Atypical Parkinson Disorders

Atypical Parkinsonism disorders are a group of diseases linked to a lack of dopamine in the brain. Dopamine controls movement. There is no ideal way to define and distinguish Parkinson’s disease from other parkinsonian syndromes.

While Parkinson’s is the most common Parkinsonism, approximately 20 percent*  of people will be diagnosed with another Parkinson’s – like condition. These conditions are often difficult to differentiate from Parkinson’s disease and each other. They include multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and Dementia with Lewy bodies (DLB). Dystonia is not a related condition; but some people living with Parkinson’s may have dystonia as a symptom of Parkinson’s disease.

Ideally, people suspected of having Parkinson’s disease or a related movement disorder, should be referred to a specialized movement disorders clinic or center for evaluation. For more information please call 1 (800) 565-3000.

* Practice Parameter: Diagnosis and Prognosis of new Onset Parkinson Disease, American Academy of Neurology, 2006.

The content on this page is provided for information purposes only, and does not represent advice, an endorsement or a recommendation, with respect to any product, service or enterprise, and/or the claims and properties thereof, by Parkinson Canada.


Multiple System Atrophy

Click here to download the PDF on Multiple System Atrophy

Multiple System Atrophy (MSA) is a progressive brain disorder caused by loss of nerve cells in specific areas of the brain. This loss causes problems with movement, balance and autonomic functions of the body.  (Autonomic functions are body functions that occur automatically, such as  bladder control.)

MSA includes conditions that were previously known individually as Shy-Drager syndrome, striatonigral degeneration and sporadic olivopontocerebellar atrophy. Researchers have learned that there is a common underlying cause in all three disorders, so they are now referred to as MSA. The term MSA stands for:

Multiple – many
System – brain structures that control different functions
Atrophy – cell shrinkage or damage


Which parts of the brain are affected in Multiple System Atrophy?

In MSA, cells are damaged in different areas of the brain which control a variety of body functions. The three areas affected are the basal gangliacerebellum and brain stem.

In MSA, brain cells in the affected areas shrink (atrophy). This can sometimes be seen on MRI scans. When brain tissue is examined under a microscope, structures called glial inclusion bodies can be seen; they contain a protein called alpha-synuclein. It is the presence of these inclusion bodies in the movement, balance and autonomic control centres of the brain that confirms a diagnosis of MSA.

 

Credit: Multiple System Atrophy Trust (UK), formerly known as The Sarah Matheson Trust used with permission www.msaweb.co.uk, 2010


What are the symptoms of MSA?

The symptoms of MSA can be put into three groups. Having a diagnosis of MSA means you have a combination of symptoms from at least two of the groups:

1.  Parkinson symptoms – relating to slowness and stiffness of movement

  • feeling slow and stiff when moving
  • difficulty starting to move
  • difficulty turning in bed
  • difficulty fastening buttons on a shirt or blouse

2. Cerebellar symptoms – relating to co-ordinating movement and balance

  • feeling clumsy, dropping things
  • inability to balance without support
  • slurred speech
  • feeling unsteady in crowds
  • difficulty writing

3. Autonomic symptoms – relating to automatic body functions

  • bladder problems
  • dizziness or fainting (blood pressure problems)
  • cold hands and feet
  • erectile dysfunction
  • constipation
  • inability to sweat

Other symptoms may include:

  • swallowing problems, difficulty chewing, choking episodes
  • restless sleep
  • noisy breathing during the day, snoring at night
  • weakness in arms and legs
  • cognitive problems, slowness in thinking, difficulty with multi-tasking
  • heightened emotional response (laughing or crying disproportionate to the situation)
  • nightmares
  • unintentional sighing
  • weak, quiet voice

What causes Multiple System Atrophy?

It is unclear why the cells become damaged in MSA. It does not appear to be inherited, although some research suggests there may be a predisposition to MSA due to the individual’s genetic make-up. What triggers the damage is unknown. Environmental toxins or a history of trauma is a focus of ongoing research. MSA is not infectious or contagious.


Who gets MSA?

MSA usually starts between the ages of 50-60 years, although it can affect people younger and older than this. The average age of onset is 54. MSA is a disorder that occurs randomly in the general population and is considered rare (4 – 5 in 100,000). There is a slightly higher incidence in men.


