Ataxia and me – living within a neurological hierarchy

Abstract

By its very nature, the term Neurodiversity promotes the concept of biodiversity, the wonderful realm of neurocognitive functioning. Why is it that a paradigm that sets out to celebrate and give voice to those belonging to the neurodiverse community ostensibly silences those with rarer conditions? Having a rare neurological condition often intrinsically means living within the neurodiverse community alongside those with more prevalent ailments yet simultaneously living diametrically opposed. More uncommon and misunderstood manifestations are often demonised and condemned as they deviate from what society deems as normal. The neurodiverse community has been limited to those with more prevalent and understood illnesses due to a lack of awareness surrounding other neurological disorders such as ataxia. This article questions the presence of a hierarchy within the neurodiverse community highlighting how it can often exacerbate feelings of inadequacy and shame for those with rarer conditions.

Keywords:

• neurodiverse
• neurological
• hierarchy
• ataxia
• rare disease
• disability

Limiting neurodiversity only to those with autism and related differences, however, resembles limiting ethnic diversity to discourse about individuals of African American descent (Baker, 2011 as cited in Runswick-Cole 2014, 1120)

Neurodiversity has been acknowledged as a natural and essential human variation, composed of infinite forms of neurocognitive functioning (Walker 2014). The paradigm and studies surrounding neurodiversity offer an essential and alternative way of exploring conditions that were traditionally pathologized by the neurotypical majority who dominate society. Following Judy Singer’s developments in the field of neurodiversity, much observational research and critical discussion have materialised within the natural and social sciences (Comberousse 2019). Exploration, interpretation, and critique of the models of disability (Comberousse 2019) have ensued as well as a scrutiny of neurodiversity itself. Walker (2014) argues that neurodiversity is a concept which embodies ‘…the infinite variation in neurocognitive functioning…’ (p.1) concurring that neurodiversity is just a natural human variation and by no means a deficit. For decades now the neurodiversity movement has fought to bridge the gap between neurodivergent individuals who deviate from the norm, and neurotypical members of society (den Houting, 2018). Neurodiversity should not be thought of as a problem that needs to be eradicated, but rather as a slight deviation from the norm that makes up our wonderfully diverse and rich society (Comberousse 2019).

Due to the invisibility of many symptoms, the challenges that many neurodiverse individuals face may go unrecognised and undetected and are often belittled or seen as trivial because others fail to spot the difficulties they are having (Holland 2017). Research conducted by Elsabbagh et al. (2012), exploring the global prevalence of autism, reported one in one hundred and sixty children suffer from a form of autism, making it one of the most accepted and known neurological disorders. Consequently, for many, the term neurodiverse is synonymous with autism. I recognised a perceived lack of insight into the lives of individuals with rare forms of neurological illnesses and have identified the crucial need to raise awareness surrounding how individuals manage in a society governed by conventions and norms. My overall experiences and in-depth research have led me to question why rarer neurodiverse conditions, such as my own, have been overlooked and overshadowed by other neurological diseases that are more acknowledged and well known. The potential presence of a neurological hierarchy has propelled me to reflect upon the lack of awareness and focus surrounding lesser-known neurological conditions.

Having a rare, often unheard of (Koenig and Moreira 2004), neurological condition myself has incited an unnerving frustration within me. This is due to more uncommon and at times more severe neurological conditions going unrecognised, overthrown by more accepted and acknowledged conditions. Runswick-Cole argued that ‘the term neurodiverse has been claimed by people labelled with autism …’ (p.1120). She contended that the neurodiverse community has been limited to those with autism due to a lack of awareness surrounding other disorders, contending that the neurodiverse community is often thought to include ‘autistics and their cousins’ (Ortega 2009 as cited in Runswick-Cole 2014, 1120). I question whether this is because autism can often imitate what society deems as acceptable and tolerable behaviour. My doubts surrounding inclusivity within the neurodiverse community may be corroborated by Runswick-Cole who discusses how the neurodiversity movement troubles the dominance of the ideal healthy, able-bodied neoliberal. ‘Neoliberalism tolerates those who can mimic the neoliberal ideal type’ (Runswick-Cole 2014, 1126).

The anxiety I felt leading up to and after being diagnosed had to do with the thought that no one would understand my condition, no one could immediately tell why a healthy looking seventeen-year-old was walking so abnormally. It was not until the summer of 2019 that I began relying on a walking stick to help me manage my ataxia, up until then my neurological illness often went undetected. These years presented a plethora of challenges for me, especially during my educational experience, as no one could easily recognise the obstacles I faced. As a result of this, no one knew when and how they could help. At seventeen years old, and after almost a decade of investigations and interventions, I was diagnosed with ataxia. This happened two months before starting university. Originating from the Greek language, ataxia refers to the loss of coordination due to muscle weakness (Burke and Hammans 2012), symptoms include slurred speech, abnormal eye movement and poor proprioception. Although research has progressed rapidly over the last few years, there is, unfortunately, no cure yet (Burke and Hammans 2012).

