I am asked frequently by people who have had parkinsonism for several years what to expect going forward. This question is usually brought on by some change in the condition of the person: maybe meds have not been lasting as long, or balance has gotten worse. It could be any number of things, but the change has usually raised concern for what is coming next. This question is really about prognosis: the likely course a disease will take. Knowing about prognosis can help you plan your life, and alert those that care for you that this is not a static condition, there will be changes. Given that Parkinson disease (PD) is progressive, it is a good idea to figure out what your resources are, and who will be with you as you navigate this illness.
There is another benefit to being armed with knowledge. This will allow you to be unsurprised by the progression of disease, and to react appropriately if and when things do change. I find that people who take this approach have better quality of life and better outcomes. They know what to expect because they discuss PD issues with their doctor at appointments. They are actively engaged in managing the problem. The opposite way is to the take the head in the sand approach, and simply learn about the disease as it happens to you. In advanced disease this can lead to bad outcomes. I informally categorize the sometimes numerous and urgent phone calls from these patients, who have waited until some symptom is out of control, as an unnecessary form of disaster management, a crisis line type call. Nobody wants that, not you, not me.
Things can seem like they are falling apart, and it is better to be in front of the situation if you can. It will also help you to be more confident and secure about what is going on if you keep up with the disease, keep up with your doctor’s appointments (on time please!*), and follow medical advice. That is the best control you can have. And, it helps to have an ally, a person who is with you for this, a person who comes with you to appointments and keeps up with how things are going.
Having said all that, keep in mind still that PD tends to be a very slow-moving problem, and the past tends to predict the future in terms of rate of progression. For most people, after several years of PD the course unfortunately tends toward a less predictable response to medication, and to fluctuations in motor function. Bearing in mind that no two people with PD are the same, there may be a variety of other signs or symptoms of disease as discussed below. Some issues have more bearing on prognosis than others. Trying to make sense of all of this and figure out what the future will hold in a moderate to advanced patient is thus difficult. There have been several studies looking at these questions however, and I tried here to summarize some of that information in a way that I hope will be helpful. This post is not meant to be exhaustive, but a brief survey of some relevant topics. I am hopeful that you will use it to check out certain issues and hopefully feel a sense of familiarity with what is going on. I am hopeful that it will help.
Cognition
Many people with PD complain that cognitive function is not what it used to be, and this is for a variety of reasons, mostly to do with reduced levels of dopamine and other “neurotransmitters” such as acetylcholine and serotonin. The typical cognitive profile of PD is one in which thinking speed feels a little slower than it used to, short term memory is not quite as sharp, and multitasking is not as easy. If a gradual decline that still does not interfere with functional independence is noted, and the person meets certain neuropsychological criteria, the person is said to have mild cognitive impairment in PD (PD-MCI). (1) The prevalence of PD-MCI is about 27%, but increases with age, disease duration, and motor severity. PD-MCI may lead to dementia in Parkinson’s disease (PDD), and studies vary in reporting of incidence, ranging from 30 to 40% in many papers on this topic (2) to 80% in at least one population study. (3)
One study in Norway followed a sample of 224 PD patients with an average age of 73, and duration of disease 10 years when the study started. At baseline 22% of the patients had dementia. However, after 8 years, when the average age in the group had reached 81, and disease duration had reached 18 years, prevalence of dementia was 78%. (4)
In the Sydney Multicenter Study 149 people with PD across Australia were followed. At 15 years 48% of the sample group developed PDD, 36% MCI, and 15% had no cognitive impairment. Cognitive decline was more common in older individuals. (5)
Predictors of PDD in patients with PD-MCI include semantic disfluency (a problem with fluency of language), or a type of visuospatial dysfunction (complicated, but this is why cognitive tests often include a drawing portion).
Risk factors for PDD generally include older age overall, older age of disease at onset, overall duration of disease, severity of disease, absence of tremor, male gender, lower baseline education, the presence of hallucinations, depression, or PD-MCI.
