Most people with Parkinson’s have Ideopathic Parkinson’s, which means there is no clear genetic or environmental cause for them developing the condition.

A much smaller number of people have Familial Parkinson’s, which means they have inherited the condition through their genes. The most common example of this is a change in the LRRK2 gene, which is more common in North African and certain Jewish (Ashkenazi) populations.

There is also a growing number of people developing Parkinson’s before the age of 40, which is called Young or Early-Onset Parkinson’s.

Find out more about the symptoms, causes, diagnosis and treatment of Parkinson’s.

Parkinsonisms

There are also several other conditions which feature Parkinson’s-like symptoms, such as tremor (shaking), muscle stiffness and slowness of movement (also known as bradykinesia).

We call these conditions Parkinsonisms. The first seven conditions below all fall into this group, and we’ve also given some extra information on Young-Onset Parkinson’s:

Corticobasal Degeneration

Corticobasal Degeneration is a rare type of parkinsonism that affects people from the age of 40, but typically between the ages of 50 and 70. It tends to affect one side of the body more than the other initially, gradually spreading over a few years.

The condition has similarities with Progressive Supranuclear Palsy (PSP) – some people with Corticobasal Degeneration go on to develop PSP, and vice versa.

Symptoms

Corticobasal Degeneration is a very individual condition and symptoms vary from person to person. It’s a progressive neurodegenerative condition, so symptoms gradually become worse over time.

The most common symptoms are:

  • Movement difficulties
    These can cover a variety of symptoms, such as:

    • Difficulty controlling the limbs on one side of the body – often known as ‘alien limb’ syndrome – as arms or legs may seem to move independently
    • Numbness and loss of coordinated movement in one hand (apraxia), making everyday tasks such as dressing, writing, and eating difficult
    • Muscle stiffness (rigidity)
    • Shaking (tremor)
    • Jerky or awkward movement and spasms (dystonia)
    • Balance and coordination problems.
  • Speech problems, like slow and slurred speech.
  • Swallowing difficulties: as swallowing becomes progressively more difficult, food may ‘go down the wrong way’. This can lead to chest infections or pneumonia.
  • Cognitive and behavioural changes: thinking may become impaired, leading to memory problems and difficulty understanding and interpreting communication. It may also be difficult to carry out complex tasks that require planning ahead. Changes in personality can also occur, such as apathy, irritability, and a decreased interest in things you previously enjoyed.

Causes
Corticobasal Degeneration occurs when cells in specific parts of the brain – the cortex and basal ganglia – are damaged as a protein called tau builds up, and over time causes harmful clumps.

In normal brains, tau is broken down to avoid a build-up, but in Corticobasal Degeneration this does not happen. It is thought there may be some weak genetic link, but the risk of other family members developing the condition is very low.

Diagnosis
Unfortunately, as with Parkinson’s, there is no single test or scan to diagnose Corticobasal Degeneration. Diagnosis is usually based on the pattern of symptoms experienced and the exclusion of other conditions that may cause similar symptoms, such as Parkinson’s or stroke.

A specialist with experience in Corticobasal Degeneration, usually a neurologist, may ask for a brain scan to rule out other causes, and carry out tests to check memory, concentration, and understanding of verbal communication.

Treatment

There is currently no cure for Corticobasal Degeneration, but certain treatments can relieve symptoms and improve quality of life:

  • Medication can improve movement, cognitive and behavioural problems. However, the improvement is usually much less than in Parkinson’s.
  • Physiotherapy can help with movement and balance problems.
  • Speech and language therapy can help with swallowing and communication difficulties.

An occupational therapist can advise on equipment and adaptations in the home, and can suggest strategies for carrying out daily tasks to retain as much independence as possible.

Further information

More information and support is available from the Frontotemporal Dementia Support Group – FTDSG, The Association for Frontotemporal Degeneration – AFTD, and the Progressive Supranuclear Palsy (PSP) Association – PSP Europe.

 


Dementia with Lewy Bodies (DLB)

Dementia with Lewy Bodies (DLB) – also known as Lewy body disease – is a form of dementia, a group of illnesses that affect the brain, causing memory and thinking problems. 

DLB is  less common than Alzheimer’s disease and vascular dementia but may account for 20% of dementia cases in people over 65 years old, becoming increasingly common over the age of 75. It is relatively uncommon in younger people.

This is an overview of Dementia with Lewy Bodies – for detailed information see The Lewy Body Society.