My type of ataxia is called AOA2 (Ataxia with oculomotor apraxia type 2), it is known as autosomal recessive ataxia, meaning that ‘two defective copies of the gene (one from each parent) are required to manifest symptoms; thus, parents as carriers are usually asymptomatic’ (Akbar and Ashizawa 2015, 227). Despite ataxia being a rare neurodegenerative condition, ‘the estimated incidence of AOA2 is… known to be around 3 per million’ (AT Society, 2021). Although there may be no cure, treatment including physiotherapy, mobility aids and educational support is available to help control manifestations (Koenig and Moreira 2004). Having this diagnosis motivated me to focus on disability throughout my studies at university. I developed an interest in labelling, stereotypes, and the detrimental effect these can have on individuals and groups who are not regarded as part of conventional society, questioning how and why they create stigma and further ostracise those who do not comply with the norm. My concern is around whether these stereotypes and conventions also define me. Am I less valuable because I deviate from the orthodox aesthetically normal woman? I am a second-class citizen because I do not conform to the norm? (LaChapelle et al. 2014).

Daily persistent falling due to loss of balance and poor coordination is a common symptom of ataxia. Such falls are predominantly injurious and detrimental to the individual’s wellbeing, recent studies have found that ‘in nearly 1 out of 10 cases, they are accompanied by severe injuries…’ (Damulin 2015) resulting in a multitude of hospital visits and often leaving them in a wheelchair, in some cases temporarily but often permanently. When not falling over ataxia can leave sufferers in a state of confusion and disorientation due to symptoms such as fatigue and ‘brain fog’. Notably, the severity of these symptoms has now become more widely acknowledged by the general public and by medical professionals due to them being symptoms of long COVID. Further to this, a lack of control over the coordination of one’s limbs results in alterations in movement and speech, prompting others to question whether they are drunk ‘many people told me I was drunk, even in the morning. I always had to convince them that I wasn’t drunk’ (patient P17 as cited in Mercadillo et al. 2015, 10). This prompted Ataxia UKs campaign ‘Disordered Not Drunk’, an initiative depicting the stories of those with ataxia who are frequently mistaken for being drunk. Many individuals with ataxia have detailed how these unintentionally hurtful misconceptions fuel prejudice and intolerance within society, how these unkind comments often exacerbate the feeling of unworthiness and inadequacy. These personal accounts embody and highlight the humiliating and often demoralising experiences that those with ataxia endure due to not only their physical symptoms but also due to the unfair labels enforced onto them because they do not conform to society’s ‘…neoliberal ideal type’ (Runswick-Cole 2014, 1126).

Ataxia affects the part of your brain called the cerebellum. Despite its size, the cerebellum can cause drastic changes in not just physical but cognitive and emotional behaviour. Cognitive changes may interfere with daily tasks of problem solving and planning, as ataxia progresses it may also cause word-finding difficulties where individuals may alternatively use a more ambiguous word or will find themselves compelled to explain what it is that they are trying to convey. Ataxia may also alter short-term memory in many individuals. A significant decrease in one’s ability to regulate emotion can also be detected amongst individuals with ataxia. This can elicit feelings of irritation and impatience that may be ‘…expressed as angry outbursts, followed by a rapid return to usual behaviour’ (Marvel 2018). These periodic and frequent emotional ebullitions can have detrimental impacts on interpersonal relationships. Researching uncommon neurological conditions such as ataxia, would help individuals to better understand them. Early detection of these manifestations may also lead to a faster, more rapid diagnosis and potentially aid with the development of treatments.

This article is not arguing that one neurological condition is more important than the other but that due to more research and publicity given to those more common illnesses many individuals, including myself, are often left feeling like a ‘…public policy afterthought rather than as a priority’ (Blackwood 2019). More needs to be done to normalise and humanise the differences between the neurodiverse community and the neurotypical majority, focusing on rarer and less accepted ailments. Belonging to the neurodiverse community myself, the lack of knowledge and understanding about rarer and often much more severe neurological disorders prompted me to question whether some form of hierarchy was present within the neurodiverse community. The neurodiversity paradigm itself is introspectively antithetical. Instead of celebrating ‘…the infinite variation in neurocognitive functioning…’ (Walker 2014, n.p.), the lack of awareness around lesser-known neurological differences has shaped and built this hierarchy between conditions. The neurodiverse community has been limited to those with more prevalent and understood diagnoses due to a lack of awareness surrounding other neurological disorders such as ataxia. The presence of a neurological hierarchy is just one example of intersectionality, the interconnected and overlapping disposition of many social categories resulting in prejudice and intolerance is apparent amongst many other communities. Historically, hierarchies have always been present across many social systems and cultures, many social groups ‘…rapidly self-organize into hierarchies…’ (Koski, Xie, and Olson 2015). This article questions whether the neurodiverse community has also adopted a hierarchical structure and suggests that further social research will help to normalise and humanise more uncommon conditions.

Disclosure statement

No potential conflict of interest was reported by the author.

Correction Statement

This article has been republished with minor changes. These changes do not impact the academic content of the article.