How is someone diagnosed with Multiple System Atrophy?

Due to the variety of symptoms present in MSA, it is often difficult, at first, to differentiate it from Parkinson’s disease. Sometimes, it can take years before a distinction can be made. Generally, MSA has a more rapid decline, no tremor, early autonomic symptoms, and motor symptoms on both sides of the body rather than one side. If someone responds poorly to medications used for treatment of Parkinson’s disease, this may be a clue that MSA is present. Also, if you still have your sense of smell, this can distinguish MSA from Parkinson’s because loss of smell is common in people with Parkinson’s and not as common in MSA.

An assessment by a movement disorder specialist can determine if you have MSA.  Included in this assessment would be a complete medical history, a physical exam and tests, including brain imaging.


What are the first signs of Multiple System Atrophy?

Both men and women often have bladder problems: urgency, frequency, incomplete bladder emptying or an inability to pass urine (retention). These problems may sometimes be incorrectly attributed to aging.
For men, the first sign may be erectile dysfunction (inability to achieve or sustain an erection) which may be incorrectly attributed to prostate disease.

Other problems can be dizziness on standing, fainting, difficulty turning in bed and changes in writing. Some people become clumsy or unsteady when walking, increasing risk for falls.

These early symptoms may be due to a range of other diseases which need to be excluded before a diagnosis of MSA can be made.


What is the progression of Multiple System Atrophy?

MSA is a progressive brain disease and everyone will experience MSA differently. However, the symptoms will change. In MSA, the changes occur more rapidly than with Parkinson’s. You will need more help to care for yourself as symptoms impact your daily life.  Lowered blood pressure can cause fainting and falls. Loss of co-ordination, slowed movements, and rigidity can interfere with activities of daily living.


How is Multiple System Atrophy treated?

Currently, there is no treatment to slow the progression of the disease. The complex nature of MSA means you should see a neurologist who specializes in movement disorders. It is important to look at and treat each symptom separately in order to maintain daily activities and quality of life. Often a multidisciplinary team, including a neurologist, social worker, speech language pathologist, physiotherapist, urologist, clinical nurse and family doctor will be involved.


Managing MSA

Suggestions that may be useful for managing symptoms of MSA:
Movement: Drugs to help stiffness and slowness are the same drugs used in Parkinson’s disease. Medications in MSA may be effective for some people and not effective for others.

Spasms:
 Medications may help treat spasms.

A drop in blood pressure (orthostatic hypotension):
 A drop in blood pressure when standing can cause dizziness, lightheadedness, fainting, or blurred vision. It may be treated with drugs that raise blood pressure. Some people find getting up slowly from a chair can reduce fainting spells. Raising the head of the bed can make it easier to get out of bed. Increasing salt in the diet and wearing pressure stockings can also help. It is important to have regular blood pressure checks.

Constipation:
 Increased dietary fiber and plenty of fluids on a daily basis. Taking laxatives may relieve constipation.

Erectile dysfunction
: Medications or penile implants may help with impotence.

Muscle coordination:
 A routine exercise program with stretching, range of movement and strength building is very important. It helps keep muscles strong and allows for safer mobility.

Swallowing:
 To improve swallowing and reduce risk of choking, food may need to be pureed. If swallowing becomes difficult, a tube may be surgically inserted into the stomach.

Breathing
: If you have problems with snoring or sleep apnea (you stop breathing while asleep), you should report this to your doctor. A breathing tube may need to be inserted.

Urinary retention:
 Medications can reduce urgency and frequency problems, improve tone of bladder muscles or reduce production of urine overnight. You should take care to avoid infection and seek treatment.  Consulting a urologist may help.


What research is being done on Multiple System Atrophy?

Parkinson Canada’s National Research Program includes applications for funding for MSA. Researchers around the world are looking to understand MSA to find better treatments and eventually a cure.


What approach can I take for the future?

Learn as much as you can about MSA. Planning ahead and talking with your care partner and family can help. Adapt your home to accommodate safety issues. Ensure you communicate with family and your health care team about your wishes (legal, financial, and personal care).


