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Benign Tremulous Parkinsonism
I just returned home from an appointment with the neurologist (MDS) who treated me from 2000-2004; she's back at my clinic in the Seattle area after spending the last 3 years at the Cleveland Clinic. Based on her examination today, and after several years between examinations (she can see no change, or progression in my PD), and since I have been symptomatic for more than 10 years, she believes I have the syndrome that has recently been identified as "Benign Tremulous Parkinsonism."
I found this one research paper; I fit every category. Anyone else find this to be familiar? link to article, and text below: http://archneur.ama-assn.org/cgi/con...3/3/354?ck=nck ---------------------------------------------- Benign Tremulous Parkinsonism Keith A. Josephs, MST, MD; Joseph Y. Matsumoto, MD; J. Eric Ahlskog, MD, PhD Arch Neurol. 2006;63:354-357. ABSTRACT Background Benign tremulous parkinsonism has never been precisely defined nor has the long-term course been studied. Objective To report the clinical features and longitudinal course of patients with benign tremulous parkinsonism encountered in our movement disorders practice. Design Computer search of medical records database. Setting Mayo Clinic, Rochester, Minn. Patients Of 116 patients identified, 16 (10 male and 6 female) had at least an 8-year history of this disease, had been examined by a senior movement disorders specialist, and had ultimately been diagnosed as having benign tremulous parkinsonism after an initial diagnosis of Parkinson disease (PD). Interventions None. Main Outcome Measures Age at onset of disease, response to levodopa therapy, tremor characteristics, and family history. Results Mean disease duration was 11 years (range, 8-25 years) at last follow-up. Mean age at onset, 58.5 years, was younger than in most PD series, and most patients had a poor levodopa response (although levodopa trials were inadequate in some). A moderate to marked postural tremor was noted in 13 of the 16 patients, including 6 with a kinetic tremor. A family history of PD and/or tremor was reported in 10 (63%) of our patients. Three patients required thalamic deep brain surgery to treat their tremor. Conclusions Benign tremulous parkinsonism may be a distinct clinical entity characterized by tremor predominance plus minimal progression of other aspects of parkinsonism. The tremor is often not very responsive to levodopa therapy. In this series, most patients had immediate family members with a diagnosis of tremor or PD. INTRODUCTION In our routine movement disorders clinical practice, we have occasionally encountered patients with features similar to classic idiopathic Parkinson disease (PD) yet with a unique clinical course. These patients fulfill minimum criteria for PD with resting tremor plus other parkinsonian signs, without evidence of ataxia, corticospinal signs, apraxia, cognitive impairment, or prominent early dysautonomia. What has distinguished them has been a consistent constellation of clinical features: (1) prominent resting tremor that is the first or among the first signs and that persistently overshadows other aspects of parkinsonism throughout the course; (2) nontremor components of parkinsonism that remain mild; (3) absence of gait disorder apart from reduced arm swing or mild stooping; (4) no more than mild progression, except for tremor, despite at least 8 years of parkinsonism; and (5) absence of disability apart from tremor. We have also noted that the tremor is typically refractory to medications, including maximally tolerated levodopa. Furthermore, most cases also display a prominent action tremor that impairs eating and writing. Limited reports have alluded to such cases as "benign tremulous PD" or "benign tremulous parkinsonism."1-2 However, previous series have not precisely defined the clinical criteria nor has there been follow-up beyond a few years of parkinsonism, except in rare cases. We report on a series of patients with benign tremulous parkinsonism to better define the characteristics and describe the long-term outcomes. METHODS We identified 116 cases from a retrospective computer search of the medical records database of the Mayo Clinic, Rochester, Minn, spanning a single decade (January 1, 1994, through December 31, 2004), using the text word search terms benign tremulous parkinsonism, benign tremulous Parkinson's disease, tremor predominant parkinsonism, or tremor predominant Parkinson's disease. The medical records of all 116 cases were reviewed to exclude any case that, by the time of last evaluation, had not had at least an 8-year history of parkinsonism. Only cases evaluated and diagnosed by a senior movement disorders specialist (J.E.A. or J.Y.M.) were included. RESULTS INITIAL SYMPTOMS AND PRESENTATION In all but 1 patient the initial symptom was a resting hand tremor; this was unilateral in 13 (confined to the thumb in 1) and bilateral in 2. One patient initially noted a resting foot tremor. At the time of first evaluation, only 2 patients were receiving levodopa treatment, while 10 patients were not using any medication for tremor or PD. The initial examination showed a resting hand tremor in all patients, which was moderate or severe in 12, including the 2 patients taking levodopa. All but 1 patient had rigidity, bradykinetic alternating motor rates, or both at this first examination. All had a normal gait with the exception of reduced arm swing in 14 patients, which was unilateral in 5, and a stooped posture in 3 patients. Patient 16 also had a hypokinetic dysarthria. A postural tremor was present in 13 patients, which persisted yet was attenuated with visually guided movements toward a target in 7. None reported tremor response to alcohol. CLINICAL FINDINGS