Hi Strelley Is there anyway of knowing what form of PSP someone has? Eg. PSP RS / PSP P could a neurologist know

[Hidden]

Hello Joey

As you may know, there have been at least 5 clinical subtypes of PSP described - according to clinical features. There is a great overlap of clinical symptoms within the many "atypical Parkinson's" conditions (like PSP, CBD, MSA etc), so it can be hard to know what form of PSP a person is exhibiting, especially in the early stages of the disease. In fact, an early diagnosed PSP sufferer can eventually show some symptoms of other similar conditions (CBD etc) later down the track.

To answer your question, I would expect a neurologist who majors in movement disorders and sees a lot of patients with Parkinsonism, should be able to eventually pick PSP-RS from PSP-P (despite the problem of overlap).

Sorry for the detail but here is "some" of what a neurologist would look for.......

PSP-RS will be identified by a lurching gait and unexplained backward falls, with more rigidity in the neck and shoulders and less in the limbs. They may show retrocollis - neck backwards (but many show antecollis - neck forwards). A cardinal sign will appear within 3 years of symptom onset of vertical gaze palsy (cannot look up or down voluntarily) and other eye problems (like slow saccades). Blepharospasm (eyelid closure) may be present or eyelids stay open. There will probably be signs of apathy, and slowness of thought (hard to find words). There is other frontal cortex and subcortical damage that displays other symptoms (dysexecutive syndrome and psuedobulbar affect like laughing and crying out of place. Plus other personality changes in some sufferers, like aggressiveness). Excessive fatigue will be present. Eventually, there will be slurring of speech and swallowing problems (that becomes excessive towards late stages of the condition, often leading to aspiration pneumonia). PSP-RS does not respond to levodopa medication (and if there is some benefit it ceases within a short time). Tremors are rarely seen in PSP-RS.

PSP-P appears initially more like Parkinson's with slowness of movement and rigidity, often with tremor, and usually symptoms usually start on one side of the body (unlike PSP-RS). They have normal eye movements (unlike PSP-RS) and respond well to levodopa (although later this becomes less effective). These signs will be good indicators of PSP-P rather than PSP-RS.

Many symptoms found in PSP-RS eventually show in PSP-P but much later and often milder for a longer time (this is why it is called "the slow form" of PSP). After 6 or 7 years or so, PSP-P begins to look a lot like PSP-RS..

(On autopsy PSP-P has less severe tau pathology than PSP-RS, that is, the damaging protein seem to be less in the brain areas responsible for the symptoms of the disease).

Hope this helps a bit.

Cheers

T.

[Hidden in reply to Hidden]

I thought I would mention the difficulty neurologists may have when making a differential diagnosis between idiopathic Parkinson's and conditions like PSP.

(Many symptoms that appear in PSP are also seen in Parkinson's).

Can anyone see any of the following symptoms in their PSP sufferers?.......

Idiopathic Parkinson disease (PD) is more than a movement disorder (with just motor damage symptoms). Many PD sufferers have the following nonmotor problems........

(some may be associated/with or exacerbated by their medications)

Apathy/depression/anxiety; impulsive behaviour; impaired sense of smell; visual problems (not the same as PSP); sleepiness; vivid dreams; restless legs; insomnia; orthostatic hypotension; excessive sweating (with cold limbs); swallowing difficulties; drooling; constipation; urinary problems; subcortical dementias (slow thinking and difficulty with problem solving; confusion)

T.

[mthteach in reply to Hidden]

I was told by someone to use coconut oil. Gargle with it. Have you heard of this.

[Hidden in reply to mthteach]

Some people use coconut oil as a mouth wash for bad breath. I don't think it has much therapeutic value when gargled. However, if it helps, it probably won't harm you.

T.

[mthteach in reply to Hidden]

Strelley,

I have ask several times on here about PSP and throat and nose pain. My husband has done the barring swallow, an EDG, and the ENT has run a scope up his nose. The only thing we have is the ENT said that his throat is swollen. He does not know why. My husband is on Senimit for now. He has a neurologist appointment this coming Wednesday for this problem. He is over drugging himself on benedryl, cough drops, etc... Just to stop the pain. Sometimes I look over and his eyes are squinting, and he is breathing little short breaths fast. He said he is hurting. Any advice. Thank you for all that you do for us on here. You are as a light at the end of a never-ending search for us on here.

[Hidden in reply to mthteach]

Hi mthteach

I don't think I can add much to your previous post on this subject (and the answers given). I assume with the raft of tests performed (hopefully including blood tests), the Drs have excluded growths, lesions, spasms and infections (of throat and sinuses). While PSP can cause muscle spasms in the throat and oesophagus, the tests performed would have shown this. If the pain is continuous it is unlikely to be spasm pain. (Most PSP symptoms are related to brain damage causing "muscle and motor" problems).

