Basis for recommendation

The recommendations on referral are based on the National Institute for Health and Care Excellence (NICE) clinical guidelines Parkinson's disease in adults: diagnosis and management [NICE, 2022] and Suspected neurological conditions: recognition and referral [NICE, 2021a], the European Parkinson's Disease Association (EPDA) Standards of care consensus statement [EPDA, 2011], a joint European Federation of Neurological Societies/Movement Disorder Society-European Section (EFNS/MDS-ES) publication EFNS/MDS-ES recommendations for the diagnosis of Parkinson's disease [Berardelli, 2013], and expert opinion in a review article [Kobylecki, 2020].

Arranging urgent specialist referral

• The recommendation to arrange urgent specialist referral before starting drug treatment is based on the NICE guideline [NICE, 2022] and the EPDA consensus statement [EPDA, 2011].
  • CKS notes that drug treatment for Parkinson's disease can mask some or all of the important signs needed for diagnosis, therefore people should be referred before treatment is started.
  • NICE cites studies that have found that the diagnosis of Parkinson's disease was incorrect in about 47% of people diagnosed in the community, 25% of people diagnosed by non-expert secondary care physicians, and 6–8% of people diagnosed by an expert in movement disorders.
  • NICE highlights that arranging urgent referral aims to prevent any unnecessary psychological distress arising from a delay in diagnosis. In addition, the EPDA consensus statement highlights that people with confirmed Parkinson's disease are offered medication early to improve their quality of life and possibly delay the progression of the disease, and the multidisciplinary team can be involved early to delay the development of complications.
• Specialist referral allows additional neuroimaging, neurophysiological, or neuropsychological testing to be arranged if needed, to clarify the diagnosis and exclude other causes of parkinsonism in people with suspected Parkinson's disease. Making an accurate diagnosis of Parkinson's disease is important in determining the person's prognosis and treatment regime [Berardelli, 2013].

Not delaying specialist referral if suspected drug cause

• The recommendation to refer a person, even if drug-induced parkinsonism is suspected and the causative drug is stopped, is based on expert opinion in a review article, that people with parkinsonism induced by antipsychotic drugs can take up to 8 weeks to improve when the dose is reduced, or anti-parkinsonian drugs are given [Hirose, 2006]. In addition, expert opinion of two previous external reviewers of this CKS topic is that parkinsonism can persist for longer than 8 weeks after drug withdrawal.

Advice about driving

• The Driver and Vehicle Licensing Agency (DVLA) states that people with a diagnosis of Parkinson's disease must notify the DVLA following diagnosis:
  • For Group 1 entitlement (car or motorcycle) — may drive as long as safe vehicle control is maintained at all times. If the individual's condition is disabling and/or there is clinically significant variability in motor function, the licence will be refused or revoked. If driving is not impaired, licensing will be considered subject to satisfactory medical reports. A licence may be issued subject to regular review.
  • For Group 2 entitlement (lorry or bus) — may drive as long as safe vehicle control is maintained at all times. If the individual's condition is disabling and/or there is clinically significant variability in motor function, the licence will be refused or revoked. If driving is not impaired, licensing will be considered subject to satisfactory medical reports and assessment. A licence may be issued subject to annual review.
• The latest information from the DVLA regarding medical fitness to drive can be obtained at Assessing fitness to drive - a guide for medical professionals.
• The Parkinson's UK information booklet Driving and Parkinson's outlines the rights and obligations of people with Parkinson's disease who drive.

[DVLA, 2022]

Basis for recommendation

The recommendations on arranging routine review are largely based on the National Institute for Health and Care Excellence (NICE) clinical guideline Parkinson's disease in adults: diagnosis and management [NICE, 2022], the joint European Federation of Neurological Societies (EFNS)/Movement Disorder Society-European Section (MDS-ES) guideline EFNS/MDS-ES recommendations for the diagnosis of Parkinson's disease [Berardelli, 2013], the American Academy of Neurology (AAN) publication Practice parameter: treatment of nonmotor symptoms of Parkinson disease [Zesiewicz, 2010], the Driver and Vehicle Licensing Agency (DVLA) publication Assessing fitness to drive: a guide for medical professionals [DVLA, 2022], the British Dietetics Association (BDA) publication Best practice guidance for dietitians on the nutritional management of Parkinson's [BDA, 2021], the General Medical Council (GMC) guidance Treatment and care towards the end of life: good practice in decision making [GMC, 2022], a Delphi study of quality of palliative care standards [Rogers, 2023], several small case surveys [Ng, 2007; Rahman, 2008; Visser, 2008], and expert opinion in review articles on Parkinson's disease [Halli-Tierney, 2020; Kobylecki, 2020], drug treatment in Parkinson's disease [Muzerengi, 2015], drug-induced parkinsonism [Hirose, 2006], and neuroleptic malignant syndrome [Berman, 2011].