The content on this page is provided for information purposes only, and does not represent advice, an endorsement or a recommendation, with respect to any product, service or enterprise, and/or the claims and properties thereof, by Parkinson Canada.


Progressive Supranuclear Palsy

This is a drug resistant parkinsonism. It is characterized by the inability to move the eyes in an up and down movement, neck rigidity (a peculiar posturing of the neck causing the head to be fixed skyward), dysphagia (difficulty swallowing) and dysarthria (difficulty speaking), and a tendency to suddenly lose balance.  Facial expression may take on a surprised look with elevation of the eyebrows. The facial expression is fixed and associated with frowning, and rapid blinking can occur. Early symptoms include problems with balance, fatigue, minor personality changes and subtle visual symptoms such as blurring or double vision. With disease progression, walking becomes slow and deliberate with broad-based steps and progressive loss of balance.

Complaints often associated with the problem moving the eyes up or down include the inability to read, write, eat properly, or dress. Going down stairs can be very difficult. In advanced stages the eyes may not move at all. As the disease progresses, falling becomes more frequent, often without warning. Swallowing often becomes the major problem putting the patient at risk of choking and developing pneumonia.

The following material is from ‘Getting Help for Progressive Supranuclear Palsy: A Guide for Patients and Families’

Progressive Supranuclear Palsy (PSP) is an uncommon neurological disease.  It is caused by damage to nerve cells in specific areas of the brain. It is most often diagnosed when a person is in their 60’s.  Early symptoms include loss of balance, stiffness of the neck, and unexpected falls (often backwards).  As the disease progresses these symptoms worsen and difficulties with eye movements, speech, swallowing, and thinking occur.

People with PSP and their family members face many challenges and these challenges change over time.  This brochure lists potential challenges as well as options that some patients and families have found helpful for improving their quality of life.  While everyone’s experience is different, the challenges are listed in the approximate order in which they may occur.  You may decide that you only want to read the first section now.

The brochure is intended to be a starting point for discussions with your health care team.  When you encounter or anticipate a specific challenge, we encourage you to talk to your health care team about whether one of the options listed would be right for you.  If possible, this team should include a movement disorder specialist.

If/When Options May Include
You or others have questions • Discussions with your health care team
• Discussion with a Parkinson Canada staff member
You feel anxious, isolated, or overwhelmed • Support and counselling from a social worker in the community or clinic
• info@parkinson.ca or 1 (800) 565-3000
• Relaxation techniques, such as meditation, Therapeutic Touch™ or imagery
• Talk with family and friends
• Ask the health team about any research opportunities
Speech is affected • Use yes/no questions, writing, or hand/eye signals
• Referral to a speech language pathologist for therapy and/or devices
• Ensure others are aware of your financial, legal, and end of life care wishes
Falls occur often and/or it becomes more difficult to move • Referral to a physical or occupational therapist who may recommend walkers, wheelchairs, equipment, exercises, home adaptations or other strategies
• Referral to a rehab program
• A personal care provider at home*
• Medications for muscle stiffness and slow movement
It becomes difficult to look down • Avoid bifocals
• Prism glasses to redirect gaze
• Mirror on a swivel base
• Raise plates and books
The neck is stiff • Massage or prescribed stretches
There is coughing or choking with food or fluids • Referral for a swallowing assessment, which may lead to suggestions for changes in head position, food texture, or utensils or use of reminders or thickened fluids
• Monitor for pneumonia
• In later stages, discuss with team the pros and cons of feeding tubes
There is excess saliva or drooling • Chew/suck on gum or candy (if safe to do so)
• Atropine drops under the tongue (by prescription)
• Botox injected into salivary glands
Eye irritation or sensitivity occurs • Artificial tears or lubricating drops
• Omega-3 supplement/foods
• Sunglasses
The eyes become difficult to open • Botox injected around eyes
• Reduction in medications
Constipation occurs • Adequate fluid and fibre
• Time and privacy (if safe) on toilet
• Reduction in medications
• Laxatives
The urge to void is frequent and/or sudden • Urine culture to rule out infection
• Prompt toileting
• Routine toileting
• Medications
• Spill-proof urinal or bedside commode
• Condom catheter
• Incontinence briefs
Sustained muscle contractions occur (dystonia) • Start or adjust medications
• Botox injections to muscles
• Relaxation techniques*
• Referral to a pain specialist
• Equipment from a therapist
Sleep is disturbed • A short nap after lunch
• Limit stimulating fluids, foods, and medications
• Toileting strategies (see previous page)
• Relaxation techniques*
• Sedatives
Apathy, depression, irritability, or mood swings occur • Counselling to support the caregiver, identify triggers, and find ways not to take the behaviors personally
• Medications
• Exercise for mental, physical and social wellbeing
Travel becomes difficult • Discuss driving safety
• Review transfer equipment or techniques with therapist
• Handicapped parking permit
• Accessible transit services
Slowness of thinking occurs • Allow time for the person to respond
• Present one idea at a time
• If the change is sudden, search for other causes
Financial assistance is needed • Discussion eligibility for various disability benefits with social worker
• Contact local charities for possible assistance
It becomes more difficult to care for the person at home • Discuss options with social worker. These may include: in-home or external respite programs, day programs, retirement homes or nursing homes
The end of life is approaching • Discuss with the health team your care wishes, eligibility for hospice or palliative care, and availability of grief counselling