PRESENT AT LAST FOLLOW-UP The mean follow-up from onset of parkinsonism to time of the last movement disorder evaluation was 11.1 years (range, 8-25 years). Four patients had had more than 12 years of symptoms. In all patients, the symptoms remained relatively unchanged compared with the initial examination with the exception of the resting tremor, which worsened in 6 patients. In patient 3 the bradykinesia and rigidity were mildly improved, which may have resulted from levodopa treatment, as this patient was taking the highest levodopa dose in this group (1200 mg). The gait and balance remained unimpaired. Severity of the postural tremor almost mirrored severity of the rest component (Table 3). With visually guided movement toward a target, a kinetic tremor was also present in 7 patients. A resting chin tremor was noted in 7 patients. Additional symptoms and signs of PD, identified in 8 patients, were mild and included stooped posture, seborrhea, difficulty with fine motor tasks, difficulty turning in bed, sialorrhea, and decreased facial expression. RESPONSE TO TREATMENT Levodopa therapy was not consistently effective in treating the resting tremor or improving other aspects of parkinsonism. Levodopa therapy provided no benefit in 9 of the cases, although only 3 were treated with doses of at least 600 mg daily during the disease course. It provided partial benefit in 3 patients and moderate benefit in 1 (patient 3). The remaining 3 patients received only a single dose of levodopa. Only 2 patients developed levodopa dyskinesias, slight in both; these included facial dyskinesias in one and arm posturing in the other. At the time of last examination, 6 patients were receiving levodopa therapy. The tremor was severe enough that 3 patients underwent thalamic deep brain surgery. All 3 patients were tremor free 1 month after surgery; however, the tremor returned in 1 patient after 1 month. One patient was lost to follow-up 1 month after surgery. The third patient (patient 13) had significant improvement of the action tremor up to 3 years after surgery with continued reprogramming of the stimulator, yet he had persistence and progression of the resting tremor, which became bilateral. FAMILY HISTORIES The family histories were remarkable, with 10 of the 16 patients reporting tremor or PD in at least 1 immediate family member. This included PD in the immediate family in 4 patients, tremor in 5, and both PD and tremor in 1. More than 1 immediate family member was affected in 6 cases (2 cases with PD in both 1 sibling and 1 parent; 4 cases with tremor in 2-4 immediate relatives). None of the family members was examined to confirm these observations. OTHER FINDINGS Dysautonomia, by report, was minimal to absent, with constipation recorded in 3 patients, erectile dysfunction in 2, and bladder dysfunction in 1. No patients developed cognitive impairment. COMMENT Following the lead of other authors, we have been recognizing patients with benign tremulous parkinsonism during the past 12 years.1 Although this syndrome does not appear to be rare, we are aware of no reports that have defined the clinical criteria or the long-term course for this diagnosis. We therefore report on our 16 patients with this diagnosis, who had symptoms for at least 8 years, to help better define consistent features and outcomes. We discourage the reader from trying to infer any prevalence data from this article. When patients were initially examined early in the course of the disease, the clinical picture was consistent with PD,4 and that was typically the initial diagnosis. Subtle clues suggesting benign tremulous parkinsonism were a predominance of resting tremor and prominent postural-action tremor (in almost all cases), with other signs of PD being present but very mild. Benign tremulous parkinsonism became more apparent, however, after several years when the tremor remained the primary problem, with minimal progression of other aspects of parkinsonism. Also suggesting this condition was the absence of a satisfactory response to levodopa therapy in most patients in whom it was administered. This condition was clearly recognizable after many years (we arbitrarily selected >8 years), by which time patients with typical PD are experiencing myriad problems, including gait difficulties, which our patients were spared. Moreover, substantial levodopa complications were not part of the clinical picture. A significant number of the patients also reported a family history of tremor and/or PD, which may be a clue to the diagnosis of benign tremulous parkinsonism if confirmed in other series. Neurodegenerative diseases are heterogeneous and display a variety of rates of progression. Could this simply represent one end of the bell-shaped curve of typical PD? Although we have no current means to be certain, as we did not have data from pathological studies, the near absence of progression apart from tremor and inconsistent response to levodopa therapy suggest that benign tremulous parkinsonism may represent a unique disorder. The benign tremulous parkinsonism syndrome must be differentiated from other somewhat similar yet distinct tremor syndromes. The monosymptomatic resting tremor is defined by a pure or predominant resting tremor without signs of bradykinesia, rigidity, or problems with stance stability sufficient to be diagnosed as PD.5 The combined resting-postural tremor syndrome described by Koller and Rubino6 and essential tremor with isolated resting tremor7 are also different, as these syndromes are not associated with PD signs apart from resting tremor. Isolated tremor with very mild parkinsonian signs related to aging8 is a somewhat nonspecific syndrome but also differs from the disorder in our patients, as the mean age at onset of our cohort was only 58 