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Multiple system atrophy (MSA) is a rare neurodegenerative disorder [1] characterized by tremors, slow movement, muscle rigidity, postural instability (collectively known as parkinsonism), autonomic dysfunction and ataxia.
They include multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). Dementia with Lewy bodies (DLB), may or may not be part of the PD spectrum, but it is increasingly recognized as the second-most common type of neurodegenerative dementia after Alzheimer's disease.
Neurodegenerative diseases include amyotrophic lateral sclerosis, multiple sclerosis, Parkinson's disease, Alzheimer's disease, Huntington's disease, multiple system atrophy, tauopathies, and prion diseases. Neurodegeneration can be found in the brain at many different levels of neuronal circuitry, ranging from molecular to systemic. [4]
In multiple system atrophy, autonomic dysfunction appears earlier and is more severe, [39] and is accompanied by uncoordinated movements, while visual hallucinations and fluctuating cognition are less common than in DLB. [153] Urinary difficulty is one of the earliest symptoms with multiple system atrophy, and is often severe. [70]
[10] [12] [13] Other symptoms of the specific synucleinopathy usually manifest within 15 years of the diagnosis of RBD, [14] but may emerge up to 50 years after RBD diagnosis. [ 10 ] Alpha-synuclein deposits can affect the cardiac muscle and blood vessels. [ 15 ]
Such "primary" dysautonomias are distinguished from secondary dysautonomias, where the dysfunction of the autonomic nervous system is believed to be caused by another disease (e.g. diabetes). [2] Diseases categorized as primary autonomic failure usually include pure autonomic failure and multiple system atrophy.
Multiple sclerosis, meningitis-retention syndrome [13] Paraneoplastic syndrome [25] Spinal cord injury [13] or traumatic brain injury [26] Synucleinopathy, a group of neurodegenerative diseases including pure autonomic failure, Parkinson's disease, dementia with Lewy bodies and multiple system atrophy [6] Surgery or injury involving the nerves [13]
Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. [1] CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years.