Developed by Theresa Moore RN MScN, Centre for Movement Disorders, 2780 Bur Oak Avenue, Markham, ON, Canada
With financial support from the Parkinson Canada PSP fund, as well as assistance from Cure PSP staff and patients and families affected by PSP
March, 2012

The content on this page is provided for information purposes only, and does not represent advice, an endorsement or a recommendation, with respect to any product, service or enterprise, and/or the claims and properties thereof, by Parkinson Canada.


Cortico-basal-ganglionic Degeneration

This is a rare drug resistant parkinsonism. It is characterized by the marked asymmetry of the parkinsonian features until late in the disease.  Individuals will frequently have apraxia (inability to properly use a limb for complex tasks despite normal power and only mild incoordination); jerky abnormal movements superimposed on normal movements;  “alien limb phenomenon” (one limb seems to have a mind of its own, sometimes actively interfering with planned movements), and involuntary jerking in response to light touch.

Early on, the condition may be misdiagnosed as Parkinson’s disease. Marked, often painful, rigidity may occur late in this disorder. Tremor is not as common as in Parkinson’s disease.

The following material was from WeMove.org  (website no longer active as same organization)Worldwide Education and Awareness for Movement Disorders

Corticobasal degeneration (CBD) is a rare neurological disease in which parts of the brain deteriorate or degenerate. CBD is also known as corticobasal ganglionic degeneration, or CBGD.

Several regions of the brain degenerate in CBD. The cortex, or outer layer of the brain, is severely affected, especially the fronto-parietal regions, located near the center-top of the head. Other, deeper brain regions are also affected, including parts of the basal ganglia, hence the name “corticobasal” degeneration. The combined loss of brain tissue in all these areas causes the symptoms and findings seen in people with CBD.

What causes the degeneration of brain tissue in CBD?
Unfortunately, the cause of CBD is entirely unknown. There is currently no strong evidence to suggest CBD is an inherited disease, and no other risk factors, such as toxins or infections, have been identified.

Studies of brain tissue of individuals with CBD show certain characteristic cell changes. Similar, although not identical, changes are observed in two other neurodegenerative diseases, Pick’s disease and progressive supranuclear palsy. These changes, involving a brain protein called tau, have provided researchers some initial clues in their search for the causes of CBD.

What are the symptoms of CBD?
Symptoms of CBD usually begin after age 60. The initial symptoms of CBD are often stiffness, shakiness, jerkiness, slowness, and clumsiness, in either the upper or lower extremities. Other initial symptoms may include dysphasia (difficulty with speech generation), dysarthria (difficulty with articulation), difficulty controlling the muscles of the face and mouth, or walking and balance difficulties. Symptoms usually begin on one side of the body, and spread gradually to the other. Some patients (probably more than commonly recognized in the past) may have memory or behavioral problems as the earliest or presenting symptoms.

