Gilles de la Tourette Syndrome: advice in the times of COVID-19

Aetiology: COVID-19

Coronaviruses are a group of viruses that mostly result in mild illnesses, similar to the “common cold”, targeting the upper respiratory tract. However, certain types of coronaviruses can affect the lower airway, causing serious illnesses such as bronchitis or pneumonia. Coronaviruses have very large single stranded RNA genomes (approximately 26-32 KB). Coronavirus particles are surrounded by a fatty outer layer called an “envelope” and usually are spherical when visualised under an electron microscope, with a “corona” of club-shaped spikes on their surface (Wu et al., 2020). Most people become infected with coronaviruses at some point in their lives, but the majority of these are harmless. The novel coronavirus, which causes the COVID-19 illness, is different, and can be extremely serious.

The virus that causes COVID-19 is known as SARS-CoV-2 (Severe Acute Respiratory Syndrome Corona Virus 2). It appears to have emerged in Wuhan, Hubei Province, China. The viruses first inhabited animals such as bats and then “jumped” to humans: this occurred in China in late 2019, from whence it has spread worldwide (Zhu et al., 2020). Coronaviruses replicate their RNA genomes using enzymes called RNA-dependent RNA polymerases, which are prone to errors, but genomic analysis to date suggests that SARS-CoV-2 is mutating slowly, thus reducing the chance of it changing to become deadlier (Wu et al., 2020). It is worth mentioning that since 2003, viral diseases have caused major pandemics. These include the coronaviruses, which have caused multiple major public health events that resulted in global pandemics such as severe acute respiratory syndrome (SARS; or “bat SARS”), Middle East respiratory syndrome (MERS) and the current coronavirus disease (COVID-19) (Kandeel et al., 2020). In addition, with a growing threat to maternal-foetal health, the Zika viral illness began in 2009 with devastating consequences and continues to spread (Towers et al., 2018). Finally, in some parts of the globe, cases of swine flu (H1N1 virus) are still on the rise such as in India, where in 2020 alone around 1500 cases have been reported to date, with 28 deaths in the period from 1 January to 1 March 2020.

Clinical features: COVID-19

COVID-19 can affect individuals of any ages and clinical symptoms may vary. The main recognised clinical features of COVID-19 include fever (52% of younger patient, 89% of older patients), cough (44% younger, 77% older; usually dry, but can be expectorant), fatigue or tiredness; more severe cases get pneumonia. In mild cases people may have only a runny nose or sore throat. Patients may also have gastro-intestinal symptoms such as nausea, diarrhoea and anorexia. It does appear that older people, on the whole, are more severely affected. Individuals with pre-existing co-morbid conditions also appear to be particularly at risk: these illnesses include hypertension, diabetes, cardiovascular disease, and malignancy (Mao et al., 2020; Wan et al., 2020; Zhang et al., 2020a; Zhang et al., 2020b; Zhang et al., 2020c; Zheng et al., 2020a). There is also recent evidence that anosmia or loss of smell is a symptom of COVID-19 and can even be the presenting symptom (Hopkins & Kumar, 2020; Lüers et al., 2020). Recent unpublished results in a series of 417 patients suggest rates of >80%, and a large European multicentre trial is currently being undertaken.

Severity has been described in various ways. Patients with abnormal chest images are older, with a higher rate of pre-existing conditions, lower rate of exposure history, and a longer time between onset and confirmation than patients without pneumonia. In addition, patients with more severe symptoms have a higher rate of fever, expectorant cough and headache, lower lymphocytes, albumin and serum sodium, and a higher total bilirubin, c-reactive protein, creatine kinase and lactate dehydrogenase, (Zhang et al., 2020c). Muscle aches, headache, shortness of breath and nausea/vomiting are also associated with disease severity. Also, biomarkers such as lower lymphocyte levels in addition to raised leukocytes, D-dimer levels, procalcitonin and serum creatinine, as well as higher radiograph score at admission are predictive factors for a severe/critical subtype (Zhang et al., 2020c). Finally, it has been shown that elevated exhaustion levels and reduced functional diversity of T cells in peripheral blood may predict progression in COVID-19 patients (Zheng et al. 2020b). In the most severe cases, patients go into respiratory and multi-organ failure. It must be borne in mind, however, that mortality has also been reported in children, adolescents and young adults. Thus, it does appear that no one is immune or not at risk.

