Dr Shahid Dadabhoy discusses some key points in the diagnosis and management of Parkinson’s disease in primary care
Read this article to learn more about:
- the impact of non-motor symptoms on people with Parkinson’s disease
- how Parkinson’s disease nurse specialists and the multidisciplinary team can help with management
- why advanced care planning is crucial.
Parkinson’s disease (PD) is the second most common neurodegenerative disorder in the world after Alzheimer’s disease.1 Age remains the greatest risk factor for the condition; incidence is low before the age of 50 years but rises exponentially thereafter to a peak at 80 years of age.2 Other established risk factors for PD include biological sex (the male to female ratio is 3:2) and ethnicity (in the US, Hispanics have the highest incidence).1 The number of people with PD is expected to increase by more than 50% by 2030, and given increasing life expectancy in the general population, the public health and social care implications are significant.1 Although some other modifiable risk factors are being evaluated, promotion of physical activity seems to be the only practical intervention for primary prevention of PD at present.2
While it is rare for PD to be immediately fatal, it can have enormous consequences for the affected individual and the carers or family entrusted with looking after them. There is much that healthcare professionals working in primary care, who are well-placed to assist and support, can do to help people with PD.
1 Consider the non-motor symptoms of PD
Always think of non-motor symptoms (NMS) when considering the diagnosis and over the course of the condition. It is said that people with PD think of their condition from the neck down and that doctors think of the condition from the neck up. Non-motor symptoms in PD are sometimes the earliest to manifest, even predating the more familiar motor symptoms. They can be the most troublesome and seem to persist throughout the entire natural history of the condition.1,3 Non-specialist colleagues in primary care and/or acute settings are more likely to be confronted with requests to help with the management of NMS. The expectation that successful treatment for motor symptoms will translate into improvement in NMS does not always follow. Box 1 covers some of the most common NMS.
Box 1 Non-motor symptoms in Parkinson’s disease3–8
- Orthostatic hypotension
- Urinary incontinence
- Anhidrosis and hyperhidrosis
- Olfactory dysfunction
- Impaired colour discrimination
- REM sleep behaviour disorder
- Restless legs syndrome
- Sudden onset of sleep
- Daytime somnolence
- Sleep fragmentation
- Panic attacks
- Blurred vision
2 Be mindful of the time frames and emphasise these to patients and their carers
NICE Guideline (NG) 71 on Parkinson’s disease in adults recommends that, if PD is suspected, people should be referred quickly and untreated to a specialist with expertise in the differential diagnosis of the condition.9 Practically, the condition is slow in its onset and the typical natural history of the condition is shown in Figure 1.1 If atypical features or symptoms develop, then the diagnosis should be re-evaluated—diagnosis should remain clinical according to the UK Parkinson’s Disease Society Brain Bank Clinical Diagnostic Criteria. Therefore, treatment with levodopa (L-DOPA) or other dopamine agonists may not be initiated immediately and may be delayed until a firm diagnosis is secured; this is important because as NICE NG71 recommends, people with suspected PD need to be referred to a specialist untreated. Titration of treatment may take many months and it is important to communicate with the patient that tangible change will be slow. Regular review to reconsider the patient’s diagnosis should be set at 6–12 months by default.9
3 Recruit the help of Parkinson’s disease nurse specialists early
Given the complexity of the condition and its many facets, it pays to seek the assistance of PD nurse specialists (PDNS) very early on. These nurse specialists ably fulfil the three recommendations from NICE NG71: that patients have access to clinical monitoring and medication adjustment, ongoing contact and support (through home visits, if necessary), as well as information regarding healthcare and social care provision available in their locality.9 There is longstanding evidence that PDNS are cost effective.10
4 Ensure the timing of treatment is correct
The timing of medication in PD is critical to controlling motor symptoms and maintaining function over a typical day. When levels of dopamine drop and patients are ‘caught short’, the symptoms of PD become uncontrolled, and the consequences can be severe. This increases care needs considerably. The charity Parkinson’s UK ‘Get it on time’ campaign emphasises this and has developed several useful resources not only for patients and healthcare professionals but also for other care settings, such as care homes (where people with PD can be over-represented) and acute care.11
Levodopa remains the mainstay of initial treatment.9 However, although it provides the best motor symptom control, it can result in more motor complications. Newer agents such as dopamine agonists and MAO-B have been evaluated, and although they have fewer motor complications, there is evidence of other increased side-effects; see Table 1 for a comparison of these treatments.1,9
|Table 1: Potential benefits and harms of dopamine agonists, levodopa, and MAO-B inhibitors9|
|National Institute for Health and Care Excellence (2017). Parkinson’s disease in adults. NICE Guideline 71. Available from: www.nice.org.uk/ng71 NICE has not checked the use of its content in this article to confirm that it accurately reflects the NICE publication from which it is taken.|
|Levodopa||Dopamine agonists||MAO-B inhibitors|
|Motor symptoms||More improvement in motor symptoms||Less improvement in motor symptoms||Less improvement in motor symptoms|
|Activities of daily living||More improvement in activities of daily living||Less improvement in activities of daily living||Less improvement in activities of daily living|
|Motor complications||More motor complications||Fewer motor complications||Fewer motor complications|
|Adverse events||Fewer specified adverse events*||More specified adverse events*||Fewer specified adverse events*|
|MAO-B=monoamine oxidase B.|
* Excessive sleepiness, hallucinations and impulse control disorders (see the summary of product characteristics for full information on individual medicines).
