritional support at times can be adjusted and specially formulated to meet their needs. In some instances where digestion is limited, elemental dietary formulations, texture modifications to support swallowing difficulties may be offered, or where gastrointestinal intake is severely restricted parenteral nutritional application may be necessary.

Nutrition and dietary intake have been established as major factors influencing the quality of life of patients diagnosed with spinocerebellar ataxia. Consequently, it is important to expand the dialog of nutrition in understanding how wellness is affected through dietary influence in this unique population. A major element of physical wellbeing is nutritional status which is directly proportional to the quality of dietary intake. Moreover, social, and emotional wellbeing are affected by dietary intake and nutritional status directly due to the components of food and indirectly because of the perceptions associated with the evaluation of personal nutritional status. Several factors affect dietary intake and include but are not limited to appetite, physiological development, health condition, the built environment, effects of a pandemic and social family settings. These factors will be explored in detail.

#### **2.5 Appetite**

Appetite is an individual's desire to eat food, the body's biological response to a lack of food is hunger. However, an individual's appetite can rise and fall due to various factors, sometimes causing people to eat less or more than their body needs [17]. Appetite can be affected by one's diet, mental health, pregnancy, medications or other health conditions. An individual experiencing a decrease in appetite may lead to a concordant decrease in the general desire to eat food and thus the person may consume less food and nutrients. Appetite is also an important factor impacting the nutritional status of patients with spinocerebellar ataxia. Appetite is primarily affected by the pharmacotherapy approaches used to manage/treat the condition. Drugs including Varenicline and Riluzole are used in the treatment of the neurological features of dystonia and ataxia [18]. The medications are reported to negatively impact on appetite and may potentiate weight loss. These concerns are necessary to be addressed systemically in view of the risks associated with weight loss in spinocerebellar ataxia. Corrective actions including eating by the clock, small frequent meals, colorful and attractive meals, and appetite stimulants may be important to address these appetite changes.

In the general population several personal and psychological factors were examined as factors that contributed to caloric intake. Hunger, appetite, and satiety were identified as important contributors to dietary intake among this population. Appetite was thought to relate to the psychological drive to eat [19]. There remains a concern regarding the factors that regulate appetite but dietary factors including protein and caloric load have been identified as features that influence satiety. At the alternate end of the spectrum, poor appetite is reported as a factor that affects the quality and quantity of food intake in older adults and influences health

outcome and morbidity risk [20]. Markedly appetite exerts an important influence across the lifespan and influences nutritional status and wellness. Appetite may also be affected by the capacity to coordinate movements and feed self especially among the psychological influences. In spinocerebellar ataxia, physiological outcomes including uncoordinated actions limit feeding independence may contribute to reductions in appetite [6, 18, 21]. As medications are introduced to correct physiological impact in these neurological conditions caution needs to be exercised in view of the physical impact that pharmacotherapy may have on appetite. Ultimately, a tight balance must be reached between the management of physical limitations to feeding and the impact on appetite, and pharmacological approaches to managing coordination and their impact on appetite. This must be done with careful observations of diet and appetite especially as the impact on weight balance and health related quality of life.

#### **2.6 The built environment**

Social changes that have led to structural advances have improved the quality of life of the global population. These changes had led to positive financial impact, changes in commerce, trading, and travel. Conversely, it has had instrumental impact on diet and nutritional intake. Furthermore, even the dynamic of the rural population has changed. Several scholars are identifying that caution should be introduced when these benefits are examined. As the referenced changes have also been recognized as significant drivers of changes in dietary quality, where wealth has led to increased dietary intake, particularly energy and serving sizes [22]. Urbanization has led to reduction in the value of home gardens, reduction in the reliance on agrarian subsistence with resulting declines in the consumption of complex carbohydrates [23]. There is also a notable increased in energy dense, nutrient poor and processed foods. Importantly as well, statisticians have associated the structural changes with dietary changes and with significant steep increases in non-communicable diseases, and sharp declines in quality of life. Moreover, there is a concern with changes in the built environment and green spaces for exercise and physical activity. With automation and innovations in construction, there has been a corresponding decline in physical activity with an increased risk of noncommunicable diseases [24]. Physical activity is directly related to nutritional status as physical activity helps to create a balance between caloric intake and expenditure especially as weight maintenance in adulthood is desired. In clients with spinocerebellar ataxia muscle strength, disuse, and incoordination impact on the capacity to engage in the physical activity [6]. Notably, physical activity has been linked to improvement in the quality of life of patients diagnosed with spinocerebellar ataxia. It has been shown to improve physical capacity of the patient, slow the progression of the disease and limit the severity of the physical symptoms of the condition [25]. Furthermore, physical activity has been found to improve antioxidant capacity and reduction in prooxidant damage in patients with spinocerebellar ataxia [26]. It is therefore important, that green spaces are maintained, gyms and other facilities are created, and physical therapy is made available to this population so that the positive influence of physical activity can be accessed by this population.

