Hello, My dad is 85 and in the end stages of psp. We live in a very rural area so our Hospice healthcare workers rarely have psp patents.

[dbmy3]

We can't get answers to many questions. New for dad are increased muscles spasms, contractions or tensing up. Not sure what to call them. He has few words that are understandable. (Except I love you). But he moans out now with the spasms. All were told is that he's not in pain, yet his hand or foot will shake with the moans. We're told that sometimes this happens. Anyone have any suggestions of what is going on and or if their loved one did this? He's getting weaker and his eyes look more distant everyday. But he's still eating his pureed foods. Thanks for you help. Very confusing and scary times for us all.

[Hidden]

Hello dbym3

So sorry to hear about your dad and his final struggles with PSP. We've chatted before on this forum about "moaning, groaning, humming and noises". While for most sufferers, as you seem to know, the noises are not connected with pain. However, we have to make sure that this is the situation. You describe very severe muscle spasms that is called dystonia (in fact one of the other names for PSP is nuchal dystonia dementia). With most (secondary) dystonia, like your dad, there will be pain, and I think his moans (in addition to a usual PSP symptom) will most likely be associated with pain. Probably talk to a neurologist about pain management for the spasms. One can start with simple over the counter meds like ibuprofen. Then botox injections have given relief, and finally muscle relaxants. The latter have side effects but at 85 and in pain, I think one can balance side effects with pain relief.

(Hand and foot dystonia is common in Parkinson's and CorticalBasal Degeneration, the latter often misdiagnosed as PSP or vice versa. In this dystonia, sometimes relief is made by dopamine medication).

Your dad's eyes are showing the typical sign of vertical (and now, possible horizontal) gaze palsy, from which the name of this disease is taken (Progressive Supranuclear Palsy).

I do hope you'll be able to minimise any pain, and give as much comfort as possible to your dear dad.

Take care

[cabbagecottage in reply to Hidden]

hi Strelly . Wonder if you can help . jOhn was diagnosed with Parkinson's over ten years ago now .

He has been so complex in as much as his symptoms are so unpredictable .

tHey fluctuate so much and although he appears to be generally worse after taking his medication , he is on Sinemet , he , what I call switches off . .

I can see his face and body gradually change . hE he chair or bed bound these days

iT can take fours before he (comes back in ) of course it's soon time to take another tablet then . dUring these times he will have lots of Myclonic jerk movement

then he will surprise me with what I call one of his better days !!!!

he sits more upright and eyes open and tries to follow the to programme .

tHe isn't any real sort of pattern .

mAkes it difficult to make any sort of plans because I don't know how he will be from one hour to the next . iTs not easy for anyone other than me to understand , He can give out the wrong body signals .

I have been in the process of assessments for CHC and one of the boxes they have to tick is about Unpredictability , Jihn certainly is this .

The CHC lady told me BECAUSE YO TELL ME HE IS UNPREDICTABLE , HE ISNT UNPREDICTABLE ,,,

I have wondered if he would be better off taking the slow release Sinemet during the day as well as at night , that the delivery of the medication would be smoother passing through the body . oR maybe the ingredients in the slow release is different to the instant ones which he talk one thee times daily .

[Hidden in reply to cabbagecottage]

I haven't been on this site since my wife passed away in May and then I had major surgery for bile duct cancer. So my answer will be brief. Often the slow release Sinemet is recommended to prevent or lessen fluctuations in motor problems (jerking movements), but the dose is usually increased due to less absorption. There is a new modified slow release medication now on the market (in USA and Europe) called Numient, which is worth investigating.

I wish you well in your caring role - it can be so hard - and the sufferer has to go through so many difficulties. Take care.

[Heady in reply to Hidden]

Hi Strelley, lovely to hear from you again. Sorry to hear about your fight with cancer. Hope that is under control, now you have had the op!

Often think about you, I was only reading an old post, the other day of yours, wondering how you were getting along.

Sending you lots of love

Heady

[goldcap in reply to Hidden]

Strelly, I hope your cancer is in remission and you are recovering and have someone to take care of YOU!

Take care.

