Types of Parkinsonisms

Parkinsonism is a term used to describe the collection of signs and movement symptoms associated with several conditions — including Parkinson’s disease (PD). Signs include slowness (bradykinesia), stiffness (rigidity) and resting tremor. Conditions other than Parkinson's disease may have one or more of these symptoms, mimicking PD. Your neurologist should assess your symptoms when making a diagnosis.

Below are the more common medical conditions that can present with Parkinsonian disorders (parkinsonism). Parkinsonism can be classified into two major groups: primary and secondary.

Primary Parkinsonism

Primary parkinsonian disorders include Parkinson’s disease and atypical parkinsonian disorders. Both can be misdiagnosed, however a neurologist with training in movement disorders can help make an accurate diagnosis and coordinate care.

Atypical parkinsonian disorders can also be sporadic or familial.
- The sporadic cases include progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD), and dementia with Lewy bodies (DLB), as well as other rarer causes.
- Familial (heredodegenerative) disorders that may present with atypical parkinsonism include Huntington disease, spinocerebellar ataxias, X-linked dystonia-parkinsonism (Lubag disease) and neuronal brain iron accumulation disorders.

Secondary Parkinsonism

This type of parkinsonism includes neurological disorders commonly caused by brain tumors, toxins or medications. Symptoms are similar to those seen in Parkinson’s disease and may sometimes go away with treatment, depending on the disorder. Unlike Parkinson’s disease, the medication Levodopa does not improve symptoms.

There are many secondary or acquired causes of parkinsonism.

Drug-induced parkinsonism can be difficult to distinguish from Parkinson’s, though the tremors and postural instability may be less severe. It is usually the side effect of drugs that affect dopamine levels in the brain, such as antipsychotics, some calcium channel blockers and stimulants like amphetamines and cocaine. If the affected person stops taking the drug(s), symptoms usually go away over time, but may take as long as 18 months.
Vascular parkinsonism is usually caused by clotting in the brain from multiple small strokes. People with vascular parkinsonism tend to have more problems with gait than tremor and have more problems in the lower body. The disorder progresses very slowly in comparison to other types of parkinsonism. People might report an abrupt onset of symptoms or symptoms that get worse then plateau for a while. Symptoms in vascular parkinsonism may or may not respond to levodopa.
- Metabolic diseases, such as Wilson’s disease
- Endocrine diseases, such as hypothyroidism
- Heavy metals, such as manganese
- Infectious diseases, such as Whipple’s disease
- Normal pressure hydrocephalus
- Toxins, such as MPTP
- Repetitive head trauma
Parkinsonism can also be categorized based on the abnormal proteins that accumulate in the brain, such as alpha synuclein.

Atypical Parkinson’s Conditions

DLB is second only to Alzheimer’s as the most common cause of dementia in elderly people. It causes progressive intellectual and functional deterioration. In addition to the signs and symptoms of Parkinson’s disease, people with DLB tend to have frequent changes in thinking ability, level of attention or alertness and visual hallucinations. They usually do not have a tremor or have only a slight tremor. The parkinsonian symptoms may or may not respond to levodopa.
PSP is slightly more common than amyotrophic lateral sclerosis (ALS), also called Lou Gehrig disease. Symptoms usually begin in the early 60’s. Common early symptoms include loss of balance while walking that results in unexplained falls, forgetfulness and personality changes.

The visual problems associated with PSP generally occur three to five years after the walking problems and involve the inability to aim the eyes properly because of weakness or paralysis of the muscles that move the eyeballs. People with PSP may have some response to dopaminergic treatment but may require higher doses than people with Parkinson’s disease. Learn more about PSP.
MSA (also referred to as Shy-Drager syndrome) is the term for a group of disorders in which one or more systems in the body stop working. In MSA, the autonomic nervous system is often severely affected early in the course of the disease.

Symptoms include bladder problems resulting in urinary urgency, hesitancy or incontinence and orthostatic hypotension (nOH). In nOH the blood pressure drops so low when standing that fainting or near fainting can occur. When lying down, blood pressure can be quite high (called supine hypertension). For men, the earliest sign may be loss of erectile function. Other symptoms that may develop include impaired speech, difficulties with breathing and swallowing, and inability to sweat.

Like the other parkinsonism conditions, MSA symptoms respond either very little, or not at all to Parkinson’s medications. Learn more about MSA.
CBS is the least common atypical parkinsonism. It usually develops after age 60. Symptoms include a loss of function on one side of the body, involuntary and jerky movements of a limb and speech problems. It may become difficult or impossible to use the affected limb although there is no weakness or sensory loss. People with CBD may feel as if their limb is not under his/her voluntary control. There is no specific treatment for CBD. Treatment is focused on reducing symptoms and improving quality of life.

Parkinsonian disorders and Levodopa

Various parkinsonian disorders can be categorized based on how they respond to the medication called levodopa. PD tends to respond well to levodopa therapies, while most atypical parkinsonian disorders do not. Sometimes people with parkinsonian symptoms who do not respond well to levodopa may be referred to as having parkinsonism. This can be confusing since parkinsonism technically refers to a set of movement symptoms, rather than a specific diagnosis.

Understanding Your Diagnosis

Despite recent research and diagnostic advances, the diagnosis of PD and types of atypical parkinsonian still relies primarily on a clinical evaluation. There are many overlapping signs and symptoms among PD, atypical parkinsonisms, and secondary parkinsonisms, making it difficult to diagnose. An accurate diagnosis can take months or even years to determine.

You may be diagnosed right away with “typical” or idiopathic Parkinson’s, or your doctor may tell you that you have parkinsonism. A “diagnosis” of parkinsonism may simply mean that you have movement symptoms, like slowness, rigidity and tremor. It might also mean that your doctor does not have enough information to know if your symptoms will respond well to levodopa.

Additionally, some doctors use the term parkinsonism interchangeably with atypical parkinsonism. Talk to your doctor if you have questions about what your diagnosis means.

Page reviewed by Dr. Jun Yu, Movement Disorders Fellow at the University of Florida, a Parkinson’s Foundation Center of Excellence and Jerome Lisk, MD, FAAN, Movement Disorders Specialist at MD Neurology.