Symptoms

DLB shares many symptoms with both Alzheimer’s disease (like confusion and memory loss) and Parkinson’s (like slow movement), so it is often misdiagnosed. 

Symptoms vary from one person to another, but some of the most common are:

  • Fluctuating from alertness to confusion
  • Poor memory, concentration and attention span
  • Difficulty finding the right words when speaking
  • Visual hallucinations, often involving children and/or animals
  • Difficulty recognising familiar faces and objects
  • Difficulty carrying out simple everyday tasks
  • Difficulty with spatial awareness, walking and balance, often leading to falls
  • Disrupted sleep and possibly nightmares
  • Slowness of movement, stiffness, tremor and facial masking
  • Swallowing difficulties
  • Depression
  • Fainting
  • Severe sensitivity to neuroleptic (anti-psychotic) medications
  • Occasionally, changes in blood pressure.


Causes

DLB is caused by the build-up of microscopic protein deposits called Lewy bodies – which are linked to nerve cell death – in the brain. They’re named after the German doctor, Friedrich Lewy, who first described them in 1913.

It is unclear why these Lewy bodies form but, when they do, they interfere with the normal functioning of the brain. They are present in the brains of people with DLB and Parkinson’s.

The genetics of DLB are still unclear, but it seems that there is a slightly higher (but still quite low) risk of developing DLB if closely related family members have the illness.

Diagnosis

There are no specific tests for DLB. Instead an experienced doctor will base diagnosis on a full medical examination and take a detailed medical history. They will ask about your symptoms: (when they occur and how long they last) and test your thinking and memory processes.  

Since symptoms can fluctuate, it may take several appointments over time to reach a diagnosis. Key features that might lead to a diagnosis of DLB are visual hallucinations, extreme and rapid fluctuations in alertness and attention span, and the presence of symptoms such as tremor, stiffness and slowness.

The doctor may carry out tests to rule out other types of dementia. These could include a brain scan and blood tests.

Treatment

There is currently no cure for DLB, but symptoms can often be alleviated with treatments such as:

  • Medications, including:
    • Alzheimer’s or Parkinson’s medications
    • Some new antipsychotic drugs like quetiapine can be used to help reduce hallucinations and psychosis, but only in certain circumstances due to potential harmful side effects. 
    • Rivastigmine may help reduce hallucinations and slow the pace of deterioration. 
    • Co-beneldopa or co-careldopa are sometimes used in low doses to help with stiffness and lack of movement.
  • A psychiatrist, psychologist or counsellor will be able to help with some of the behavioural and psychological aspects of DLB.
  • Physiotherapy will be able to help with problems related to muscle stiffness and movement
  • Occupational therapy can suggest specialist equipment and strategies to overcome everyday difficulties as well as ways to stay motivated and to carry on doing things you enjoy.


 


Drug-induced parkinsonism

A small number of people develop parkinsonism after taking certain medications. People with Parkinson’s may also find their symptoms get worse after they use these medicines. This is known as drug-induced parkinsonism.

The medicines involved are generally those that block the action of dopamine, the chemical that the brains of people with Parkinson’s gradually stop producing. They include:

  • Neuroleptic or antipsychotic drugs used to treat schizophrenia and other psychiatric problems
  • Prochlorperazine (Stemetil) used to treat dizziness and nausea
  • Metoclopromide (Maxolon, Plasil) used to treat nausea, vomiting and indigestion.

The symptoms of drug-induced parkinsonism tend to stay the same. Only in rare cases do they progress as the symptoms of Parkinson’s do. Most people will recover within months, and often within hours or days, of stopping the drug that caused the dopamine block.

 


Essential Tremor (ET)

Everyone has a slight tremor in the hands, especially when stressed or anxious – this is normal, and is known as physiological tremor.

Some people, however, develop an abnormal (or “pathological”) tremor, which is caused by conditions such as Parkinson’s, and a relatively common condition called Essential Tremor (ET). 

This is much more common than Parkinson’s: in the UK population of 64 million, research suggests that up to one million people have ET, while 80-120,000 have Parkinson’s.

Symptoms

Essential Tremor causes trembling in the hands or arms, which in some cases can then spread to cause tremor of the head, legs, torso or voice.