years. Parkinson disease initially manifesting as essential tremor9-10 is defined by initial presentation with a postural tremor or head tremor, with the subsequent development of signs and symptoms consistent with PD. In none of our patients did postural tremor predate resting tremor. Combined essential tremor with PD11 is unlikely because of the lack of progression of parkinsonism. "Tremor-predominant PD"12 is a very broad category that, as originally defined, required only tremor greater than gait-posture deficits. Thus, in the article by Jankovic et al,12 more than half of the DATATOP (Deprenyl and Tocopherol Antioxidative Therapy of Parkinsonism) cohort of 800 patients fit the criteria for tremor-predominant PD. A striking finding in our cases was the family history. An immediate family member with either tremor or PD was reported by 10 (63%) of our 16 patients, including 5 (31%) with PD. This contrasts with recent epidemiologic studies in which approximately 10% to 16% of patients with classic PD reported a history of PD in an immediate family member.13-15 Similarly, a family history of essential tremor has been reported to occur in 3% to 17% of patients with PD,16-17 contrasting with 38% in our cohort. This raises speculation that this disorder might have more substantial genetic underpinnings than typical PD. The neuropathology of benign tremulous parkinsonism, and specifically whether it is similar to classic PD, is unknown. Previous striatal dopaminergic imaging studies, however, have identified reduced uptake, typical of PD in patients with this phenotype.1-2 Despite this similarity, identification of these patients in clinical neuroprotective therapy trials may be important, since disproportionate distribution of these patients in treatment groups will distort the outcomes. The term benign tremulous parkinsonism, as originally used, highlights the course of the syndrome and is not necessarily inappropriate. However, the tremor can be very disabling, such that 3 of our patients opted for surgical intervention for their tremor. AUTHOR INFORMATION Correspondence: Keith A. Josephs, MST, MD, Department of Neurology, Mayo Clinic, Rochester, MN 55902 (josephs.keith@mayo.edu ). Accepted for Publication: June 24, 2005. Author Contributions: Study concept and design: Josephs and Ahlskog. Acquisition of data: Josephs, Matsumoto, and Ahlskog. Analysis and interpretation of data: Josephs and Ahlskog. Drafting of the manuscript: Josephs and Ahlskog. Critical revision of the manuscript for important intellectual content: Matsumoto and Ahlskog. Administrative, technical, and material support: Josephs. Study supervision: Matsumoto. Funding/Support: This study was supported by grant P01 NS40256-06 from the National Institute of Neurological Disorders and Stroke, Bethesda, Md. Author Affiliations: Division of Movement Disorders, Department of Neurology, Mayo Clinic, Rochester, Minn. REFERENCES 1. Brooks DJ, Playford ED, Ibanez V, et al. Isolated tremor and disruption of the nigrostriatal dopaminergic system: an 18F-dopa PET study. Neurology. 1992;42:1554-1560. FREE FULL TEXT 2. Marshall V, Grosset DG. Role of dopamine transporter imaging in the diagnosis of atypical tremor disorders. Mov Disord. 2003;18:S22-S27. PUBMED 3. Department of Neurology. Mayo Clinic. Clinical Examinations in Neurology. St Louis, Mo: CV Mosby Co; 1998. 4. Hughes AJ, Daniel SE, Blankson S. A clinicopathological study of 100 cases of Parkinson's disease. Arch Neurol. 1993;50:140-148. ABSTRACT 5. Deuschl G, Bain P, Brin M, Ad Hoc Scientific Committee. Consensus statement of the Movement Disorder Society on Tremor. Mov Disord. 1998;13(suppl 3):2-23. PUBMED 6. Koller WC, Rubino FA. Combined resting-postural tremors. Arch Neurol. 1985;42:683-684. ABSTRACT 7. Louis ED, Jurewicz EC. Olfaction in essential tremor patients with and without isolated rest tremor. Mov Disord. 2003;18:1387-1389. FULL TEXT | ISI | PUBMED 8. Lee MS, Kim YD, Im JH, Kim HJ, Rinne JO, Bhatia KP. 123I-IPT brain SPECT study in essential tremor and Parkinson's disease. Neurology. 1999;52:1422-1426. FREE FULL TEXT 9. Chaudhuri KR, Buxton-Thomas M, Dhawan V, Peng R, Meilak C, Brooks DJ. Long duration asymmetrical postural tremor is likely to predict development of Parkinson's disease and not essential tremor: clinical follow up study of 13 cases. J Neurol Neurosurg Psychiatry. 2005;76:115-117. FREE FULL TEXT 10. Geraghty JJ, Jankovic J, Zetusky WJ. Association between essential tremor and Parkinson's disease. Ann Neurol. 1985;17:329-333. FULL TEXT | ISI | PUBMED 11. Lance JW, Schwab RS, Petersen EA. Action tremor and the cogwheel phenomenon in Parkinson's disease. Brain. 1963;86:95-110. FREE FULL TEXT 12. Jankovic J, McDermott M, Carter J, et al. Variable expression of Parkinson's disease: a base-line analysis of the DATATOP cohort. Neurology. 1990;40:1529-1534. FREE FULL TEXT 13. Payami H, Larsen K, Bernard S, Nutt J. Increased risk of Parkinson's disease in parents and siblings of patients. Ann Neurol. 1994;36:659-661. FULL TEXT | ISI | PUBMED 14. Rocca WA, McDonnell SK, Strain KJ, et al. Familial aggregation of Parkinson's disease: the Mayo Clinic family study. Ann Neurol. 2004;56:495-502. FULL TEXT | ISI | PUBMED 15. Elbaz A, Grigoletto F, Baldereschi M, et al, EUROPARKINSON Study Group. Familial aggregation of Parkinson's disease: a population-based case-control study in Europe. Neurology. 1999;52:1876-1882. FREE FULL TEXT 16. Lang AE, Kierans C, Blair RD. Association between familial tremor and Parkinson's disease. Ann Neurol. 1986;19:306-307. ISI | PUBMED 17. Cleeves L, Findley LJ, Koller W. Lack of association between essential tremor and Parkinson's disease: lack of association between essential tremor and Parkinson's disease. Ann Neurol. 1988;24:23-26. FULL TEXT | ISI | PUBMED |
Familiar --But ....