GC

[Yahashem26 in reply to Hidden]

My husband has p.s.p and he ha z sex ev d ry day with different women persian men wow

[dbmy3]

Thank you Strelley for that information. I greatly appreciate it. I'll be talking to his hospice nurse today about these options. Wish I'd found this site long ago.

[Sugardart]

My mother experienced the same symptoms. She was prescribed baclofen which seemed to alleviate some of the spasms and rigidity. I believe that she did experience pain. I am hopeful you will find some medication that will help with your fathers pain.

[wifemo in reply to Sugardart]

My husband Tony was also helped with baclofen.

Mo

[Hidden]

Mum had contractions in her hip and it was the only thing I ever heard her complain about. It seemed to be relieved by pain patches, which can still be topped up with paracetamol in between. Does warmth help? I was thinking maybe a warm water bottle and massages. Even ibuprofen gel rubbed in can help.

I hope it eases soon,

Dianne x

[Baddie in reply to Hidden]

Hi my dad is 69 and in the last stages stages of psp.he is in so much pain.

The palative care nurse came to see him this week.he is on morphine but it not helping so he is going on pain relief patches and morphine too.we are waiting for him to start on Botox injections to help him

This is a horrible illness and its so hard watching our dad suffer.

I wish everyone well who is going through what my dad is going through.

The hardest part is when he goes to hospital we have to carry a psp card with us to show the doctors what he has as not a lot of people know about this illness.this is my first time to reply to a message.but I read all the letters.

[dbmy3]

Thanks for the suggestions. Saw the fill-in nurse earlier. So far they only what him to have Tylenol as needed. And Lorazepam .05 mg as needed. The Hospice we're dealing with have no clue about PSP. They keep telling us he has Parkinson and are treating him as such. He was dx in 2010 by Neurologist & Later by the University of MD. He is in the end stages of this disease, but not yet to the active stage. I'll be contacting them with more questions / suggestions for pain relief. Your help is greatly appreciated as I purse the hope for his last days to be as easy and painless as possible.

[Hidden]

hello dbmy3, for the pain i would suggest ice packs, i believe the spasms are caused by inflamation which tighten the muscles which also add to lack of blood supply to said muscles, i would start with the shoulders icing each one separately for 15 minutes twice a day, you can also ice the neck, middle of the back and lower back which will give relief, my mother in law says it helps and also she has been sleeping a lot better since we started doing this, pain medication work only short term, i also believe there is nerve damage of sorts coming from the head/neck area causing the body to go into spasm, a personal opinion of mine is the 12 cranial nerves in some way are part of this conundrum especially the vestibulocochlear oculomotor and trochlear nerves which come from the brain into the neck through the spine and throughout the body, the question i ask is 'p.s.p, can it be caused by head trauma? damage to the cerebellum can induce balance problems also damage to some of the cranial nerves [ those that i've mentioned] can lead to problems with the eyes, as with the tau proteins buildup and other debris in the brain, maybe the brain can not get rid of this buildup of waste because of the damage to nerves supplying the brain. these are all just my opinion and i like many many others are trying to come to understanding WHY! i hope you can help heal your father's pain dbmy3,

[jimandsharynp in reply to Hidden]

Hi, My first wife died of another brain disease, Cruetzfelt-Jacob Disease (CJD). It moves quickly and the patient dies within a matter of months. There was a story on ABC news about CJD and how it can be transmitted by operating insturments not properly cleaned. The heat level to kill the virus so it doesn't get transmitted has to be extremely high and most operating rooms don't go that hot when cleaning. The bit on TV said they had warned patients who had surgery during the time the original CJD patient had been operated on at that hospital that they may have been exposed to CJD. What a terrible thought to be given. I mention all this because I sort of thought my wife got CJD from some dental surgery he had a couple of years in advance of diagnosis with PSP and passing away. No way to know for sure. That brings me around o asking if anyone can make a connection between having a surgery a few years in advance of being diagnosed with PSP. Just a thought. I'm sure Strelly can clear this up for us.

