Article Figures & Tables
Figures
- Figure 1:
Diagnosis and prognosis of Parkinson disease (PD). Note: CT = computed tomography, MRI = magnetic resonance imaging, MSA = multiple system atrophy, NPH = normal pressure hydrocephalus, PSP = progressive supranuclear palsy.
Tables
- Box 1: Grading scheme from SIGN, EFNS and NICE*
Grade of recommendation Evidence A At least 1 meta-analysis, systematic review or RCT rated as 1++, and directly applicable to the target population; or
A body of evidence consisting principally of studies rated as 1+, directly applicable to the target population, and demonstrating overall consistency of resultsB A body of evidence including studies rated as 2++, directly applicable to the target population and demonstrating overall consistency of results; or
Extrapolated evidence from studies rated as 1++ or 1+C A body of evidence including studies rated as 2+, directly applicable to the target population and demonstrating overall consistency of results; or
Extrapolated evidence from studies rated as 2++D Evidence level 3 or 4; or
Extrapolated evidence from studies rated as 2+GPP Recommended best practice based on the clinical experience of the guideline development group Levels of evidence 1++ High-quality meta-analyses, systematic reviews of RCTs or RCTs with a very low risk of bias 1+ Well-conducted meta-analyses, systematic reviews or RCTs with a low risk of bias 1− Meta-analyses, systematic reviews or RCTs with a high risk of bias. High-quality systematic reviews of case–control or cohort studies 2++ High-quality case–control or cohort studies with a very low risk of confounding or bias and a high probability that the relationship is causal 2+ Well-conducted case–control or cohort studies with a low risk of confounding or bias and a moderate probability that the relationship is causal 2− Case–control or cohort studies with a high risk of confounding or bias and a substantial risk that the relationship is not causal 3 Nonanalytic studies (e.g., case reports, case series). Expert opinion 4 Meta-analyses, systematic reviews or RCTs with a high risk of bias. High-quality systematic reviews of case–control or cohort studies Note: EFNS = European Federation of Neurological Societies,14,15 GPP = good practice point, NICE = National Institute for Health and Clinical Excellence,7,8 RCT = randomized controlled trial, SIGN = Scottish Intercollegiate Guidelines Network.13
* When no grade was assigned or when a recommendation was created from a systematic review or RCT, the SIGN grading table was used.
Recommendation number Recommendation Source Grade C1 Communication with people with PD should be aimed at empowering them to participate in the judgments and choices about their own care. NICE7 D C2 Discussions should be aimed at achieving a balance between the provision of honest, realistic information about the condition and the promotion of a feeling of optimism. NICE7 D C3 Because people with PD may develop impaired cognitive ability, a communication deficit or depression, they should be provided with both verbal and written communication throughout the course of the disease — which should be individually tailored and reinforced as necessary — and consistent communication from the professionals involved. NICE7 D
GPPC4 Families and caregivers should be given information about the condition, their entitlements to care assessment and the support services available. NICE7 D
GPPC5 People with PD should have a comprehensive care plan agreed upon between the individual, their family and caregivers, and all health care providers. NICE7 D
GPPC6 People with PD should be offered an accessible point of contact with specialist services. NICE7 D
GPPNote: GPP = good practice point, NICE7 = National Institute for Health and Clinical Excellence 2006 PD Guidelines,7 PD = Parkinson disease.