Often, changing the timing of medication rather than changing the drugs themselves can dramatically improve motor symptoms that may seem puzzlingly resistant; PDNS colleagues or the patient’s consultant neurologist may be able to provide guidance on this. For explicit clarity, it is important to transcribe the exact times of dosing on prescriptions, repeat medication lists, and on any communication where patients are admitted to respite, long-term, or acute care.
5 Call on the assistance of the multidisciplinary team
The multi-faceted and holistic needs of people with PD require a range of skills including physiotherapy, occupational therapy, and speech and language therapy.9
Physiotherapy in people with PD is aimed at improving gait and stability, initiation of movement, breathing, and overall function to assist in daily living. Occupational therapy in people with PD should be focused on improving the activities of daily living, transfers (i.e. between sitting, standing, or lying down), improving environment, and assessment of cognition. Finally, speech and language therapy has a crucial role in improving the quality of communication, improving the safety and integrity of swallowing (including the risk of aspiration), and providing advice on technologies that can help to overcome communication difficulties.9 The benefits on PD progression of occupational therapy and intensive exercise are well established.12,13
This list is not exhaustive and the assistance of other colleagues may be necessary depending on the skills and expertise required to address the individual’s needs.
6 Raise the issue of advanced care planning
Given the nature of PD and its inexorable progression, it is prudent that patients and carers are aware that they can consider planning ahead about financial, healthcare, and social care decisions and make contingencies entrusting that their wishes are carried out when they are unable to do so themselves.
The NHS Advanced Care Planning (ACP) guide for health and social care staff defines ACP as ‘a process of discussion between an individual and their care providers irrespective of discipline’. It states that: ‘the process of ACP is to make clear a person’s wishes and will usually take place in the context of an anticipated deterioration in the individual’s condition in the future, with attendant loss of capacity to make decisions and/or ability to communicate wishes to others.’14
Advanced care planning should be documented, reviewed, and shared with key professionals involved in the patient’s care and, if the patient is willing, with family and friends. The spectrum of documentation can range from non-legally binding statements of wishes and preferences, through an advance decision to refuse treatment, to legal instruments such as Lasting Power of Attorney;14 start the discussion on ACP and empower people with PD to make decisions about their own care.
7 Recognise that carers are key to successful management
As with any long-term condition, the support of carers is critical to maintaining the independence of people with PD and usually this role is unpaid. Carers need to understand PD and their role in relation to the condition. Carers should be:9
- advised of what support they can receive from healthcare and social care professionals in their role
- supported to maintain their personal resilience
- advised of access to (if applicable) employment rights, benefits, and other resources.
As with any important role, training should be available. Parkinson’s UK has a wide range of resources to assist carers and their informational resources are the best first port of call.15
8 Always consider mental health
Given the pathophysiological basis of PD, it follows that the disease will cause a wide variety of neuropsychiatric symptoms (see Box 1).3,4,16 Mood symptoms may predate motor symptoms by years. Depression is the most common of these and develops independently of a situational response to a long-term condition. Despite the frequency of depression in PD there is no real guidance on preferable treatments from the options available.16 Nevertheless; treatment should be aggressively sought from familiar therapies.17
In addition, the treatments for PD can also have significant neuropsychiatric side-effects, including impulsive and compulsive behaviours, pathologic overuse of dopaminergic agonists, and psychosis. It is well worth informing patients and their carers that all neuropsychiatric symptoms are intrinsic to the PD and its management. After ruling out delirium, review the patient’s medication, and consider pharmacological options but do not forget about non-pharmacological approaches such as interpersonal therapies.