Jimbo

[Hidden in reply to jimandsharynp]

Hi Jimbo

Just seen your post. As far as I know there is no direct evidence to relate a PSP diagnosis after a few years of surgey.However, the only connection I might suggest would be that aneasthesia can affect a protein in a similar way to PSP (it can hyperphosphorylate tau protein that is considered one of the problems that starts the process of neuron death). This protein problem is connected with aneasthesia that causes the core body temperature to drop. Often one reads about older people losing some memory function after operations, and some even connect this with a catalyst to Alzheimer's (increase dementia risk).So there may be a remote possiblility that surgery (in rare cases) may hasten on a pre-existing condition of PSP.

All the best

[jimandsharynp in reply to Hidden]

Strelley, Thanks for the input. I always appreciate your responses and posts.

Jimbo

[TamNJ in reply to Hidden]

Your post is old, but yes, I firmly believe my.moms PSP is related to anesthesia from multiple surgeries in a short amount of time.

[LizzieF in reply to Hidden]

Hi vjessv I was interested to read your suggestion about head trauma maybe being involved in the cause/development of PSP. My 82 year old mother has PSP (misdiagnosed as Parkinson's in 2006 and diagnosed as PSP in January 2009), as a child she was assaulted many times by one of her teachers! She was bashed on the head with a heavy book and had her head smashed on the blackboard amongst other assaults. It does make me wonder if any of this was responsible. It worries me as I have also sustained a head injury on the back of my skull as I fell out of the bath and hit my head on the toilet whilst on holiday in Malaysia in 1995.

[Hidden in reply to LizzieF]

Wow lizzieF, is there something to this? my mother in law slipped on ice dec 2010 and by apr 2011 she started to show there was something wrong, she hit the back of her head where the cerebellum joins the brainstem, so hard she knocked herself out, went to hospital x-ray'd for FRACTURES to head and spine got the all clear and was then sent on her merry way, she was diagnosed p.s.p nov 2012, it can take years for any type of head trauma to manifest, cerebellum controls your balance, hitting the back of the head causing a whiplash effect does that result in a knock-on effect? the cerebellum jamming into the pons area of the brain causing nerve damage? dr john bergman says over 70% of people with parkinson's is due to head trauma and as you stated i believe parkinson's and psp are very similar thats why neurologists give sinemet to patients with psp in case it has effect, the neck is the connection between the head and the rest of the body and if you damage it? if you have a subluxation of the atlas/axis area of the spine it will cause nerve damage resulting in many problems, if you google subluxation of the atlas/axis you will see what i mean, which leads me to thinking and its just my opinion an upper cervical chiropractic just might help with nerve damage to head/neck and mobility as well as balance or gait, nerves not firing properly causing reduced mobility which then leads to shuffling when walking slowly leading to reduced movement and so on, i for one are looking towards a different type of pain management,( my little rant) i do not know what causes psp, many people on here are into the science of this disease and can't tell you how to manage it, just like the neurologist who gave my mother in law sinemet ' but you told us she had p.s.p not parkinson's? it just might help he said, well it didn't now what do you suggest? we're still waiting! and for people on here who quote from pubmed it doesn't help my mother in-law does it! and it doesn't give us the answers we need. i came to this forum for help and suggestions and i would like to think that some of my mine are that! suggestions, another way to look at this, lizzieF thanks for your reply

[Hidden]

Just an addition to my post above: Dystonia is a sustained muscle (set of muscles) contraction unlike spasms and cramps. I am not sure if your dad is taking "dopamine" medication, but this can also cause dystonia when its effects wear off.

PSP is called atypical Parkinson's because of some silmilar characteristic with Parkinson's. The movement disorders (including dystonia) arises from damaged areas in the midbrain and basal ganglia. One of these areas is the substantia nigra which is responsible for dopaminergic nerves that (in a very complex way) "regulate" movement (with input from other areas like the cerebellum). In Parkinsons it is believed the main culprit for neuron damage is a problem with a protein called alpha synuclein, while in PSP it is a protein called tau.

