follow up on blog tomorrow…here and on Family circle as well as blog on PDF.org this week
Okay, don’t panic you come to the right place!
First of all, Parkinsonism simply means you have features of Parkinson’s disease (PD) but not the entire clinical picture based on the UK a brain criterion which includes 4 main symptoms: rest tremor; rigidity (stiffness); bradykenisia (slowness); and gait abnormality. Usually people labeled ‘parkinsonian’ have two or three of these features.
What Causes This?
3 scenarios are possible for why this occurs.
1) You either have an underlying illness which affects the basal ganglia – structure involved in PD – which then mimics PD. Things that affect these structures of the brain whether it’s through blood loss, loss of oxygen, pressure, swelling from trauma or infection can then mimic symptoms of Parkinson’s because remember the basal ganglia is important in movement, learning and balance. So common primary neurological diseases like strokes, multiple sclerosis, brain tumors must be ruled out and can be a reason why you have received a diagnosis of Parkinsonism. Non- neurological causes which can affect the brain are things like lupus which cause brain inflammation and other types of infections such as lymes disease or syphilis, and HIV. NPH (Normal pressure hydrocephalus) and lower body parkinsonism caused by multiple strokes (considered to be its own disease type) can be other less known causes of parkinsons-like symptoms in an individual. This is why we often order an MRI of the brain at time of diagnosis as well as lumbar puncture / spinal tap to make sure no infections are present.
2) Your Parkinson’s disease is very early in its presentation at time of first evaluation and has not developed all of its features; this is especially common in younger people with YOPD who may present usually with dystonia (abnormal contraction of muscles) along with more non- motor symptoms in conjunction with stiffness and slowness. I have had many young patients take 5 years or longer to present with full blown PD symptoms- initially receiving diagnosis of Parkinsonism or focal even segmental dystonia. I myself did not start having rest tremors till 7 years after onset of other PD symptoms; even now my rest tremors are not very prominent. They are more pronounced in my toes than in my hands which is not very common or typical.
3) You may have an atypical Parkinson’s syndrome or Parkinson’s plus disease such as Lewy body dementia (LBD), corticobasalganglia degeneration (CBGD), progressive supranuclear palsy (PSP), multi system atrophy (MSA) which could either be Shy-drager or olivopontocerebellodegeneration or atrophy (OPCA). All of these “atypical” PD illnesses take time to develop into full blown disease that can be recognized sometimes even by experts if the right “key” feature is not present from the beginning like “alien-hand” is characteristic of CBGD. Plus we must remember there are many other hereditary illnesses which are less typical and common that can present this way some of which may not have characteristic imaging findings and can’t be recognized unless an expert does a specific blood test such as SCA6 . However, because we don’t have a diagnostic blood test or an objective way of differentiating among the various types of Parkinson’s we must rely on the ability of MDS doctors to recognize the other clinical signs which point to the fact that one is not dealing with ‘typical’ Parkinson’s disease. The signs and symptoms such as dementia, memory loss, personality changes, or apathy from the beginning of disease, bladder issues severe and out of proportion for time of other symptoms like tremors, stiffness .as well as greater swallowing difficulties, increase falling, speech impediments, difficulty with coordination or action tremors are all signs that we are dealing with a Parkinson’s plus syndrome. In this category, a good MDS can be of great benefit. He or she should be able to tell that your Parkinsonism is due to an atypical syndrome as opposed to a secondary cause like in 1. However, knowing a person has a Parkinson’s plus syndrome and being able to pinpoint with 100% certainty which one may still require frequent visits with your physician.
Getting A Dat scan does not help in these situation because all it can tell you is that there is a dopamine problem if normal it would only point to a non -dopaminergic cause and if MRI normal- NPH, infection (individual would be sick with fevers, rash etc), and even psychogenic causes need to be entertained.
At this point since we do not have an assortment of drugs to treat different types of Parkinsonism or to treat even between the different subtypes of PD the treatment is always Levodopa! All types of Parkinsonism except psychogenic requires levodopa. However, the prognosis for each and the amount of response vary on the type and underlying cause. The best response to levodopa is with typical PD- a big clue for physicians.
The key to successful treatment is early initiation of medication independent of cause except psychogenic/stressed induced. Since if its early PD – the sooner the treatment the better the prognosis and greater quality of life, if its Parkinson’s plus it will initially give quality but because of nature of disease it will progress independent of medication and will not lose anything by starting dopa early. In the one’s caused by an underlying disease, patients will fell symptomatically better but once underlying disease or culprit found they may or may not need further dopa but if they do unlike the other dopaminergic illnesses there will not be a need for escalation of does and medications because symptoms are for the most part static.
Nevertheless, all Parkinsonism patients will benefit from a neurologist care. Those with Parkinson’s and those with Parkinson’s plus syndromes need a movement disorder specialist which can manage the subtleties as well as coordinate a team care approach. Those with Parkinsonism due to other underlying cause need to seek care from a specialist in that area such as stroke doctor, MS specialist, or rheumatologist. In some cases, the Parkinsonism can resolve completely once cause is identified like treating infection or NPH.