Ensuring specialist review of symptoms and medication

• The NICE clinical guideline states that a Parkinson's disease nurse specialist may provide clinical monitoring, advice on medication adjustment, a point of contact for support, and a source of information for the person and their family/carers. In addition, it recommends that the diagnosis of Parkinson's disease should be reviewed regularly (every 6–12 months) by the person's specialist, and reconsidered if any atypical clinical features develop [NICE, 2022].
• The EFNS/MDS-ES joint guidelines highlight that the diagnosis of Parkinson's disease may change at specialist follow-up, for example, if a person develops atypical signs suggesting an alternative diagnosis, or if there is an insufficient response to dopaminergic drug treatment [Berardelli, 2013].
• Similarly, expert opinion in a review article notes that the diagnosis of Parkinson's disease should be regularly reviewed during follow-up for 'red flag' features which may suggest an alternative diagnosis, such as progressive supranuclear palsy or multiple system atrophy, which tend to have a more aggressive disease course and reduced response to dopaminergic drug therapy. In addition, it highlights the importance of multidisciplinary team input for both motor and non-motor symptoms [Kobylecki, 2020].

Advising on sources of information and support

• This recommendation is based on expert opinion in a review article [Kobylecki, 2020]. It is also pragmatic, based on what CKS considers to be good clinical practice.

Advising about driving

• This recommendation is based on the Driver and Vehicle Licensing Agency (DVLA) publication [DVLA, 2022] and expert opinion in a review article [Kobylecki, 2020].

Arranging a medication review

• NICE noted that people with Parkinson's disease are at high risk of vitamin D deficiency due to potentially being more sedentary, and at increased risk of osteoporosis and falls [NICE, 2022].
  • NICE cites limited evidence of a theoretical reduction in the risk of complications from falls and the benefits of improved bone health with vitamin D supplementation, although NICE acknowledges that neither of these outcomes was reported in the analyzed study evidence. The available study (n = 112) did, however, show potential benefits in the Unified Parkinson's Disease Rating Scale (UPDRS) for people taking vitamin D, which is an overall measure of disease activity, however NICE noted that the improvement in UPDRS score was below the minimal clinically important difference, so this is of uncertain clinical benefit.
• In addition, the BDA best practice guidance notes that people with Parkinson’s disease have been found to have a lower bone mineral density (BMD) and increased risk of osteoporotic fracture [BDA, 2021].
• The recommendations on ensuring the person and also carers/hospital staff are aware of the need for the correct timing and dosage of anti-parkinsonian medication is based on expert opinion in a review article, which notes that a sudden reduction or withdrawal of medication may cause severe morbidity or rarely mortality due to neuroleptic malignant syndrome [Kobylecki, 2020].

Seeking specialist advice regarding anti-parkinsonian medication

• The recommendation to only start or alter anti-parkinsonian medication on the advice of a specialist is pragmatic, based on the fact that the drug treatment of Parkinson's disease becomes increasingly complex as the disease progresses.
• The recommendation to ensure that changes to repeat medication are made promptly in primary care is based on a small retrospective survey of case notes (n = 49) which found that 29% of medication changes recommended at specialist outpatient clinic appointments were not completed [Ng, 2007].
• The recommendation on not suddenly stopping anti-parkinsonian medication due to the risks of acute akinesia and neuroleptic malignant syndrome is based on expert opinion in the NICE clinical guideline [NICE, 2022] and review articles [Berman, 2011; Muzerengi, 2015].

Assessing motor and non-motor symptoms

• The recommendation to identify and manage worsening motor symptoms and complications is also based on the EFNS/MDS-ES joint guidelines [Berardelli, 2013].
• The recommendation to identify and manage any non-motor symptoms is based on a small self-completed postal survey (n = 130) of Parkinson's disease patients attending specialist clinics. It found that non-motor symptoms contribute significantly to poor quality of life, and recommended that they should be 'targeted for intervention' [Rahman, 2008]. In addition, a small longitudinal cohort study of hospital patients (n = 320) found non-motor symptoms had a significant impact on health-related quality of life scores [Visser, 2008].
• In addition, the importance of the management of non-motor symptoms is highlighted in the EFNS/MDS-ES joint guidelines [Berardelli, 2013], the AAN publication [Zesiewicz, 2010], and expert opinion in a review article [Halli-Tierney, 2020].

Advising about drugs that can exacerbate parkinsonism

• The recommendation to avoid or use with caution any drugs that can exacerbate parkinsonism or that interact with anti-parkinsonian medication is based on expert opinion in a review article [Hirose, 2006], and is pragmatic based on what CKS considers to be good clinical practice.

Discussing advance care planning and end-of-life care

• The recommendation to offer planning for future and end-of-life care is based on the NICE clinical guideline [NICE, 2022], the Department of Health's End of Life Care Strategy. Fourth Annual Report [DH, 2012], the GMC guidance [GMC, 2022], and a quality of palliative care Delphi study [Rogers, 2023].
  • The NICE guideline recommends that referral to the palliative care team can be offered to a person with any stage of Parkinson's disease, to give them and their family members or carers the opportunity to discuss palliative care and advance care planning.

Specialist management of motor symptoms

Specialist management of motor symptoms and complications of Parkinson's disease should be individualized, and may include the following drugs, depending on the person's symptoms and wishes, comorbidities, other medications, and potential benefits and harms of the different drug classes.