[Hidden]

The brain damage in PSP (and similar diseases) is not analogous to blocked arteries. It's far more complex. Balance and falling in PSP has little to do with cranial nerves (the vestibularcochlear nerve is rarely damaged in PSP and the type of balance and apraxia problems, if they do occur, are not the same those caused by brainstem and cerebellum damage in PSP). It's not so much damage with cranial nerves as it is with all the areas and mechanisms that control such nerves. There are very complex nerve pathways that are damaged, and these affect other nerves that are NOT damaged. The fact of the movement disorder in PSP (and Parkinson's) comes mainly (but not exclusively) from an area called the substantia nigra where up to 80% of neurons are dead before symptoms occur. This area produces "nerves" that carry dopamine to other "nerve" systems, and when it fails, the motor nerves (not cranial) do not work properly and cannot send the right signals to muscles that help us with body movements like walking.

Tau is a protein that wraps itself around the internal protein skeleton of neurons, and stabilises it so that it can fire impulses and carry neurotransmitters (like dopamine) to the end of the neurons that are needed to fire the next neurons (neurons are contained in nerves). In diseases like PSP, this tau protein becomes "hyperphosphorylated" and "comes off "the protein skeleton" of the neuron, and then the latter falls apart. The tau (now damaged) eventually (over many years) forms clumps called neurofibrilliary tangles, that the cells cannot break down and remove. These tangles are thought to eventually be the cause of the death of the neurons.

All the research into PSP "cures" are aimed at 1. Stopping the tau being hyperphosphorylated (it's thought this may be caused by the mitochondria not working properly, and this may be responsible for certain proteins being produced, called kinases, that cause the hyperphosphorylation 2. Stopping the tangles forming or helping the cells degrade (dissolve) and excrete them. 3. Find a genetic answer to change the type of tau found in the brain of PSP sufferers (because it's sort of different to that in normal brains).

So it's not like a blocked artery. While some cranial nerves are affected in PSP (with associated symptoms) these nerves are often "controlled" by other areas in the brain. It's similar to having an electrical wire (cranial nerve or upper cortex motor nerve etc) that is not damaged, but a transformer at the beginning of it (that little box often seen between the electrical plug and the device you are using) has damage within it. So you may have no power, or intermiitent power, as bits work "on and off". Not a good ananolgy, but hopefully it helps!

[jimandsharynp in reply to Hidden]

Strelly, excellent description of what Tau is doing in the brain.

[Hidden]

Hi vjessv

The cerebellum is damaged in PSP and it does have an effect on balance (the dendate nucleus of the cerebellum is mainly damaged). Additionally, PSP has damage like Parkinson's where dopaminergic nerve pathways are damaged in the basal ganglia region (in particular the substantia nigra) that causes hypokinesia.

Please remember that in such conditions as PSP there are very many areas affected with neuron death and lesions. While the central nervous system controls many functions in the body, the brain does not usually have one discreet system for each function. Many neural pathways have to link together from different areas in the brain, and all those areas have to be healthy. When we think of swallowing and talking problems in PSP we usually think of damage directly or indirectly to the hypoglossal and glossopharyngeal nerves, but they may not function if there are other damaged areas (like certain lesions in the frontal cortex). When we think of vertical gaze palsy in PSP we think of the ocularmotor nerve, but one area (amongst others) of the brain that's damaged in PSP has a direct effect on whether that ocularmotor nerve actually works (with a big name -called the rostral interstitial nucleus of the medial longitudinal fasciculus - due to midbrain atrophy).

It's true that PSP can only be diagnosed during life by clinical signs, but MRI is used as a diagnostic tool to eliminate other possibilities, and confirm (in most PSP patients) a type of midbrain atrophy that is very typical in PSP. Remember that PSP (and similar conditions) can only be definitively diagnosed at autopsy.

There is new research on radioactive labelled "tau" compounds that can show up "tau tangle deposition" with a PET (Positron Emission Tomography) scan of the brain. This is being used for Alzheimer's (that also has abnormal tau) and related tauopathies like PSP. This is quite exciting.

All the best.

[Hidden]

thank you for your reply strelley, can i ask you 'how do you think the cerebellum is damaged to cause PSP or is it just part of this affliction after said damage?