Recommendation number Recommendation Source Grade C7 PD should be suspected in people presenting with tremor, stiffness, slowness, balance problems or gait disorders. NICE7 D
GPPC8 PD can be diagnosed using the MDS Clinical Diagnostic Criteria. CAN GPP C9 Clinicians should be aware of the poor specificity of a clinical diagnosis of PD in the early stages of the disease, and consider this uncertainty when giving information to the patient and when planning management. SIGN13 C C10 Patients should be offered long-term, regular follow-up to review the diagnosis of PD. This should include a review of the ongoing benefits in those started on dopamine replacement therapy. SIGN13 GPP C11 Patients initially considered to have a possible diagnosis of PD may benefit from a trial of dopamine replacement therapy to assist with an accurate diagnosis. SIGN13 GPP C12 Patients with suspected PD, with substantial disability or exclusion criteria or red flags as per the MDS diagnostic criteria, should be seen by a clinician with sufficient expertise in movement disorders to make the diagnosis. SIGN13 C
GPPC13 Acute challenge testing with either levodopa or apomorphine should not be used in the diagnosis of PD. Patients with suspected PD should be considered for a trial of chronic levodopa treatment. SIGN13 A C14 Objective olfactory testing is not recommended in the diagnosis of PD. SIGN13 B C15 Routine use of functional imaging is not recommended for the differential diagnosis of PD and Parkinson plus disorders such as progressive supranuclear palsy and multiple system atrophy. SIGN13 C C16 PET scanning is not recommended as part of the diagnostic work-up of parkinsonian syndromes, except within a research framework. SIGN13 GPP C17 123I-FP-CIT SPECT scanning should be considered as an aid to clinical diagnosis in patients where there is uncertainty between PD and nondegenerative parkinsonism or tremor disorders. SIGN13 B C18 Computed tomography or MRI brain scanning should not be routinely applied in the diagnosis of idiopathic PD. SIGN13 C C19 Vitamin E should not be used as a neuroprotective therapy for people with PD. Co-enzyme Q10 should not be used as a neuroprotective therapy for people with PD. NICE8 A C20 Levodopa (grade: GPP), amantadine (grade: GPP), dopamine agonists (pramipexole, ropinirole, rotigotine, apomorphine, bromocriptine) (grade: A), or MAO inhibitors (selegiline, rasagiline) (grade: A) should not be used as neuroprotective therapies for people with PD, except in the context of clinical trials. CAN Varied C21 Genetic testing for monogenic parkinsonism is not recommended in routine clinical practice. SIGN13 GPP C22 Patients who request genetic testing, particularly those with young-onset parkinsonism, should be assessed in a specialist movement disorders clinic for consideration of counselling and testing. SIGN13 GPP Note: 123I-FP-CIT = 123I-ioflupane, CAN = new Canadian Guideline recommendation, GPP = good practice point, MAO = monoamine oxidase, MDS = Movement Disorder Society Evidence-Based Medicine Review,16 MRI = magnetic resonance imaging, NICE7 = National Institute for Health and Clinical Excellence 2006 PD Guidelines,7 NICE8 = National Institute for Health and Clinical Excellence — 2017 PD Guidelines,8 PD = Parkinson disease, PET = positron emission tomography, SIGN13 = Scottish Intercollegiate Guidelines Network,13 SPECT = single-photon emission computed tomography.
Recommendation number Recommendation Source Grade General considerations C23 People with PD should have regular access to the following: Clinical monitoring and medication adjustment
A continuing point of contact for support, including home visits, when appropriate
A reliable source of information about clinical and social matters of concern to people with PD and their caregivers, which may be provided by a PD nurse specialist.
NICE7 C C24 Antiparkinsonian medication should not be withdrawn abruptly or allowed to fail suddenly owing to poor absorption (e.g., gastroenteritis, abdominal surgery), to avoid the potential for acute akinesia or neuroleptic malignant syndrome. NICE7 D
GPPC25 The practice of withdrawing patients from their antiparkinsonian drugs (so-called “drug holidays”) to reduce motor complications should not be undertaken because of the risk of neuroleptic malignant syndrome. NICE7 D
GPPC26 In view of the risks of sudden changes in antiparkinsonian medication, people with PD who are admitted to hospital or care homes should have their medication: i) given at the appropriate times, which in some cases may mean allowing self-medication; ii) adjusted by, or adjusted only after discussion with, a specialist in the management of PD. NICE7 D
GPPC27 Surveillance for dopamine dysregulation syndrome should be undertaken in patients receiving levodopa or intermittent apomorphine. SIGN13 GPP C28 When starting dopamine agonist therapy, people and their family members and caregivers (as appropriate) should be given verbal and written information about the following, and the discussion should be recorded as having taken place: The increased risk of developing impulse control disorders when taking dopamine agonist therapy, and that these may be concealed by the person affected
The different types of impulse control disorders (e.g., compulsive gambling, hypersexuality, binge eating and obsessive shopping)
Who to contact if impulse control disorders develop
The possibility that if problematic impulse control disorders develop, dopamine agonist therapy will be reviewed and may be reduced or stopped.
