What is atypical parkinsonism?

Atypical parkinsonism is a term that refers to diseases that share some features of parkinsonism (slowness of movement, muscle stiffness, tremor, or walking/balance problems), but tend to be more rapidly progressive, while less likely to respond to the drugs used to treat Parkinson disease (PD).

Early in disease, a person with atypical parkinsonism may have features that mimic PD, and this commonly will lead to a wrong diagnosis. Usually over time features unique to each form of atypical parkinsonism will declare themselves and therefore allow a doctor to correct the diagnosis. Forms of atypical parkinsonism are not usually genetic diseases, or at best do not appear to spread in a genetic pattern through families. The first four disorders discussed below were formerly called “Parkinson-plus” syndromes, though the name was changed in part due to the fact that they are not forms of Parkinson disease, thus a misleading term. Drug-induced, or tardive parkinsonism will not be discussed here, though these too, are forms of parkinsonism that are not PD. Also not discussed , due to the fact that they are very rare conditions which only sometimes mimic PD, are illnesses such as certain forms of spinocerebellar ataxia, neurodegeneration with brain iron accumulation, Fahr’s disease, the Westphal variant of Huntington disease, and many others. This article lists the illnesses which are usually implied when a doctor refers to atypical parkinsonism.

Corticobasal degeneration (CBD) (also known as corticobasal syndrome) typically affects one side of the body more than the other, classically resulting in apraxia, the inability to carry out learned movements in the absence of weakness or impaired sensation. For example, a person with CBD may not be able to turn a car key, use a spoon properly, or use scissors. In these patients another common issue is dystonia, a semi-sustained contraction of a pair of opposing muscles. Dystonia may cause painful twisting and cramp-like pain in a limb. Myoclonus, a sudden, brief, jerking movement, is common. Another unusual feature of CBD is alien limb phenomenon, wherein a person feels like an arm or leg is not their own, and they may not be able to control it. Similar to Alzheimer disease and PSP (below), CBD is a tauopathy, in which abnormal tau protein accumulations collect in key locations of the brain. Dystonia is treated with botulinum toxins (Botox, Dysport, Xeomin, or Myobloc). Myoclonus may sometimes be treated with levetiracetam (Keppra), valproic acid (Depakote), or clonazepam (Klonopin).

Dementia with Lewy bodies (DLB) is the second most common form of neurodegenerative dementia (after Alzheimer disease), and tends to start in the 50s, though it may occur at any age after 50. These patients have progressive cognitive problems in the first year of disease, with or before motor features as described above. The parkinsonism tends to be symmetric. Patients with DLB often describe illusions, in which one object or a pattern, takes on the appearance of something else. For example, one might see a person in the branches of a tree, or a face in a pattern on wallpaper. Visual hallucinations tend to come after illusions, and are common. Usually these are small animals or children, or moving shadows in the periphery of the visual field, so-called “passage hallucinations.” DLB patients tend to fluctuate, having good days and bad days (or weeks), which may be severe. These patients tend to have delusional thoughts, believing something that could not possibly be true, such as a newscaster talking directly to through the television. Paranoia is also common. And, importantly, these patients are very sensitive to neuroleptics, drugs which can cause parkinsonism, such as metoclopramide (Reglan),and the antipsychotic medications which block dopamine receptors. These drugs should be avoided in patients with DLB. Newer atypical antipsychotics may be tolerated in these patients. DLB is characterized by an abnormal accumulation of misfolded alpha-synuclein protein in brain cells, which result in Lewy bodies.

Multiple system atrophy (MSA) is a form of symmetric parkinsonism in which autonomic function tends to be biggest problem: urinary symptoms such as urgency, retention, or incontinence, sexual dysfunction, GI issues such as constipation, blood pressure problems resulting in lightheadedness when standing or after eating a large meal, or cardiac issues. Patients may notice red hands and feet, and they may feel they do not tolerate cold temperatures well. There are two main forms of MSA. MSA-C (formerly known as OPCA), affects the cerebellum and results in very poor balance, gait problems, and poor coordination. MSA-P (formerly known as striatonigral degeneration), mimics PD, but is more rapid and is unresponsive to dopaminergic medication because the disease harms the striatum, where dopamine is used. In MSA misfolded alpha-synuclein accumulates in key locations that are associated with damage. The low blood pressure issues are usually treated by stopping antihypertensives (blood pressure lowering medications), lowering doses of carbidopa/levodopa (if taken), liberalizing salt, and adding drugs such as midodrine, fludrocortisone (Florinef), or droxidopa (Northera).

