Parkinsonism-Plus Syndromes
Dear Readers/Bloggers: My goal is to present the Parkinsonism-Plus Syndromes in as simple and clear terms as possible. Easier said than done. This is an original essay I composed based on over six years of reading and writing about Parkinsonism-Plus Syndromes.
Parkinsonism-Plus Syndromes by Patient-Online
© 2012 "We Will Go On" - Used by Permission Only
Introduction
Parkinsonism-Plus Syndromes are conditions which involve the degeneration of the Basal Ganglia, the movement center of the brain. Parkinson’s Disease and Parkinsonism-Plus, will generally affect this part of the brain in that both will have a loss of the cells that produce a chemical called dopamine. Dopamine is required by the brain in order to bring about normal and properly coordinated movements. This lack of dopamine causes the symptoms of Parkinson’s Disease that are sometimes found in Parkinsonism-Plus, such as tremors, postural instability (balance difficulty), rigidity (stiffness) and bradykinesia (slowness of movement).
Although the Parkinsonism-Plus Syndromes typically have some aspects of these symptoms resulting from dopamine loss, Parkinsonism-Plus is not merely another name for Parkinson’s Disease or a more serious version, but it is a disease which includes Parkinson’s Disease symptoms plus other serious conditions.
As brain cells are lost or damaged, the symptoms will also worsen and affect speech, swallowing, balance, walking, tremors, eye movements, bodily functions, stiffness and facial expression, to name a few of the issues that are more prevalent. Cognition (the thinking process) is affected, and is one of the least noticed, but most severe aspects of Parkinsonism-Plus.
Differences Between Parkinsonism-Plus and Idiopathic Parkinson's Disease
Patients with Parkinsonism-Plus Syndromes have a worse prognosis than those with Parkinson's Disease (PD), and Parkinsonism-Plus syndromes respond poorly to the standard anti-Parkinson's Disease treatments. Although the medications used may be similar to those used to treat Parkinson’s Disease, Parkinsonism-Plus Syndromes typically do not respond as well to these medications. An inadequate response to treatment in a patient with Parkinson's Disease-like symptoms may indicate that a Parkinsonism-Plus Syndrome is developing. This should trigger medical tests and a thorough examination in search of any signs of degeneration in other brain structures beyond the Basal Ganglia.
Parkinsonism-Plus syndromes typically progress more rapidly than Parkinson’s Disease. There is no cure for these conditions, so treatment focuses on managing symptoms.
Diagnosing Parkinsonism-Plus
It is often difficult to diagnose a Parkinsonism-Plus condition to the point of determining which of the particular syndromes it is, specifically. Like Parkinson’s Disease, identifying specific bio-markers is difficult and the diagnosis of these diseases is primarily a clinical process of observation. In some cases, the diagnosis is confirmed after death when the brain is then studied and it becomes possible to differentiate. The parts of the brain affected with cell loss or shrinkage, will determine which of the symptoms are manifested, thus the diagnosis is based on the symptoms that are emphasized in each condition.
The term “levodopa” refers to the most important and common medication used to treat hallmark symptoms, and it is usually most effective in cases of regular Parkinson’s Disease. Levopoda is only moderately helpful in the case of Parkinson’s Plus. This distinction about the effectiveness of levodopa is one of the most important differences between regular (Idiopathic) Parkinson’s when compared to Parkinsonism-Plus.
Multiple System Atrophy
Multiple System Atrophy (MSA) is a rare degenerative condition and has historically been called Shy Drager Syndrome, as well. In MSA, more widespread neurological damage occurs than in the case of Parkinson’s. Both Parkinson's Disease and Multiple System Atrophy produce a build up of α-synuclein* proteins in brain cells of the Basal Ganglia where dopamine is produced. In Multiple System Atrophy the damage to cells is more widespread within the brain. When compared with Parkinson's Disease, Multiple System Atrophy affects more areas that control important autonomic functions such as heart rate, breathing, blood pressure, urination, digestion, body temperature, eye movements and sweating. Multiple System Atrophy does not respond as well to anti-Parkinson's Disease medicines, if it responds at all. Parkinson's Disease responds well to these medications, which helps doctors to make that diagnosis. Multiple System Atrophy progresses more quickly than Parkinson's. Multiple System Atrophy has an average survival rate of 7-9 years, while Parkinson's Disease does not necessarily affect the longevity of the patient. These are key differences between these two diseases, but by no means all of them.
