Parkinsonism: Other Disorders That Resemble Parkinson's
By National Institute of Neurological Disorders & Stroke
A number of disorders can cause symptoms similar to those of Parkinson's Disease (PD). People with symptoms that resemble Parkinson's but that result from other causes are sometimes said to have parkinsonism. Some of these disorders are listed below.
Just after the first World War, a viral disease, encephalitis lethargica, attacked almost 5 million people throughout the world, and then suddenly disappeared in the 1920s. Known as sleeping sickness in the United States , this disease killed one third of its victims and led to post-encephalitic parkinsonism in many others. This resulted in a particularly severe form of movement disorder that appeared sometimes years after the initial illness. (In 1973, neurologist Oliver Sacks published Awakenings, an account of his work in the late 1960s with surviving post-encephalitic patients in a New York hospital. Using the then-experimental drug levodopa, Dr. Sacks was able to temporarily "awaken" these patients from their statue-like state). In rare cases, other viral infections, including western equine encephalomyelitis, eastern equine encephalomyelitis, and Japanese B encephalitis, have caused parkinsonian symptoms.
A reversible form of parkinsonism sometimes results from use of certain drugs, such as chlorpromazine and haloperidol, which are prescribed for patients with psychiatric disorders. Some drugs used for stomach disorders (metoclopramide), high blood pressure (reserpine), and epilepsy (valproate) may also produce parkinsonian symptoms. Stopping the medication or lowering the dosage of these medications usually causes the symptoms to go away.
Some toxins — such as manganese dust, carbon disulfide, and carbon monoxide — can cause parkinsonism. The chemical MPTP also causes a permanent form of parkinsonism that closely resembles PD. Investigators discovered this reaction in the 1980s when heroin addicts in California who had taken an illicit street drug contaminated with MPTP began to develop severe parkinsonism. This discovery, which showed that a toxic substance could damage the brain and produce parkinsonian symptoms, caused a dramatic breakthrough in Parkinson's research: for the first time, scientists were able to simulate PD in animals and conduct studies to increase understanding of the disease.
Sometimes known as pseudoparkinsonism, vascular parkinsonism, or atherosclerotic parkinsonism, arteriosclerotic parkinsonism involves damage to the brain due to multiple small strokes. Tremor is rare in this type of parkinsonism, while dementia — the loss of mental skills and abilities — is common. Antiparkinsonian drugs are of little help to patients with this form of parkinsonism.
Parkinsonism-Dementia Complex of Guam
This disease occurs among the Chamorro populations of Guam and the Mariana Islands and may be accompanied by a motor neuron disease resembling amyotrophic lateral sclerosis (Lou Gehrig's disease). The course of the disease is rapid, with death typically occurring within 5 years.
Also known as post-traumatic encephalopathy or "punch-drunk syndrome," parkinsonian symptoms can sometimes develop after a severe head injury or frequent head trauma that results from boxing or other activities. This type of trauma also can cause a form of dementia called dementia pugilistica.
Essential tremor, sometimes called benign essential tremor or familial tremor, is a common condition that tends to run in families and progresses slowly over time. The tremor is usually equal in both hands and increases when the hands are moving. The tremor may involve the head but usually spares the legs. Patients with essential tremor have no other parkinsonian features. Essential tremor is not the same as Parkinson's, and usually does not lead to it, although in some cases the two conditions may overlap in one person. Essential tremor does not respond to levodopa or most other PD drugs, but it can be treated with other medications.
Normal Pressure Hydrocephalus (NPH)
NPH is an abnormal increase of cerebrospinal fluid (CSF) in the brain's ventricles, or cavities. It occurs if the normal flow of CSF throughout the brain and spinal cord is blocked in some way. This causes the ventricles to enlarge, putting pressure on the brain. Symptoms include problems with walking, impaired bladder control leading to urinary frequency or incontinence, and progressive mental impairment and dementia. The person also may have a general slowing of movements or may complain that his or her feet feel "stuck." These symptoms may sometimes be mistaken for PD. Brain scans, intracranial pressure monitoring, and other tests can help to distinguish NPH from PD and other disorders. NPH can sometimes be treated by surgically implanting a CSF shunt that drains excess cerebrospinal fluid into the abdomen, where it is absorbed.
Progressive Supranuclear Palsy (PSP)
PSP, sometimes called Steele-Richardson-Olszewski syndrome, is a rare, progressive brain disorder that causes problems with control of gait and balance. People often tend to fall early in the course of PSP. One of the most obvious signs of the disease is an inability to move the eyes properly. Some patients describe this effect as a blurring. PSP patients often show alterations of mood and behavior, including depression and apathy as well as mild dementia. The symptoms of PSP are caused by a gradual deterioration of brain cells in the brainstem. It is often misdiagnosed because some of its symptoms are very much like those of PD, Alzheimer's disease, and other brain disorders. PSP symptoms usually do not respond to medication.
Corticobasal degeneration results from atrophy of multiple areas of the brain, including the cerebral cortex and the basal ganglia. Initial symptoms may first appear on one side of the body, but eventually affect both sides. Symptoms are similar to those found in PD, including rigidity, impaired balance and coordination, and dystonia. Other symptoms may include cognitive and visual-spatial impairments, apraxia (loss of the ability to make familiar, purposeful movements), hesitant and halting speech, myoclonus (muscular jerks), and dysphagia (difficulty swallowing). Unlike PD, corticobasal degeneration usually does not respond to medication.
Multiple System Atrophy (MSA)
MSA refers to a set of slowly progressive disorders that affect the central and autonomic nervous systems. MSA may have symptoms that resemble PD. It also may take a form that primarily produces poor coordination and slurred speech, or it may have a mixture of these symptoms. Other symptoms may include breathing and swallowing difficulties, male impotence, constipation, and urinary difficulties. The disorder previously called Shy-Drager syndrome refers to MSA with prominent orthostatic hypotension — a fall in blood pressure every time the person stands up. MSA with parkinsonian symptoms is sometimes referred to as striatonigral degeneration, while MSA with poor coordination and slurred speech is sometimes called olivopontocerebellar atrophy.
Dementia with Lewy Bodies
Dementia with Lewy bodies is a neurodegenerative disorder associated with abnormal protein deposits (Lewy bodies) found in certain areas of the brain. Symptoms can range from traditional parkinsonian symptoms, such as bradykinesia, rigidity, tremor, and shuffling gait, to symptoms similar to those of Alzheimer's disease. These symptoms may fluctuate, or wax and wane dramatically. Visual hallucinations may be one of the first symptoms, and patients may suffer from other psychiatric disturbances such as delusions and depression. Cognitive problems also occur early in the course of the disease. Levodopa and other antiparkinsonian medications can help with the motor symptoms of dementia with Lewy bodies, but they may make hallucinations and delusions worse.
Parkinsonism Accompanying Other Conditions
Parkinsonian symptoms may also appear in patients with other, clearly distinct neurological disorders such as Wilson's disease, Huntington's disease, Alzheimer's disease, spinocerebellar ataxias, and Creutzfeldt-Jakob disease. Each of these disorders has specific features that help to distinguish them from PD.
MSA, corticobasal degeneration, and progressive supranuclear palsy are sometimes referred to as "Parkinson's-plus" diseases because they have the symptoms of PD plus additional features.
The National Institute of Neurological Disorders and Stroke (NINDS) conducts and supports research on brain and nervous system disorders.