Aetiology and epidemiology: Gilles de la Tourette Syndrome

Gilles de la Tourette syndrome (GTS) is a complex neurodevelopmental disorder that has aetiological contributions from genetic influences in pregnancy and birth difficulties, and immunological variables (Robertson et al., 2017). Whereas the PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections) hypothesis is increasingly being discarded, an immunological trigger is possible in a subset of patients with GTS as they might show differences in immune responses compared to controls (Martino et al., 2015); however, how these might possibly affect response to viral infections and, more specifically SARS-CoV-2, remains as yet unknown (see below).

Clinical features: Gilles de la Tourette syndrome

The generally recognised diagnostic criteria according to the DSM-5 for GTS are multiple motor and at least one or more vocal (phonic) tics over the course of at least 12 months in a patient less than 18 years old (APA, 2013). The phenotypic spectrum also includes chronic (persistent) motor or vocal tic disorder and transient (provisional) tic disorder. The most common motor tics are facial (such as eye blinking and grimacing), whereas the most common phonic tics are coughing, sniffing and throat clearing (Robertson et al., 2017). Individuals may have the relatively rare (around 15–20%) but “press-worthy” symptom of coprolalia (Freeman et al., 2009).

Neuropsychiatric comorbidities are common (around 85% of patients) and include obsessive-compulsive disorder or behaviours (OCD/OCB), attention-deficit hyperactivity disorder (ADHD), learning disabilities, depression, anxiety, and autism (Rizzo et al., 2014; Robertson et al., 2017).

It is also worth noting that quality of life in patients with GTS has been demonstrated to be reduced (Elstner et al., 2001; Eddy et al., 2011). In addition, Eapen et al. (2016) reviewed the literature, pointing out that both tics and comorbidities in GTS may result in poorer psychosocial functioning. Much of the stigma and social maladjustment associated with GTS resulting in exclusion, bullying and discrimination, can be largely caused by misperceptions of the disorder by teachers, peers and the wider community. Thus, in the current “COVID-19 climate,” we must be mindful to remain attuned to the needs of our patients with GTS.

Gilles de la Tourette syndrome in the context of COVID-19

Potential consequences of the COVID-19 pandemic on patients with GTS can be classified according to four axes:

Management and treatment

Accurate recognition of the symptoms of coughing or sniffing as related to GTS as opposed to COVID-19 is critical in providing the right intervention and support. Similarly, spitting or forced touching etc. can be part of tic-related behaviours that may get the patient in trouble with the law. Hence awareness and appropriate support (e.g. the GTS patient may have to carry a card, informing authorities and other appropriate people about the condition) is important. Further, accurate identification and treatment of tics and comorbidities should continue as usual and according to established guidelines, if possible (Pringsheim et al., 2019). It is likely that medications will need to be increased probably transiently until the COVID-19 crisis resolves. That is obviously true for anti-tic medication as it is for psychostimulants (for ADHD) and selective serotonin reuptake inhibitors (for anxiety and OCD). There are currently unprecedented challenges in healthcare delivery and some of the specialists who would normally give this advice in many countries may be unavailable for a time due to redeployment, potentially causing further stress to families. Also, CBT for tics (van de Griendt et al., 2013) should be maintained by videoconferencing whenever possible (Andrén et al., 2019). The same applies to supportive psychotherapy for those who suffer from anxiety, depression, and loneliness. Finally, telehealth will play an increasing role in the medical follow-up of patients with TS, likely beyond the end of the pandemic. It will be important to establish whether this type of care will be well accepted by patients and families alike.

The National Patient Advocacy Groups for GTS in the UK and USA have published factual information and advice for individuals with GTS, their families and health-care professionals. These represent invaluable resources in their own right and, moreover, underline a sense of (global) community that is vital in these times.

Concerning the points raised above, we propose the following measures:

Conclusion

GTS, as a complex neuropsychiatric disorder, offers many angles of attack for the current COVID-19 pandemic and its consequences (social distancing, home schooling, confinement/quarantine, and living in a general climate of fear). Also, some patients might expose themselves to social conflict because of behavioural features specific to GTS. Thus, they need our reassurance and help in these difficult times, supported by a network of informed health-care professionals, although already rare in ‘normal’ times. Ideally, specialist medical services will be able to organise in the face of acutely changing roles in hospitals to provide advice when needed, to avoid a feeling of professional abandonment (which obviously is not special to patients with GTS). With this short paper, we hope to have contributed a few small pieces to the puzzle which will hopefully help us navigate through the upcoming weeks and months.

Data availability

No data are associated with this article.