First-line treatments

• Levodopa, which is usually given with a dopa decarboxylase inhibitor, as co-beneldopa or co-careldopa.
  • Levodopa is usually offered to people in the early stages of Parkinson's disease whose motor symptoms impact on their quality of life.
  • Levodopa typically provides more improvement in motor symptoms and daily functioning, fewer adverse effects such as excessive sleepiness, hallucinations, and impulse control disorders, but may cause more motor complications such as dyskinesias than other drug classes.
• Oral monoamine oxidase-B (MAO-B) inhibitors (selegiline, rasagiline, or safinamide) — these do not cause an interaction after consumption of tyramine-rich foods.
  • These typically provide less improvement in motor symptoms and daily functioning, fewer motor complications, and fewer adverse effects such as excessive sleepiness, hallucinations, and impulse control disorders than other drug classes.
• Oral dopamine agonists, such as pramipexole or ropinirole; or transdermal dopamine agonist, such as rotigotine.
  • These typically provide less improvement in motor symptoms and daily functioning, fewer motor complications, but more adverse effects such as excessive sleepiness, hallucinations, and impulse control disorders than other drug classes.
  • A transdermal preparation may be useful if a person has a high tablet burden or swallowing problems.
  • Ergot-derived dopamine agonists such as cabergoline and pergolide should not be used as first-line treatment due to the risk of cardiac fibrosis with long-term use and need for additional monitoring.

Adjuvant treatments

• Oral catechol-O-methyl transferase (COMT) inhibitors (such as entacapone or opicapone).
  • These may be used as an adjunct to levodopa for people who have developed dyskinesia or motor fluctuations despite optimal levodopa therapy.
  • These typically provide more improvement in motor symptoms and daily functioning, more adverse effects such as excessive sleepiness and impulse control disorders, but a lower risk of hallucinations than other drug classes.
• Oral amantadine.
  • This may be considered if dyskinesia is not adequately managed by modifying existing antiparkinsonian therapy.
  • Adverse effects may include confusion, hallucinations, insomnia, nightmares, and dry mouth.
• Subcutaneous apomorphine (a potent dopamine agonist).
  • This may be offered to people with advanced Parkinson's disease and freezing episodes on optimal oral treatment, as intermittent subcutaneous bolus injections and/or continuous subcutaneous infusion.
  • Adverse effects include nausea, vomiting, and orthostatic hypotension.
• Deep-brain stimulation (DBS) surgery of the subthalamic nucleus.
  • This may be considered for people with advanced Parkinson's disease with motor complications refractory to optimal medical treatment who are fit, levodopa-responsive, and have no co-morbid mental health conditions.
  • Surgery involves the insertion of electrodes, usually bilaterally, into deep nuclei within the brain. These are connected to a battery-powered generator via leads that are tunnelled beneath the skin. The generator is usually implanted into the chest wall and it delivers an electric current to the electrodes placed in the targeted areas of the brain. The battery unit needs to be replaced using a simple surgical procedure every few years.

[Oertel, 2011b; Ferreira, 2013; Muzerengi, 2015; NICE, 2022; Halli-Tierney, 2020; Kobylecki, 2020; EMC, 2021; BDA, 2021; Pringsheim, 2021; BNF, 2023]

Basis for recommendation

The recommendations on managing motor symptoms and complications in Parkinson's disease are largely based on the National Institute for Health and Care Excellence (NICE) clinical guidelines Parkinson's disease in adults: diagnosis and management [NICE, 2022] and Falls in older people: assessing risk and prevention [NICE, 2013]; an evidence-based review article Summary of the recommendations of the European Federation of Neurological Societies (EFNS) and the Movement Disorder Society-European Section (MDS-ES) review on therapeutic management of Parkinson's disease [Ferreira, 2013], the American Academy of Neurology (AAN) publications Practice Parameter: treatment of nonmotor symptoms of Parkinson disease [Zesiewicz, 2010] and Dopaminergic therapy for motor symptoms in early Parkinson disease practice guideline summary [Pringsheim, 2021]; the British Dietetic Association (BDA) publication Best practice guidance for dietitians on the nutritional management of Parkinson's [BDA, 2021], various Cochrane systematic reviews Treadmill training for patients with Parkinson's disease [Mehrholz, 2015], Physical exercise for people with Parkinson's disease: a systematic review and network meta-analysis [Ernst, 2023], and Interventions for preventing falls in Parkinson's disease [Allen, 2022], expert opinion in a neurology textbook [Oertel, 2011a; Oertel, 2011b], and expert opinion in review articles on Parkinson's disease [Halli-Tierney, 2020; Kobylecki, 2020] and on drug treatment of Parkinson's disease [Muzerengi, 2015].