Progressive supranuclear palsy (PSP) is a symmetric parkinsonian condition in which early eye movement problems (especially downgaze, such as looking down at your feet), and falls usually bring patients to the neurologist. In PSP there is typically no flexion of gait, and patients develop a high degree of rigidity of the spine, so-called “axial rigidity.” There is usually no tremor in PSP. PSP is a tauopathy similar to Alzheimer disease and CBD. The balance issues in PSP are initally treated with physical therapy. Sometimes the vision issues can be treated with prism glasses.

Vascular parkinsonism is usually caused by stroke or small vessel disease (damage to tiny blood vessels in the brain). In most cases gait/balance issues are the most prominent problems, and often it seems to be predominantly lower extremity disease. Tremor is uncommon in vascular parkinsonism, and dopaminergic medications are usually unhelpful. High blood pressure is the leading cause of small vessel disease.

If you or a loved one have an atypical form of parkinsonism, please see the article Janet Edmunson on the atypical parkinsonism support group.

What is parkinsonism?

I am frequently asked why I use the term “parkinsonism,” rather than “Parkinson disease” (PD) for most patients. It is a fair question, and one that I hope to clear up here. This issue is also relevant for veterans seeking service connection for PD through the U.S. Department of Veteran Affairs (VA). If notes from a doctor’s office indicate parkinsonism as the diagnosis, the vet may be denied because the VA interprets the use of “parkinsonism” as an indication the patient “does not have a diagnosis of Parkinson disease,” or so the denial letters read. In my practice I have written a form letter to explain the situation to the VA.

Parkinsonism is an umbrella term most movement disorder neurologists use to refer to a group of conditions that share features (physical signs) of PD. People who have parkinsonism may have PD, or may have an alternative diagnosis which can mimic PD, such as vascular parkinsonism, drug-induced parkinsonism, or multiple systems atrophy – to name a few. When we say that someone has parkinsonism, we are talking about these signs: slowness of movement (bradykinesia) plus either stiffness of muscles (rigidity) or tremor at rest. Balance problems and gait changes are often considered as well. All of these are so-called “parkinsonian features.” There are a variety of other early motor and non-motor signs and symptoms which may support a diagnosis, but are not required to say that one has parkinsonism.

Parkinsonism is a less specific descriptor than PD, and is not an actual diagnosis, but a category reflecting a set of symptoms. The term is sometimes used out of necessity, even when it seems pretty likely that a person has PD. But there is a world of difference between pretty likely and definite.

Under diagnostic criteria by the American Academy of Neurology (AAN) the best we can say in the vast majority of cases is that a person has “probable” PD (1). However,when doctors diagnose patients they have to choose an ICD-10 (Medicare billing) code. There is no code for probable PD, but there are codes for parkinsonism and PD. Under the guidelines the only coding choice that makes sense most of the time is parkinsonism.

It is also true that in rare cases, some mimickers look identical to PD, especially early in disease. It may take a while, sometimes years, for atypical forms of parkinsonism to “declare” themselves and show signs or symptoms that agree with something other than PD.

Because of this overlap in presentation, under National Institute of Neurological Disorders and Stroke (NINDS) criteria (2), brain biopsy would be the only way we could be certain, and diagnose definite PD in a living person. However, NINDS does not condone brain biopsy, no one does (see below). NINDS therefore tells doctors the highest degree of certainty they can have is “probable PD.” The internationally recognized Movement Disorder Society (MDS) guidelines also recognize “probable PD” as a diagnostic endpoint for the same reason. There are a variety of guidelines around the world which are very similar: PD is a clinical diagnosis and brain biopsy, while it would diagnose with certainty, would be unethical (4). You are using your brain, all of it, and would probably miss pieces that were removed. Likewise, this would place you at risk of infection, bleeding, or other issues.