This is why it is extremely important to seek initial treatment with a neurologist and follow- up with the same person consistently in order to increase as well as expedite accurate and proper diagnosis.
Dat Scan (Ioflupane I 23 injection also known as phenyltropane) is a radio pharmaceutical agent injected into the veins of a patient known as SPECT nuclear medicine test. When this test was approved in was under the premise that it will be an added tool in the armamentarium of the neurologists/ movement disorder specialist to help decipher difficult cases.
So, the answer to the question…can it tell you have PD? – NO!
The test can’t confirm you have PD! – it can only tell if there is abnormality in the dopamine system which can include any and all of the Parkinson’s plus syndromes including Parkinson’s disease. If abnormal it means there is a problem in the dopamine system period.
Furthermore, like any test is user dependent. My husband who is a neuroradiologist in a small community who has extensive experience in reading PETs for lots of disease and specializes in the brain would not give an accurate reading of a DATSCAN as his colleagues up the road in Houston at the medical center who do hundreds of them. Since the only PET scans of the brain that have been FDA approved are for diagnosis of dementia- there are standard things that the radiologists look for by guidelines set by the Academy of Radiology who also mandate general training in reading of these tests across the border to all practicing radiologist to maintain their credentials. However, no such mandate has been given in the reading in SPECT or DAT scans for diagnosis of PD and only those that are in academic centers who have seen hundreds of these studies are actually the only ones qualified to give an expert opinion as to the “quantitative” measure of the uptake in the brain.
If you have received a diagnosis of PD from an expert specialists in movement disorders and are improving or responding to dopamine therapy there is no reason or gain by getting a Dat scan. Likewise, if someone suspects of Parkinsonism due to PSP, MSA etc. no added benefit will be obtained by getting this scan, you might in fact be wasting your money ($2500 to $5000) and time.
This test was only intended to be used as another diagnostic tool to help decipher between dopamine and non dopamine diseases which can mimic PD. Now it is over used unfortunately for the wrong reasons. The FDA only intended to be used to differentiate between essential tremors and PD. In my humble professional opinion, Dat scans are not required for treatment or diagnosis and only place for a Dat scan is in academics for studies or in rare cases where a procedure like DBS or Pallidotomy is being considered and physician is not sure if treating essential tremors vs. PD; which if this is the case, I would be reluctant to have a brain surgical procedure when clinical diagnosis is in question! This invariable will lead to poor outcome… many other treatments can be employed until diagnosis is certain.
Another thing because the trace used to measure dopamine activity is radioactive and expensive is not ordered till the day or night before the test. Thus, if you decide to cancel at the last minute because not feeling well you are causing the facility to lose a lot of money and some facilities may even charge you for it. If you have history of thyroid disease or take thyroid replacement you may not be able to do the test.
So short and long …Doing a DAT SCAN CAN NOT TELL ANYONE THEY HAVE PD – do not be fooled by those that claim otherwise!!! Parkinson’s unfortunately still remains primarily a clinical diagnosis and ONLY way to diagnosed with 100% certainty is brain biopsy or at autopsy. However, there is a caveat, with more studies being done in academic centers understanding and standardization of DAT scan reading is increasing slowly among those involved in the field. at the same time we are slowly gaining knowledge of PD and its varying presentations. Therefore, it is conceivable that in the near future, we might be able to combine the knowledge of two to predict and detect patients who will develop PD.
According to a new study, Danna Jennings, MD, Clinical Research Director at the Institute for Neurodegenerative Disorders in New Haven, and colleagues have attempted to do just this via the Parkinson Associated Risk Syndrome (PARS) study to identify a large-scale cohort of individuals at risk for Parkinson’s disease using olfactory testing and DAT imaging. What they have found is that although no one had PD symptoms at baseline despite abnormal DAT scans or reduced ability to smell ; 46% of individuals with loss of sense of smell combined with a deficit on the DAT scan have shown to develop clinical features of Parkinson’s disease within four years.
“The knowledge that comes from this study will have important implications for the recruitment of individuals for future neuroprotective trials,” stated Dr. Anthony Lang, Director of the Movement Disorders Clinic at Toronto Western Hospital. Remember, in a previous blog “Thinking Outside the Brain for a Parkinson’s Cure,” I commented that often trials fail or are doomed to fail from the start when it comes to finding neuroprotective agents because we often don’t even have the right diagnosis. If we are able to successfully predict who will develop PD from these early markers: 1) we can institute treatment a lot earlier in hopes of retarding or slowing progression and 2) trials may have a greater chance of success than previously; because we may no longer have to wait until a patient’s disease evolves to the motor stage causing obvious manifestations of Parkinson’s disease in order to include in “early –stage” trials which by definition is no longer early since by then these patients have lost at least 50% of their dopamine producing neurons.
Source: Olfactory Testing and DAT Imaging May Lead to Early Detection of Parkinson’s disease. Neurology Reviews. 2014 22(8):18-21.
