A short time ago, in a country far, far away… a mysterious pneumonia-like illness transcended upon its people. Outbreaks of a novel coronavirus associated with severe acute respiratory syndrome (SARS-CoV-2) quickly took the world by storm. Entire regions were placed under lockdown to minimize “COVID-19” spread. Social distancing and self-quarantine, masking, and at-home test kits became the norm. Life as we knew it had completely changed.  

The medical field had to adapt in response to this global pandemic. Hospitals were forced to prioritize critically urgent, life-or-death cases, while virtual care became an essential tool for family doctors, specialists, and rehabilitation services. The field of research was racing to find out as much as it could about the virus and its effects. During this time, studies discovered that the nervous system is particularly vulnerable to SARS-CoV-2 infections. We knew that some of the common symptoms of COVID-19 were neurological, like headaches, fatigue, or even stroke. Some could even persist for a long time, known as long COVID-19 syndrome. Long COVID-19 was found to affect pre-existing neurological conditions negatively: patients were more likely to develop infection and they experienced faster disease progression. However, the effects of SARS-CoV-2 are unknown in patients with cerebellar ataxias. The researchers of this paper wanted to understand how COVID-19 affected the mental health and motor functions of patients with spinocerebellar ataxia type 2 (SCA2). 

This study found that SCA2 patients were equally likely to be infected with COVID-19 as the general population. Two types of Cuban individuals were included in designing this study: SCA2 patients and community members with no family history of cerebellar ataxias. The participation of community members provided the baseline for exposure risks to the virus. All participants estimated their risk of exposure to COVID-19 in a standardized questionnaire. In this questionnaire, they were asked whether they had any symptoms of COVID-19, whether they had close contact with anyone confirmed to have the virus, and if the participant had tested positive themselves. Any reports of a positive encounter were nasally swabbed and confirmed using a polymerase chain reaction (PCR) test. When the risk of exposure and confirmed positive or confirmed negative COVID-19 infection cases were compared, no differences between the general public and SCA2 patients were found. Overall, SCA2 patients were not at an increased vulnerability to COVID-19 infections compared to the general population, but does infection alter motor function in patients? 

Researchers found worsened speech function in SCA2 patients with viral infection. When assessing ataxia and motor symptoms, the Scale for the Assessment and Rating of Ataxia (SARA) is used. The SARA was used at baseline (first) and follow-up appointments. Researchers could access previous pre-pandemic SARA scores for their SCA2 patients, acting as a true control. The average time between these retrospective assessments and the first appointment was 16 months, similar to the time between the first and second visits. This portion of the study focused on three main traits: walking posture and ability, movement coordination, and ease of speech. There were only significant differences between the baseline assessment and the second visit from the infected SCA2 patients. This group experienced worsened speech compared to the infection-negative group. There was a small, but significant decline when combining every aspect of the SARA assessment. In conclusion, the coronavirus had negative impacts on motor function in SCA2 patients. 

This study linked COVID-19 diagnosis to a faster SCA2 disease progression. Researchers made this conclusion based on the decline in motor function. There are a couple of possible explanations for why speech difficulties seem to deteriorate faster than the other categories measured. The first is because coronavirus is a respiratory virus. If there are long periods where the parts of our body needed for speech are swollen, it could damage them. Another theory could be due to a specific pathway in the brain, which ensures speech and swallowing are happening correctly. It is possible that this pathway is at a higher risk of deterioration in cerebellar ataxias and could be susceptible to the accelerating effects of this viral infection. Although these might provide some biological explanation for speeding up disease progression in patients, it’s impossible to ignore the psychological factors that were also at play.   

Researchers found that coronavirus infection does impact mental health in SCA2 patients, using the Hospital Anxiety and Depression Scale (HADS). HADS is another self-assessment tool aimed at picking up the severity of emotional distress. This multiple-choice tool asks questions like: “I still enjoy the things I used to enjoy” to assess depression and “worrying thoughts go through my mind” for anxiety. This assessment tool was used in COVID-19-positive and COVID-19-negative patients at a baseline (first) and follow-up appointment approximately 14 months later. At the baseline appointment, there were no differences in reported anxiety or depression between the viral positive and negative groups. However, after their second visit, patients who were classified as COVID-19-positive experienced worsened depression and anxiety. Those confirmed negative did have slight decline in their mental health as well, but not to the same extent. From this article, we learned that once SCA2 patients were infected with the virus, they experienced deterioration in depression and anxiety. 

The coronavirus pandemic was a time of great uncertainty and heightened emotions for many people. As the pandemic progressed, it’s not surprising that almost all participants reported more anxiety and depression-related symptoms. But what was interesting was that SCA2 patients with coronavirus had even greater mental health difficulties. There are two possible explanations for this. The first explanation could be that there are already mental health problems in ataxia patients. Because the coronavirus is worsening the disease progression of other symptoms, it could also be worsening these feelings of depression and anxiety. The other contributing factors are the external, pandemic-related circumstances. Patients were experiencing lockdowns and home-confinement, and they were forced to withdraw from or miss rehabilitation programs, like counselling or speech therapy. Participants were fearful for their health and for the health of their loved ones, many of whom are also ataxia patients.  

Although the authors have linked COVID-19 infections with faster SCA2 disease progression, we still don’t know a lot. We need more research to fully understand what is causing this more rapid progression, whether biological, psychological, or some combination of the two. Studies like these provide important information for the ataxia community so that precautions can be taken. For example, it sets the rationale for organizing virtual, telehealth appointments to reduce chances of coronavirus infection.