• Expert opinion in a review article notes that if there are uncontrolled motor fluctuations and dyskinesia persists despite optimal oral therapy, specialist consideration of advanced therapies may be appropriate [Kobylecki, 2020].
• The information on the possible benefits of a protein redistribution diet is based on the NICE clinical guideline, which found limited evidence that this may improve motor fluctuations for some people, although the mechanism of action on levodopa absorption and action are unclear [NICE, 2022]. This is supported by the BDA best practice guidance, which notes that dietary protein can delay gastric emptying and competes with the absorption of levodopa, and the manipulation of dietary protein may reduce fluctuations in some people [BDA, 2021].
• Gait problems may include reduced stride length as well as speed, festination and freezing. Physiotherapy interventions such as exercise, cueing, and other strategies can improve a person's function and maintain independence [NICE, 2022]. This is supported by the findings of two Cochrane systematic reviews.
  • One systematic review of 156 randomized controlled trials (RCTs, n = 7939) found evidence of beneficial effects on motor symptoms and quality of life for most types of physical exercise in people with mainly mild-to-moderate Parkinson's disease, with little evidence of harm [Ernst, 2023].
  • Another systematic review of 32 RCTs (n = 3370) found that exercise interventions may reduce the risk of falls in people with mild-to-moderate Parkinson's disease [Allen, 2022].
• The information on the Alexander Technique is based on high-quality evidence from one randomized controlled trial (RCT) cited in the NICE clinical guideline, which found significant improvements in self-assessed disability ratings with this self-management intervention compared with usual care [NICE, 2022].
• The information on treadmill training is based on a Cochrane systematic review of 18 RCTs with moderate to low risk of bias (n = 633), which found that the use of treadmill training in people with Parkinson's disease may improve clinically relevant gait parameters such as gait speed (moderate-quality evidence) and stride length (low-quality of evidence). It noted, however, that the study results were heterogeneous and there were variations in patient characteristics, the duration and amount of training, and types of treadmill training applied [Mehrholz, 2015].

Constipation, nausea and vomiting

Constipation

• Consider a stepped approach to the management of constipation due to colonic dysmotility in Parkinson's disease. Where possible:
  • Increase the person's dietary fibre and fluid intake.
  • Increase exercise levels within the person's capacity.
  • Consider the use of laxative drug treatments.
  • Consider the use of enemas if laxative treatments are ineffective and symptoms persist.
  • The Parkinson's UK booklet Looking after your bladder and bowels when you have Parkinson's may be helpful.
• If constipation persists, liaise with the person's specialist team, as alteration of the person's anti-parkinsonian medication may be helpful.
• For more information, see the CKS topics on Constipation and Faecal incontinence in adults.

Nausea and vomiting

• Initially, if nausea or vomiting is mild and related to starting or increasing the dose of levodopa or a dopamine agonist: 
  • Reassure the person that nausea often settles over time as tolerance to adverse effects builds up. 
  • Advise the person to take their medication with food.
• If nausea or vomiting persists, is severe, or is unrelated to levodopa or dopamine agonist drug treatment: 
  • Do not use metoclopramide or prochlorperazine anti-emetics — these can cause or exacerbate parkinsonism. 
  • Consider prescribing low-dose domperidone, and reduce or stop it when the nausea or vomiting settles.
    • Be aware that domperidone has been associated with an increased risk of ventricular tachyarrhythmias and sudden cardiac death.
    • Advise the person to seek urgent medical attention if symptoms such as syncope or palpitations occur during treatment with domperidone.
    • If domperidone is taken with high doses of apomorphine, there is an increased risk of QT prolongation — only consider concomitant prescribing after assessment of cardiac risk factors and ECG monitoring.
    • See the electronic Medicines Compendium (eMC) or the British National Formulary (BNF) for complete information on the contraindications and cautions when prescribing domperidone.
  • If domperidone is ineffective or not tolerated, seek specialist advice, as one or more of the following options may be recommended: 
    • An increase in the proportion of decarboxylase inhibitor to levodopa (for people taking co-careldopa specifically). 
    • Slower titration of the anti-parkinsonian drug. 
    • A switch to an alternative anti-parkinsonian drug.
    • A switch to an alternative anti-emetic drug.

Pain

• Liaise with, or arrange a review with, the person's specialist team for the following types of pain, as they may co-exist and require an alteration to anti-parkinsonian medication: 
  • Dystonic pain. The Parkinson's UK information sheet Muscle cramps and dystonia may be helpful.
  • Primary or central neuropathic pain.
  • Akathisia-related pain.
• Offer management for the following types of pain in primary care. Seek specialist advice or arrange a specialist review if pain remains uncontrolled: 
  • Musculoskeletal pain
    • Treat with simple analgesia and regular exercise within the person's exercise capacity. See the CKS topics on Analgesia - mild-to-moderate pain and NSAIDs - prescribing issues for more prescribing information.
    • Consider referral to physiotherapy if appropriate. 
    • See the CKS topics on Shoulder pain and Back pain - low (without radiculopathy) for more information, if appropriate.
  • Radicular neuropathic pain
    • Treat with simple analgesia and regular exercise within the person's exercise capacity. See the CKS topics on Analgesia - mild-to-moderate pain and NSAIDs - prescribing issues for more prescribing information.
    • Consider referral to physiotherapy if appropriate.
    • Consider prescribing drugs specifically for neuropathic pain. See the CKS topic on Neuropathic pain - drug treatment for more information. See the CKS topics on Neck pain - cervical radiculopathy and Sciatica (lumbar radiculopathy) for more information, if appropriate.
  • The Parkinson's UK information sheet Pain in Parkinson's may be helpful and covers the management of multiple common types of pain in Parkinson's disease.