The two Different MSA subtypes are identified based on which areas of the brain are most affected. MSA may appear to be a typical case of PD in the first few years of disease onset, but as it progresses extreme drops in blood pressure upon rising from a chair (accompanied by dizziness), difficulty with bowel and bladder control, exaggerated pulse rate fluctuations and sexual impotence will give rise to this diagnosis. Milly Kondracke, whose life was presented in a made for TV movie called SAVING MILLY, was diagnosed with Parkinson’s Disease initially, but was believed to have had a case of MSA when she died.
There are two subtypes of MSA, as described below.
Multiple System Atrophy-Parkinsonism (MSA-P)
This subtype is difficult in the initial years to distinguish from Parkinson’s Disease. Tremors, balance issues, rigidity, speech and swallowing are all eventually affected. It will be distinguished from idiopathic PD by its faster onset and inadequate response to Levodopa therapy. The autonomic issues will also become progressively worse as the disease progresses.
Multiple System Atrophy-Cerebellar (MSA-C)
This one is also similar to PD, but because of damage to the area of the brain called the cerebellum, balance, walking/gait issues, eye movement abnormalities and speech are affected more severely. The walking gait difficulty that surfaces with MSA-C is called ataxia. Challenges with the overall coordination of bodily movements is the striking difference between this and the other subtype. The prominent features are unsteady/staggering walk with slurred speech. Autonomic system functions will eventually be harmed, as well.
Progressive Supranuclear Palsy
Progressive Supranuclear Palsy (PSP) often resembles Parkinson's disease. Changes in the protein tau lead to neural degeneration. These changes lead to an aggregation of fibrillar polymers, known as taupathies. Its unique features include early development of a severe loss of balance, unsteady walking and frequent falls. People with PSP also may develop blurred vision and impaired eyesight, especially the inability to look up or down. This lack of eye movement causes difficulty for the patient to see where he or she is going, and interferes with reading and eating. Other symptoms include a gradual but significant impairment in speech and swallowing. Apathy, depression and a reduced ability to think are common among patients with PSP.
No medication has been found to treat this disorder. As with the other Parkinson’s Plus conditions, PD drugs such as levodopa and dopamine agonists are sometimes mildly beneficial and worth attempting as treatments. Currently, medical interventions are aimed at reducing the impact of specific symptoms, such as: balance, eating and swallowing. Support, counseling and education for patients and care partners can improve the patient’s quality of life. The average survival from onset of symptoms is 7 years.
Corticobasal Ganglionic Degeneration
(Note: I have been diagnosed with this syndrome in the last several months.--DB, 3/2017)
CBD is a movement disorder that usually starts after age 60. Like Progressive Supranuclear Palsy, it is a taupathy (see above in first paragraph of PSP explanation). First, symptoms often appear on one side of the body and spread to the other side. They include stiffness, rigidity, slowness, tremor, jerky movements and loss of sensation. Later, patients can develop problems with walking and maintaining balance, dementia, memory loss and “alien limb” phenomenon (a condition in which a limb appears to move on its own). Depression and other emotional changes may also develop.
Levodopa treatment is rarely successful. Other medicines may help manage specific symptoms. Physical, occupational and speech therapy can help patients cope with the illness. Education and support can improve quality of life. The average survival from onset of symptoms is 8 years.
Lewy Body Disease
Lewy bodies are abnormal protein deposits found in brain cells. In Parkinson’s disease, Lewy bodies form in only one area of the brain, the Basal Ganglia (where dopamine cells are lost, reducing the dopamine needed for normal movement). In Lewy Body Disease (LBD), these deposits are found in other important areas of the brain, as well.
Symptoms of LBD are similar to Parkinson’s and Alzheimer’s diseases, with an overwhelming emphasis on dementia characteristics. In addition, patients can have repeated visual hallucinations, and experience varying levels of alertness and mental ability. Depression, apathy, anxiety and delusional thoughts also are common. Some patients develop a sleep disorder years before developing LBD that may cause them to violently and loudly act out their dreams.
Treatment with levodopa and some Alzheimer medications are sometimes helpful. Support, education and counseling for the patient and family are important. The average survival from onset of symptoms is 5-7 years.
*signifies "alpha"-synuclein
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