First Symptom Survey Comment by Strelley

Again, thank you everyone for replying to my survey. I trust you have read the experience of others and, like me, found them to be more than just clinical facts, but touching and emotional journeys that link us together on this site.

This was not a "scientific clinical survey", but it has served as a general guide in one of my little investigations. Hope the next bit is not too clinical.

Although we all know PSP can only have a definitive diagnosis at autopsy, I have assumed all respondents have PSP (and no other co-morbidity exists that involves any form of dementia, like Alzheimer's).

Changes in personality, mood, behaviour and thinking (slowness of thought), along with dysexecutive syndrome, have been noted as symptoms in most literature on PSP. It has often been given an umbrella title of subcortical dementia (this can include changes in emotion due to damage in the basal ganglia and midbrain areas). However, a scientific paper written in Australia in 2011, found that there was far more cortical atrophy in PSP, affecting more frontal lobe regions, than had previously been identified.

This meant the possibility of more personality/behavioural type changes.

In our little survey it seems that some form of personality/behavioural change was one of the first symptoms in about 56% of sufferers. While we cannot apply any meaningful statistics to these anecdotal results, it may mean that clinicians should be more aware of such symptoms. There is a tendency to highlight the motor problems first (balance, gait, posture and falls). As seen from our survey, these do occur first in many sufferers, and thus often give rise to an initial diagnosis of idiopathic Parkinson's, or other motor/muscle disease (with mostly ineffective and thus, unnecessary medications). Additionally, those clinicians that simply see personality and thinking changes first, have been known to diagnose as Alzheimers (or depression).

I hope I'm not being unfair to our medical doctors, but observations from literature about the time it takes to diagnose this class of diseases (as difficult as they may be) probably means that more clinician education is required (especially GP's who are usually our first port of call). As we live longer, these type of diseases will appear more abundantly. PSP is already under diagnosed, and there may be 4-6 times more sufferers at this current time who have a wrong diagnosis.

(We know an early diagnosis will not prevent its natural unremitting progression, but I'm sure carers and sufferers alike want the certainty of a diagnosis as early as possible).

[dorothy-thompson]

Dear Strelley

I completely agree with your summing up and having the diagnosis at a much early stage would save such a lot of heart ache for the sufferer and the carer alike.

I also agree that as more and more of us are living longer, there has to be more research into the function of the brain and why things go wrong. What puzzles me is that we know that PSP is relatively rare, knowing that, seeing a neurologists can be no more than every 6-9 months and the changes in the course of the disease can be immense in that time, there should be more intervention. But money and clinical inclination is the problem.

Thank you for an interesting survey.

Dorothy thompson

[Hidden in reply to dorothy-thompson]

I agree with you so much and I am disappointed that the neurological team do not show enough interest in the patients given the apparent rarity of psp . You would think that the information would be useful to the future diagnosis. I wonder if they just don't like the idea of committing themselves because of the inability to use concrete tests as yet?

[shasha]

HELLO STRELLY - I TOO AGREE THAT THERE SHOULD BE MORE EDUCATION OF OUR CLINICIANS (? ) BUT THE WHOLE THING IS THAT PEOPLE WHO ARE SUFFERING WITH THIS VILE DESEASE ARE STILL LEFT WONDERING WHAT THEY HAVE ..# LIKE ME I WAS ON DRUGS FIR PD FOR NEARLY 3 YEARS . THE FIRST TIME I QUESTIONED MY DX WAS AFTER ABUT 6/9 MONTHS - THEN AGAIN AFTER ANOTHER 6 MONTHS AS I KEPT BEING GIVEN DIFFERENT DRUGS AND HAD TI WAIT TO SEE IF THEY HAD ANY EFFECT - SO I THINK THAT THE EDUCATION OF NEUROLOGISTS AND GP'S IS VITAL

[CarlaL48]

Thanks for all your efforts, Strelley. Interesting conclusions.

