Atypical parkinsonism aka Parkinson’s Plus conditions
Atypical Parkinson’s or atypical parkinsonisms are a group of rare neurological disorders that share some symptoms with Parkinson’s disease but have distinct features and typically a more rapid progression. They also often do not respond well to standard Parkinson’s medications. These disorders include Multiple System Atrophy (MSA), Progressive Supranuclear Palsy (PSP), Corticobasal Degeneration (CBD), and Dementia with Lewy Bodies (DLB). There is also Drug-induced Parkinsonism, which unlike PD is a reversible condition caused by medications or drugs that interfere with dopamine transmission in the brain.
Each condition has its own set of symptoms, which may include balance problems, stiffness, difficulty with movement, and cognitive changes. Diagnosing atypical parkinsonisms can be challenging and may require specialised testing and evaluation by a neurologist or movement disorder specialist familiar with these conditions. Treatment focuses on managing symptoms and improving quality of life, often involving a multidisciplinary approach tailored to individual needs.
Corticobasal degeneration (CBD), also known as corticobasal syndrome, is a rare neurological disorder that causes progressive damage to specific areas of the brain, including the cerebral cortex and the basal ganglia. These regions are responsible for various functions such as movement, memory, and cognition. The condition typically leads to symptoms that may initially affect one side of the body but eventually progress to both sides.
Symptoms of CBD can include:
- Difficulty moving, which worsens over time
- Poor coordination and balance issues
- Stiffness and muscle jerks
- Abnormal postures of the hands or feet
- Difficulty swallowing
- Abnormal eye movements
- Trouble with thinking and language skills
- Speech problems, such as slow and halting speech
The disease progresses over six to eight years, and as it advances, individuals with CBD may lose the ability to walk. The underlying cause of CBD is often associated with the buildup of the protein tau in brain cells, leading to their deterioration. This is similar to other neurodegenerative diseases like Alzheimer’s disease and progressive supranuclear palsy (PSP).
There is currently no cure for CBD, and treatment focuses on managing symptoms. This may include medications, physical therapy, speech pathology, and dietary changes to help improve quality of life. If you or someone you know is experiencing symptoms that could be related to CBD, it is important to consult a healthcare provider for a proper diagnosis and management plan.
Dementia with Lewy bodies (DLB) is a type of progressive dementia that leads to a decline in cognitive abilities, affecting memory, thinking, and behaviour. It is characterised by the presence of abnormal protein deposits called Lewy bodies in the brain’s cortical neurons. These deposits disrupt normal brain function, leading to symptoms such as visual hallucinations, cognitive fluctuations, and problems with movement similar to those seen in Parkinson’s disease.
Treatment for DLB focuses on managing symptoms, as there is currently no cure:
- Medications like cholinesterase inhibitors (e.g., rivastigmine) can help improve cognitive function and reduce hallucinations.
- For motor symptoms, carbidopa-levodopa may be prescribed, although its effectiveness can vary.
- Antipsychotic medications are sometimes used cautiously to manage behavioral symptoms, but they come with risks and should be used with caution.
- Non-drug treatments, such as speech pathology, physical and occupational therapy, can also support daily functioning and quality of life.
Support from caregivers and a multidisciplinary approach involving neurologists, psychiatrists, and other healthcare professionals are crucial in managing DLB effectively.
Drug-induced parkinsonism is a condition where certain medications cause symptoms similar to Parkinson’s disease. Unlike Parkinson’s disease, which is a degenerative disorder, drug-induced parkinsonism is a reversible condition caused by medications that interfere with dopamine transmission in the brain.
What Causes It?
The primary cause of drug-induced parkinsonism is the use of medications that block dopamine receptors. These medications are often used to treat conditions such as schizophrenia, bipolar disorder, and nausea. Common culprits include antipsychotics (e.g., haloperidol, risperidone) and anti-nausea medications (e.g., promethazine). Symptoms appear rapidly after starting the medication, unlike the gradual onset in Parkinson’s disease. It is more common in older adults due to decreased dopamine receptor sensitivity.
Symptoms
The symptoms of drug-induced parkinsonism are similar to those of Parkinson’s disease and include:
- Tremors: Shaking or trembling, especially when at rest.
- Bradykinesia: Slowed movements and difficulty performing fine motor tasks.
- Muscle Stiffness: Tightness in the limbs or torso.
- Balance Problems: Difficulty walking and maintaining balance.
- Impaired Speech: Difficulty speaking clearly.
Treatment
The primary treatment for drug-induced parkinsonism is to stop or reduce the offending medication, if possible. In some cases, switching to a different medication with fewer side effects may be necessary. For patients who cannot stop the medication, managing symptoms with other treatments, such as physical therapy and medications to alleviate symptoms, may be considered. Unlike Parkinson’s disease, drug-induced parkinsonism can improve or resolve once the medication is stopped.
