Tag: MSA

Types (Atypical Parkinsonisms)

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
MSA-P more closely resembles Parkinson’s, but it progresses more quickly and stops responding to Parkinson’s drugs sooner.

MSA-C
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

Types (Parkinsonism Syndrome)

Lowrey… stated about this article: ( Posted on August 13, 2018 by MyParkinsonsTeam) Volumes of objective, clear and succinct info about PD, with out the border line hysteria often associated with cognitive impairment. Most refreshing! Many thanks for sharing this.

I agree! But, knowing my readers, I have broken the article into three days, to hopefully avoid overload and ‘shut down’ without reading… day 2 will be “Secondary Parkinsonism” Day 3 outlines five “Atypical Parkinsonisms.”

Parkinsonism is a syndrome, or collection of symptoms, characterized by motor issues – bradykinesia (slowed movements), tremors, loss of balance, and stiffness. There are many types of parkinsonism classified by their cause and how they progress. Knowing which type of Parkinson’s someone has helps neurologists prescribe effective treatments and better predict how the disease will progress.

Parkinson’s types

There is no conclusive test to identify what type of parkinsonism someone has. For some people, years may elapse between experiencing the first symptoms and receiving a definitive diagnosis of a specific type. Since all parkinsonisms share similar motor symptoms, Parkinson’s diagnosis can be very difficult. A correct diagnosis is more likely when performed by an experienced neurologist who specializes in movement disorders. Some people have multiple chronic conditions, making it difficult for doctors to identify whether parkinsonian symptoms are caused by a disease or a medication. In some cases, it is possible to have more than one type of parkinsonism.

Parkinson’s disease

The most common type of parkinsonism is Parkinson’s disease (PD), which accounts for about 80 percent of cases. No one is sure what causes most cases of Parkinson’s disease, so it is also known as idiopathic Parkinson’s. Idiopathic means “cause unknown.”

Deep inside the brain, regions called the basal ganglia and substantia nigra work together to ensure that the body moves smoothly. The substantia nigra produces a neurotransmitter – a chemical that helps nerves communicate – called dopamine. Messages sent by the brain to muscles to cause movement pass through the basal ganglia with the help of dopamine. In Parkinson’s disease, cells in the substantia nigra gradually stop producing dopamine and die off. With too little dopamine, the basal ganglia cannot facilitate movement as well. Researchers believe parkinsonian symptoms begin when the level of dopamine falls to about half of normal levels.

Subsets of Parkinson’s disease include:

  • Late-onset Parkinson’s disease

Symptoms develop after age 50.

Most PD is late-onset.

  • Early-onset or young-onset Parkinson’s disease

Symptoms develop before age 50.

Accounts for approximately 10 percent of PD cases

Tends to have slower progression, more medication side effects

Dystonia (painful spasms and abnormal postures) is more common in early-onset PD.

  • Juvenile-onset Parkinson’s disease

Symptoms develop before age 20.

Extremely rare

Often strong family history of Parkinson’s

  • Familial Parkinson’s

Directly caused by genetic variants inherited from parents

Accounts for 10 to 15 percent of Parkinson’s disease cases

Parkinson’s disease dementia (PDD)

Between 50 and 80 percent of those with Parkinson’s disease eventually develop Parkinson’s disease dementia. On average, most people begin to develop PDD about 10 years after they receive a Parkinson’s disease diagnosis. PDD is often confused with Alzheimer’s and dementia with Lewy bodies. Parkinson’s disease dementia is usually diagnosed when motor symptoms occur first, at least a year before dementia symptoms.

Antipsychotics such as Haldol (Haloperidol) and Thorazine (Chlorpromazine)

Anti-nausea medications such as Reglan (Metoclopramide)

Antidepressants in the serotonin specific reuptake inhibitors (SSRI) class such as Prozac (Fluoxetine) and Zoloft (Sertraline)

Calcium channel blockers such as Flunarizine and Cinnarizine (not approved for use in the U.S.)

Reserpine

 

Orthostatic tremor

I learned about a type of tremor I hadn’t heard about, before… from a team member with a MSA diagnosis, typing on the myparkinsonsteam.com site.

“Orthostatic tremor (OT) is a rare syndrome characterized by unsteadiness on standing due to a high-frequency tremor involving the legs. Symptoms usually start in the sixth decade. Typically, the symptoms rapidly improve on sitting or walking, and the need to sit down or to move can be so strong that patients avoid situations where they have to stand still. A polygraphic recording of a fast and synchronous tremor of the legs, between 13 and 18Hz, is mandatory to confirm the diagnosis of OT. Many patients also suffer from tremor, often involving lower frequencies, of the face, hands, or trunk. Recent studies suggest that this is perhaps due to subharmonics of the high-frequency tremor spreading through the body. Most cases of OT seem to be idiopathic, though symptomatic forms have been occasionally described. Several cases of OT have been reported in Parkinson’s disease.”

