Parkinson's - Clinical Presentation

Original Editor - Bhanu Ramaswamy as part of the APPDE Project

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Clinically Relevant Neuroanatomy

In keeping with current, person-focused, best practice for communicating with and about people with or without a disability, Physiopedia uses the terms "person/people with Parkinson's" (pwP) to emphasise the dignity of the person, rather than define them by their clinical diagnosis. This is a move away from the historic terms of Parkinson's Disease (PD). At times, when describing the pathophysiological process of the disease, Physiopedia may then use the term Parkinson's Disease.

For rehabilitation professionals who work with people with Parkinson's, it's always worth revisiting the main set of nuclei that govern learnt movement and their function: the basal ganglia.

To review the anatomy and physiology of the basal ganglia, please see this optional reading page.

Basal Ganglia Function

The Basal Ganglia nuclei

With regards to movement, the basal ganglia control well-learnt, long and complex movement sequences by coordinating or ensuring certain actions, including: pre-movement planning and preparation (putting plans into actions), initiation of movement, and sequencing and timing of movement. They also maintain cortically selected movement amplitude—i.e., the frontal cortex is involved in the choice of movement, after which the basal ganglia take over and communicate with other areas of the brain. The scale of a required movement is then calibrated through sensorimotor integration. For example, a person may start to walk with a normal step length, but if the amplitude is incorrectly executed, their steps soon shorten, progressing to a shuffling gait.

They are also involved in the control of various non-motor behaviours, including emotions, language, decision making, procedural learning, and working memory. 

Basal Ganglia Dysfunction

Basal ganglia dysfunction affects the automatic (involuntary) nature of our movements. This includes impaired performance of well-learnt motor skills and movement sequences, problems maintaining sufficient movement amplitude, difficulty in performing more than one task simultaneously (dual-tasking), difficulty in shifting motor and cognitive sets, slower mental processing, and perseveration (repetition) in thought and action.

Parkinson’s is characterised by a decrease in dopaminergic innervation in the basal ganglia. This results in a range of motor and non-motor symptoms.

Parkinson's Clinical Presentation

While Parkinson's causes various non-motor symptoms, it used to be considered primarily a motor (movement) condition. Most clinicians still diagnose Parkinsonism by observing for the following movement changes:

• bradykinesia (experienced as slow and fatigable movement) plus one or more of:
  • tremor (involuntary shaking of parts of the body)
  • rigidity (experienced as muscle stiffness)

Clinicians also take into consideration the non-motor symptoms

Non-motor symptoms used to be considered as a separate or lesser issue. However, it is now recognised that non-dopaminergic and non-motor symptoms often present before the appearance of motor symptoms, with further non-motor symptoms emerging as the condition progresses. They can dominate the life of a person living with Parkinson's, contributing to their ability to engage in or participate in their lifestyle choices, subsequently impacting their quality of life, as well as those close to them, and shortening life expectancy.

Non-motor symptoms remain hard to treat despite increased attention on the recognition and quantification of symptoms.

In the prodromal phase of Parkinson’s (before movement is noticeably affected), the common non-motor symptoms that people may experience are: sleep disturbances like REM sleep behaviour disorder, changes in gut function (e.g., constipation), olfactory dysfunction (loss of smell), mood disorders such as depression and anxiety, and cognitive impairment. These can appear 15-20 years before motor symptoms develop.

Tonal changes that occur in the prodromal phase, many years before Parkinson’s is diagnosed, might cause shoulder problems such as frozen shoulder, capsulitis, etc.

Over the course the of the condition, commonly experienced non-motor symptoms tend to be divided into the following categories (some symptoms are listed for each category).

Cognitive changes: thinking, reasoning and decision-making skills are usually affected. Persons with Parkinson's may also have problems with multi-tasking, concentration, learning and remembering, understanding and using language, planning and carrying out activities.

Sleep problems: this can include REM sleep disorder, which causes daytime tiredness and sleepiness. Sleep is disturbed as the condition progresses due to the altered ability to move automatically in sleep, or because the person is woken by cramps or by a need to go to the toilet. Once disturbed, returning to sleep becomes more problematic.

Neuropsychiatric changes: this includes changes in mood (e.g. depression, anxiety, apathy), and psychotic symptoms that can include illusions, hallucinations and delusion.

Physiological symptoms: this can include autonomic dysfunction, such as pain, genitourinary problems, constipation, ophthalmologic dysfunction, excessive sweating, drooling of saliva, restless leg syndrome and irregular heartbeat.

Early identification and effective management of non‐motor symptoms may be able to enhance the quality of life of people who have Parkinson's. It is, therefore, important to elicit from the individual whether they have any such (or other) symptoms; this can be done using the Non-Motor Symptoms Questionnaire. If this questionnaire is used, it is important to assure the pwP that they are not expected to get each of the mentioned symptoms over time.