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After 4.75 years with primarily a tremor but no meds and being on an upswing lately I am not contemplating taking anything in the near future. Names for my version of PD are nice but really doesn't have any meaning unless it means different treatment or the not very likely fix. Interesting and please post if you find more. thanks Carey ,,,,, ken |
wow
Carey,
I don't think I fit the profile (I wish I did ...) , but this is very good news for many who do -- you included of course. :hug: There is always something new to learn about this disease, isn't there? Good luck to you and all. |
Carey, so how do you feel about this change in dx?
:) |
Change
I am not sure whether it should be regarded as a change in diagnosis.Surely it is just one of the many ways P.D can present itself.
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a long and detailed response
ECD – you are correct; it is still Parkinson’s – it now has a more precise definition and potential prognosis for me. I believe it is one of many distinct variations of PD that currently lie within the term “Parkinson’s.” Many researchers and clinicians are beginning to view PD as a syndrome, or a collection of syndromes, rather than a disease.
Carolyn - to be clear, my doctor’s conclusion was not pronounced in a grand way, nor as saying that I no longer have PD. It came after I had been examined, and as a result of an extended discussion we had about my activities as a patient advocate for the Parkinson’s Action Network and the Parkinson’s Disease Foundation, and how that dovetailed with the clinical work she has been doing, and the conclusions she has reached since leaving Seattle several years ago. She firmly believes PD is a collection of syndromes, and that the only way patients can be treated effectively is if each person who walks through her door is seen as an individual, completely free of any pre-packaged version of Parkinson’s. She also is a strong promoter and practitioner of the team approach to PD care. (See her article in this summer’s issue of the PDF newsletter http://www.pdf.org/Publications/news...m_approach.cfm) In our conversation I confessed that I had expected to be dead, or at least very physically disabled, eight years after diagnosis. I told her I had never imagined life after my daughter left for college (which she did just last week), that I had been unable to plan anything in the future. But, here I was, feeling better than I had felt in a long time. Where was the dyskinesia? Why did I not experience on/offs? How come my voice was still strong, my gait even, my balance intact, my movement unfrozen? When would the other shoe drop? She replied that maybe, in fact probably, it never would. She had the benefit of not seeing me as a patient for several years; she could see little if any change in how I look and behave. I had history on my side – eight official years of little to no progression apart from tremor. She just kind of casually mentioned there was research identifying at least one distinct PD syndrome called “Benign Tremulous Parkinsonism” that seemed to fit my situation, which she then described. Pressed further by me asking, “does this mean I can plan to be active for some time into the future?” she said, “yes – probably at least for two more decades, as far as your PD is concerned.” I said, “wow! Does this mean I can go back to school or get a job?” She laughed and said, “Carey, you’re way too intense! Keep the stress at a minimum – maybe start with one class – but not that PhD or trying to keep a job!” She then said she didn’t want to change any of my meds, but I was to exercise, exercise, exercise, maybe add meditation practice and yoga or tai chi for stress reduction, and get back into therapy for depression. I'll see her again in two months along with the occupational therapist. So that’s about it. I am cautiously optimistic. Other notes: Besides its importance to the care and treatment of patients, another reason to pay attention to the emergence of PD syndromes is the impact on clinical trials. The study states: “identification of these patients in clinical neuroprotective therapy trials may be important, since disproportionate distribution of these patients in treatment groups will distort the outcomes.” The study’s claim to have identified a distinct syndrome leaves some wiggle room. “Neurodegenerative diseases are heterogeneous and display a variety of rates of progression. Could this simply represent one end of the bell-shaped curve of typical PD? Although we have no current means to be certain, as we did not have data from pathological studies, the near absence of progression apart from tremor and inconsistent response to levodopa therapy suggest that benign tremulous parkinsonism may represent a unique disorder.” -------------------------------------------------------------------- For those who want to know more, below is a breakdown of why I think the term Benign Tremulous Parkinsonism correctly describes my experience with PD. The study states “Benign tremulous parkinsonism may be a distinct clinical entity characterized by tremor predominance plus minimal progression of other aspects of parkinsonism. The tremor is often not very responsive to levodopa therapy. In this series, most patients had immediate family members with a diagnosis of tremor or PD. . . . What has distinguished them has been a consistent constellation of clinical features: (1) prominent resting tremor that is the first or among the first signs and that persistently overshadows other aspects of parkinsonism throughout the course; (2) non tremor components of parkinsonism that remain mild; (3) absence of gait disorder apart from reduced arm swing or mild stooping; (4) no more than mild progression, except for tremor, despite at least 8 years of parkinsonism; and (5) absence of disability apart from tremor.” Longevity and progression: I had a tremor in my right hand for about 5-6 years before I sought a diagnosis (it was only when others noticed it that I decided to seek a medical opinion). The diagnosis of PD was in April, 1999 when I was 41. Eight years later, my symptoms have very slowly progressed, if at all, other than tremor. I have lived at least 13-14 years with a right hand resting tremor. Symptoms: I presented with right hand resting tremor; this is the only symptom that has shown significant change – I now also have a left hand tremor (resting and action, which hinders my writing); it’s not as bad as on the right. So far, I do not have tremor in my lower extremities or any other part of my body. I also presented with the absence of an arm swing on the right side, a symptom of which I was unaware, but was immediately discerned by my neurologist. My writing had begun to deteriorate, and is now illegible. I have mild, non-progressive bradykinesia that responds well to levadopa; I have no balance problems; I occasionally drag my right foot, otherwise I have