Lewy body dementia (LBD) is an umbrella term for two related conditions: dementia with Lewy bodies (DLB) and Parkinson’s disease dementia (PDD). Dementia with Lewy bodies is characterised by chronic cognitive deterioration due to the presence of Lewy bodies in the cortical neurons. This leads to symptoms such as memory loss, visual hallucinations, and cognitive fluctuations.
On the other hand, Parkinson’s disease dementia occurs in the later stages of Parkinson’s disease, where cognitive decline is marked by Lewy bodies in the substantia nigra, a region of the brain that affects movement. Here, movement issues typically appear before dementia symptoms.
Multiple system atrophy (MSA) is a rare, progressive neurodegenerative disorder that affects the body’s involuntary functions, such as blood pressure, heart rate, bladder control, and motor control or striatonigral degeneration. MSA shares many symptoms with Parkinson’s disease, including slow movement, rigid muscles, and poor balance. The symptoms of MSA typically begin in adulthood, usually in the 50s or 60s, and can include:
- Stiff muscles and difficulty moving
- Poor coordination and balance issues
- Autonomic function failure, leading to problems like postural hypotension (dizziness or fainting when standing up)
- Urinary incontinence and bowel dysfunction
- Changes in sweat production and body temperature control
- Sleep disorders, such as acting out dreams or abnormal breathing at night
- Visual disturbances
- Difficulty swallowing or chewing
- Sexual dysfunction
- Psychiatric problems, such as difficulty controlling emotions
MSA is categorised into two types based on the most prominent symptoms at diagnosis:
- Parkinsonian type (MSA-P): Similar to Parkinson’s with symptoms like tremors, slow movement, and stiffness.
- Cerebellar type (MSA-C): Mainly affects muscle coordination, leading to gait disturbances and loss of balance.
The exact cause of MSA is not fully understood, but it involves the accumulation of the protein alpha-synuclein in the brain cells, which is also seen in other neurodegenerative diseases. There is no cure for MSA, and treatment focuses on managing symptoms to improve quality of life. You may take medications, go to physical therapy, and make healthy lifestyle changes. If you or someone you know is experiencing symptoms that could be related to MSA, it is important to seek medical attention for a proper diagnosis and management plan.
Parkinson’s Disease Dementia (PDD) is a form of dementia that affects some people who have been living with Parkinson’s disease for at least a year. It is characterised by cognitive decline, including memory difficulties, problems with attention, and behavioural changes such as apathy, delusions, and hallucinations. The cognitive deterioration in PDD is linked to the presence of Lewy bodies in the substantia nigra, a part of the brain that controls movement.
Treatment for PDD focuses on managing symptoms, as there is currently no cure:
- Medications like cholinesterase inhibitors (e.g., rivastigmine) can help improve cognitive function, while carbidopa-levodopa may be prescribed for motor symptoms.
- Antipsychotic medications are sometimes used cautiously to manage behavioural symptoms, but they come with risks and should be used with caution.
- Non-drug treatments, such as physical, occupational, and speech therapy, can also support daily functioning and quality of life.
Support from caregivers and a multidisciplinary approach involving neurologists, psychiatrists, and other healthcare professionals are crucial in managing PDD effectively.
Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is an uncommon brain disorder that causes serious problems with walking, balance, eye movements, and later with swallowing. It is a neurodegenerative condition that leads to the deterioration of brain cells, particularly in the midbrain and frontal lobe, which control movements and thinking.
The symptoms of PSP can include:
- loss of balance while walking
- inability to aim the eyes properly, often resulting in difficulty looking downward
- sensitivity to light
- slurred speech
- difficulty swallowing
- changes in behaviour
- memory problems
- sleep disturbances
- depression.
The exact cause of PSP is unknown, but it involves the accumulation of abnormal levels of the protein tau in the brain cells. This is similar to other neurodegenerative diseases like Parkinson’s and Alzheimer’s disease. There is no cure for PSP, but treatment options aim to manage symptoms and may include medications, physical therapy, speech and language therapy, and dietary changes.
If you or someone you know is experiencing symptoms that could be related to PSP, it is important to consult a healthcare provider for a proper diagnosis and management plan.
Other movement disorders
Dystonia is characterised by involuntary muscle contractions that cause repetitive or twisting movements. The condition can affect one part of the body (focal dystonia), two or more adjacent parts (segmental dystonia), or all parts of the body (general dystonia). The muscle spasms can range from mild to severe and may be painful, interfering with daily tasks.
Symptoms of dystonia can vary but often include:
- Abnormal postures and movements
- Muscle spasms that are sustained or intermittent
- Pain and discomfort
- Difficulty with voluntary movements
- Impact on tasks such as writing (writer’s dystonia) or playing musical instruments (musician’s dystonia)
The exact cause of dystonia isn’t fully understood, but it may involve changes in the brain’s communication pathways. Some forms of dystonia are genetic, while others may result from conditions like Parkinson’s disease, stroke, or traumatic brain injury. Treatments for dystonia aim to improve symptoms and may include medications, physical therapy, and in some cases, surgery.
If you or someone you know is experiencing symptoms that could be related to dystonia, it’s important to consult a healthcare provider for a proper diagnosis and management plan. Early intervention can help manage the symptoms and improve quality of life.