The wide-ranging symptoms mean that health professionals refer to Parkinson's as a syndrome—i.e., more than one disease. This also means that the experience of living with Parkinson's is different for all individuals, especially when you add in their upbringing (including cultural and faith influences), their way of coping, their support network, as well as whether they have any other health issues.

The increasing drive towards using the personalised medicine approach means that the differences in how people present their Parkinson's symptoms are coming under scrutiny, with aspects of the person and their symptoms being researched as phenotypes or subtypes of Parkinson’s. The six most common ways Parkinson's are subtyped are differentiated as follows (see Table 1).

Table 1. Common Methods to Create Subtypes of Parkinson's

Method	Clinical Features
Parkinson's and other types of Parkinsonism	There may be other early changes in a person’s cognition, their speech or balance, leading to falls, which should make a clinician question a diagnosis of Parkinson’s, if this happens early on after diagnosis. For example, whilst people with Idiopathic Parkinson’s initially respond very well to dopaminergic medication, not all the other Parkinsonian conditions respond as well.
Age of onset	There are an increasing number of people diagnosed below the age of 50, this is referred to as Young or Early Onset Parkinson’s. For many, the medication type they are prescribed earlier in the condition may differ from a person diagnosed at an older age; they may experience more dystonia and pain, plus have different needs if trying to bring up children whilst maintaining employment on top of their own health and social needs. The initial movement symptoms of someone with Young Onset is generally considered as milder, and the rate of Parkinson’s progression and cognitive decline seems to be slower than someone diagnosed after the age of 50.
Genetic/familial or idiopathic	A large proportion of those diagnosed with Young Onset Parkinson’s have been found to have a specific gene thought to be responsible for their symptoms. There is a lot of research trying to understand the mechanism by which the genetic mutation causes Parkinsonian symptoms, and from this, work out a way to treat, repair, or even stop the process.
Which symptoms are most prominent	Tremor dominant Parkinson’s: people with tremor as their most noticeable symptom may have a better prognosis and a slower progression rate than people with other types of Parkinson’s. However, for some people, they feel an acute embarrassment being in public with a tremor, affecting their socialisation. People who experience more of a bradykinetic-rigid Parkinson’s have been shown to be slower and stiffer not just in movement, but in their cognition. They are more likely to have difficulties moving and be more prone to falling. Postural instability gait disorder: describes a cohort of people that have affected balance and postural changes, so are likely to fall more. This group have been found to have altered autonomic symptoms (including issues with their gut, heat regulation, and interrupted sleep). More recently, further work has been done to look at differences in age and gender—the older the person, the worse their cognitive and autonomic function, as well as quality of life. Other findings are that women tend to report more anxiety and issues with autonomic functioning and worse quality of life, which increases in severity as they age. They also have increased depressive symptoms, compared to men who generally experience worse cognitive functioning.
Whether a person's Parkinson's began in the gut or the brain	Gut-first: typically involves peripheral autonomic symptoms (sympathetic/parasympathetic nervous systems) then spreads to the brain. People with gut-first Parkinson's would be expected to show gastrointestinal and other peripheral autonomic symptoms early in the disease course. Brain-first : typically begins in the brain, then spreads to the brainstem and peripheral nervous system. People with brain-first Parkinson's would be expected to show central nervous system symptoms (motor, cognitive) earlier in the disease course. This information has more relevance for future research into targeting treatments.
Severity and variety of symptoms	A study by Johansson et al. subdivided pwP into three subtypes: mild-motor predominant intermediate diffuse-malignant   Further, the authors state: ‘the mild-motor predominant subtype resembles a brain-first type of α-synucleinopathy, which has been associated with relatively benign and focal motor symptoms. In contrast, the diffuse-malignant subtype resembles a body-first type of α-synucleinopathy that is characterized by diffuse motor and non-motor symptoms in combination with a more aggressive disease course.’

Subtyping (also known as phenotyping) Parkinson’s is still an emerging area of research, and not all practitioners agree with this approach—some argue that few pwP fit neatly into any single clinical category.

Clinically, some people may differ in their presentation if they are well medicated (on) compared to when their medication is ineffective (off). Also, it needs to be remembered that much research has been conducted on people of a white racial background, and those who present themselves for research trials; thus, the data is not often reflective of the global population.  As PD progresses over the years, the person's symptom profile may evolve, requiring adjustments to treatment approaches and management strategies.

However, understanding that there are likely to be differences in how a person presents will help us make treatment more specific to their needs. For example, for those with significant bradykinesia in the morning, a research study showed their medication to be suboptimal during the rest of the day—this can be better managed.