NICE8 GPP C29 It should be recognized that impulse control disorders can develop in a person with PD who is on any dopaminergic therapy at any stage in the disease course. NICE8 GPP Pharmacologic therapy in early PD C30 Before starting treatment for people with PD, the following should be discussed: The person’s individual clinical circumstances; for example, their symptoms, comorbidities and risks from polypharmacy
The person’s individual lifestyle circumstances, preferences, needs and goals
The potential benefits and harms of the different drug classes.
NICE8 GPP C31 Levodopa may be used as a symptomatic treatment for people with early PD. NICE7 A C32 The dose of levodopa should be kept as low as possible to maintain good function in order to reduce the development of motor complications. NICE7 A C33 Controlled-release formulations of levodopa or adding entacapone are not effective for delaying motor complications. EFNS14 A C34 Dopamine agonists may be used as a symptomatic treatment for people with early PD. NICE7 A C35 A dopamine agonist should be titrated to a clinically efficacious dose. If adverse effects prevent this, another agonist or a drug from another class should be used in its place. NICE7 D
GPPC36 Ergot-derived dopamine agonists should not be used as first-line treatment for PD. SIGN13 B C37 When an ergot-derived dopamine agonist is used, patients should undergo: Baseline echocardiographic screening and regular follow-up echocardiographic testing to identify cardiac abnormalities
Baseline laboratory (ESR, serum creatinine) and radiological (e.g., chest x-ray) investigations with regular follow-up surveillance to identify serosal fibrosis.
SIGN13 GPP C38 MAO-B inhibitors may be used as a symptomatic treatment for people with early PD. NICE7 A C39 There is insufficient evidence to support the use of amantadine in the treatment of patients with early PD. SIGN13 A C40 Anticholinergic drugs should not be used as first-line treatment in patients with PD. SIGN13 B C41 Beta-adrenergic antagonists may be used in the symptomatic treatment of selected people with postural tremor in PD, but should not be drugs of first choice. NICE7 D
GPPPharmacologic therapy for motor symptoms in later PD C42 The choice of an adjunct to levodopa for people with PD who have developed dyskinesia or motor fluctuations despite optimal levodopa therapy should take into account: The person’s individual clinical circumstances; for example, their PD symptoms, comorbidities and risks from polypharmacy
The person’s individual lifestyle circumstances, preferences, needs and goals
The potential benefits and harms of the different drug classes.
NICE8 GPP C43 Catechol-O-methyltransferase inhibitors (entacapone) and MOA-B inhibitors (rasagiline) may be considered for the reduction in off-time in patients with advanced PD who have motor fluctuations. SIGN13 A C44 Dopamine agonists (oral [pramipexole, ropinirole] or transdermal [rotigotine]) may be considered for the management of motor complications in patients with advanced PD. SIGN13 A C45 Levodopa controlled release may improve wearing-off (grade: C) and nighttime akinesia (grade: GPP). EFNS14 Varied C46 Subcutaneous apomorphine infusions or injections may be considered for the management of severe motor complications, but should be provided only in units that have sufficient experience and resources. SIGN13 C C47 Intrajejunal levodopa-carbidopa enteric gel administered through percutaneous gastrostomy may be considered for the reduction of off-time or to reduce dyskinesia. EFNS14 C C48 Amantadine is recommended for the treatment of dyskinesia in PD (200–400 mg/d). EFNS14 A Surgery C49 Deep brain stimulation of the STN or the GPi is effective against motor fluctuations and dyskinesia. EFNS14 A C50 With the current evidence, it is not possible to decide if the STN or GPi is the preferred target for deep brain stimulation for people with PD, or whether 1 form of surgery is more effective or safer than the other. NICE7 D C51 Thalamic deep brain stimulation may be considered as an option in people with PD who predominantly have severe disabling tremor. NICE7 D C52 Unilateral pallidotomy is efficacious at reducing contralateral dyskinesia. EFNS14 A C53 Unilateral thalamotomy improves contralateral tremor and rigidity but has no consistent effect on akinesia. EFNS14 D C54 Preoperative response to levodopa should be considered as a factor predictive of outcome after deep brain stimulation of the STN. AAN17 B C55 Age and duration of PD may be considered as factors predictive of outcome after deep brain stimulation of the STN. Younger patients with shorter disease durations may possibly have improvement greater than that of older patients with longer disease durations. AAN17 C Rehabilitation C56 Consideration should be given to referring people who are in the early stages of PD to a physiotherapist with experience of the disease for assessment, education and advice, including information about physical activity. NICE8 B C57 Physiotherapy specific to PD should be offered to people who are experiencing balance or motor function problems. NICE8 A C58 Consideration should be given to referring people who are in the early stages of PD to an occupational therapist with experience of PD for assessment, education and advice on motor and nonmotor symptoms. NICE8 B C59 Occupational therapy specific to PD should be offered to people who are having difficulties with activities of daily living. NICE7 A C60 Speech and language therapy should be offered to people with PD who are experiencing problems with communication, swallowing or saliva. Therapy should include: Strategies to improve the safety and efficiency of swallowing to minimize the risk of aspiration, such as expiratory muscle stress
Strategies to improve speech and communication, such as attention to effort therapies.