ET is often confused with Parkinson’s because people with the condition also experience tremors, but in Parkinson’s the trembling is usually more apparent when the hands are resting or while walking. ET, on the other hand, is a form of ‘action tremor’ – it is more pronounced when the hands are performing an action, for example holding a cup or using cutlery.

Causes

The cause of ET is not known. However, it often runs in families (about 50% of people with ET have a family member with the condition), and it becomes more common with age. 

Diagnosis

If you believe you have symptoms of Essential Tremor, you should see your doctor for an assessment, which will usually include:

  • Taking a detailed personal and family history.
  • A thorough physical examination
  • A blood test to check your thyroid function, as an overactive thyroid can cause an exaggerated tremor of the hands. 
  • Asking you about the medications you take, as some medications can cause an abnormal tremor of the hands.

It can be difficult to distinguish between Parkinson’s and Essential Tremor, but there are some key differences:

  • In ET, shaking usually first appears in both hands, but in Parkinson’s, tremor usually starts on one side of the body (typically beginning in a hand). 
  • Other Parkinson’s symptoms are not seen in ET, for example rigidity, slowness of movement or gait disturbance. 
  • A particular scan, called a dopamine transporter scan, (DaTSCAN) can distinguish between tremors caused by ET and Parkinson’s, as the scan is abnormal in Parkinson’s and normal in ET.

An accurate diagnosis usually becomes clearer over time, so you may need to visit the specialist several times before a definitive diagnosis can be made.

Treatment

There are various ways to treat Essential Tremor.

Medication:

  • Usually (unless you have asthma) your doctor will prescribe a beta-blocker called propranolol. In more severe cases of ET, your doctor should refer you to a neurologist or specialist doctor for a more detailed assessment.
  • Primidone is another drug that may be prescribed, as well as topiramate, gabapentin, alprazolam, clonazepam and other beta-blockers (such as atenolol and sotalolol).
  • Botulinum toxin injections can be effective, particularly for tremor of the head and voice. 

Surgical treatments:

  • Deep Brain Stimulation (DBS) can help some people with severe tremors that do not respond to ET medications.
  • A new non-invasive surgical technique involving highly focussed ultra-sound beams (called MR guided Focussed Ultrasound, or MRgFUS) has been used to treat people with ET by alleviating tremor in one of their arms.

Lifestyle changes:

  • Avoiding stimulants such as caffeine and also some recreational drugs
  • Trying to limit  emotional or physical stress, anxiety, fatigue and other illness. 
  • In about 50% of cases, ET can be suppressed for a few hours with alcohol, however excessive alcohol consumption causes various serious health problems, so it is recommended to follow responsible drinking guidelines.

 


Multiple System Atrophy (MSA)

Multiple System Atrophy (MSA) is a rare illness of the nervous system, which causes damage (or “atrophy”) to nerve cells in several areas (or “multiple systems”) of the brain. 

This damage can result in a number of problems related to movement, balance and unconscious body functions like bladder control. These symptoms are very similar to Parkinson’s, but MSA progresses faster and does not respond as well to medication.

Symptoms

Symptoms vary according to which part of the brain is affected by loss of nerve cells, and are different for everyone, but some of the most common symptoms include:

  • Slowed movements
  • Rigidity or stiffness
  • Difficulty turning in bed
  • Difficulty in initiating a movement
  • Difficulty writing – writing may become small and spidery
  • Loss of balance and poor coordination or clumsiness (ataxia)
  • Speech difficulties, including soft voice (particularly when tired) which over time becomes slurred, then incomprehensible
  • Difficulty with fine motor skills such as doing up buttons
  • Impotence
  • Loss of bladder control, including urgency, frequent urination or incomplete emptying, often getting up several times at night to pass urine
  • Bowel problems, including constipation
  • Inability to sweat
  • Cold hands and feet
  • Drop in blood pressure when standing (postural hypotension), leading to dizziness, fainting or blurred vision
  • Coat hanger pain’ (pain in neck or shoulders) as a result of low blood pressure
  • Problems with swallowing
  • Emotions that are easily aroused or tend to change quickly
  • Depression, anxiety or a feeling of being overwhelmed
  • Restless sleep
  • Noisy breathing, including snoring when sleeping

Causes

Both men and women can be affected by MSA. It usually starts between the ages of 30 and 60, although it can also affect older people. 

MSA is caused by the overproduction of a protein in the brain called alpha synuclein, which mainly damages three areas of the brain: the basal ganglia, the cerebellum and the brain stem. What triggers this process is the subject of ongoing research – at present, the cause is unknown.