I agree that education within the medical community ought to be a high priority. In my experience, however, it's not just ignorance about PSP that hinders diagnosis, there's a downright resistance to learning. We've had both an internist and a neurologist essentially ask, "What difference does it make?" In their opinion, they want to treat the symptom, whether it emanates from PD, PSP, Alzheimer's, etc.

I don't know if they're lazy or if they've reached the level of their incompetence (wasn't that the "Peter principle"?), or they truly believe that we shouldn't care about an accurate diagnosis.

As we all know, it's enormously frustrating.

Again, thank you for your work, and please continue sharing your insightful analyses on all things having to do with PSP.

Blessings,

Carla

[cabbagecottage in reply to CarlaL48]

My husband hasn't need diagnosed with PSP YETTTT!! he has Parkinson's ??

I have my doubts because he has been on Sinemet along with a Neuro patch now for five years . The results of taking these are all over the place , very difficult to tell if they are working or not .

I don't think so and never have , but each Time we go they catch hold of his arm bend it and tell him he is doing good .

He is either in his wheelchair, armchair or bed which he is hoisted into .

What do they know

This morning after he took his meds and had his breakfast he switched off jumping and jerking , his head turning with tongue protruding , then grimacing . He has never had the grimace before .

He can be very sensitive to some meds so much so I get afraid to give him any

thing .

Do you think if it is PSP taking the meds would make him worse .

D

[CarlaL48 in reply to cabbagecottage]

Hi, Cabbagepatch

I think Strelley pretty well summed it up. I will add that when my husband Dale was first diagnosed with idiopathic Parkinson's in late 2011, he tried two different agonists on separate occasions: ropinirole and pramipexole. In each case, he didn't take more than a day's dosage. They made him extremely dizzy and took away his appetite. After that, I put my foot down on trying any more agonists.

Also, Dale was immediately put on generic Sinemet. We couldn't tell that it was particularly helpful, so when he saw the new movement disorders specialist in late July, this doctor started weaning him off. Dale should be completely off this medication by November. As of now, Dale's actually feeling a bit better. He actually has the strength to get off his scooter and use his walker for two or three turns down the hallway.

We are both pleased, even though Dale reminds himself that he won't get better -- he'll just be the best he can be at whatever his current plateau is.

I've noticed his speech is getting worse, more garble, the past couple of months. Also, he's had a few rough coughing fits (several days in between thus far). I don't know if this is the start of the swallowing issues. We'll see.

I would suggest that you be very direct with your doctor about your husband's meds. Your doc may want to make some adjustments or wean him off some, too. (Not practicing medicine here; strongly urging you to talk with your neurologist.)

Blessings to you,

Carla XO

[Hidden]

Hi cabbagepatch

I would assume by now that any neurologist would know if his medications are working or not. If not, then it's possible that your hubby does not have classical Parkinson's. Remembering that even when a person has Parkinson's, the effects of such medication will also wear off after many years (and cause dyskinesia - involuntary movements). One diagnostic test to distinguish Parkinson's from PSP is to give such medications (as your husbands) and if they don't work or stop working within a short time (by 1-2 years max) then it is more likely to be PSP (or other similar neurological condition).

I'm probably not the one to answer questions about your hubby's meds because I tend to be cautious about medications for diseases like PSP (CBD).

In my limited understanding it seems to me that the side effects (if any are present) mimic PSP symptoms so that one is hard pressed to know whether it's a side effect or the PSP causing a symptom.

For instance (Sinemet)...Used on a long-term basis, (levodopa) may also cause restlessness, confusion, or abnormal movements (restless muscle movements in your eyes, tongue, jaw, or neck)...etc etc.

For instance "Neuro patches contain the active ingredient rotigotine, which is a type of medicine called a dopamine agonist. Rotigotine works by mimicking the activity of a substance in the brain called dopamine".

Although some may not experience side effects, I find some of them for rotigotine very interesting:

1. This medicine can occasionally cause your blood pressure to drop when you move from a lying down or sitting position to sitting or standing, especially when you first start using the patches. This may make you feel dizzy or unsteady.