no gait problems; I have stooped posture, but have always slumped – never have had good posture. My fine motor skills are impaired; gross motor are not. I have had no change to my voice. The study does not address depression, but I have suffered from, and been treated for depression throughout. I exhibit no decline in cognition – although stress can adversely affect certain cognitive functions for short periods of time (concentration, word retrieval, etc). My memory, long and short term, remains strong. Medications: I was on agonists alone for 6 years; they controlled my symptoms fairly well (not my tremor), but caused significant problems of daytime sleepiness, certain obsessive-compulsive behaviors, and foggy thinking, so I stopped and began taking levadopa two years ago. It improves my overall movement, but has no impact on my tremor. I started amantadine a year ago, and it controls my tremor. I take Effexor for depression and anxiety. Family history: No Parkinson’s or other neurological disorders. However, my father has had a hand tremor for years; never progressed or been diagnosed (I can’t think of a time he has ever been to a doctor). He is an active 73 years old. Disability: I stopped working full time in August, 2002. I had a horrible situation at my job at the University of Washington after 18 years of wonderful employment there. The situation led to severe stress, major depression, and nervous breakdown – all mental and emotional symptoms that went untreated for several months before I dragged myself back into the doctor. I was placed on immediate disability leave and began intensive physical, occupational, and psychological interventions. My physical symptoms were exacerbated by the stress I was under; conversely, the Parkinson’s made it harder for me to deal with problems at work, and impaired my usual resiliency. It was difficult to recover, and that, combined with University politics, led to my layoff six months later (I sued the UW a year later and was successful in obtaining a modest out of court settlement). Trying to start over was virtually impossible for me (my divorce, initiated by me, was finalized at the same time I was losing my job). I opted to apply for SSDI, which was granted two years ago. I am only now regaining a sense of control over my life. Bottom line on disability: depression and stress, not physical symptoms, were, and continue to be, mainly responsible for my disability. Tremor interferes with my ability to write and type, further contributing to disability. |
A am in the dark.
I do not know what average progression is.How well are most people after 3 years,6 years,9 years etc. As P.D does seem to be a syndrome it is difficult to get an answer.My husband has had a tremor in his right(parkinsons side) foot when excited or annoyed for over 25 years.Was this Parkinson's?How do we judge his progression rate,how do we judge each one of us?Is there a norm against which we can judge ourselves?
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Carey
Thank you for posting this. I just left a rant on Tena's thread about how I think I ended up with a pD diagnosis in the first place...but that's another story. I learned that the people who were my parents were likely not my biological parents (truth is stranger than fiction) and so I have no reliable family history to look at. I am tremor dominant and my tremor responds well to a heavy dose of sinemet. But I have no other signifigant signs of PD. My tremor has gotten worse over time and is hugely affected by my blood sugar levels and no amount of complaining to neuro or family doctor gets me anywhere. My A1C is under 6. The magic number in Canada for Diabetes.
The latest "odd" manifestation of my disease is that I wake up in the morning tremor free and can read, or knit or whatever, as long as I don't stand up or move around much I am fine. I take 50/200 + .5 mirapex, and as soon as that stuff hits my brain It kicks in my tremor in my legs. As soon as that starts I have to get out of bed and start moving around or it escalates into an unmanageable tremor and I'm stuck for who knows how long. I am frustrated and angry because so far nobody can explain why this happens. When I'm on, I am fully functional. People who don't know I have PD can't tell there is anything wrong with me. My handwriting is still very good, I get compliments on it regularly. I'm reasonably cognizant (??) but sitting at a computer or other "no movement" activities cause my meds to get quite unreliable. My treadmill is my best friend. My neuro has all sorts of ideas about stretching out my sinemet, but we can't get my body to budge off its two hour schedule. I rarely have dyskinesia unless I seriously overdose...I could go on, but I bore the hell out of myself. .. so do I fit this criteria too? To say PDs a strange disease is a gross understatement! |
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Even what my doctor told me, that I could plan on another couple of decades of being active with PD, was an educated guess - there's no long term documentation. But there was no reason for her to believe that I would begin to progress at a different rate than I have for the 14 years since my tremor first appeared. Nor did she think that I would begin to acquire other symptoms after living so long without them. Seems to me, the designation of Benign Tremulous Parkinsonism can only be made several years after initial PD dx, as longevity of tremor without other symptoms is part of the definition. It's nothing I could have known or prepared for as I started on the PD journey. Believing at the outset that "maybe I'll only have a really bad tremor and never experience many of the other symptoms that are PD" would have seemed unrealistic if not downright nuts. I think my doctor has the right idea - labels are not as important as treating the symptoms of each individual person who walks through the door. There's a more nuanced approach to treating PD than just throwing more sinemet at it. The lesson for me is more psychological - start living my life without the fear of losing my life to things that haven't even happened yet, and might not ever happen. It's hard for me to do. |
I have been trying to get the message out for years
I was given a genetic test of my Parkin gene and had 2 mutations, which is extremely rare. Young onset Parkinson patients have a 50% chance of having one mutation which helps to confirm the diagnosis. In Europe what I have is called Parkin Disease. It is different than idiopathic parkinson disease. It starts with weakness on one side as well as tremor. As it progresses, the tremor disappears, the progression is slower than idiopathic Parkinson's and dystonia is a more debilitating symptom than tremors. In the more advanced state of the disease, the autonomous system becomes affected causing problem with blood pressure, heart, swallowing and lungs. DBS surgery is the best option to treat this form of Parkinson's disease.
