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Atypical Parkinsonism

Table of Contents

    What Is Atypical Parkinsonism?

    Atypical Parkinsonism disorder, also called Parkinson’s Plus, is a set of progressive, neurodegenerative movement disorders with signs and symptoms that resemble those of Parkinson’s disease, but that generally do not respond well to the primary medication used to treat PD, levodopa.

    Technically, Parkinson’s disease is a form of parkinsonism, and while patients with atypical Parkinsonism develop many of the same parkinsonism symptoms as those with Parkinson’s disease, atypical parkinsonism patients can also experience a range of additional symptoms including:

    • early balance problems
    • problems with falling
    • early cognitive problems
    • and impaired control of their blood pressure, bowel, and bladder

    Facts About Atypical Parkinsonism

    • Atypical parkinsonism is considered a neurodegenerative disease except in patients where it’s drug-induced.
    • Progressive Supranuclear Palsy is the most common type of Atypical Parkinsonism, but it is only about one-tenth as common as Parkinson’s disease.
    • Multiple System Atrophy is considered to be “rare” — it affects about .003% – .004% of the population.
    • Corticobasal Degeneration was only first identified in 1968 and is also considered to be a “rare” disease; it usually affects one side of the body much more than the other.

    What Are the Symptoms of Atypical Parkinsonism?

    Many of the symptoms of atypical parkinsonism resemble classic Parkinson’s disease, including tremors, muscle stiffness, and problems with walking, balance, and coordination. Moreover, atypical parkinsonism patients can also have problems with speaking, swallowing, and drooling. 

    But there are additional symptoms associated with atypical parkinsonism that are driven by the underlying disease that’s causing the disorder.

    You Are More Than Your Symptoms

    Movement disorders are incredibly complex diseases affecting multiple systems in the body. At Neurology Solutions, we believe that these challenging diseases require a comprehensive approach that treats the whole patient— not a list of symptoms. 

    What Causes Atypical Parkinsonism?

    Several known diseases can cause atypical parkinsonism, each affecting a different region of the brain and presenting with a different set of symptoms.

    Diseases that Cause Atypical Parkinsonism

    Progressive Supranuclear Palsy (PSP)

    Progressive Supranuclear Palsy (PSP) is the most common form of atypical parkinsonism. It affects men and women equally and typically first appears in patients in their early 60s. When PSP is just beginning, it may look very similar to Parkinson’s disease, making it difficult to diagnose accurately. The only definitive way to diagnose PSP is by examining the brain tissue of the patient post-mortem during an autopsy.

    The cause of PSP is unknown but is associated with the accumulation of tau protein in the frontal lobes, brain stem, cerebellum, and substantia nigra of patients. PSP is not usually considered hereditary, nor has it been clearly associated with any environmental exposures.

    PSP patients typically don’t experience a tremor, but the most common PSP symptoms include:

    • Difficulties with eye movements, particularly when looking down.
    • Difficulty with balance, especially when descending stairs
    • A tendency to fall backward

    Multiple System Atrophy (MSA)

    Multiple System Atrophy (MSA) is a rare neurodegenerative disorder that primarily affects the autonomic nervous system — the part of the nervous system that controls internal functions such as the heartbeat, blood pressure, digestion, and urination.

    MSA symptoms include:

    • Urinary urgency, retention, and incontinence
    • Constipation
    • Lightheadedness when standing (orthostasis)
    • Significant erectile dysfunction in men that is otherwise unexplained
    • Some patients may experience ataxia

    Corticobasal Degeneration (CBD)

    Corticobasal Degeneration (CBD) is a rare, progressive neurodegenerative disease that’s very complex, with a wide variety of signs and symptoms. Similar to PSP, CBD involves a buildup of tau protein. It typically starts in patients between 60 and 70 years old and tends to affect one side of the body more than the other. CBD patients often have trouble seeing and navigating through space.

    CBD symptoms may include:

    • Dystonia
    • Sudden jerking (myoclonus)
    • Cognitive difficulties, such as problems with simple arithmetic
    • An inability to recognize or use familiar, everyday objects (such as a can opener or even using a knife and fork to eat)
    • Alien limb phenomenon

    Dementia with Lewy Bodies (DLB)

    Dementia with Lewy bodies, also known as Lewy body dementia (LBD), is the third most common cause of dementia, behind Alzheimer’s and vascular dementia. Similar to other forms of atypical parkinsonism, LBD is associated with the buildup of the alpha-synuclein protein in the brain.

    DLB symptoms may include:

    • Parkinson-like movement symptoms, such as rigidity or lack of spontaneous movement
    • Fluctuation changes in mental functioning, particularly attention or alertness
    • Visual hallucinations

    Our Approach to Treating Atypical Parkinsonism

    Treating atypical parkinsonism can be more complicated than treating traditional Parkinson’s disease (PD). With PD, you can typically replace the dopamine deficiency with dopamine-stimulating medications, and the patient’s symptoms will usually respond well.

    In contrast, the various atypical parkinsonisms tend to have varying structural abnormalities in the patient’s brains — therefore, replacing dopamine does not always work as well in these patients. Nevertheless, with most cases of atypical parkinsonism, traditional PD treatments are still attempted but often with limited success.

    Because of this inherent difficulty with treating atypical parkinsonism, we believe that metabolic optimization with nutrition, lifestyle changes, and physical therapy is often the best approach for atypical parkinsonism patients. Reducing risks to improve quality of life is also a focal point. 

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