NICE8 A C61 Consideration should be given to referring people for alternative and augmentative communication equipment that meets their communication needs as PD progresses and their needs change. NICE8 GPP C62 Discussion should take place about a diet in which most of the protein is eaten in the final main meal of the day (a protein redistribution diet) for people with PD on levodopa who experience motor fluctuations. NICE8 GPP C63 People with PD should be advised to avoid a reduction in their total daily consumption of protein. NICE8 GPP C64 Consideration should be given to referring people with PD to a dietitian for specialist advice. NICE8 GPP C65 People with PD should be advised to take a vitamin D supplement. NICE8 B
GPPC66 People with PD should be advised not to take over-the-counter dietary supplements without first consulting their pharmacist or other health care professional. NICE8 GPP Note: AAN = American Academy of Neurology practice parameters,18 EFNS14 = European Federation of Neurological Societies — Motor Guidelines,14 ESR = erythrocyte sedimentation rate, GPi = globus pallidus interna, GPP = good practice point, MAO-B = monoamine oxidase B, NICE7 = National Institute for Health and Clinical Excellence 2006 PD Guidelines,7 NICE8 = National Institute for Health and Clinical Excellence — 2017 PD Guidelines,8 PD = Parkinson disease, SIGN13 = Scottish Intercollegiate Guidelines Network,13 STN = subthalamic nucleus.
Recommendation number Recommendation Source Grade Autonomic dysfunction C67 Botulinum toxin A is efficacious for the symptomatic control of sialorrhea in PD. MDS16 A C68 General measures for treating urinary urgency and incontinence include before bedtime, avoiding coffee and limiting water ingestion. When symptoms appear suddenly, exclude urinary tract infection. EFNS15 GPP • Nocturia: reduce intake of fluid after 6 pm. Sleep with head-up tilt of bed to reduce urine production. • Nighttime dopaminergic therapy should be optimized. • For urinary urgency (overactive bladder), anticholinergic or antispasmodic drugs may be useful, but care must be taken regarding central adverse effects. • Botulinum toxin type A injected in the detrusor muscle. C69 For orthostatic hypotension, general measures would include the following: EFNS15 GPP • Avoid aggravating factors such as large meals, alcohol, exposure to a warm environment and drugs known to cause orthostatic hypotension, such as diuretics or antihypertensive drugs. Levodopa and dopamine agonists may also worsen orthostatic hypotension. • Increase salt intake in symptomatic orthostatic hypotension. • Ensure head-up tilt of the bed at night. • Wear elastic stockings. • Highlight postprandial effects. In some patients, hypotension occurs only postprandially. Warning the patient about this effect and taking frequent small meals may be helpful. C70 For orthostatic hypotension, drug therapy includes the addition of: • Midodrine EFNS14 A • Fludrocortisone EFNS14 GPP • Domperidone. CAN GPP C71 For gastrointestinal motility problems in PD, general measures for treating constipation should be applied: EFNS14 Varied • Increased intake of fluid and fibre is recommended (grade: GPP). • Increased physical activity can be beneficial (grade: GPP). • Polyethylene glycol solution (macrogol) is recommended (grade: A). • Fibre supplements such as psyllium (grade: B) or methylcellulose and osmotic laxatives (e.g., lactulose) are recommended (grade: GPP). • Short-term irritant laxatives for selected patients are recommended (grade: GPP). • The use of drugs with anticholinergics activity should be reduced or discontinued (grade: GPP). • Domperidone should be added (grade: B). C72 For individuals with PD and erectile dysfunction: EFNS15 Varied • Drugs associated with erectile dysfunction (e.g., α-blockers) or anorgasmia (e.g., selective serotonin reuptake inhibitors) should be discontinued. Dopaminergic therapy can have both negative and positive effects on this symptom (grade: GPP). • Sildenafil 50–100 mg, 1 h before sex, can be tried in patients with PD with these problems (grade: B). • Other drugs of this class, such as tadalafil (10 mg, 30 min–12 h before sex) or vardenafil (10 mg, 1 h