2.can cause drowsiness and sleepiness

3.Changes in behaviour have been reported

4.disturbances to your vision

All these are symptoms of PSP, so how will a doctor tell the difference ? (especially if he/she is unsure of the full range of PSP symptoms - many of which have been described in our little survey).

Hope others will help you out with your concerns.

All the best

[cabbagecottage]

My husband was diagnosed with Parkinson's EVENTUALLY at the age of seventy four he is now 79 . Although On reflection think he had been struggling Unknowingly for many years . Apart from that considered himself lucky with his health .

We are now in an unfortunate position .

The consultant who dealt with Parkinson's in the care of the elderly clinic retired last Xmas and the GP who was supporting us moved away . That meant the two people we knew and who knew him best and had been with him from the beginning had gone , we felt very much in Limbo .

They have advertised for a new consultant ?.

We have never had the opportunity to see a Parkinson's nurse .

[cabbagecottage]

He is so changeable his Sinemet has been reduced from Sinemet plus to Sinemet 110 because I thought like you they were rebounding almost , It is a lot of guesswork as far as I can see .

His blood pressure is usually low , the GP told me it usually is with Parkinson's . he cannot stand , partly because Of very painful knee , he has had two knee ops . chicken and egg comes to mind

When I ask he always says he isn't dizzy .v

The drowsy sleepiness has been with him for a long time And he does seem to have most of the symptoms . poor coordination .. His memory all round is excellent he is the one who reminds me lol.

[jimandsharynp in reply to cabbagecottage]

Hi, how is his handwriting? Just curious.

Jimbo

[cabbagecottage in reply to jimandsharynp]

That was one of the first things we noticed along with a struggle walking a painful dropped right shoulder had that for very many years , and no swing . he started to notice a very slight tremor in his right arm . Mouth drop open and a l sided face . I thought he hAd had a stroke or maybe even had a tumour . He just thought it was a stiff neck . .

[khils]

Hi All. This is my first post, and i read with interest. My mother at the age of 63 was diagnosed with PSP in November 2012. We had about 12 months on being told it was "ageing" and then got to see a professor of neurology who straight away spotted the symptoms...speech, slowness of movement, eye movement, unsteadiness diagnosed PSP. Our GP and Neurologist since then have shown great interest in my mother and we have regular appointments and telephone conversations with them both. Infact the GP surgery have taken a real interst as they have only ever seen 1 patient before. Maybe my experiences so far has been fortunate..but i guess what i'm trying to say, is the medical professionals (in my case) have been switched on to PSP and have so far given us 1st rate care of both my mum and me.

I am a young carer (30something!) who up until last month, looked after my mum with a care package, worked full time and tried to maintain normality. I found it hard because although i joined the carers association, most of them there were able to be at home and caring for their husbands / wives /elderly mothers/ fathers. The care package worked well, but it was the time inbetween carers visits that became the problem and after much soul searching and tears from me, my mum and i made the decision to have some respite and to move her into residentail / nursing care. When i returned to collect her she didnt want to come home! However guilty i feel about it, it has been the best decision we have made. As the PSP continues to progress, i have been able to regain that mother/daughter relationship and enjoy the 'good' days which is so special and on the "bad" days she and I have support from all the staff. I jokingly say i now have visiting rights only...and her face beems when i see her everyday! I dont think i could face the future without the wonderful work of our GP practise, the neurologist, and the home and its staff. The future doesnt look great for my mum, but we're living it like a ray on sunshine and making the most of it!

[Hidden in reply to khils]

PSP is never good news, but your experience is indeed a good news story with a reasonably quick diagnosis, focused and interested clinicians, together with a mum who has settled well in a nursing facility.

I'm sure you've read the experience of others, and noted that their journey with physicians, carers and health professionals has not always been a happy one.

Nevertheless, I'm pleased your mum is being cared for in such wonderful way, because it must take some stress out of your very busy life. As you say, the future will be tough, but with your positive approach and help from family, friends and caring staff, I trust you'll continue to face the challenges ahead as you are facing them at present.

All the best.