Sleep disturbance and daytime sleepiness

Sleep disturbance

• Take a full sleep history and advise on sleep hygiene measures. See the CKS topic on Insomnia for more information.
• Identify whether any of the following are present and manage them appropriately. Seek specialist advice, or arrange a specialist review, if needed:
  • Depression and/or anxiety — see the section on Depression and anxiety for more information.
  • Restless legs syndrome. See the CKS topic on Restless legs syndrome for more information. The Parkinson's UK information sheet Restless legs syndrome and Parkinson's may be helpful.
  • Periodic leg movements of sleep.
  • Rapid eye movement (REM) sleep behaviour disorder — the person's specialist may consider clonazepam or melatonin drug treatment (off-label indication) if possible drug causes have been excluded.
  • Nocturnal akinesia (inability to turn over in bed) — the person's specialist may consider levodopa or oral dopamine agonist drug treatment, or rotigotine second-line if these agents are ineffective. Occupational therapy and physiotherapy assessment may also help.
  • Nocturia — see the section on Bladder and sexual problems.
  • Vivid dreams, nightmares, or hallucinations — may be caused by anti-parkinsonian medication (such as selegiline and dopamine agonists). Seek specialist advice about altering drug treatment.
  • The Parkinson's UK information Sleep and Parkinson's may be helpful.

Daytime sleepiness

• Advise people who have daytime sleepiness and/or sudden onset of sleep without awareness or warning signs, not to drive, to inform the Driver and Vehicle Licensing Agency (DVLA), and to consider any other occupational hazards.
  • The latest information from the DVLA regarding medical fitness to drive can be obtained at Assessing fitness to drive - a guide for medical professionals.
• The initial management of daytime sleepiness depends on its likely cause:
  • Anti-parkinsonian medication (such as dopamine agonists) — seek specialist advice regarding reducing or stopping specific medication.
    • The person's specialist may consider modafinil drug treatment for excessive daytime sleepiness if a detailed sleep history has excluded reversible drug and physical causes.
  • Sedating medication (such as antihistamines, antipsychotics, and some antidepressants) — reduce, stop, or use an alternative medication; seek specialist advice if necessary. 
  • Inadequate rest at night. See the CKS topic on Insomnia for more information.
  • Dementia — see the section on Dementia and cognitive impairment for more information.

Depression and anxiety

• In general, assess and manage depression and anxiety in the same way as for people without Parkinson's disease.
  • Symptoms and signs of depression may be wrongly assumed to be caused by the person's underlying Parkinson's disease. See the section on Mental health problems for more information.
    • Family/carers may be able to provide useful supplementary information to help diagnose depression.
    • For people with motor fluctuations, assessment for depression should be made during 'on' periods.
  • It may be necessary to liaise with the person's specialist for advice about adverse effects and potential drug interactions, if antidepressant medication is being considered.
    • Selective serotonin reuptake inhibitors (SSRIs) are most commonly used, but they can worsen motor symptoms such as restless legs, periodic limb movements, and rapid eye movement (REM) sleep behaviour disorder.
    • Tricyclic antidepressants may be more effective than SSRIs, but adverse effects, such as cognitive impairment and risk of falls may limit their use.
    • The Parkinson's UK information sheets Depression and Parkinson's, Anxiety and Parkinson's, and Fatigue and Parkinson's may be helpful.
  • See the CKS topics on Depression, Generalized anxiety disorder, and Obsessive-compulsive disorder for more information on management strategies for depression and anxiety.

Dementia and cognitive impairment

• If possible, obtain a collateral history from family or carers to help assess the impact of cognitive impairment on the person's daily functioning.
  • Treat any underlying condition that may be causing or exacerbating cognitive impairment, such as depression, constipation, infection, dehydration, or electrolyte disturbance.
    • The Parkinson's UK information sheet Mild memory and thinking problems may be helpful.
  • Reduce, stop, or use an alternative medication if the person is taking any drugs that may be causing or exacerbating cognitive impairment; seek specialist advice if necessary. Contributory drugs may include:
    • Antimuscarinic drugs, such as tricyclic antidepressants, tolterodine, and oxybutynin. 
    • H₂-receptor antagonists such as ranitidine.
    • Benzodiazepines. 
    • Amantadine or dopamine agonists — arrange a specialist review of the person's anti-parkinsonian medication. 
  • Liaise with the person's specialist and consider referral to a specialist memory assessment service if ongoing cognitive impairment or dementia is suspected. See the CKS topic on Dementia for more information.
    • The Parkinson's UK information Thinking and memory changes may be helpful if the diagnosis of Parkinson's disease dementia is confirmed.
    • Acetylcholinesterase inhibitor drug treatment (off-label indication) may be offered by a specialist for people with Parkinson's disease dementia. Memantine drug treatment (off-label indication) may be offered second-line if cholinesterase inhibitors are not tolerated or are contraindicated.

Impulse control disorders and psychotic symptoms

Impulse control disorders

• Impulse control disorders may be an adverse effect of dopaminergic medication (particularly dopamine agonist anti-parkinsonian medication) and may result in the person compulsively over-using this medication. If suspected and problematic:
  • Arrange a review with the person's specialist team; the urgency depending on clinical judgement.
  • After confirmation of the diagnosis, offer information and support to the person and their family/carers. The Parkinson's UK information Impulsive and compulsive behaviours in Parkinson's may be helpful.
  • Specialist management may include:
    • Reducing or stopping dopamine agonist medication, or switching to an alternative drug, after discussing the risks and benefits of this approach.
    • Reducing the dose of levodopa medication.
    • Using amantadine or an atypical antipsychotic drug (off-label indications).
    • Cognitive behavioural therapy (CBT) targeted at impulse control disorders, if modifying dopaminergic therapy is not effective.