Since there is evidence of a "swollen" throat, there must be some inflammation present. It is possible to have inflammation without infection. Some forms of pharyngitis can be caused by acid reflux, irritants and even some medications. Perhaps the doctors will suggest a one off steroid dose to see what happens.

While trying all sorts of medications to prevent the pain is a natural response, the cause of the swelling and pain needs to be identified. I think your husband has been taking medication for excess mucus and the compensatory medicine for dry mouth. This may be a problem, and I'm sure the Drs will advice on such an action.

Remember that sinemet has some rarer side effects of shortness of breath and swelling of the mouth region.

One has to consider other conditions apart from PSP when identifying such things as throat and nose pain, accompanied by shortness of breath.

It's a tough one, but I really hope the Drs will find a solution in order to give your husband the pain relief he needs at this time.

All the best

T.

[Hidden]

Thank you strelley you are such a fantastic person to have on board with all your knowledge

[snapper]

Thanks for your explanation Strelley. My wife has all the classic RS symptoms with the Psuedobulbar being very pronounced. I have never had this explained to me before.

Many thanks

[gwend]

Strelley

great explanations thank you

do we know if co-Q10, turmeric , coconut oil - work better on one or the other?

just curious

it is probably more related to the P symptoms?

Gwend

[Hidden in reply to gwend]

Hello Gwend

Since the disease pathology of PSP-RS and PSP-P is basically the same, then if these supplements have any benefit at all "this benefit" will apply to both conditions. In general, such supplements are used for many types of diseases, including degenerative neurological diseases (because they may have some neuroprotective properties).

All the best

T.

[gwend]

Thank you Strelley

[Heady]

Hi Strelley

Thank you for that. My husband obviously has PSP-RS. You mention that PSP-P being the slow one. Does that mean that RS is more aggressive and therefore a shorter life span?

Heady

[Hidden in reply to Heady]

Hello Heady

Most recent studies with larger numbers of patients show that PSP-RS has a much shorter survival time than PSP-P. An example would show survival range for PSP-RS as 6.3 +/- 2.4 years and for PSP-P as 11.7+/-4.9 years.

So PSP-RS is generally "more aggressive".

Cheers

T.

[Heady]

Thanks Strelley, I think! Just more "good news" for this foul condition.

Lots of love

Heady

[Charliesyoungest]

Strelley

I totally appreciate everything you share here. You are such a benefit in helping understand this terrible disease. I had no idea PSP-RS patients routinely have a shorter life span.

Thank you again!

[jillannf6]

hi strelley/joey

htanks fo ryour explanation

i think i have the PSP P (the slow type) but all my movements have speeded

d up not slowed down - it is a real problem 4 me as i tend to move around too quickly and then i fall and my speech has go much quicker rand more garbled ( and quieter too)

bu ti am GOOD and am finding ways of adapting to this lifestyle i now have to endure!

i am using a wheelchair 4 outsiide

my typing will never get better bui have a word check for it nad have the app onmy ipad "proloque to go "4 when my speech goes .

my swalllowing has not really got worse since dxd so that is good (i bolt my food like it is the las tmeal i will have )- ask peter jones australlia about this!

loljill

ps ia m takign coq10 and turmeric - been falling less since taking them

[marytea13]

Hi Strelley I saw my neurologist recently he was 2 hours late & apologised being on the phone to Canberra. I asked him if I had progressed he gave me a cursory glance & said about the same but since then I have developed shakes mostly of my upper limbs but some of my lower limbs. My eyesight also has been affected - I'm having problems putting in correct numbers so have to teach internet banking to my husband & I am not very patient with him poor man! swallowing also can be difficult. I cant wright these notes without using spell check all the time. Do I sound like I am progressing? & if I am I think it will be very rapid.Mary

[Hidden in reply to marytea13]

Hi Mary

I think only your neurologist can give you a possible answer about the "rate" of progression. However, Parkinsonian conditions (whether PSP/CBD or the like) are all "progressive" degenerative neurological diseases. They "progress" at different rates in each sufferer and with different severities. Has your neurologist applied the PSP rating scale to your set of symptoms? It was devised by neurologist Lawrence Golbe and helps show the progression rate (over set times). He should know about it, so you could ask next time you see him (or phone him or have someone write/email him with this query).

You sound very anxious, so I trust you'll make every effort to find answers to your concerns. If you do not feel your neurologist is giving you the answers perhaps call the Parkinson's Association of WA and ask to talk to one of their nurse specialists.

All the best

T.

[marytea13 in reply to Hidden]

Thanks Strelley I am anxious I had one of the neuro nurses visit me but not really a good one then I had the HACC coordinator visit me she is an OT but not practised as such I find her v good & she is trying to set care in place for me - personal care I need. Hopefully I will get to see R Stell, Mary