Dr. M. De Leon is a movement disorder specialist on sabbatical, PPAC member and research advocate for PDF (Parkinson’s Disease Foundation); Texas State Assistant Director for PAN (Parkinson’s Action Network). You can learn more about her work at http://www.facebook.com/defeatparkinsons101 you can also learn more about Parkinson’s disease at www.pdf.org or at www.wemove.org; http://www.aan.org, http://www.defeatparkinsons.blogspot.com All materials here forth are property of Defeatparkinsons. without express written consent, these materials only may be used for viewers personal & non-commercial uses which do not harm the reputation of Defeatparkinsons organization or Dr. M. De Leon provided you do not remove any copyrights. To request permission to reproduce release of any part or whole of content, please contact me at deleonenterprises3@@yahoo.com contributor http://www.assisted-living-directory.com Contributor http://www.lavozbrazoriacounty.com
For some time me and others like myself have been toying with the idea could it be possible that Parkinson’s is yet another autoimmune disease like diabetes or thyroid disease or even pernicious anemia? Or even an inflammatory disorders like ulcerative colitis? Perhaps some genetic subtypes can more easily fall into this category than others. For instance, it is interesting that the majority of Parkinson’s Disease (PD) patients have B12 deficiency as a co morbidity. Or that the type of Gaucher patients that are most likely to develop and have an increase risk of developing PD are type I patients – those without central nervous system involvement.
Patients who posses Gaucher and are carriers of a GBA gene mutation have increased risk of developing Parkinson’s disease and parkinsonism. Gaucher patients carry a deficiency of the enzyme glucocerobrosidase. This enzyme is typically acts on the glycolipid glucocerobroside. So, when the enzyme is defective, glucosylceramide aggregates and accumulates in white cells (which are responsible for mounting immune attack but particularly like to congregate in the macrophages. The macrophages are like “Pac Man” hungry white blood cells gobbling up invading bacteria. They are formed in response to an infection or accumulating damage or dead cells. Thus, they are unable to break down fatty acids and they have abnormal accumulation into the white cells and macrophages which are the ones responsible for being able to mount an appropriate immune response against a foreign attack like bacteria or virus. But, if these cells who are to destroy the offending viruses etc. are unable to take them up because already full with unwanted stored up material then more white cells, T cells and macrophages have to be created jamming up the system and thus indiscriminately attacking normal cells throughout the body and brain. Some evidence of this is present in the preliminary data in a study I am involved at the University Of Texas Houston Center. My friend and colleague has informed me that my blood levels of T cells and other inflammatory and immune markers have been measured and found to be elevated and have improved as my disease has been treated. Data hopefully will be released soon.(this is all preliminary and confidential).
The other subtype of PD which points to an inflammatory and autoimmune component is the LRRK2 gene phenotype. Many of these patients with this type of phenotype have a history of inflammatory bowel disease most often ulcerative colitis. Ulcerative colitis (UC) is another autoimmune disease characterized by T-cells infiltrating the colon. Although Crohn’s disease another inflammatory bowel disease which is much more extensive beyond the colon as compared to UC has also been seen in this group of PD patients, once again making a case for a possibility of our immune system going haywire and attacking the nervous system. In medicine, it is dogma to say that once a patient has one autoimmune disease they are at higher risk for contracting another and we frequently see this in our practices. But, until recently, no one had really dared to contradict the underlying notion that “neurons” were somehow protected from attacks from the immune system. Although, we have clear evidence of instances of where there is an autoimmune reaction in the central and peripheral nervous system after vaccine injections causing “Acute disseminated encephalomyelitis” and also MS but these have only targeted the connections not destroyed or actually damaged actual Neurons! Therefore, if it is true that Parkinson’s neuronal loss is a result of attack of the immune system itself it would revolutionize not only our thinking but our way of preventing and going after treatments for Parkinson’s.
So, recently researchers tested this hypothesis to see if indeed living neurons would display antigens (like bacteria or viruses) which then trigger an immune mediated response to neutralize this force. Drs. Sutzer and Cebrian from Columbia University used in vitro mouse and human neurons from embryonic stem cells. Their studies revealed that under certain circumstances- including those known to occur in PD. These neurons produced a special protein which presented an antigen which was recognized by the T cells and triggered an attack on these neurons. They prove an autoimmune process can happen and neurons can be attacked but is not known if this is the initial or final response or if all Parkinson’s is started this way or only those subtypes I alluded to previously. One thing is for sure future is bright and field is ripe for new and novel treatment options!
For more information on the subject go to:
Dr. M. De Leon is a movement disorder specialist on sabbatical, PPAC member and research advocate for PDF (Parkinson’s Disease Foundation); Texas State Assistant Director for PAN (Parkinson’s Action Network). You can learn more about her work at http://www.facebook.com/defeatparkinsons101 you can also learn more about Parkinson’s disease at www.pdf.org or at www.wemove.org; http://www.aan.org, http://www.defeatparkinsons.blogspot.com
All materials here forth are property of Defeatparkinsons. without express written consent, these materials only may be used for viewers personal & non-commercial uses which do not harm the reputation of Defeatparkinsons organization or Dr. M. De Leon provided you do not remove any copyrights. To request permission to reproduce release of any part or whole of content, please contact me at email@example.com