Psychotic symptoms

• Have a low threshold for suspecting psychosis in a person with Parkinson's disease, and arrange a specialist review if this is suspected.
  • See the CKS topic on Psychosis and schizophrenia for more information on how psychosis may present.
  • Consider possible underlying causes, such as:
    • Parkinson's disease itself and anti-parkinsonian medication (particularly dopamine agonists).
    • Delerium caused by infection, constipation, pain, dehydration, electrolyte disturbance, or medication, for example.
    • Dementia. See the CKS topic on Dementia for more information.
    • Depression. See the CKS topic on Depression for more information.
  • Mild psychotic symptoms related to Parkinson's disease itself may not require treatment if they are well tolerated by the person and their family/carers.
  • More severe psychotic symptoms may require a gradual withdrawal of anti-parkinsonian medication which may be causing symptoms, or the use of an atypical antipsychotic drug such as quetiapine (off-label indication) for people without associated cognitive impairment, under specialist supervision. Rarely, clozapine drug treatment may be initiated by a specialist if standard treatment is not effective.
  • The Parkinson's UK information sheet Hallucinations and delusions in Parkinson's may be helpful.

Orthostatic hypotension

• Consider a stepped approach for the management of orthostatic hypotension for people with Parkinson's disease:
  • Advise the person to increase dietary salt and fluid intake (if appropriate); avoid caffeine at night; eat small, frequent meals; and avoid alcohol if possible.
  • Advise the person to elevate their bedhead by 30–40 degrees if possible.
  • Consider prescribing compression stockings after excluding arterial insufficiency. See the CKS topic on Compression stockings for more information.
  • Reduce the dose or stop any contributing medication, if possible and appropriate, such as antihypertensives (including diuretics), anticholinergics, and antidepressants.
  • Liaise with the person's specialist, or arrange a review regarding whether reducing or altering anti-parkinsonian medication may help. In addition, midodrine first-line or fludrocortisone second-line drug treatment (off-label indications) may be considered for some people, depending on potential drug interactions, contraindications, and monitoring requirements.
  • The Parkinson's UK information sheet Low blood pressure may be helpful.
• If symptoms persist or are troublesome, refer the person to a specialist falls service for further assessment and management. See the CKS topics on Blackouts and syncope and Falls - risk assessment for more information.

Speech and swallowing problems and weight loss

Speech and communication problems

• Consider referral to a speech and language therapist (SALT).
• The Parkinson's UK information sheet Speech and communication problems may be helpful.

Swallowing problems

• Refer the person promptly to a SALT for: 
  • A full swallowing assessment and strategies to improve the safety and efficiency of swallowing to minimize the risk of aspiration, such as expiratory muscle strength training (EMST).
  • Further investigations such as videofluoroscopy or fibre-optic endoscopic examination of swallow safety (particularly if silent aspiration is suspected).
  • Advice on possible food texture modification and the provision of thickeners, if appropriate.
• Liaise with the person's specialist team for advice on altering anti-parkinsonian medication, which may help symptoms in some people.
• If dysphagia is severe, the specialist team may consider options such as enteral feeding (for example, via a nasogastric tube for short-term feeding or percutaneous endoscopic gastrostomy [PEG] for longer-term feeding), if appropriate, depending on the person/family/carer wishes.
• The Parkinson's UK information sheet Eating, swallowing and saliva control may be helpful.

Unintended weight loss

• Monitor body weight, body mass index (BMI), and nutritional status regularly, and consider referral to a SALT for a full swallowing assessment.
• Liaise with the person's specialist team for advice on altering anti-parkinsonian medication, as weight loss may correlate with the severity of dyskinesia, if present.
• Consider referral to a dietician for advice on dietary changes such as food fortification, high energy/protein advice, and regular small energy-dense meals. Oral nutrition support may be advised if the person can no longer maintain their body weight through oral intake alone. The Parkinson's UK information sheet Diet and Parkinson's may be helpful.

Excessive salivation and sweating

Excessive salivation

• Consider referral to a speech and language therapist (SALT) for: 
  • A full swallowing assessment and strategies to improve the safety and efficiency of swallowing, such as expiratory muscle strength training (EMST).
• Consider referral to the person's specialist team, especially if SALT input has not improved symptoms.
  • Specialist treatments may include anticholinergic treatment with glycopyrronium bromide, or injection of the salivary glands with botulinum toxin A (both off-label indications).
• The Parkinson's UK information sheet Eating, swallowing and saliva control may be helpful.

Excessive sweating

• Excessive sweating may occur as an end-of-dose 'off' phenomenon. It may also occur during the 'on' period, when it is usually associated with dyskinesia.
• Exclude other causes of excessive sweating. See the CKS topic on Hyperhidrosis for more information.
• Liaise with the person's specialist, or arrange a specialist review, as sweating may respond to alterations in anti-parkinsonian medication.
• The Parkinson's UK information sheet Skin and sweating problems may be helpful.