It’s important to note that both ET (Essential Tremor) and Parkinson’s are movement disorders and sometimes they can be mistaken for each other. However, there are many differences between the two conditions, and proper and early diagnosis is important for receiving the right treatment and support.
Prevalence:
ET is approximately 8 times more common than Parkinson’s.
Age of Onset:
Parkinson’s is most commonly diagnosed in people over the age of 60, while ET can occur at any age, even in childhood.
Tremor Characteristics:
In Parkinson’s, the tremor is mostly seen at rest and may be referred to as “resting tremor”. In ET, the tremor occurs mostly during action or movement, though this occurs in Parkinson’s as well.
Body Parts Affected:
The tremor in Parkinson’s usually starts on one side of the body and may develop on the other side as the disease progresses. In ET, the tremor usually affects both sides from the start of the condition.
Other Symptoms:
In ET, tremor is the primary symptom. In Parkinson’s, there are four primary symptoms that affect movement, including tremor, stiffness/rigidity, bradykinesia (slowed movements), and balance issues. Parkinson’s is also accompanied by many non-motor symptoms.
Genetics and Family History:
In cases of Parkinson’s, there is rarely a family history (estimated 10-20% of cases), but in ET, a family history of tremor is seen in more than 50% of cases.
Rapid Eye Movement (REM) Sleep Behaviour Disorder (RBD) is often associated with Parkinson’s. Here’s how they are related:
RBD and Parkinson’s:
RBD is characterised by acting out dreams or moving a lot in the REM stage of sleep. About 80% of people diagnosed with RBD develop synuclein-related disorders such as Parkinson’s or other atypical parkinsonism at some point in their lives.
Prevalence:
In the general population, the incidence of RBD is around 0.5%, compared to the prevalence of RBD in Parkinson’s patients, which has been reported to be around 50%.
Symptom Onset:
The diagnosis and symptom onset of RBD typically precedes the onset of motor or cognitive symptoms of Parkinson’s by years to decades.
Research:
There is a lot of research into RBD, and the next stage is figuring out how to treat it.
Please note that having RBD does not necessarily mean the person will have Parkinson’s later in life. It is the combination of RBD plus other early symptoms that can lead to a prodromal diagnosis.
Restless leg syndrome (RLS), also known as Willis-Ekbom disease, is a neurological condition that causes an uncontrollable urge to move the legs, typically due to uncomfortable sensations. It often occurs in the evening or nighttime hours when you’re sitting or lying down, and moving your legs can temporarily ease the unpleasant feeling.
Symptoms of RLS may include:
- Sensations that begin while resting, often after being seated in a car, plane, or movie theatre for an extended time.
- Relief that comes with movement, such as stretching, jiggling the legs, pacing, or walking.
- Symptoms get worse in the evening.
- Nighttime leg twitching, which may be associated with periodic limb movement of sleep, causing the legs to twitch and kick throughout the night while you sleep.
People with RLS typically describe the sensations as crawling, creeping, pulling, throbbing, aching, itching, or electric. Sometimes the sensations are difficult to explain, but they consistently describe the desire to move the legs. The exact cause of RLS is often unknown, but it may be related to an imbalance of the brain chemical dopamine, which sends messages to control muscle movement. Heredity can play a role, especially if the condition starts before age 40. Treatment for RLS may involve lifestyle changes, self-care steps, and medications to help relieve symptoms and improve sleep quality. If you think you may have RLS, it’s important to talk with your healthcare provider to confirm the diagnosis and discuss potential treatment options.
Vascular Parkinsonism is a movement disorder caused by one or more small strokes in the brain regions that control movement. These strokes result in damage to the brain’s blood supply, leading to symptoms similar to Parkinson’s disease but with some key differences.
Symptoms
The main symptoms of vascular parkinsonism include:
- Slow movements and walking difficulties: People often have a shuffling gait and trouble with balance.
- Muscle stiffness and rigidity: This is particularly noticeable in the lower body.
- Limb weakness: Weakness in the arms and legs can occur.
- Postural instability: Difficulty maintaining balance while standing or walking.
- Speech and swallowing difficulties: Some people may experience pseudobulbar palsy, which affects speech and swallowing.
- Cognitive impairments: Memory loss and difficulty thinking clearly can also be present.
Treatment
Treatment for vascular parkinsonism focuses on managing symptoms and preventing further strokes. This includes:
- Physical and occupational therapy: These therapies help improve muscle function and daily activities.
- Medications: While standard Parkinson’s medications may not be as effective, other medications can help manage symptoms.
- Lifestyle changes: Controlling risk factors such as high blood pressure, cholesterol, and diabetes is crucial.
- Regular exercise: Staying active can help maintain muscle strength and improve balance.
Managing vascular parkinsonism requires a comprehensive approach involving healthcare professionals, caregivers, and the individual’s active participation in their treatment plan.
Parkinson’s Australia National Conference
6-8 April 2025, National Convention Centre Canberra, ACT