Sometimes people donate their brains to science (after they have finished using them). At that point, it can be established conclusively whether or not a person had suffered from PD in their lifetime. This is often helpful for other family members and does a lot to move neuroscience forward. Several of my patients have in recent years used the Mayo Jacksonville lab for this reason. When donated brains are examined, pathologists are looking for Lewy bodies (LB), the pathologic hallmark of disease (see footnote) in key locations of the brain. Location is important, because PD is not the only condition with LB. Other diseases that have LB in a pattern different from PD include pure autonomic failure (PAF), multiple systems atrophy (MSA), and Lewy body dementia (LBD).

Back to the living. Diagnosis can sometimes be supported by dopamine transporter scanning (DaTscan) but this is still not definitive. DaTscan tells doctors that dopamine is low, but not the reason why. The FDA indication for DaTscan is to distinguish essential tremor from parkinsonian syndromes. That is usually not a hard call.

So, to sum up the problem, we can’t biopsy your brain, and there is no blood test, imaging, cerebrospinal fluid examination, or other electrophysiology test that makes the diagnosis of PD. Until we have a test or a biomarker, PD will remain a clinical diagnosis, and patients will have “probable” PD or parkinsonism.

Footnote: For more on Lewy bodies, read the spring 2016 MPDN article “What’s so bad about alpha-synuclein.”

REFERENCES

Suchowersky, et al. Practice Parameter: Diagnosis and prognosis of new onset Parkinson disease (an evidence-based review) Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2006 Apr 11;66(7):968-75.
Gelb, et al. Diagnostic criteria for Parkinson’s disease. Arch Neurol. 1999;56:33-39.
Postum and Berg, MDS Clinical Diagnostic Criteria for Parkinson’s Disease (I1.010) 2016; 86 (16 Supplement).
Marsili, et al., Diagnostic Criteria for Parkinson’s Disease: From James Parkinson to the Concept of Prodromal Disease. Front Neurol. 2018; 9: 156.

Getting the most out of seeing your neurologist

Whether you are a first time neurology patient or recurrent visitor to a movement disorders specialist, it would help to take a few minutes to think about your history so that you and your doctor can make the most of your visit. It is important to us that you feel understood, and likely important to you too, that that we learn as much as possible about your symptoms. Because we do this all the time, and are very familiar with these neurologic problems, neurologists have some insight into how to have this conversation. This article is a sort of a guide to what to do, and what not to do.

First, let’s clear up the idea that doctors are talking to each other on computers, or that doctors can see everything on other computers at other offices.

We are not, though when we ask about history, we are often told, “it should be in the computer,” or “in Dr. So-and-so’s chart.” If this is your first time seeing a neurologist, you shouldn’t assume your new doctor can see your referring doctor’s chart in a computer. We usually cannot for reasons given below. Most of the time the records must be sent from one office to another, and it is a task choosing what to send, and literally a task for someone in the referring office to send those files. You may think this is all squared away because your doctor made the referral. But, don’t assume that records from referring doctors have actually arrived and that the new doctor knows the medical facts of your case. That might also be a problem. Let’s walk through how this works in 2019.

A neurologist is consulted by another doctor. This means that other doctor has asked for help. But, the communication is not always clear. In spite of the fact that we have moved into the age of electronic medical records (EMR) on the dreaded computer (frankly how a lot of us feel about it), we are not all connected.

Due to legal and technical issues often the consultant has not been sent anything through a computer.

Instead, they have been faxed a few pieces of demographic information about who you are, where you live, what your insurance is, and hopefully the question being asked. Sometimes there are a few office notes. Amazingly, the reason for consult is not always included (though Medicare and insurance carriers usually require this information). This issue slows down time for getting an appointment. There are also a huge variety of formats for the forms sent. Sometimes the consultant cannot find needed information because it is buried on page five (or page 50 for that matter). Sometimes the name of the person has been altered to protect identity. Great, but who is this? Sometimes the computer coding is so heavy that a practically unreadable document is sent. Sometimes a copy of a copy is faxed and all abnormal (and therefore highlighted) lab values have become illegible.

A lot of this has to do with huge changes in medicine over the last couple decades, resulting in a clunky system that is far from perfect. We are required to use computers, and under Medicare guidelines can actually be fined for not using them. Still, we are unable to communicate as easily as you might think. Many different software vendors employ armies of information technology people who write complex software that results in systems that don’t talk and are not easy to learn. Most doctors recoil at the phrase “mandatory computer training.” Aside from the steep learning curve, software changes all the time, meaning two things: you’ve gotta keep up (who has the time?), and there will be unintended consequences of software upgrades, which some of us call “downgrades.” And, seemingly well-intentioned laws are not so easy to navigate.