It does not appear to be inherited, although it may be that some people are genetically more predisposed to developing it..

Diagnosis

MSA can take time to diagnose, especially early on when symptoms appear very similar to Parkinson’s – but it’s important to get an accurate diagnosis as the two conditions are treated differently.

A neurologist or a movement disorder specialist will use a specific set of criteria to make careful observations over a period of time in order to reach a diagnosis, so several visits may be needed.

There is no specific test to confirm MSA, but brain scans and blood tests may help to rule out other possible causes. 

Treatment

Currently there is no cure for MSA, but there are treatments that can help to manage some of the symptoms. Because MSA is a complex illness, a combination of approaches will probably be suggested, which may include:

  • Medication, often including Parkinson’s medicines, can treat movement symptoms, regulate blood pressure and improve bladder control.
  • Occupational therapists, physiotherapists and speech and language therapists can advise on specialist equipment and strategies to help overcome everyday difficulties like turning in bed and eating.
  • Complementary therapies can help with relaxing stiffness and rigidity in muscles and relieving stress.

Further information

Find out more

More information and support is available from local MSA organisations, for example The MSA Trust in the UK. To find out if there is an MSA organisation in your area, ask your healthcare professional or search online.

 


Progressive Supranuclear Palsy (PSP)

Progressive supranuclear palsy (PSP) is a rare neurodegenerative brain condition which causes problems with balance, thought processes, eye movements, speech and the ability to swallow.

To decode the name: Progressive means “gradually getting worse”. Supranuclear refers to the area “above the nuclei of the brain”, and Palsy means “paralysis”.

PSP is sometimes misdiagnosed as Parkinson’s, Alzheimer’s, or other forms of dementia. However, PSP progresses much faster, causes more severe symptoms, responds very poorly to Parkinson’s medication, and has a significantly reduced life expectancy.

This is an overview of Progressive Supranuclear Palsy – for detailed information see PSP Europe and Cure PSP.

Symptoms

The course of the illness varies from one person to another, as does the order in which symptoms appear, but some of the most common symptoms of PSP are:

  • Poor balance and unsteady gait or walking
  • Frequent falls, generally backwards
  • Slowed movements
  • Rigidity or stiffness
  • Cramped writing
  • Blurred eyesight, gaze palsy (the inability to look up or down), slow blinking, difficulty in maintaining eye contact, tunnel vision and sensitivity to light
  • Soft, slow, and slurred speech
  • Difficulty in swallowing
  • Anxiety and/or depression, and loss of motivation
  • Forgetfulness
  • Personality changes, like loss of interest in ordinary pleasurable activities or increased irritability, difficulty resisting impulses, and inappropriate behaviour
  • Difficulty synthesising several different ideas into a new idea or plan
  • Slowing of thought
  • Difficulty finding words (aphasia)

Causes

PSP affects people over the age of 40, but symptoms tend to begin after the age of 60. Men are affected slightly more often than women.

The condition is caused by the build-up of a protein called tau in certain areas of the brain, forming clumps, which gradually damage nerve cells. An overproduction of tau also causes Corticobasal Degeneration (see above) and Alzheimer’s, and these conditions are referred to collectively as tauopathies.

Unfortunately, we don’t yet know what causes PSP, and it is a very rare condition. There is some evidence that chemicals in the environment or poor diet may be a factor, and some research also suggests that some people may be more genetically disposed to it. 

Diagnosis

PSP can be difficult to diagnose and is quite easy to confuse with other conditions with similar symptoms. The means diagnosis may take up to three years, which can be frustrating, but research into faster, more accurate diagnosis is ongoing.

There is no specific test to definitively diagnose PSP, but a neurologist or movement disorder specialist may try the following:

  • Taking a thorough medical history
  • A physical and neurological exam
  • Scanning techniques, such as brain magnetic resonance imaging, which may show some shrinkage at the top of the brain stem. 
  • Other imaging tests, which can help to display activity in the brain areas which are affected by neurodegeneration in general.