#### **2.7 Health condition**

Non-communicable diseases account for more than half of the annual deaths in middle eastern countries [22]. The resultant public health advice has been targeted at reducing obesity through dietary and lifestyle changes to produce nutritional and general wellness. Despite the drive to improve dietary intake of

#### *Impact of Nutrition in Spinocerebellar Ataxia DOI: http://dx.doi.org/10.5772/intechopen.96904*

complex carbohydrates, fruits and vegetables and reduce simple sugars, saturated fats and total cholesterol the rates of overweight and obesity continue to rise with a concordant increase in the Disability adjusted life years (DALY) [23]. Neurological disorders categorized as other account for approximately 2.5% of all neurological disorders between 1990–2015 and remains a significant concern especially in developed countries (**Figure 2**). Nevertheless, health condition remains and important

**Figure 2.** *The global burden of neurological disorders ([27], p. 258).*

factor that influences dietary requirements and recommendations. In clients with neurological disorders especially spinocerebellar ataxia, there are several health conditions that impact on nutrition particularly requirements and dietary intake. Importantly the health conditions of these patients are also affected by nutritional status and intake. Individuals diagnosed with spinocerebellar ataxia experience, dysphagia, loss of lean body mass with weight loss, uncoordinated movement, micronutrient deficiencies including- niacin (Vitamin B3), Tocopherol (Vitamin E) and thiamine (Vitamin B1) as well as impaired fat metabolism and increased metabolism and prooxidant activity [3, 4, 14–16]. Dysphagia, uncoordinated movement, and loss of skeletal muscle mass may together create a challenge with feeding and limit dietary intake potentiating weight loss and reducing quality of life. Consequently, texture modification through thickening of food with caregiver support may help to treat swallowing difficulties and improve dietary intake. Limited muscle mass may be treated by introducing branched chain amino acids from supplements and complete proteins [28]. Micronutrient deficiencies constitute a health concern in spinocerebellar ataxia insomuch as they may influence negatively immune response, antioxidant capacity and energy metabolism ataxia [14–16]. It is therefore imperative that biochemical profile assessments are regularly conducted with a view to guide nutritional support. Increases in complete protein may supply adequate niacin levels, while thiamine levels can be improved from animal-based protein and tocopherol levels can be bolstered by consuming vegetable oils, green leafy vegetables and fortified cereals [29, 30]. Impaired fat metabolism is a critical concern in adults in general, and clients with spinocerebellar ataxia in particular, as this anomaly may lead to cardiovascular health risks including atherosclerosis and may also play a role in non-communicable disease development [6, 15]. To mitigate the negative health outcomes of impaired fat metabolism, caution needs to be taken with respect to meal preparation styles, limiting fry and trimming fats, and the selection of foods items [31]. Foods best suited in this case, should limit trans-fat, thereby reducing processed foods as well as increasing the intake of polyunsaturated fats from fish and nut oils while reducing saturated fats from red meats and animal fat. Finally, prooxidant activity in spinocerebellar ataxia can be managed through the reduction of processed foods, which are thought to have higher levels of prooxidant species and include foods with higher antioxidant capacity such as allium vegetables, fruits and a controlled amount of red wine [32]. Furthermore, when the immune status and antioxidant capacity of clients with spinocerebellar ataxia falls to suboptimal level, several nutritional approaches can be employed to improve these health statuses including supplementation and immunonutrition. Diet has been used to positively influence wellness through positive links with supplemental nutritional support, emotional wellness and immunonutrition. Supplemental nutrition includes the addition of a substance or product with the express goal of improving the intake of key micronutrients including vitamins and minerals [33]. This additional intake is aimed at improving nutritional status and wellness and has been a feature of the public health response in LMICs. It may also be employed in dietary options for clients who have low levels of micronutrients or those who are found to be clinically deficient, which is possible in clients with spinocerebellar ataxia. Supplemental nutrition may be formulated as tablets, pills, shakes and other products. Moreover, immunonutriton is a process or product of bolstering the immunity of individuals through the introduction of a targeted nutrients such as amino acids, especially essential and branched chain, essential poly unsaturated fatty acids, nucleotides, and antioxidants. It is particularly useful in reducing recovery time, improving quality of life and health of individuals who are immunosuppressed [21, 34]. This is another strategy that can be coupled with