I have listed several studies to back this up. Folks, just learn as much as possible about your illness. Do what you feel is the best to treat your symptoms. If you disagree with a neurologist bring as many studies you have found online that apply to your situation to direct the neurologist observation on you as an individual. They have become accustumed to treat diseases; not patients. If you want to be treated as a patient, educate yourself by searching for studies that apply to your symptoms. Learn of the latest technology which can rule out or confirm your symptoms and ask your neurologist to order them. Be an active member of your medical team and not passive. When you understand your disease, and part of the nature of your disease is neither you nor your neurologist can, with today's technology, improve or control your symptoms, accept it and find other things to focus on in your life. God laughs loudest at man's folly when making plans. The hardest thing in the world is to admit to yourself you have no control of your physical wellbeing. After this lesson is learned, you once more learn to enjoy life within the limitations and stop working so hard to control your world and those around you. It has taken me 53 years to reach this point. I get unhappy and lonely with the emotional rollar coaster of living with illness, and how hard it is to retain friendships when you are up for a couple weeks, and then have a setback and find yourself striking out in anger at friends. Heck, the disease leaves ourselves loving our lives one day and hating ourselves the next. I hate my inconsistent behaivior, but must accept it as well as be understanding of the difficulties it places on relationships. But I never was happy with who or what I was before my diagnosis. Peace to you all, Vicky |
Thanks Vicky for posting that and Carey that's good news!
Vicky do you or anyone else reading this know much about the autonomic nervous system in what I've been told in my case is idiopathic young onset P.D.? I have a very low pulse rate and perspire profusely. The sweating is annoying and a tad embarrassing but what I'd like to know more about is the pulse rate being so low, usually around 40 - 50. Does anyone know if it will get lower as I reach middle age, the consequences etc? Thanks, Lee |
Autonomous System
Dear Lee,
I would reccomend bring that up with your physician. I also have a problem with sweating, but I have learned to live with it. Low pulse rate could be caused by many different factors, such as cardiovascular exercise. Again consult your physician. Neurologist are only now beginning to take patients seriously when they complain of autonomous problems. Autonomous problems usually do not show up until well advanced into the disease. I complained to my neurologist several times that I could not breathe. I was brought to the hospital several times, they would check my heart and exray my lungs, then send me home with a nebulizer. Not one neurologist, not even the one on call, would come in to see me. One told the emergency physician on the phone that Parkinson's had nothing to do with breathing difficulties. This was the same physician who diagnosed me with delayed stress syndrome after evaluating me for DBS surgery. He has yet to acknowledge his error in diagnosis even after I prooved the Parkinsons Diagnosis with the gene test and a modertly sever result of a FDOPA Pet scan done at Mount Zion in New York. My son, a paramedic, watched as I tried to force myself to keep breathing while we waited for the paramedics to arrive. He swears only my diaphram muscles (strong from playing woodwind instruments) was moving, he saw no movement in the upper chest walls. He also described my breathing as having what is known as a death rattle. His crying and telling me to keep breathing was all that got me through it. Still the neurologist refused to take it seriously and stated to the emergency room physicians the Parkinson's did not cause breathing problems. A few months later I went into a malignant syndrome because of overinjestion of drugs to control my symptoms, not the disease. I was thrashing around uncontrolably, screaming for heip. The emergency room physicians were great. They finally, after watching me go through this for 19 hours, got a neurologist to come in to see me. I was put into a coma to give my body time to detoxify. During the 19 hours of waiting for the neurologist to come, my kidneys began to shut down because it was unable to handle the protein from my constant movements. They had to try 3 times to bring me out of the coma. Afterwords the neurologist came to see me when I was moved to a regular room from ICU. Her diagnosis, Anxiety. She blamed me for not controlling myself and allowing myself to get upset and bringing the disease on myself. When I saw my regular neurologist for my next appointment, she also pulled out the anxiety diagnosis, but I was ready for her and brought several copies of studies proving the involvement of the autonomous system and papers written by Emergency room physicians of difficulty getting help from Neurological staff who refused to acknowledge symptoms as Parkinson's related. When my neurologist attempted to refuse me the opportunity to have DBS, she told me no. She said based on her experience with patients, I fell into the category that would more than likely commit suicide after surgery. I told her she may have seen many patients, but she had no idea what or who I was. I refused to let her slot me as a type of patient, and make her see me. She said that she would allow me to have the surgery, but it was against her better judgement basically putting the responsibility for the outcome straight on me. I had the surgery and it has been a miracle for me. People who see me say they are amazed at how much better I am. Lee, one suggestion. At your next appointment, ask the nurse to take your blood pressure both sitting and standing. If there is a big drop when you stand or you feel dizzy, you may be having autonomous problems, and others possible reasons should be ruled out before your neurologist can be convinced of this possibility. Good Luck, Vicky |
Benign Tremulous Parkinson's Disease
This has been such an interesting thread that I have suggest PwP on the P.D.S forum read it.I hope nobody minds.