…..What follows was written by a DR in the Neurology Dept at Mayo Clinic…..

“The term tremor implies an involuntary, rhythmic body movement. Orthostatic tremor affects the muscles in a person’s legs, and sometimes also radiates from the legs upward to other parts of the body. The term “orthostatic” refers to standing. Orthostatic tremor occurs when a person stands up. The condition can be difficult to diagnose and, although medication helps some people, it can be a challenging condition to treat effectively.

Orthostatic tremor is an uncommon disorder that can significantly affect the quality of life for those who develop it. Usually, the main symptom is a feeling of leg shakiness and unsteadiness when standing still. Orthostatic tremor can create difficulty in performing daily activities that require a person to stand freely without support. For example, standing in line at a store, standing at a kitchen counter to prepare a meal, or standing at a workbench to enjoy a hobby can be very hard or impossible for people who have orthostatic tremor. Most people don’t fall as a result of the condition. However, approximately 15 percent become so unsteady that falling is a problem.

Orthostatic tremor often starts shortly after standing and stops when a person sits or lies down. Contrary to what common sense might normally suggest, typically the tremor decreases when a person is walking. Leaning on an object such as a chair or a countertop when standing also may help reduce the tremor.

Orthostatic tremor is a high-frequency tremor, which means the tremor rhythm is very rapid and there can be as many as 16 to 20 tremor cycles in one second. That’s significantly faster than other tremor types. For example, essential tremor usually involves eight to 12 tremor cycles per second, and the tremors associated with Parkinson’s disease typically occur at a rate of four to eight cycles per second.

Because the tremor is so fast, orthostatic tremor can be difficult to see. That can make the diagnosis challenging. In some cases, when patients report that they feel unsteady on their feet, the physicians may overlook the leg tremor and may pursue other possible causes of unsteadiness. One additional diagnostic strategy is to place a hand on the thigh, feeling for the tremor; however, the clinician must have an appropriate level of suspicion.

If a physician suspects orthostatic tremor, the diagnosis can be confirmed by assessing the electrical activity in the leg muscles. At Mayo Clinic, that analysis is conducted in our Movement Disorders Neurophysiology Laboratory. During orthostatic tremor, the leg muscles show no electrical activity when a person is sitting. When he or she stands up, though, the muscles immediately begin firing rhythmic bursts of electrical activity.

Because the cause of orthostatic tremor is unknown, a cure currently isn’t available. Instead, treatment focuses on the symptoms. Once the condition is diagnosed, the first line of treatment is the medication clonazepam or a related drug. This medication moderately to markedly reduces orthostatic tremor in about one-third of people who have the disorder. For some, it eliminates orthostatic tremor almost entirely.

Unfortunately, for those who don’t respond to clonazepam, no single alternative medication has a high likelihood of decreasing orthostatic tremor. Although numerous other medications may be tried, these may be no more than mildly helpful.

Fortunately, orthostatic tremor tends not to be a progressive disorder. Once the symptoms are fully developed, it doesn’t progress to a more pervasive neurological condition. It does tend to be persistent, however, and usually does not resolve on its own.”

— J. Eric Ahlskog, M.D., Ph.D., Neurology, Mayo Clinic, Rochester, Minn.

Gasp

I experience a complication listed under ‘additional’ symptoms of MSA… A Parkinsonism… Multiple System Atrophy.

  • Involuntary, uncontrollable sighing or gasping

This happens unpredictably where I suddenly suck in air VERY quickly. Usually it is of no consequence, however I did experience one pretty significant choking episode, exactly one year ago, which I documented.

May 26, 2018   As indicated previously, there are times I experience an involuntary sudden intake of air (gasp), followed by a release (sigh).  Today, the ‘gasp’ timing occurred just as I had taken a bite of a toasted grill cheese sandwich… inadvertently sucking crumbs of whole wheat toast into my air passage, which precipitated an inordinate amount of violent coughing.  Although breathing was labored, the passageway was never entirely blocked. Within a few minutes, the toast was finally coughed up.

This morning, as I was showering, it happened… that I gasped while attempting to rinse shampoo from my hair. Yikes! No matter that it was Melaleuca Herbal Shampoo… Bubbles impede air flow! I don’t know what kind of sound I made, as I struggled to breathe, but it was enough to panic my sweetheart.

It has been eight hours, and I still have shampoo breath and my voice is raspy, but the coughing has subsided.

I know there is something called aspiration pneumonia… so I looked it up: Aspiration pneumonia is a type of pneumonia that might occur if a person breathes something in instead of swallowing it. The germs from food particles, saliva, vomit, or other substances may infect the airways and lead to aspiration pneumonia.

I suppose it was good that I ingested an herbal concoction… shouldn’t have to worry about infection.