MDC Publication Highlights - June-July 2020

Roadmap to effective advance care planning in Parkinson’s disease and atypical Parkinsonism

People with Parkinson’s can often effectively manage their symptoms with dopaminergic therapy for many years. However, over time the disease progresses and patients may enter a palliative phase, in which dopaminergic medication is no longer effective, advanced therapies (e.g. surgery) are not suitable and additional disability, such as dementia, may develop. People with Parkinson’s may therefore benefit from planning their preferred advanced care options relatively early in the course of the disease. Advanced care planning allows people to plan for their future care needs, including medical, psychological and social aspects and discuss, review and record these plans with family and health-care providers. Despite the importance of advanced care planning there are no clear guideline as for how these should be addressed by healthcare providers and little is known about how they are regarded by people with Parkinson’s and their family carers. The current review, led by MDC researcher Prof. Anette Schrag and colleagues from the UCL Centre for Ageing Population Studies, sought to explore the experiences of advanced care planning for people with Parkinson’s or atypical Parkinsonian disorders and their family carers, with the goal of identifying the effective components of optimal advanced planning for this population. A total of 27 articles were included in this analysis, addressing advanced care planning from the perspective of the patients, the family carers or healthcare providers. The analysis examined what is involved in advance care planning discussions, when and how they are initiated, what are the roles of healthcare professionals and family carers in these discussions and what are the barriers to advance care planning. The researchers were able to identify several components which are important for optimal advance care planning in Parkinsonian disorders which could inform best practices. These included the timing and approach to advance care planning discussions, the importance of appropriate training for healthcare professionals, provision of support to family carers, clear documentation of plans and their accessibility to relevant service providers. The study indicates that advance care planning can help people with Parkinsonian disorders take control of their future, but in order to be effective it must be proactively discussed by healthcare professionals in a way that is individualized and best suited to the unique circumstances of each patient.

• Nimmons et al. ‘Experiences of advance care planning in Parkinson’s disease and atypical Parkinsonian disorders: a mixed methods systematic review’. European Journal of Neurology, First Published: 30 June 2020, DOI: 10.1111/ene.14424.

High prevalence of neuropsychiatric symptoms in late-stage Parkinson’s

Late-stage Parkinson’s is defined as an advanced disease stage, in which patients have become dependent on caregivers for activities of daily living. Patients with late-stage Parkinson’s experience multiple motor and non-motor symptoms, including neuropsychiatric problems such as psychosis, depression, anxiety, apathy, and behavioral problems. Early small scale studies have indicated that neuropsychitric symptoms may be very common among people at late stage of Parkinson’s and their presence is associated with a decreased quality of life and increased caregiver burden. However, there has been little investigation of neuropsychitric symptoms in this population to date and our understanding of their scale and effects are very limited. The current study is a multi-national collaboration led by MDC researcher Prof. Anette Schrag, designed to assess the prevalence of neuropsychiatric symptoms in late-stage Parkinson’s and to identify their clinical markers and predictors. The researchers used data collected through the “Care of Late-Stage Parkinsonism” study, which is a longitudinal cohort study aimed to evaluate the needs of patients in late-stage Parkinson’s. They found that an overwhelming majority of people with late-stage Parkinson’s, 92% of 625 study participants, experienced at list one neuropsychiatric symptom, and most of them experienced two or more symptoms. The most common symptoms were depression, apathy, anxiety and hallucinations. Although each psychiatric symptom had a unique set of disease-related determinants, the strongest predictors for any of them was the presence of other neuropsychiatric symptom/s. The findings of this study indicate that clinicians involved in the care for patients with late-stage Parkinson's should be aware of the frequent occurrence and high comorbidity of neuropsychiatric symptoms in this patient group and proactively explore and address them.

• Hommel et al. ‘The Prevalence and Determinants of Neuropsychiatric Symptoms in Late-Stage Parkinsonism’. Movement Disorders Clinical Practice, First Published: 21 May 2020, DOI: 10.1002/mdc3.12968.

Insights into brain mechanisms underlying dystonia

Dystonia is a movement disorder characterised by involuntary muscle contractions that lead to abnormal movements, postures and tremor. There are many different types and causes of dystonia, and it can appear either as a primary symptom or as part of the clinical picture in a broader neurological syndrome. The abnormal brain activity patterns that underlie dystonia are not fully understood, but several mechanisms are thought to be involved. These include over-activity and over-excitability of brain circuits controlling movement, and also dysfunction of neural mechanisms that process and integrate sensory information. An effective treatment for dystonia is Deep Brain Stimulation (DBS) in one of a few specific brain regions (globus pallidus interna, subthalamic nucleus and the thalamus). Beyond its therapeutic effect, DBS has brought the opportunity to directly record brain activity to gain insight into the pathpophysiology of dystonia and its amelioration by DBS. This article reviews the breadth of research into the neurophysiology of dystonia and how it is affected by DBS treatment.

• Tisch & Limousin, ‘Neurophysiological insights in dystonia and its response to deep brain stimulation treatment’. Experimental Brain Research, First Published: 7 July 2020, DOI: 10.1007/s00221-020-05833-8.

The spectrum of neurological complications associated with COVID-19 infection

Since December 2019, over 16 million cases of the novel coronavirus, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), have been reported worldwide. Although the effects of COVID-19 on the respiratory system have been the most frequent and life threatening, there are increasing reports of nervous system involvement in the form of a range of neurological complications. Responding to this, the National Hospital of Neurology and Neurosurgery in Queen Square established systematic clinical and paraclinical data collection of cases of COVID-19 referred to the hospital due to presentation of neurological symptoms. The current publication describes the detailed emerging spectrum of neurological disorders encountered in 43 COVID-19 patients, their courses of treatment and clinical outcomes. This analysis indicates that SARS-CoV-2 infection is associated with a wide spectrum of neurological syndromes, which in some cases, respond well to immunotherapies. Notably, the neurological complications were not related to the severity of the respiratory COVID-19 disease. Early recognition of COVID-19-related neurological disease proved challenging and further studies are essential to determine the underlying pathobiological mechanisms, which will guide treatment. Furthermore, longitudinal follow-up studies will be necessary to ascertain the long-term neurological consequences of this pandemic.

• Paterson et al. on behalf of the UCL Queen Square National Hospital for Neurology and Neurosurgery COVID-19 Study Group, ‘The emerging spectrum of COVID-19 neurology: clinical, radiological and laboratory findings’. Brain, First Published: 8 July 2020, DOI: 10.1093/brain/awaa240.