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Benign tremulous Parkinson's disease
I was diagnosed with PD in November 1996 at 40 years of age. A resting tremor of my left hand and armr are really my only symptoms, I drag my left foot a bit too. I have had little progression in 14 years. I recently vsited Mayo Clinic in Rochester and was re-diagnosed with Benign tremulous Parkinson's disease. The only posts I can find are old, does anyone else share the diagnosis or know "indigogo" is still active in this blog? Curious!
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Carey,
What can I say? After being diagnosed for ten years I have only just given up on the notion that what I have is not really PD. I had finally decided I was dilusional and of course had it. NOW THIS? Even if it is only a very slight change in diagnosis, it sounds like it means a release from the posibility of rapid decline over the next few years (which I have been expecting as retribution for the slow progression over the last ten.) Honestly, as I read the piece I could feel the denial returning,(something I don't necessarily consider a bad thing.) By this evening I will be in full blown denial and dreaming of adventure vacations. Thanks Carey, even though I don't exactly fit I taking it on anyway. At least there are possibilities. Pam |
Holy Cow
I am so slow, I only just realized the original post is almost three years old. Carey does it still stand with you?
I think I'm going to adopt it anyway. I just love denial. Thanks again anyway. |
I've never received an official dx as having Benign Tremulous Parkinsonism, but almost 3 years later, I still have not progressed much further. My toes cramp a little more, but I am taking less medication just because I'm lazy. I wonder if some of my symptoms might be side effects; I know I feel better when I'm physically active, but am lazy that way too. Depression and anxiety continue to plague me and are my greatest obstacles to having a high quality of life.
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Any further research on this?
I'm just 3 years into the the PD journey but have not progressed at all that I can discern. I'm beginning to hopefully wonder if Benign Tremulous Parkinsonism may apply to me. I'm only taking Azilect and have done so since my dx in 2009. Is anyone aware of further research on the topic? Thanks!
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I am wondering what Datscan results would look like in "Benign Tremulous Parkinsonism." I have had a resting tremor for 7 years. (I take only Azilect). Have some minor right side stiffness which may or may not be PD-related. My MDS was stumped until we got the Datscan results. Clearly PD.
Why didn't the study on "Benign Tremulous Parkinsonism" perform a Datscan on those patients? The test was available in Europe a decade ago. Parkinson's disease appears to be an umbrella term for a myriad of disorders with different root causes, mostly genetic. |
PD or BTP
I was in denial in my first years of PD. I lasted 5 years with just a mild tremor generally caused by a bout of stress During those first 5 years I managed a large chemical factory, making organic peroxides which are very inflammable and hazardous. You don't get more stressful than that. They were used by the London bombers. I did not progress at all during this 5 year period. I retired at 60, and went on endless world holidays during the 15 years since I retired. I have progressed relatively slowly. My tremor has almost gone but my PD symptoms have progressed steadily. Main difficulty is walking. I also perspire a lot, and have difficulty swallowing. I have just given up driving in spite of feeling confident when behind the steering wheel. Since I was diagnosed 20 years ago, I was prescribed Sinemet from day 1, which was far too early. This has now given me raging dyskinesia, and writhe for hours on even a half stavelo.
So if you are below 5 years since diagnosis, be careful not to get too excited, believing you have BTP. it can be PD and you are in what some call he honeymoon period" of up to 5 years. I hope however for peoplr like littlesky that it is BTP not PD. I wonder what the ratio of BTP is out of total cases? Ron |
PD or BTP
Ronhutton, I will take your wise advice, keep with the healthy lifestyle, take my Azilect (for whatever it's worth) and try not to get excited. I asked my MDS and she, at a prominent MD clinic, hadn't seen the 2006 Mayo Clinic study. The one observation she made worth sharing is that one's rate of progression will remain constant. So if slow or almost nonexistent from the start, that is what you can expect. No sudden kick into high gear. She said that those of us with apparent BTP drew a lucky number in the PD lottery.