Differentiating between PSP and Parkinson’s is hard, but there are some key differences:

  • Tremor (shaking) affects about 70% of people with Parkinson’s, but only about 10% of people with PSP. It’s more noticeable when the hands are in use in PSP, and at rest in Parkinson’s.
  • Posture-wise, people with Parkinson’s tend to bend forwards, whereas people with PSP stand straight or tilt backwards
  • People with PSP find it hard to look up or down, whereas people with Parkinson’s may experience other eye-related problems, including double vision, uncontrolled blinking or excessive watering. 
  • Problems with speech and swallowing tend to be more common and severe in PSP than in Parkinson’s and are often more apparent earlier
  • The sense of smell may remain intact in PSP whereas it is often lost in Parkinson’s.

Treatment

There is currently no specific treatment for PSP, but various things can help with symptoms:

  • Medication: Sadly, few medications treat PSP effectively. The Parkinson’s medication levodopa may help in 20-30% of cases. Botox or Myobloc can sometimes be injected to help involuntary movements like closure of the eyelids (blepharospasm) and turning or bending of the head, but this is only suitable for some people.
  • Supportive therapies: an occupational therapist or physiotherapist will be able to provide gait assessment and suggest equipment and practical strategies. A speech and language therapist can advise on overcoming difficulties with speech, eating and swallowing.
  • Palliative medicine can be a great help. For example, muscle relaxants or gentle massage can help with cramps; bathing eyes with cotton wool dipped in cooled boiled water can ease the extreme dryness of the eyes due lack of eyelid blinking. 
  • Feeding: as the illness progresses, there is an increased risk of complications such as pneumonia due to an inability to swallow, so it may be necessary to introduce a feeding or PEG tube

Further information

Find out more

More information and support is available from PSP organisations like PSP Europe and Cure PSP. To find out if there is a PSP organisation in your area, ask your healthcare professional or search online.

 


Vascular (Arteriosclerotic) parkinsonism

Several small strokes in the part of the brain that controls position and movement can cause Parkinson’s-like symptoms, such as rigidity and slowness, walking with short, shuffling steps, speech and memory or thinking problems.

Vascular (Arteriosclerotic) parkinsonism can be difficult to distinguish from Parkinson’s. However, stroke symptoms tend to appear suddenly and do not progress, whereas the symptoms of Parkinson’s appear gradually and get worse over time. Vascular parkinsonism usually affects the legs more than the upper part of the body.

Doctors believe that high blood pressure and diabetes are the most likely risk factors, being the same causes underlying stroke and heart disease.

 


Early onset/Young onset Parkinson’s (YOPD)

Parkinson’s can occur at any age. Early onset Parkinson’s, also known as young onset Parkinson’s (YOPD), is defined as occurring in someone below the age of 40. 

Managing a neurological condition at a much earlier age and for a longer period of time can lead to challenges relating to financial planning, career adjustments and family or parenting responsibilities. But being younger also means adapting to change can be easier.

One of the most important steps you can take is to prioritise what is important to you, whether that’s exercise, nutrition, leisure time, relationships, or emotional wellbeing. Discussing these goals with those close to you can help you to prioritise and enable them to support you.

Symptoms

In early or young onset Parkinson’s, the symptoms you experience and how you respond to medication may differ slightly from older onset, although for some people these can be very similar.

Dystonia (involuntary muscle spasms) is a more common early motor symptom in early or young onset, whereas some of the non-motor symptoms that occur in older onset Parkinson’s, such as memory problems, are less common.

Causes

The causes of YOPD are the same as Parkinson’s in general, however research suggests that genetics may play more of a role in people who are diagnosed at an early age.

Diagnosis

Diagnosis for YOPD is usually via the same method as Parkinson’s diagnosed later in life.

Treatment

Much of the treatment for Young Onset Parkinson’s is the same as other forms of Parkinson’s, but there are some key differences:

  • Medication: motor (movement) symptoms generally respond well to medication in both young and older onset Parkinson’s. However, the Parkinson’s drug levodopa can make people with YOPD have earlier motor fluctuations like involuntary movements (dyskinesia) and “wearing off” (where your symptoms get worse as your medication wears off.) For this reason, young onset is usually treated initially with alternatives to levodopa, such as MAO-B inhibitors or dopamine agonists. Levodopa is generally only added in when other medications do not provide adequate symptom control. 
  • A surgical treatment called Deep Brain Stimulation (DBS) has also been shown to be effective at an earlier stage of Parkinson’s if medication no longer controls motor symptoms so well, and you may want to discuss this option with your care team. 
  • Levodopa/carbidopa intestinal infusion delivered directly to your small intestine by a pump may also be an option to discuss with your doctor if oral medication is no longer managing symptoms well.