biochemical analysis to improve the immune status of the client when per os dietary support has not significantly improved antioxidant capacity, immune status, or micronutrient profile.

#### **3. Summary and recommendations**

Diet is associated with Quality of Life and Wellness. Several factors impact on the quality of dietary intake including social, physiological, and psychological issues. The dietary quality has important health outcomes ranging from impacts on Disability Life Years to an increased impact on disease burden, risk of concomitant diseases and impact of disease progression. Nevertheless, diet has been shown to improve body weight, muscle strength, immune capacity, disease progression, life expectancy, recovery, and general wellness especially in clients with spinocerebellar ataxia. Given the significance of diet in influencing the illness wellness continuum, public health officials are constantly challenged to improve the population outcomes using diet. The benefits of diet particularly its impact on improving the quality of life of clients with spinocerebellar ataxia, can be best achieved if portion control, nutrient density and meal planning were to be engaged in this population. Portion control addresses the concerns of caloric intake, through a general reduction in the size and amount of a meal at any single sitting and matches caloric requirements with intake goals [35]. This is associated with a strong positive linear relationship with weight maintenance and disease related quality of life. As energy balance is reached, caloric intake equilibrium is achieved, and weight maintenance is maintained even in high metabolic states. This strategy is particularly important as weight loss is mitigated and weight maintenance is achieved. Weight stabilization is an important factor in spinocerebellar ataxia and is an important determinant of disease progression. Nutrient density is a concept of increasing the range of nutrients consumed, particularly micronutrients, for the smallest value of calories [36, 37]. This allows for the increased functional activity of antioxidants, immune supportive micronutrients, and biological supportive nutrients [38]. The concordant impact on recovery, immune support, and reduction in prooxidant activity is important in wellness especially in clients diagnosed with spinocerebellar ataxia in view of the morbidity risk. Meal planning includes multiple principles that benefit overall dietary pattern through the improvement with calorie intake, through energy control, improving diversity and variety and instituting moderation. These result in planned meals that are better aligned with nutritional guidelines and associated with better health outcomes. Generally, these three principles of portion control, nutrient density and meal planning improve the metabolic profile of the individuals who consistently institute these practices, with improved Body Mass Index (BMI) values, more ideal body weight, healthier body fat and lipid profile with lower chronic disease risk profile. The benefits to be had from diet are best achieved with consistency, moderation and diligence and provide significant sustainable advantages to quality of life and wellness. In clients with neurological disorders, coordinated nutritional support and control are positively associated with better disease related quality of life through weight control, healthy micronutrient status and calorie control. It is essential that these activities are guided by appropriate biochemical and clinical tests and involve personalized and individualized strategies for the most beneficial impact. When instituted in spinocerebellar ataxia, dietary programmes involving principles of meal planning, nutrient density and energy balance redound to significant health outcomes such as weight maintenance, with slowed disease

progression, improved immune status and healthy micronutrient. Therefore, a multi-team approach is recommended if the best health outcomes are to be achieved in spinocerebellar ataxia ensuring the involvement of the nutritionist/ dietician, the biochemist/ phlebotomist, physiotherapist, the neurologist and the general medical practitioner.

### **Author details**

Donnette Alicia Wright\* and Kadiann Peta-Gay Hewitt-Thompson The University of the West Indies, Mona, Kingston, Jamaica

\*Address all correspondence to: donnette.wright02@uwimona.edu.jm

© 2021 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

*Impact of Nutrition in Spinocerebellar Ataxia DOI: http://dx.doi.org/10.5772/intechopen.96904*

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### Section 2