Bladder and sexual problems

Bladder problems

• Exclude a urinary tract infection if there is a sudden change in voiding pattern, such as frequency, urgency, or nocturia. See the CKS topics on Urinary tract infection (lower) - men and Urinary tract infection (lower) - women for more information.
• If frequency and polyuria are troublesome, consider underlying causes such as diabetes mellitus, prostate disease, or detrusor overactivity. See the CKS topics on Diabetes - type 2, Incontinence - urinary, in women, LUTS in men, and Prostatitis - chronic for more information.
• If urinary symptoms persist:
  • Liaise with the person's specialist to consider alteration of anti-parkinsonian medication or use of an antimuscarinic drug, to help symptoms.
  • Consider referral to a urologist, if appropriate, for further specialist assessment, such as urodynamics, if clinically appropriate.
  • The Parkinson's UK information Bowel and bladder problems may be helpful.

Sexual problems

• If there is hypersexuality:
  • Liaise with the person's specialist, or arrange a specialist review of anti-parkinsonian medication, as hypersexuality may be caused by dopaminergic drug treatment (even when erectile dysfunction is reported).
• If there is erectile dysfunction or anorgasmia:
  • Screen for underlying depression.
  • Exclude co-morbid endocrine causes, such as hypothyroidism or hyperprolactinaemia.
  • Consider stopping drugs that may be contributing (for example, alpha-blockers may cause erectile dysfunction; selective serotonin reuptake inhibitors may cause anorgasmia).
  • Consider prescribing a phosphodiesterase type-5 inhibitor — available on the NHS for men with Parkinson's disease. See the CKS topic on Erectile dysfunction for more information.
  • Consider referral to a urologist if clinically appropriate.
  • The Parkinson's UK information Sex and Parkinson's may be helpful.

Basis for recommendation

The recommendations on managing non-motor symptoms and complications in Parkinson's disease are largely based on the National Institute for Health and Care Excellence (NICE) clinical guideline Parkinson's disease in adults: diagnosis and management [NICE, 2022], the American Academy of Neurology (AAN) publication Practice parameter: treatment of nonmotor symptoms of Parkinson disease [Zesiewicz, 2010], the British Dietetic Association (BDA) publication Best practice guidance for dietitians on the nutritional management of Parkinson's [BDA, 2021], and an evidence-based review article Summary of the recommendations of the European Federation of Neurological Societies (EFNS)/ Movement Disorder Society-European Section (MDS-ES) review on therapeutic management of Parkinson's disease [Ferreira, 2013].

Constipation, nausea and vomiting

• Recommendations are also based on the BDA best practice guidance [BDA, 2021], expert opinion in review articles [Chou, 2008; Muzerengi, 2015; Halli-Tierney, 2020; Kobylecki, 2020], and the Medicines and Healthcare products Regulatory Agency (MHRA) Drug Safety Updates Domperidone: risks of cardiac side effects [MHRA, 2014] and Apomorphine with domperidone: minimising risk of cardiac side effects [MHRA, 2016].

Pain

• Recommendations are also based on expert opinion in review articles [Drake, 2005; Coelho, 2008; Nègre-Pagès, 2008; Valkovic, 2015].

Sleep disturbance and daytime sleepiness

• Recommendations are also based on expert opinion in review articles [Halli-Tierney, 2020; Kobylecki, 2020].

Depression and anxiety

• Recommendations are also based on expert opinion in the NICE clinical guideline Depression in adults with a chronic physical health problem [NICE, 2020], the American Academy of Neurology's Practice parameter: treatment of nonmotor symptoms of Parkinson disease [Zesiewicz, 2010], and expert opinion in review articles [Lemke, 2008; Rihmer, 2014; Kalia, 2015]  [Halli-Tierney, 2020; Kobylecki, 2020].

Dementia and cognitive impairment

• Recommendations are also based on the NICE clinical guideline Dementia: assessment, management and support for people living with dementia and their carers [NICE, 2021b] and the evidence-based guidelines European Federation of Neurological Societies (EFNS)/ Movement Disorder Society-European Section (MDS-ES) recommendations for the diagnosis of Parkinson's disease [Berardelli, 2013].
• Recommendations are also based on expert opinion in review articles [Chou, 2008; Lim, 2008; Weintraub, 2008; Wolters, 2008; Kalia, 2015; Kobylecki, 2020].

Impulse control disorders and psychotic symptoms

• Recommendations are also based on expert opinion in review articles [Halli-Tierney, 2020; Kobylecki, 2020].

Orthostatic hypotension

• Recommendations are also based on the British Dietetic Association (BDA) publication [BDA, 2021] and expert opinion in review articles [Halli-Tierney, 2020; Kobylecki, 2020]. They are also pragmatic, based on what CKS considers to be good clinical practice.

Speech and swallowing problems and weight loss

• Recommendations about speech and communication problems are based on the NICE guideline [NICE, 2022] and expert opinion in a review article [Kobylecki, 2020].
• Recommendations about swallowing problems and weight loss are also based on the British Dietetic Association (BDA) publication, which notes that swallowing problems should be addressed promptly to prevent weight loss and malnutrition [BDA, 2021].