before sex) can be alternative choices (grade: GPP). • In some patients, apomorphine injections (5–10 min before sex) can also be an alternative treatment (grade: GPP). • Intracavernous injections of papaverine or alprostadil can be considered in selected patients (grade: GPP). Cognitive impairment C73 The diagnoses of dementia associated with PD and of mild cognitive impairment in PD can be made using the Movement Disorder Society Clinical Diagnostic Criteria. These require reports of subjective cognitive decline and difficulties on psychometric testing. CAN GPP C74 For PD dementia, cholinesterase inhibitors could be added: rivastigmine (grade: A), donepezil (grade: A), or galantamine (grade: C). There may be idiosyncrasy in clinical response and adverse effects, so it is worth trying an alternative agent (grade: GPP). Memantine can be added or substituted if cholinesterase inhibitors are not tolerated or lack efficacy (grade: C). EFNS14 Varied C75 No interventions have been proven to reduce the risk of progression of PD from mild cognitive impairment to dementia but lifestyle modifications, such as engaging in cognitive and social activities and physical exercise, are encouraged. CAN GPP Sleep disorders C76 A full sleep history should be taken from people with PD who report sleep disturbance. NICE7 D
GPPC77 Good sleep hygiene should be advised in people with PD with any sleep disturbance and includes: NICE7 D
GPP• Avoidance of stimulants (e.g., coffee, tea, caffeine) in the evening • Establishment of a regular pattern of sleep • Comfortable bedding and temperature • Provision of assistive devices, such as a bed lever or rails to aid with moving and turning, allowing the person to get more comfortable • Restriction of napping in the late afternoon and early evening • Advice about taking regular and appropriate exercise to induce better sleep • Advice to avoid remaining in bed for long periods of time if unable to sleep • A review of all medication and avoidance of any drugs that may affect sleep or alertness, or may interact with other medication (e.g., selegiline, antihistamines, H2 antagonists, antipsychotics and sedatives). C78 Optimization of nighttime dopaminergic treatment (grade: B), melatonin (grade: B) and low doses of sedating antidepressants such as doxepin or trazodone (grade: GPP) may be beneficial for subjective symptoms of insomnia in patients with PD. EFNS14 Varied C79 Care should be taken to identify REM sleep behaviour disorder in people with PD. Melatonin or clonazepam may be useful, if pharmacologic treatment is required. NICE8 GPP C80 Care should be taken to identify and manage restless legs syndrome in people with PD and sleep disturbance. NICE8 GPP Patients with bothersome restless legs syndrome should be screened for iron deficiency. CAN GPP Potential treatments include optimization of dopaminergic therapy or GABAergic agents such as pregabalin. C81 People with PD who have daytime sleepiness or sudden onset of sleep should be advised not to drive, and to consider any occupational hazards. Their medicines should be adjusted to reduce its occurrence. NICE8 GPP C82 Modafinil should be considered for the treatment of excessive daytime sleepiness in people with PD, only if a detailed sleep history has excluded reversible pharmacologic and physical causes. NICE8 B
GPPDepression C83 Clinicians should have a low threshold for diagnosing depression in PD. NICE7 D
GPPC84 Clinicians should be aware that there are difficulties in diagnosing mild depression in people with PD because the clinical features of depression overlap with the motor features of PD. NICE7 D
GPPC85 Self-rating or clinician-rated scales may be used to screen for depression in patients with PD. SIGN13 C • Diagnosis of depression should not be made on the basis of rating scale score alone. SIGN13 GPP • Assessment or formulation of depression should be carried out via clinical interview, with a focus on low mood, and with due caution in relation to interpretation of cognitive or somatic symptoms that may be symptoms of PD rather than depression. SIGN13 GPP • Relatives or caregivers who know the patient well should be invited to provide supplementary information to assist the diagnosis, particularly in the context of cognitive impairment. SIGN13 GPP C86 The management of depression in people with PD should be tailored to the individual — in particular, to their co-existing therapy. NICE7 D