Progression of Parkinson's

Clinically, the progression of Parkinson's can be measured by various standards, including:

1. Hoehn and Yahr Scale
2. MacMahon and Thomas Clinical Staging Model

Hoehn and Yahr Scale

The Hoehn and Yahr Scale (H&Y Scale) is commonly used to describe how the motor symptoms of Parkinson’s progress. The original scale was published in a 1967 article by Melvin Yahr and Margaret Hoehn, and included stages 1 to 5.

Since then, a modified Hoehn and Yahr scale has been proposed with the addition of stages 1.5 and 2.5 to help describe the intermediate course of the disease, although a Task Force looking into the use of the scale suggest that the original form be used when looking at demographic presentations.

Table 2. Progression of Parkinson’s motor symptoms

Stage	Hoehn and Yahr Scale	Modified Hoehn and Yahr Scale
1	Unilateral involvement ONLY, usually with minimal or no functional disability	Unilateral involvement ONLY
1.5		Unilateral and axial involvement
2	Bilateral or midline involvement WITHOUT impairment of balance	Bilateral involvement WITHOUT impairment of balance
2.5		Mild bilateral involvement with recovery on pull test
3	Bilateral involvement Mild to moderate disability WITH impaired postural reflexes Physically independent	Mild to moderal bilateral involvement Some postural instability Physically independent
4	Severely disabling Still able to walk or stand unassisted	Severe disability Still able to walk or stand unassisted
5	Confinement to bed or wheelchair unless aided	Wheelchair user or bed confined unless aided

As noted in the Hoehn and Yahr Scale, at diagnosis, motor signs are usually unilateral, but they become bilateral as the condition progresses. Later, in the course of the Parkinson’s, additional signs may be present, including postural instability (e.g., tendency to fall backwards after a sharp pull from the examiner - the ‘pull test’) and orthostatic hypotension (OH).

MacMahon and Thomas Scale

MacMahon and Thomas Scale

MacMahon and Thomas (1998) provided a clinical staging classification. Their model is based on four stages of progression from a state of gaining the best health through to requiring support and comfort. The four stages are:

• diagnosis
• maintenance
• complex
• palliative

Unlike the Hoehn and Yahr scale, there is more fluidity with this model. It allows for periods when the person might deteriorate during an illness, whether related to Parkinson’s or not (e.g., chest infection, rehabilitation, post-fall and fracture), but regains their prior ability on recovery.

The authors tabulate the aims and priorities as follows (see Table 3). They also provided criteria for each stage.

Table 3. Stages, Aims and Priorities of the MacMahon and Thomas Clinical Staging Model

Stage	Name	Aims	Priorities
1	Diagnostic	To facilitate acceptance of the diagnosis	Provision of accurate, sensitive information; reduce distress, and relieve symptoms
2	Maintenance	Relief of morbidity and the prevention of complications	Maintenance of good health: anticipatory care, such as advice on medications, and referral and liaison with multidisciplinary team
3	Complex	Symptom management, reduce both physical and psychological distress of patients and carers	Drug therapy becomes more complicated Careful titration Neurosurgical options, pallidotomy, thalamotomy etc.
4	Palliative	Relief of symptoms and distress in patient and carers	Progressive dopaminergic drug withdrawal, analgesia, sedation, and other palliative techniques

MacMahon and Thomas criteria for entry into clinical staging categories

Conclusion

Parkinson’s is a complex neurodegenerative disorder. It causes a variety of symptoms that change over the course of the condition.

When considering the ways Parkinson’s can present clinically, physiotherapists should consider more than just the movement problems; non-motor symptoms affect mood and motivation, fatigue and interrupted sleep patterns can affect concentration and health, plus autonomic dysfunction (especially when related to continence). In addition, social isolation can impact rehabilitation potential.

Physiotherapy focuses on how a person's clinical presentation affects their ability or need to improve transfers, posture, limb function, balance (and falls), walking and general physical capacity. Symptoms such as fatigue, apathy, low mood, as well as slower and stiffer movement can impact how a person performs these activities. These symptoms can lead to poor fitness from inactivity, which impacts an individual’s independence and safety, as well as increasing their risk of developing co-morbidities. All of these, in turn, affect well-being and quality of life.

Resources

1. For physiotherapy-relevant information, refer to the European Physiotherapy Guideline for more information, including a breakdown in Table 2.5.2 on page 25 of the sub-types of Parkinson’s.
2. Quick Reference Cards In addition to the new Quick Reference Cards in the European Guideline, UK-specific cards can still be viewed for consideration. Reference and source: Ramaswamy B, Jones D, Goodwin V, Lindop F, Ashburn A, Keus S, Rochester L, Durrant K (2009).Quick Reference Cards (UK) and Guidance Notes for physiotherapists working with people with Parkinson’s disease. Parkinson’s Disease Society, London.
   

Related pages

• Parkinson's
• Anatomy, Pathology, Prognosis and Diagnosis
• Physiotherapy - Referral and Assessment
• Physiotherapy - Management and Interventions
• Key Evidence and resources