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What are Parkinson-Plus Syndromes?

Parkinson-Plus Syndromes

Parkinson-Plus syndromes, also known as disorders of multiple system degeneration, is a group of neurodegenerative diseases featuring the classical motor features of Parkinson's disease (tremor, rigidity, akinesia/bradykinesia, and postural instability) with additional features that distinguish them from simple idiopathic Parkinson's disease (PD).

The atypical parkinsonian or Parkinson-Plus syndromes are often difficult to differentiate from PD and each other. They include multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD).

Additional Parkinson-Plus syndromes include Pick's disease and olivopontocerebellar atrophy.

The Centre will focus on these Parkinson-Plus syndromes:

  • Progressive Supranuclear Palsy (PSP)
  • Corticobasal Syndrome (CBS)
  • Multiple System Atrophy (MSA) 
  • Related disorders, which share common mechanisms and present new opportunities to develop treatments that may benefit patients with Parkinson-Plus.

 

Progressive Supranuclear Palsy (PSP)

Progressive Supranuclear Palsy (PSP) is a progressive disease that causes problems with balance, movement, vision, speech, and swallowing. In its early stages, symptoms can resemble those of other neurological conditions such as Parkinson’s disease, Multiple System Atrophy, or depression, and misdiagnosis is common. Some of the main symptoms of PSP include:

    • problems with balance and mobility; frequent falls
    • changes in behaviour (e.g., apathy, impulsivity)
    • muscle stiffness 
    • difficulty to look up and down, or blink
    • slow, quiet or slurred speech
    • slowness of thought, and irritability

 

Corticobasal degeneration (CBD) and corticobasal syndrome (CBS)

Corticobasal degeneration (CBD) and corticobasal syndrome (CBS) are caused by degeneration in the cerebral cortex (that affects thinking, sensation, and coordination) and the basal ganglia (causing slow, stiff or jerky movements).

Corticobasal Degeneration (CBD) is a degenerative brain disease affecting people from the age of 40 onwards. There are neurological similarities to PSP, but the classical clinical picture is often distinct. Patients diagnosed with CBD may also develop features of PSP and vice versa.

Cognitive problems are common in CBD and are often one of the first symptoms, such as apathy, impulsive behaviour, changes in empathy, and language symptoms. Other signs are progressive numbness and loss of use of one hand. There may also be jerking of the fingers, slowness and awkwardness and the feeling of having an ‘alien limb’ – with complex unintentional movements of one limb causing problems with normal motor tasks. Gradually the arm and/or leg on one side is affected and then the arm and/or leg on the other.

 

 

Multiple Systems Atrophy (MSA) 

Multiple System Atrophy (MSA) also causes slow movement and stiffness, with additional problems with coordination, bladder, and blood pressure control. It is characterized by tremors, slow movement, muscle rigidity, and postural instability (collectively known as parkinsonism), dysfunction of the autonomic nervous system (which regulates bodily functions such as blood pressure, the bladder and bowels), and unsteadiness of walking (ataxia). It can also cause problems with speech and breathing. MSA is caused by progressive degeneration of neurons in several parts of the brain including the substantia nigra, striatum, inferior olivary nucleus, and cerebellum.