GPPPsychosis C87 All people with PD and psychosis should receive a general medical evaluation and treatment for any precipitating condition. NICE7 D
GPPC88 For patients with PD and psychosis, polypharmacy should be reduced. EFNS14 GPP • Anticholinergic antidepressants should be reduced or stopped; anxiolytics or sedatives should be reduced or stopped. • Antiparkinsonian drugs should be reduced. Anticholinergics should be stopped, amantadine should be stopped, dopamine agonists should be reduced or stopped, MAO-B and COMT inhibitors should be reduced or stopped and, lastly, levodopa should be reduced. C89 Hallucinations and delusions should not be treated if they are well tolerated by the person with PD and their family members and caregivers (as appropriate). Even minor hallucinations or delusions should be considered a marker of disease progression, and should warrant a general medical evaluation and treatment for any precipitating factors. NICE8 GPP C90 For patients with PD and psychosis needing treatment: • Quetiapine is possibly useful. EFNS14 GPP • Clozapine is useful but requires monitoring. EFNS14 A C91 With the exception of quetiapine and clozapine as described in recommendation C90, all other antipsychotics should be avoided in PD psychosis (grade: GPP). Olanzapine (grade: A), risperidone (grade: C) and aripiprazole (grade: GPP) can worsen parkinsonism (harmful). EFNS14 Varied C92 Pimavanserin could be considered as a treatment for PD psychosis. CAN B Note: CAN = new Canadian Guideline recommendation, COMT = catechol-O-methyltransferase, EFNS14 = European Federation of Neurological Societies — Motor Guidelines,14 EFNS15 = European Federation of Neurological Societies — late Guidelines,15 GABA = γ-aminobutyric acid, GPP = good practice point, MAO-B = monoamine oxidase B, MDS = Movement Disorder Society Evidence-Based Medicine Review,16 NICE7 = National Institute for Health and Clinical Excellence 2006 PD Guidelines,7 NICE8 = National Institute for Health and Clinical Excellence — 2017 PD Guidelines,8 PD = Parkinson disease, REM = rapid eye movement, SIGN13 = Scottish Intercollegiate Guidelines Network.13
Recommendation number Recommendation Source Grade C93 People with PD and their family members and caregivers (as appropriate) should be offered opportunities to discuss the prognosis of their condition. These discussions should promote people’s priorities, shared decision-making and patient-centred care. NICE8 D C94 People with PD and their family members and caregivers should be given appropriate verbal and written information about the following, and it should be recorded that the discussion has taken place: Progression of PD
Possible future adverse effects of medicines for PD
Advance care planning, including orders for advanced decisions to refuse treatment and do not attempt resuscitation, and lasting power of attorney for finance and health and social care
Options for future management
What could happen at the end of life
Available support services; for example, personal care, equipment and practical support, financial support and advice, care at home and respite care.
NICE8 D C95 When discussing palliative care, it should be recognized that family members and caregivers may have different information needs from the person with PD. NICE8 D C96 Consideration should be given to referring people at any stage of PD to the palliative care team to give them and their family members or caregivers (as appropriate) the opportunity to discuss palliative care and care at the end of life. NICE8 D C97 Palliative care requirements of people with PD should be considered throughout all phases of the disease; this includes an option of medical assistance in dying. CAN GPP Note: CAN = new Canadian Guideline recommendation, GPP = good practice point, NICE8 = National Institute for Health and Clinical Excellence — 2017 PD Guidelines,8 PD = Parkinson disease.
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