Up to 50% of patients with PD will demonstrate cognitive deficiencies within 5 years. In addition to non-pharmacological approaches, cholinesterase inhibitors may be of benefit; however, only rivastigmine has clear data to support its efficacy in improving cognitive impairment in PD dementia.16
9 Treat late-stage Parkinson’s disease with multiple agents
The treatment of advanced PD in people established on L-DOPA involves an assessment of whether the symptoms are predominately motor, dyskinetic, or non-motor; NMS typically become far more obtrusive to quality of life in later disease compared with motor symptoms.17
The mainstay of dealing with motor and dyskinetic symptoms involves review of, and attention to levodopa timing, dosages, and absorption. Note that treatment using multiple agents may be necessary. Dyskinesia may respond to the addition of amantadine. Throughout the natural history of PD, there always remains a significant and effective role for non-pharmacological therapies.17
10 Know that surgery is available, but manage expectations
Surgery can be offered to people with advanced PD whose symptoms are not satisfactorily managed by medication alone.1,9 Deep brain stimulation (DBS) is a well-established surgical treatment for PD—it involves the implantation of programmable multi-contact electrodes directly into the brain to stimulate target areas connected by wires running under the skin to a subcutaneous neurostimulator, which is typically implanted below the clavicle.1.18 Deep brain stimulation does offer significant improvements (including a reduction in dopaminergic drug burden) in people who have motor symptoms that are responsive to levodopa but also have disabling motor fluctuations and dyskinesia.1,18,19
It should be strongly emphasised to patients and their carers that DBS does not represent a cure for PD—it is a symptomatic treatment that can slow disease progression, but not arrest it.12,19 Deep brain stimulation will not improve symptoms in advanced PD that levodopa has not addressed, such as cognitive impairment, gait instability, mood disorders, speech impairment, and autonomic dysfunction, and may even worsen symptoms.17
- Kalia L, Lang A. Parkinson ’s disease. Lancet 2015; 386 (9996): 896–912.
- Ascherio A, Schwarzschild M. The epidemiology of Parkinson’s disease: risk factors and prevention. Lancet Neurol 2016; 15 (12): 1257–1272.
- Goldman J, Postuma R. Premotor and non-motor features of Parkinson’s Disease. Curr Opin Neurol 2014; 27 (4): 434–441.
- Beitz J. Parkinson’s disease: a review. Front Biosci (Schol Ed) 2014; 6: 65–74.
- Parkinson’s UK. Skin and sweating. www.parkinsons.org.uk/information-and-support/skin-and-sweating (accessed 12 September 2017).
- Parkinson’s UK. Restless legs syndrome.www.parkinsons.org.uk/information-and-support/restless-legs (accessed 12 September 2017).
- Pettorruso M, Martinotti G, Fasano A et al. Anhedonia in Parkinson’s disease patients with and without pathological gambling: a case-control study. Psychiatry Res 2014; 215 (2): 448–452.
- Parkinson’s UK. Anxiety. www.parkinsons.org.uk/information-and-support/anxiety (accessed 12 September 2017).
- NICE. Parkinson’s disease in adults. NICE Guideline 71. NICE, 2017. Available from: www.nice.org.uk/ng71
- Jarman B, Hurwitz B, Cook A et al. Effects of community based nurses specialising in Parkinson’s disease on health outcome and costs: randomised controlled trial. BMJ 2002; 324 (7345): 1072–1080.
- Parkinson’s UK. Get it on time. www.parkinsons.org.uk/content/get-it-time (accessed 12 September 2017).
- Foster E, Bedekar M, Tickle-Degnen L. Systematic review of the effectiveness of occupational therapy-related interventions for people with Parkinson’s disease. Am J Occup Ther 2014; 68 (1): 39–49.
- Frazzitta G, Balbi P, Maestri R et al. The beneficial role of intensive exercise on Parkinson disease progression. Am J Phys Med Rehabil 2013; 92 (6): 523–532.
- NHS End of Life Care Programme (EoLC) and the University of Nottingham. Advance care planning: a guide for health and social care staff. NHS EoLC, 2007 (updated 2008). Available at: www.ncpc.org.uk/sites/default/files/AdvanceCarePlanning.pdf
- Parkinson’s UK. Caring for someone with Parkinson’s. www.parkinsons.org.uk/information-and-support/caring-someone-parkinsons (accessed 12 September 2017).
- Cooney J, Stacey M. Neuropsychiatric issues in Parkinson’s disease. Curr Neurol Neurosci Rep 2016; 16 (5): 49–62.
- Giugni J, Okun M. Treatment of advanced Parkinson’s disease. Curr Opin Neurol 2014; 27 (4): 450–460.
- Fang J, Tolleson C. The role of deep brain stimulation in Parkinson’s disease: an overview and update on new developments. Neuropsychiatr Dis Treat 2017; 13: 723–732.
- Kaliaa S, Sankarb T, Lozanoa A. Deep brain stimulation for Parkinson’s disease and other movement disorders. Curr Opin Neurol 2013; 26: 374–380.