What can you do about it? Sleep well. Don’t drink excessive amounts of alcohol. Stay away from high blood pressure and other chronic illnesses-or control them with precision. Stay physically, emotionally, and mentally healthy and active. Keep up with interests, have hobbies, be engaged. Finally, a medication may be prescribed for this.
Hallucinations
Hallucinations are known to be common in PD and are often benign. Some patients will have what are known as passage hallucinations, fleeting images in the periphery which are typically thought of more as misinterpretations by the brain than something worrisome. We can all have them, but they seem to be more common in older people with PD or Lewy body dementia (LBD). However, formed visual hallucinations, in which a person clearly sees, and can describe something that is not there, are more concerning. Sometimes these hallucinations are benign, sometimes not. In the Sidney Multicenter Study formed visual hallucinations at some time were seen in half of patients by 15 years of disease. It was recognized that some of the medications that improve the motor symptoms of PD might also exacerbate hallucinations. When medication adjustments were made hallucinations dropped to 21%. Only 6% required an atypical antipsychotic medication to control the hallucinations. The average time to the onset of hallucinations was close to 11 years since time of diagnosis. If you have them, tell your neurologist.
Unified Parkinson’s Disease Rating Scale
Doctors tend to use tools such as the Unified Parkinson’s Disease Rating Scale (UPDRS) to measure the advance of PD or the severity of signs and symptoms. When looking at just the motor subscore of the UPDRS, a change of 3.5 % per year is typical.
Predictors of impairment of motor function or disability include age of the patient overall, age at onset of PD, overall disease duration, excessive daytime sleepiness at baseline, or cognitive impairment at baseline. (6, 7)
Advance of motor dysfunction usually includes dyskinesia: the presence of excess movement that is not tremor. Medication adjustments in the form of smaller, more frequent doses or the addition of amantadine may make a positive difference. Other patients may experience motor fluctuations in the form of medication failure or unpredictable off time. When these events occur sometimes an extra half tab of immediate release carbidopa/levodopa is helpful, but may take 30 minutes or longer to kick in. Rescue drugs act much more quickly, in typically just a few minutes. These include inhaled levodopa and sublingual apomorphine hydrochloride (sounds like a narcotic, but it is not).
Sometimes patients go to the ER for motor fluctuations, but this is not usually a helpful move. ER doctors don’t tend to have a great deal of expertise in dealing with advanced movement disorders, and while a neurologist might be on-call covering the ER, that person is not usually the neurologist who is familiar with you, and usually would not consider this an emergency.
A medically appropriate strategy would be to keep regular appointments with a neurologist, make drug changes appropriate to the advance of your disease, and plan for change in the future. If you can’t manage symptoms between appointments, call your neurologist’s office.
Age
Older age itself as a risk factor has been evaluated. One meta-analysis of 45 studies, including 27,458 patients with PD, showed typically the duration from onset of disease to death ranged from 7 to 14 years, though a great variety of reasons for death and disability were listed, and the range of lifespan was considerable. Older age at onset of disease and the presence of dementia were the most consistently found predictors of death. (8) Bear in mind also that people in their 70s and 80s tend to die from many different causes, such as heart or lung disease. See the section on survival below.
Older age at onset of PD is not all bad either. Studies have shown that disease onset in older age is associated with better quality of life, lower rates of depression, and a better sense of wellbeing compared with those who had the average age of onset of PD.
Psychosis
Psychosis, which may include hallucinations and delusions, occurs in upwards of 30% of PD patients. Onset tends to be beyond 10 years since the time of diagnosis, and the impact on quality of life is negative for both the patient and caregiver. Psychosis increases the odds of emergency room visits, hospitalizations, and placement in nursing homes.
