Summary about Disease
Parkinson-Plus Syndromes (PPS) are a group of neurodegenerative disorders that share some of the motor symptoms of Parkinson's disease (PD) – such as slowness of movement, rigidity, and balance problems – but also have additional features that distinguish them from PD and often indicate a more rapid progression. These syndromes are typically less responsive to levodopa, the primary medication used to treat PD. Common Parkinson-Plus Syndromes include Multiple System Atrophy (MSA), Progressive Supranuclear Palsy (PSP), Corticobasal Degeneration (CBD), and Dementia with Lewy Bodies (DLB).
Symptoms
Symptoms vary depending on the specific Parkinson-Plus Syndrome but may include:
Motor Symptoms: Slowness of movement (bradykinesia), rigidity, balance problems, tremors (though often less prominent than in PD).
Autonomic Dysfunction (MSA): Problems with blood pressure regulation (orthostatic hypotension), bladder control, bowel control, and sexual function.
Eye Movement Problems (PSP): Difficulty with vertical eye movements, especially looking down.
Cognitive Impairment (DLB, CBD): Problems with memory, attention, executive function, visual-spatial skills. Hallucinations are common in DLB.
Speech and Swallowing Difficulties: Dysarthria (slurred speech) and dysphagia (difficulty swallowing).
Muscle Jerks and Dystonia (CBD): Involuntary muscle contractions, jerks, and abnormal postures.
Behavioral Changes: Apathy, depression, anxiety, impulsivity.
Sleep Disturbances: REM sleep behavior disorder.
Causes
The causes of Parkinson-Plus Syndromes are largely unknown. They are generally considered to be sporadic disorders, meaning they occur randomly and are not directly inherited. However, some genetic factors may increase susceptibility in certain cases. The underlying pathology involves the accumulation of abnormal proteins (e.g., alpha-synuclein in MSA, tau in PSP and CBD) in the brain, leading to the degeneration of specific brain regions.
Medicine Used
There is no cure for Parkinson-Plus Syndromes, and treatment focuses on managing symptoms. Medications may include:
Levodopa: Can provide some benefit for motor symptoms, but often less effective than in Parkinson's disease.
Amantadine: May help with dyskinesias (involuntary movements).
Medications for Autonomic Dysfunction (MSA): Fludrocortisone, midodrine, or droxidopa for orthostatic hypotension; medications for urinary problems.
Antidepressants: To treat depression and anxiety.
Cholinesterase inhibitors or memantine (DLB): for cognitive symtoms.
Botulinum toxin (Botox): For dystonia or sialorrhea (excessive drooling).
Other Medications: To address specific symptoms such as sleep disturbances or behavioral problems.
Is Communicable
Parkinson-Plus Syndromes are not communicable. They are not caused by infections and cannot be spread from person to person.
Precautions
There are no specific precautions to prevent Parkinson-Plus Syndromes, as the causes are largely unknown. General healthy lifestyle choices, such as maintaining a balanced diet, exercising regularly, and avoiding exposure to toxins, may be beneficial for overall brain health. Occupational and physical therapy can help to maximize mobility and independence, and speech therapy can address communication and swallowing difficulties. Fall prevention strategies in the home are critical to reduce risk of injury.
How long does an outbreak last?
Parkinson-Plus Syndromes are not outbreaks. These are chronic, progressive conditions, that worsen with time.
How is it diagnosed?
Diagnosis of Parkinson-Plus Syndromes can be challenging, as early symptoms may overlap with Parkinson's disease. Diagnosis typically involves:
Neurological Examination: Assessment of motor skills, reflexes, coordination, and cognitive function.
Medical History: Detailed review of symptoms and family history.
Brain Imaging: MRI scans to look for structural changes in the brain that are characteristic of specific syndromes. Sometimes a DaTscan is used initially to rule out Parkinson's Disease.
Autonomic Testing: To assess blood pressure regulation and other autonomic functions.
Neuropsychological Testing: To evaluate cognitive function.
Response to Levodopa: Assessing the degree of improvement with levodopa treatment (less improvement suggests a PPS).
Observation Over Time: Monitoring the progression of symptoms to see if additional features emerge that are characteristic of a specific PPS.
In some cases, a brain biopsy or autopsy is required for definitive diagnosis, but this is not typically done during life.
Timeline of Symptoms
The timeline of symptom progression varies depending on the specific Parkinson-Plus Syndrome. In general, PPS tend to progress more rapidly than Parkinson's disease.
Early Stages: Motor symptoms similar to PD may be present, but other features (e.g., balance problems, autonomic dysfunction, cognitive changes) often emerge earlier.
Middle Stages: Motor symptoms worsen, and non-motor symptoms become more prominent. Difficulties with speech, swallowing, and mobility increase.
Late Stages: Significant disability, requiring substantial assistance with daily activities. Increased risk of falls, aspiration pneumonia, and other complications. Cognitive impairment may become severe.
Important Considerations
Early and accurate diagnosis is important for appropriate management and planning.
A multidisciplinary approach involving neurologists, physical therapists, occupational therapists, speech therapists, and other specialists is crucial.
Support groups and counseling can provide emotional support for patients and their families.
Advance care planning (e.g., creating a living will or durable power of attorney) is important to ensure that the patient's wishes are respected as the disease progresses.
Research is ongoing to better understand the causes, diagnosis, and treatment of Parkinson-Plus Syndromes.