Under Federal HIPAA law, your medical history is protected, and unless you have signed a release of information, it will not be available. HIPAA is a big reason why computers at different offices or organizations are almost never connected.

Even if you have signed a release, your records may be incomplete.

Your referring doctor might think you have Parkinson disease (PD), yet the records that arrive at the neurology office may be incomplete to the point of little or no supporting documentation about the signs or symptoms (this is often unintentional). The consulting neurologist wants to know what the other doctor has seen to lead to this consultation. Another scenario is that your referring doctor might have requested a second opinion. In that case, sometimes the notes that arrive do not include the first neurologist’s evaluation. These examples are problems on many levels. Consider these actual examples I have seen in referrals for which I was sent no records (and was therefore unaware) of a dangerous reaction to a medication, a critical lab value, or a brain aneurysm. I would recommend that you make sure the notes from your prior neurologist are sent to the consultant’s office to avoid a counterproductive and potentially dangerous situation.

There is also a simple fact of modern healthcare that time is limited.

Doctors see a lot more patients in a day than they used to, and are often unable to complete all of the notes, paperwork, etc., that piles up each day. An appointment without records will probably waste at least some of the appointment, and might result in a second “get to know you” visit in the future. No one thinks that is ideal. Consider it this way: you’ve waited weeks or months for an appointment only to find the doctor has no idea about your complex history, and can’t do much to help until they do. When is that next appointment going to be open?

Brain imaging (MRI, CT)

Brain images are not always necessary in PD, but sometimes are ordered when there is a question about some other cause of your symptoms. MRI of the brain is a very complex test which takes hundreds of images. To a neurologist this a way to see the anatomy and take into account many different factors. But, the way we see these films has changed. In the “old days” before computers were everywhere in medicine, images were on film, often transported by the patient if going from one institution to another. These days brain images are stored as huge digital files with hundreds of images too big to “send” over the internet. If your images are from another hospital or network, they are probably not “on the computer” at the new doctor’s office. The images are almost never sent by referring doctors for a variety of reason, firstly, they don’t have them. Referring doctors are not usually neurologists or radiologists, and often haven’t seen the images. Many referring doctors rely on radiology to read the films and give a report. They will then tell the patient something about the study, and the patient will tell the neurologist about that conversation. However, “I was told it was fine” just will not do.

Sometimes the radiology report is faxed with the referral. That is helpful, but neurologists usually want to see the images. Your brain anatomy is highly unique and may tell a neurologist a great deal about you. A good plan is for you to call the film library at your hospital or radiology department and get the images on a computer disc to bring to your appointment. Otherwise, waiting to see the images at the next visit slows your care down and leaves your doctor with an incomplete understanding of you and your workup.

Here I should mention that I have met a few patients who have intentionally tried to get a “fresh start” with no prior records. It is not a good idea. If anything, intentionally withholding prior records is a red flag to doctors. Both sides need to be completely open and honest. Give your new doctor a chance to read why the prior one came to the conclusion they did, even if you disagree.

Even when records have been forwarded, they may not tell the complete story. This is why it is very helpful to think about your PD history and come prepared to answer questions about it. Interview styles vary, but in my own practice when first meeting a patient, I will typically ask dozens of “closed-ended” questions which can be answered with a single, or just a few words. I can learn a lot quickly this way. So, be succinct. For example, if I ask what year the tremor started, the answer should be something like “1995.” The answer should not be expansive or tangential, such as this response in which names and details have been changed to illustrate a point:

“Let’s see, I was visiting my cousin Ed. I do every spring. He has an apple orchard up in Camden. We were going to walk around the property, and take in the sunset. It’s beautiful up there. Anyway, later that day after we had been all around the place and eaten dinner, I was drinking coffee. Ed makes good coffee. I don’t usually like it, but his is so good. Ed asked me if I wanted another and I said “no,” because I don’t usually drink it, and especially not that late. Then Ed said he had been reading on the internet about tremors. Then he said he had been noticing some shaking with me. We both first thought it was something else. Of course, it could have been the coffee, but then I haven’t had coffee today, and I’m shaking. And, it’s getting worse, so, probably not the coffee. After Ed I went to my doctor and first he thought it was essential tremor. But then I started reading on the internet myself…” and so on.

