Continued from article: (Posted on August 13, 2018 by MyParkinsonsTeam)
Some types of parkinsonian movement disorders have similar motor symptoms as Parkinson’s disease and are also caused by progressive damage to the brain, but do not improve when treated with medications that are effective in Parkinson’s disease. These conditions are known as atypical parkinsonisms or “Parkinson’s plus.” Atypical parkinsonisms may show slight differences in motor symptoms from Parkinson’s disease. For instance, motor symptoms may start on both sides of the body instead of one side, or problems with thinking, memory, and mood may occur first, before motor symptoms.
Multiple system atrophy (MSA)
MSA is a rare condition with about 1,900 new cases diagnosed each year in the U.S. MSA is also known as Shy-Drager syndrome (SDS). MSA seems to affect men and women at equal rates. MSA has motor features in common with other types of Parkinson’s but is more likely to present with symptoms related to the autonomic nervous system. The autonomic nervous system regulates blood pressure, digestion, and temperature, and people with MSA are more likely than those with Parkinson’s disease to experience bladder or bowel problems, excess sweating, and orthostatic hypotension (fainting or dizziness after standing).
In MSA, an abnormal protein called alpha synuclein builds up in regions of the brain including the basal ganglia, the cerebellum, and the brain stem. Alpha synuclein buildup also occurs in Parkinson’s disease, but is usually seen later in the course of the condition, and mostly confined to the substantia nigra region of the brain. MSA affects different types of brain cells than those affected by Parkinson’s.
There are two subtypes of MSA:
MSA-P more closely resembles Parkinson’s, but it progresses more quickly and stops responding to Parkinson’s drugs sooner.
In MSA-C, progressive loss of coordination and balance are prominent. People with MSA-C may show an “action tremor,” or tremor that happens when they reach for an object. Muscle weakness can cause slurring and trouble swallowing. MSA-C can develop as early as a person’s 40s.
Progressive supranuclear palsy (PSP)
Also known as Steele–Richardson–Olszewski syndrome, PSP causes motor symptoms very similar to those seen in Parkinson’s, but they tend to be much more severe and progress much more quickly. Most people develop severe disabilities within three to five years of a PSP diagnosis.
In addition to motor symptoms, people with PSP are likely to have mood and personality changes and cognitive difficulties. Tremors are rare in PSP. In progressive supranuclear palsy, people are more likely to tilt and fall backward, while people with Parkinson’s lean and fall forward.
PSP is also considered a type of frontotemporal dementia (FTD), a collection of conditions that cause progressive damage to the frontal and temporal lobes of the brain. In healthy brains, there is a normal protein called tau that helps form the structure of cells. In PSP, tau protein tangles together in abnormal clumps, and brain cells are destabilized.
Unlike other forms of parkinsonism, PSP can significantly reduce life expectancy. With treatment, a person with PSP may live 10 years after diagnosis.
Dementia with Lewy bodies (DLB)
DLB is characterized by the early development of cognitive symptoms (related to memory, attention, and thinking) and psychotic symptoms such as hallucinations. Parkinsonian motor symptoms occur later in the progression of the disease. After Alzheimer’s, DLB is the leading cause of dementia. DLB typically does not occur before the age of 65. In DLB, alpha synuclein protein builds up throughout the cerebral cortex of the brain, forming collections called Lewy bodies.
DLB is often misdiagnosed as Alzheimer’s. Symptoms of DLB may respond to medications for Parkinson’s or Alzheimer’s, but certain Alzheimer’s medications carry high risk for dangerous side effects if given to those with DLB. DLB and Parkinson’s disease dementia have many features in common, and together they are known as the Lewy body dementias.
Corticobasal degeneration (CBD)
CBD, also called corticobasal syndrome, is a rare type of parkinsonism that usually progresses more quickly than Parkinson’s disease. In CBD, brain cells in the cerebral cortex and the basal ganglia shrink and die. CBD affects men and women approximately equally. Symptoms usually begin between the ages of 50 and 70. Corticobasal degeneration may be considered a type of frontotemporal dementia (FTD).
Motor symptoms in CBD are nearly always asymmetrical – occurring on one side of the body. CBD may also cause cognitive and behavioral symptoms. People with CBD may also have Parkinson’s disease, dementia with Lewy bodies, progressive supranuclear palsy, frontotemporal dementia, and Alzheimer’s-like dementia