In a study of 230 people with PD followed over 12 years it was found that psychosis was most likely with older age of onset, higher dose of dopaminergic drugs, or significant REM sleep behavior disorder. Lowering the dose of dopaminergic medications can sometimes reduce psychosis. (9)
Nursing Home Placement
The most common cause of nursing home placement in PD is hallucination. Other causes include older age, advanced PD with more severe motor symptoms resulting in impairment of activities of daily living, falls, cognitive impairment, and living alone. (10)
Disability
Risk factors for increased disability with PD include psychosis, depressive disorder, severity of depression, apathy, sleepiness, motor impairment, and percentage of time with dyskinesia. (11)
Survival
Multiple studies have shown that some factors are associated with a shorter survival. Risk factors for shorter survival include non-tremor dominant type of PD, PDD, or early autonomic dysfunction, such as early severe orthostatic hypotension. (12-14)
A study of 230 community patients in Norway followed from 1993-2005 showed the average survival time from motor onset of disease was close to 16 years, though the range was from 2 to 36 years. (15) Bear in mind this placed the average patient at mid to high 70s, typical lifespan.
Overall, normal cognitive function at onset of parkinsonism is associated with a normal life span. In a Swedish study in which patients had an average age of 71 years at baseline entry to the study, patients without MCI survived another 12 years, and those with MCI another 8 years. Causes of death overall included pneumonia (19.5%), dementia (15.6%), unknown (11.7%), heart attack (9.1%), all types of cancer (7.8%), heart failure (5.2%), and other causes (31.2%). (16)
Generally speaking, better prognosis is also associated with good diet, regular exercise, regular engagement in life and interests, and healthy relationships with others. In moderate to advanced disease it is important to establish a caregiver, whether that person is a loved one or a professional. There may be a team involved, such as that caregiver, a therapist, a home health nurse, spiritual support, a neurologist, and a palliative care specialist. I would recommend palliative care for any adult with a chronic illness in order to specify goals of care and to improve quality of life for both the patient and the family.
I hope these facts have been useful. They are offered in the spirit of understanding this illness and trying to plan for the future.
Footnote*A strange phenomenon I have noticed from time to time is that the more complicated the problem, the less prepared for the appointment the patient tends to be, and the more likely that they will show up late for the appointment. Often this happens at a scheduled follow up, and the patient wants to discuss some new urgent issue. This places doctors and nurses at a huge disadvantage that is in turn a disadvantage for the patient. It is counterproductive for you. Advice: bring relevant records such as images (not just reports) of your brain that were taken while you were at some other emergency room last week. Don’t assume “it’s all in the computer.” And, please do not use the sentence “You can get my records.” All of that should have been present before you were to be seen by a health care provider. It is a great policy to call ahead and tell the nurse if you want to discuss some urgent issue at your upcoming appointment, and to make sure records are in order if you don’t have them. Don’t blindside your doctor, let them be prepared. I feel like a quote is order here: “Help me, help you.” Jerry McGuire.
REFERENCES
1. Cammisuli, et al. Front Aging Neurosci. 2019;11:303. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6856711/#B69
2. Emre. Lancet Neurol 2003; 2: 229–37
3. Litvan et al. Movement Disorders 2011 :26;1814-1824
4. Aarsland et al. Arch Neurol. 2003;60:387-392
5. Emre. Lancet Neurol 2003; 2: 229–37
6. Jankovic, et al. Arch Neurol. 2001;58(10):1611-5
7. Alves, et al. Neurology. 2005;65(9):1436-41
8. Knipe, et al. Mov Disord. 2011 Sep;26(11):2011-8
9. Rabey, et al. Parkinsonism Relat Disord. 2009, Suppl 4:S105-10.
10. Aarsland, et al. J Am Geriatr Soc. 2000 Aug;48(8):938-42
11. Weintraub, et al. J Am Geriatr Soc. 2004 May;52(5):784-8
12. Macleod, et al. Mov Disord 2014;29:1615–1622
13. Levy, et al. Neurology 2002;59:1708–1713
14. De Pablo-Fernandez, et al. JAMA Neurol 2017;74:970–976
15. Forsaa, et al. Neurology. 2010;75(14):1270-6
18. Bäckström, et al. Neurology Oct 2018, 00:e1-e12. doi:10.1212/WNL.0000000000006576