After that paragraph we still do not know the answer to the question of what year the tremor started (though Ed’s place sounds nice). Mainers tell me this is the “all the way around barn” approach to answering a question. I am probably not going to make it through my dozens of other questions if the responses keep up like this. I would advise patients to listen to the question being asked and answer it, only it, one question at a time.

When taking a history, a neurologist is looking for a timeline of specific details from your past in order to piece together your case in a way that leads them back to the proper diagnosis and an understanding of the progression of your illness. In the case of PD there are several illnesses or drug exposures that might be mimics, and one of the things neurologists who interview patients is trying to do is eliminate those other items by asking focused questions.

Like a detective, a meeting with a neurologist sometimes feels like less of a conversation than an interrogation. But there are good reasons for this.

I would recommend you not try to direct the conversation, but simply let the neurologist ask the questions they need to cover. If something is left out, then let them know at the end. In your own best interest, you should prepare and come armed with lots of information about your own history. I have included a form with this article which covers most of the things we will ask about. Please fill out the form, it will help a lot.

For your consult, you should specifically think about what year your first symptoms started, and what those symptoms were, for example: loss of sense of smell, constipation, REM behavior disorder (acting out vivid dreams in the night, thrashing in your sleep, screaming), tremor, stiffness of muscles, slowness of movement, changes in the way you walk, balance problems, falls, soft speech, memory issues, mood issues, what studies were done. If you have had gene testing or other unusual labs, bring the results. Three labs neurologists usually want to know about in PD are vitamins B6, B12, and D. Other labs we commonly might want to see include CMP, CBC, ESR, A1C, TSH.

Make a list of medications and doses of those medications you have tried for PD.

Write down any side effects you may have had with medications. Think about how the symptoms of your disease changed over time. If we established for example, that your hand tremor started in 1995, arrange your disease as it progressed over time, such as this: “It slowly worsened and my handwriting changed in 1996 or so. In 1998 my left hand started to shake and the same year I noticed shuffling when I walked.”

Also think about your family history.

If others in your family have had PD or something like it, ask them about it, and make a list of those family members along with any known diagnosis.

Who should be present at my consult or office visits?

I try whenever possible to collect history directly from the patient. Sometimes, especially if one has memory problems, it is helpful to have someone else present who can give a focused history. Someone else does not mean everyone else however. It is generally a very good idea to limit the number of other people present in the room at the time of a neurology visit, as it is difficult to get a clear history from multiple sources. Also, if you bring someone else, be consistent. Bringing a different friend or family member to every appointment creates a lack of continuity, and typically consumes a lot of the appointment time in order to bring the new person up to speed, or go over history that has already been covered. That is usually not helpful for the patient and is often counterproductive.

So that your neurologist can spend the entire visit with you, arrive on time.

It seems obvious, but every day in my clinic one or more patients will arrive late. Sometimes late patients cannot be seen. In my office, new patient consults are asked to arrive 15 minutes early for the first visit. This time is important for the staff to make sure certain records are correct, go over medications, check vital signs, and so on. However, a significant number of patients ignore this request, and that is not helpful.

If you have a follow-up visit, and there is some new issue, a change of some kind, or specific questions, let your doctor know at the beginning of the appointment. Urgent issues should be discussed first. Don’t wait until the end of the appointment to mention you were recently in the emergency room, for example.

Do not change, stop, or start PD medications without your neurologist.

Call if there is a problem.

Phone calls

Most offices have a medication refill line and a nurse triage line. Triage is for urgent issues. Routine questions should be addressed at follow up, not the phone. But, if you are not sure how serious something is, it is better to run it by a nurse. Neurology nurses tend to have a very good idea of what is important and what is not. That is what is triage is all about. There is also a neurologist on-call all the time. If your doctor is on-call they are probably busy tending to hospital or emergency room patients. After-hours issues in PD rarely warrant paging a doctor. Medical emergencies should of course be directed to 911.

I hope this helps.

New Parkinson disease patient checklist Download

Thanks to Grace Plummer, LCSW, Sarah Savard, RN, and Liz Stamey, RN, LMT for review and commentary.

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