CBD is a progressive disease, meaning the symptoms worsen over time. Over the course of one to several years, most people with CBD gradually worsen, with symptoms progressing to involve upper and lower extremities and other body regions. Symptoms of advanced CBD include:

– parkinsonism (rigidity, slow movements, postural instability)
– tremor
– myoclonus (sudden, brief jerky movements)
– dystonia, including blepharospasm
– speech difficulty
– mild-to-moderate cognitive impairment (memory loss, difficulty planning or executing unrehearsed movements, dementia)
– sensory loss
– “alien hand/limb” phenomenon (difficulty controlling the movements of a limb, which seems to undertake movements on its own, sometimes combined with a feeling that the limb is not one’s own)

How is CBD diagnosed?
Early in the disease course, it is often difficult to distinguish CBD from similar neurodegenerative diseases. Diagnosis of CBD involves a careful neurological exam, combined with one or more types of laboratory evaluations. Electrophysiological studies, including an EEG (electroencephalogram), may show changes in brain function over time that are consistent with the neurodegeneration. CT or MRI scans can also be used in this way, providing images of asymmetric atrophy of the fronto-parietal regions of the brain’s cortex, the regions most frequently involved in the disease.

How is CBD treated?
Unfortunately, there are no drugs or other therapies that can slow the progress of the disease, and very few that offer symptomatic relief. Tremor and myoclonus may be controlled somewhat with drugs such as clonazepam. Baclofen may help reduce rigidity somewhat. Levodopa and other dopaminergic drugs used in Parkinson’s disease are rarely beneficial, but may help some CBD patients.

Physical therapy exercises may be useful to maintain range of motion of stiff joints. This may prevent pain and contracture (muscle shortening), and help maintain mobility. Occupational therapy may be used to design adaptive equipment that supports the activities of daily living, thus helping to maintain more functional independence. Speech therapy is used to improve articulation and volume.

What is the usual course of CBD?
A person with CBD will usually become immobile due to rigidity within five years of symptom onset, and may require a gastrostomy tube for feeding at some point before that. Most often, within ten years of onset, pneumonia or other bacterial infections may lead to life-threatening complications.

The content on this page is provided for information purposes only, and does not represent advice, an endorsement or a recommendation, with respect to any product, service or enterprise, and/or the claims and properties thereof, by Parkinson Canada.


Diffuse Lewy Body Disease

Parkinsonism preceding dementia by a period of 1 year or more is termed Parkinson Disease Dementia (PD-D), and dementia that precedes or accompanies the onset of parkinsonism is labeled Dementia with Lewy Bodies (DLB). Individuals with DLB have an older age at onset and shorter disease duration than those with typical PD. Resting tremor is less common (55% verses 85%).


The following material is from Parkinson Society British Columbia.

Diffuse Lewy Body Disease (DLBD) is a degenerative disorder of the brain that leads to loss of memory and difficulty concentrating (dementia). After Alzheimer’s disease, it is the second most common cause of degenerative dementia. In the early stages of the disease there is often simply a problem with attention, e.g. sustaining a line of thought. Memory loss does not necessarily occur in the early stages, but usually appears as the disease progresses. Patients with DLBD are often anxious and may be depressed. These may even be the first symptoms of the disease.

Other features of Diffuse Lewy Body Disease are:

  • Fluctuations in thinking. Especially early in the disease, patients may be very confused at some times and be able to think clearly at others.
  • Hallucinations. These are most often well formed and detailed visual hallucinations; the patient will see animals or people that are not really there.
  • Symptoms of Parkinson’s Disease, e.g. tremor, stiffness, slowness of movement. DLBD may first present itself with these symptoms. It is therefore not unusual that the initial diagnosis is Parkinson’s Disease and later, when dementia develops, is changed to DLBD.

Most people have never heard of Diffuse Lewy Body Disease. It was only in the 1980s that DLBD was first described. Pathologists looking at post mortem brains of some people who had dementia before they died noticed that some of the brains didn’t have the classical features of Alzheimer’s disease (plaques and tangles) that they expected to see. These patients were then found to have had different features of dementia according to their clinic records, so researchers realized that these patients had something different, while they were alive. What the pathologists found were Lewy bodies. This new disease was called ” Diffuse Lewy Body Disease”.


What is a “Lewy body”?

