Atypical parkinsonisms are conditions in which an individual experiences some of the signs and symptoms of Parkinson's disease (PD) -- tremor, slowness, rigidity (stiffness), and/or walking and balance problems -- but does not have PD. Atypical parkinsonism can be due to certain medications (some anti-nausea and antipsychotic drugs), other brain disorders (repeated head injury or multiple small strokes) or neurodegenerative diseases.

The neurodegenerative diseases, which cause damage or death of brain cells, include corticobasal degeneration, Lewy body dementia, multiple system atrophy and progressive supranuclear palsy. These conditions are often referred to as "Parkinson's plus" because they mimic PD but have extra associated symptoms (the "plus"). They can be misdiagnosed as Parkinson's disease because no blood or imaging test can, on its own, make a definitive diagnosis. (As with PD, the diagnosis is based on a person's medical history and physical examination.) Early in the course, people with Parkinson's plus syndromes also may get some benefit from levodopa, the drug most commonly used to treat PD. A poor response to levodopa, development of additional symptoms and more rapid progression of disease may eventually differentiate Parkinson's plus from PD, although it can take years for these differences to emerge. As with PD, no disease-modifying therapy has been discovered for any of the neurodegenerative atypical parkinsonisms so treatment is symptomatic and supportive.

Corticobasal degeneration (CBD) leads primarily to motor and cognitive (memory/thinking) symptoms. Motor symptoms mainly affect one arm and/or hand and include:

• slowness,
• stiffness,
• myoclonus (rapid muscle jerks), and
• dystonia (an abnormal, fixed posture).

The dystonic posture may cause the arm to be held close to the body and bent at the elbow and the wrist and fingers to be flexed toward the palm. Dystonia can cause pain and palm sores and interfere with regular daily activities (such as brushing teeth or preparing meals). Cognitive problems can affect speech, memory and/or behavior. Brain-processing difficulties can make performing complex motions, such as combing hair or turning a key in a lock, challenging or impossible. People with CBD may also experience "alien limb phenomenon," which is involuntary activity of a limb and a feeling that the limb is foreign or has a will of its own. (An alien hand could take one's eyeglasses off after the other hand has put them on, for example.)

Lewy body dementia (LBD), also known as dementia with Lewy bodies (DLB) is a form of dementia associated with PD, typically occurring early in the course of disease. LBD involves motor symptoms of Parkinson's (usually stiffness and slowness) and significant impairment of thinking and/or memory abilities that interferes with daily activities. Additional symptoms may include:

• visual hallucinations (seeing things that aren't there),
• unpredictable fluctuations in levels of alertness or attentions, and
• mood, behavioral and/or personality changes.

REM sleep behavior disorder, in which a person acts out his or her dreams, and orthostatic hypotension (a decrease in blood pressure when changing positions that can cause dizziness or lightheadedness) are other common symptoms.

Multiple system atrophy (MSA) patients may experience:

• parkinsonism -- usually slowness, stiffness and walking/balance difficulties (rather than tremor);
• cerebellar symptoms -- incoordination, imbalance and/or slurred speech; and
• autonomic nervous system dysfunction -- problems with the body's automatic activities such as blood pressure regulation, bladder emptying and sexual functions.

Other features of MSA include abnormal postures (head and neck tilted forward, hand held in a grasping position, or foot and ankle turned inward); speech and swallowing problems; episodes of uncontrolled laughter or crying (pseudobulbar palsy); cognitive (memory/thinking) problems; and sleep disturbances, including REM sleep behavior disorder (acting out one's dreams) or sleep apnea (breathing pauses during sleep).

Progressive supranuclear palsy (PSP) causes imbalance, gait difficulties and a tendency to fall backwards. It also restricts normal eye movements, which can lead to reading difficulties, falls when walking down stairs and visual disturbances (blurred or double vision, or light sensitivity). Involuntary eyelid closure (called blepharospasm); memory and behavior changes (such as decreased motivation and emotional fluctuations); and speech and swallowing problems also may occur.

Management of Parkinson's Plus Syndromes

These diseases are complex conditions that progress over time. As ongoing symptoms worsen and new symptoms arise, a person's needs will change and caregivers' roles and responsibilities will evolve. A team approach involving the person with disease, caregivers, family members and multiple medical professionals, is necessary to address the multitude of symptoms. As with PD, no disease-modifying therapy has been discovered for the neurodegenerative atypical parkinsonisms. Treatment relies on medications to lessen symptoms, allied health care services, assistive devices (canes or walkers) when necessary and caregiver support. Palliative care specialists can be especially helpful consultants for managing symptoms and coordinating goals of care.

Levodopa is usually the initial therapy for motor symptoms, although most people do not get a significant or long-term response. Other Parkinson's medications are sometimes used in conjunction with or instead of levodopa, but in general these are not very effective either. For dystonia, Botox injections can be helpful, and for associated non-motor symptoms (such as memory, behavioral or sleep disturbances), doctors may prescribe a variety of other medications.

Physical and occupational therapy are beneficial, specifically for dystonia, gait and balance problems, and falls. In earlier stages of disease, therapists can develop programs aimed at maintaining mobility, preventing falls or falling in ways to minimize injury. They can also assess the need for a cane or walker. In advancing disease, therapists can teach exercises to maintain joint range of motion, evaluate the home for safety and suggest modifications or adaptive equipment (such as shower grab bars or a raised toilet seat), and determine the appropriate type of wheelchair if one is necessary.

Speech therapists can recommend language exercises for speech disturbances and dietary and/or mealtime adjustments for swallowing problems. If swallowing problems are particularly severe (leading to weight loss, choking or pneumonia), your therapist or doctor may discuss starting a feeding tube. While not always required, it's worth thinking about this possibility early on so that a person (and their caregiver’s) thoughts can be taken into full consideration.

Throughout the course, social workers can provide educational resources, link to support groups and assist with finding in-home care services or alternative living situations. Palliative care providers can be consulted at any point for help with managing symptoms and determining goals of current and future care. In conjunction with a person's movement disorder specialist, palliative care experts can aid in optimizing medical therapy while lending extra emotional and spiritual support, and coordinating communication among the patient, family and medical providers.

Watch a webinar on atypical parkinsonisms.

Register with Fox Trial Finder to find recruiting clinical trials for atypical parkinsonism and Parkinson's disease. Already on Fox Trial Finder? Make sure your profile is up to date to be matched with the latest studies in your area.

Thank you to our 2016 Parkinson's Disease Education Consortium Supporter and Steering Committee Members. While our generous sponsors make our educational offerings possible, their support does not influence MJFF's content or perspective.