Excessive salivation and sweating

• CKS notes that the NICE clinical guideline did not make any specific recommendations as to the optimal route of administration of the anticholinergic glycopyrronium bromide to minimize the risk of adverse effects, as this drug may be delivered sublingually or in injection form [NICE, 2022]. As a result, CKS recommends referral to the specialist team if this is being considered, based on what CKS considers to be safe clinical practice.
• The recommendations are also supported by expert opinion in review articles [Halli-Tierney, 2020; Kobylecki, 2020].

Bladder and sexual problems

• Recommendations are also based on an international guideline for the management of bladder dysfunction in Parkinson's disease [Sakakibara, 2016] and the AAN practice parameter publication, which provides evidence from a small study that dopamine agonist medication can improve voiding efficiency and flow rates [Zesiewicz, 2010]. They are supported by expert opinion in a review article [Halli-Tierney, 2020].

Basis for recommendation

The recommendations on when to suspect end-stage Parkinson's disease are largely based on the National Institute for Health and Care Excellence (NICE) clinical guideline Parkinson's disease in adults: diagnosis and management [NICE, 2022] and the Gold Standards Framework Proactive Identification Guidance [Thomas, 2016].

• The Gold Standards Framework lists a number of general indicators of decline and increasing needs, and also specific features of Parkinson's disease that may suggest a person is nearing the end of life [Thomas, 2016].

Advance care plans

• An advance care plan should address:
  • Anticipatory prescribing of medication to manage end-of-life symptoms.
  • Discontinuing inappropriate medication or interventions.
  • Needs for psychological and spiritual care.
  • Care of the family (before and after the person's death).
  • When, who, and how to call for help if there is a crisis, and what the options are for management.
  • The person's preferences for the place they wish to be cared for, and whether resuscitation should be attempted if they were to have a life-threatening deterioration. This information should be available to out-of-hours and ambulance services.
  • Any expressed views on organ or tissue donation, including the possibility of donating tissue to a brain bank for diagnostic confirmation and research.
• An advance care plan should be reviewed and updated as the person's situation or views change.
  • For more information on advance care planning, see the CKS topic on Palliative care - general issues.

[DH, 2012]

Advance decisions

• Healthcare professionals are responsible for determining whether a valid advance decision or advance directive exists. These are statements that:
  • Allow the person to specify (before they have lost the capacity to decide) what treatments they would not want and would not consent to (for example, cardiopulmonary resuscitation).
  • Cannot demand treatments.
  • Must be respected by clinicians.
  • Can be withdrawn if the person retains (or regains) capacity.
  • Can be made verbally, except for decisions that refuse life-sustaining treatment (such as artificial ventilation), which must be written, signed, and witnessed.
  • Cannot refuse basic care, such as the provision of warmth, shelter, hygiene, food, and water. However, clinically-assisted nutrition and hydration (that is given intravenously, subcutaneously, or via a gastrostomy), which are considered in law to be medical treatments, can be refused.
• An advance refusal of treatment is binding if:
  • The person making the advance refusal was at least 18 years of age and had the necessary mental capacity.
  • It specifies treatment to be refused, and the applicable circumstances.
  • It has not been withdrawn.
  • Nobody has subsequently been given Lasting Power of Attorney to make treatment decisions on the person's behalf.
  • The person making the advance refusal has not subsequently given reason to believe they have changed their mind.
• The legal framework for advance decisions is provided by the Mental Capacity Act 2005, which also provides for the resolution of disputes and disagreements about advance decisions. For further information, see the British Medical Association (BMA) Mental Capacity Act toolkit and the General Medical Council (GMC) publication Treatment and care towards the end of life: good practice in decision making.

Basis for recommendation

The recommendations on managing end-stage Parkinson's disease are largely based on the National Institute for Health and Care Excellence (NICE) clinical guidelines Parkinson's disease in adults: diagnosis and management [NICE, 2022] and Pressure ulcers: prevention and management [NICE, 2019], the General Medical Council (GMC) publication Treatment and care towards the end of life: good practice in decision making [GMC, 2022], the British Dietetic Association (BDA) publication Best practice guidance for dietitians on the nutritional management of Parkinson's [BDA, 2021], a Delphi study of quality of palliative care [Rogers, 2023], and a palliative care textbook A guide to symptom relief in palliative care [Regnard, 2022].

• A Delphi study of the quality of palliative care for people with Parkinson's disease states that opportunities for advance care planning should be available early in the disease trajectory, depending on the needs and wishes of the person and their family/carers. These discussions should be sensitive, flexible, and offered at regular intervals, including wishes about withdrawal of care at the end of life. The study also highlighted that the benefits of anti-parkinsonian and other medication should be weighed against potential adverse effects in the last weeks of life. In particular, it notes that dopaminergic medication may be less effective, cause adverse effects, and be difficult to deliver by the oral route in the last weeks of life. Specialist advice may be needed to guide whether medication is continued or withdrawn [Rogers, 2023].
• The recommendation about considering withdrawing artificial nutritional support is based on the BDA best practice guidance [BDA, 2021].
• The information that some people with Parkinson's disease may not show typical symptoms of pain, distress, or agitation at the end of life is based on recommendations from a Delphi study of quality of palliative care for people with Parkinson's disease, which notes that a person may be unable to communicate their needs effectively, and may not have a carer/advocate to do this for them [Rogers, 2023]. It is also pragmatic, based on what CKS considers to be good clinical practice.