I asked about doing the Datascan. She said that was my choice, that insurance would cover, but that the results wouldn't alter treatment recommendations. |
respectfully disagree
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I don't mean to scare you at all, and know this is not something anyone wants to hear. But I share it because it's true. Some stresses you cannot avoid, like death of a beloved family member or very close friend, we had several of those just right on top of each other, and I could definitely see the effect on PD, and it's permanent. Other stresses you really need to avoid like the plague, because that is what they are. They will take you down. Tell your friends and family why you cannot do this or that, and they should understand. If they don't or won't, too bad-it's your life! We have had to learn to be able to say "we're just not up for that", "today is not working out to be a good day", "meds are not working today" (this one, unfortunately, is very common for us, we could say it every day), you get the idea. PD varies day to day and for us, hour to hour. Your circle of people needs to understand this. If they can't, get a new circle...we have found that even having to repeatedly explain that we can't do something and why, to people who already well know, is very stressful in itself. I think PD actually makes people more susceptible to stress in the first place-things that may not have stressed you out before dx now do, and in a much more pronounced way than they ever would have pre-pd. It's vicious. You are wise to exercise, also key. You may have also read about how important humor is, get some good belly-busting laughs in every day (check out the radio or netflix/equivalent for the stand-up comedian channels, cheap and very worth it). I would also add that if you don't have an animal in your life, get one. Heck, get two :) If an animal is not allowed where you live, or you feel you cannot take on that responsiblity, check out surrogate or foster programs in your area. Or just go to the animal shelter and volunteer if you are able. There is a reason nursing homes have animals brought in for their residents to pet, hold, and love on a regular basis. |
I wonder about me...
At the NIH, the neurologist said I had the "slowest progressing form of PD." So I wonder if this puts me in the BTP category? I don't see the Dr Lungu each visit, but I suspect I'll see him by the last trip (May) at least. I'll be sure to bring the BTP info with me each of these final 2 trips.
I go to the NIH again this week and May is my last trip for the Kinetics clinical trial. I'll be glad to have it over -- monthly 5 hour plane rides to and from the NIH are no laughing matter! :eek: just found this article: archneur.ama-assn.org/cgi/reprint/63/3/354.pdf I don't think I have it - but the article above describes it pretty well. Jean PS I have had SPECT scans (like DATSCAN) that show I have PD) |
PD or BTP
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For now, I prefer denial. Along with exercise, meditation, and lots of veggies. |
littlesky ...
I'm with you on the vegetables however my Neuro maintains that the rate of progression is pretty constant and while stress etc may magnify the symptoms while you are experiencing the stress, it will not make the underlying disease move any faster.
Interesting to know if this constant progression theory can be confirmed by the real world !! It seems to be so in my case over the last 9 years since dx. Neil. |
Update
I went to see a neuro yesterday - the first time in more than a year. I saw the new MDS at the Booth Gardner Clinic in Kirkland, WA because I wanted a fresh pair of eyes to look at my old file that has collected for 13 years in the same place.
He examined me thoroughly and talked to me at length, and determined that I was progressing as slowly as before, and that there was no reason to believe that the rate should increase in the future. (I imagine that any great stressor to my life might give my PD a negative jolt - but that same stressor would probably negatively impact my health even without PD). He did not suggest I change my current drug regimen, which is one carb25/levo100 3 times a day; one amantadine 3 times a day; one- 1mg requip twice a day. We doubled my antidepressant from one - 20mg celexa a day to one 40mg celexa (20mg was the lowest dose possible). I told him that I usually skipped the middle dose of cd/ld and amantadine if I was at home all day with nothing on my schedule that required I be on my best physical game. I said I was worried about the side effects, mainly dyskinesia, from too much dopamine. He said the only damage I do to my health is in quality of life - but if I can get away with it, then less cd/ld was a good thing over the long haul. We didn't discuss "Benign Tremulous Parkinsonism", but I am less interested in labels than the actual state of my disease/symptoms. My most troublesome one is tremor in my hands, which can become very violent and tiresome and interferes with anything I do with my hands like eating, food preparation, dressing, and typing. I sweat a lot. My nighttime dose of medication is actually more important than my morning dose because I cannot sleep at all with the tremor or my body as rigid as it can become. I need the dopamine to relax and the tremor to be calmed if I am to sleep at all. I have not developed any gait or balance problems (knock on wood!). I am only 54; have had a PD diagnosis for 13 years, and was symptomatic for 6 years before that. I hope to have many more years ahead of me - the hard part is preserving my mobility over a long period of time. If I had been 60 at diagnosis, then I think I would view my medication management differently. While a slow course of progression is largely a good prognosis, it remains difficult to know how to manage well for the long term - and I don't think many medical professionals have a good idea either. It's all a big experiment influenced by our own individual situations. |
I saw my movement disorder specialist yesterday and, for the first time, she said I had benign tremulous parkinsonism. I have had the DATscan and definitely do have PD. I have had tremor for 7 years and it is slowly but steadily worsening. Now 52. Some stiffness in shoulders and arms but she couldn't see it and who knows if it is just age or PD. I asked when she thought I would start major meds (besides Azilect), 2 years, 5 years? She didn't know but seemed to think I would need something eventually.
She asked me to donate some cells for Parkinson's stem cell research, so I went and had a skin biopsy. Now I feel a bit more optimistic than usual and very virtuous. A good day. |
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