Lewy bodies are named after Dr. Friederich Heinrich Lewy who first described these structures that he discovered in brains of patients who had died with Parkinson’s disease. In Parkinson’s disease patients, these structures are found in brain cells in the brain stem (at the base of the brain), which are involved in the control of movement. In DLBD, Lewy bodies are also found in the outer layer of the brain called the cortex, which is responsible for mental function. These “cortical Lewy bodies” are the essential pathological feature of Diffuse Lewy Body Disease.


What causes Diffuse Lewy Body Disease?

We don’t know the cause of DLBD. Genetic factors may play a role in some cases, but there is no clear pattern of inheritance. There is evidence that suggests that DLBD is caused by the same mechanism that causes Parkinson’s disease. Unfortunately, we still don’t know the cause of most cases of Parkinson’s disease nor do we know why some people develop Parkinson’s disease and others Diffuse Lewy Body Disease.


How is the diagnosis of Diffuse Lewy Body Disease made?

The only way to be certain about the diagnosis is to examine the brain after death. In life the diagnosis of DLBD is made based on a patient’s symptoms. There are no tests such as a blood test or brain scan that can provide a certain diagnosis of DLBD. A doctor will often order some blood tests, or a brain scan, to exclude other illnesses that may cause similar symptoms. The following symptoms are used to make a clinical diagnosis:


Dementia must develop during the course of the disease -specific difficulties with inattention, visuospatial skills and lack of initiative.

Two of the following must be present:

  • Fluctuations in memory
  • Visual hallucinations
  • Parkinsonism

Features that further support the diagnosis are:

  • Difficulty with balance -may lead to repeated falls
  • Transient loss of consciousness
  • Inability to tolerate neuroleptic medications

Many different names are given for different subtypes of Diffuse Lewy Body Disease. They include: dementia with Lewy bodies, Lewy body variant of AD, and Lewy body disease. These subtypes are all variants of the same disease. Physicians may use these names interchangeably. The precise subtype does not matter in clinical practice, as treatment is the same.


How is Diffuse Lewy Body Disease different from AD?

Although dementia is present in both AD and DLBD, the features of dementia differ between the two. It’s unusual to have visual hallucinations in Alzheimer’s disease and fluctuations in intellectual functioning are more prominent with Diffuse Lewy Body Disease. Visuospatial skills and attention are more affected in DLBD, whereas language function is more affected in AD. Patients have problems with memory in both DLBD and Alzheimer’s disease, but they are different. In AD, the patients have difficulty consolidating memory (forming new memories) whereas in DLBD patients have difficulty retrieving memory (accessing previously memorized information). These differences stem from pathological differences between the two disorders. In AD there is wide spread cell loss in the cortex producing degeneration throughout the brain. If you look at a MRI or CT scan of a patient with Alzheimer’s disease the brain appears to have shrunk in size compared to a normal brain. In contrast a MRI scan of a patient with DLBD looks normal. This is because a MRI shows structural abnormalities and in Diffuse Lewy Body Disease the problem is chemical. It is possible to show the difference between brains with DLBD and normal brains using a PET scanner. The remarkable feature of PET scanning is its ability to show levels of specific chemicals in the brain.


How is Diffuse Lewy Body Disease different from Parkinson’s disease?

In Parkinson’s disease, dementia occurs in about 30% of patients and usually late in the disease. Memory problems in Parkinson’s disease are often quite mild. Since some patients with Parkinson’s disease have dementia and some patients with DLBD have Parkinsonism, there is considerable overlap between the symptoms of the two diseases. Do all Parkinson’s disease patients who have dementia have DLBD? No (not according to our current criteria). A patient with Parkinson’s disease can for instance also develop AD, in which the pattern of dementia is different as described earlier.


Is there a Cure for Diffuse Lewy Body Disease?

Unfortunately, there is no cure for DLBD. There are, however, some drugs that can help some symptoms of the disease. Patients with parkinsonian symptoms may be helped by some of the drugs used to treat Parkinson’s disease. There are also new medications that seem particularly helpful for the thinking problems of diffuse lewy body disease.


Source: Pacific Parkinson’s Research Centre, University of British Columbia, Vancouver, B.C.

Credits: Parkinson Society British Columbia


Additional Resources:


The content on this page is provided for information purposes only, and does not represent advice, an endorsement or a recommendation, with respect to any product, service or enterprise, and/or the claims and properties thereof, by Parkinson Canada.