[Hidden]

Hello again vjessv

While still being researched, it is thought that both normal tau and toxic tau can move from neuron to neuron along defined neural pathways (This has been shown in Alzheimer's and thus should apply to other tauopathies like PSP). It's thought the toxic tau causes normal tau to become toxic (and some eventually turns in tangles). It's been thought for a long time that the toxic tau and not the tangled form is responsible for neuron (and other brain cell) death. Just to complicate matters, researchers talk about a mutant tau seen in aggressive diseases like PSP, that kills the neurons without having to travel through the brain.

Although there is still a lot to understand why some areas of the brain are affected by tau toxicity and other not, it does seem that the recent discovery of "travelling tau" would explain why the cerebellum could be affected (since it has many pathways linking it to other areas like the basal ganglia and midbrain where there is a lot of damage that is considered to be due to toxic forms of tau).

So PSP is actually caused by over a dozen areas of the brain having neuron death (and each area of "atrophy" will, in general, but not always, show a clinical sign e.g. dopaminergic neuron death in the substantia nigra and globus pallidus results in the movement disorders we see in PSP).

This is why PSP has so many "symptoms" and why neurologists say there is no medication to stop the progression.

The researchers think there'll be a new class of drugs develped to "stop" the tau or toxic tau spreading through the brain, and if successful, it may halt or slow down dramatically the progress of the disease.

Hope this helps a bit!

Cheers

[Hidden]

i've read many postings here and the majority of people or carers complain of pain, i offered in an earlier post that using ice packs in affected areas to lower inflamation would be of help, it has been of great benefit to our loved one who now sleeps better and has slightly better mobility which has helped with mood, to find the cause of pain my question is 'do neurologists do a nerve conduction study test and if not why not?

[2twin4]

Hello,I have been taking care of my husband with PSP for the last 8 years, he is in the last stage and it is getting very hard for me.. he is extremely loud with his moans and groans, that I had a bedroom build in the basement with double dry wall, and I still could hear him. H e was diagnosed at the age of 55, the neurologist had tryed all kinds of meds for the pain that we dont know exactly where it hurts, also tryed morphine but did not work, let me tell you that I do not know where this energy is coming from, but yet cannot do anything on his own,, he does not speak, walk, read or write, he is with a feeding tube for the last 3 years, his eyes do not move up down or sideways, and he never blinks, all he could do is thumbs up or down for yes and no. Life is very rough right now

[oops54 in reply to 2twin4]

hello twin ,I also have my husband in the last stages of psp. he also cannot speak , he is incontinent, with both, his eyes are always closed ,he groans almost all day and night,he dose not sleep ,his feet are nealy blue in the mornings ,if i put more blankets on him he gets two hot and clamy ,i leave his socks on at night.he has been this way for a year now but still listens to the cricket and the tv .he also listens to audio books.he uses the thumbs up and down as well ,he cannot eat with a spoon or fork,but has to be fed but he can eat with his fingers,very slowly but he enjoys it that way and then we have a big clean up after,I have a carer come in to help me with him to get him out of bed and to toilet him,that is the worst part of the day on the other days two come in to help shower him ,he is very heavy he is all hunched over he is like and old man but he is just sixty, but i am gratefull for the carers ,i couldn`t do it on my own. regards christine.

[JB569 in reply to 2twin4]

Bless you, thinking of you. It is so hard to watch our loved ones suffer this way. Words are not enough to describe what it's like. xx

[Baruli in reply to 2twin4]

Hi Twin,

Such a difficult place to be but be assured you are not alone . My Dad is suffering from this awful disease and lives in a country without the facility of carers or a medical team knowledgable about PSP. It's as hard on my Mum, his principal carer as it is for him. Stay strong

[dad94]

My mom had a massage therapist come to the home. This seemed to help. He was also put on an antidepressant when he could still talk some. Not sure if he was on it in the last stages.

[2twin4]

Hello, my husband that is 62 has exactly the same thing his hand shakes when he moans, I ask him if he is in pain but I am not sure of his answer, he is very lost these days, could only do yes and no with his thumb up and down and even that is not sure..what an awful desease, he is going on his 9th year and its getting harder and harder, had to build him a room in the basement so I could have some relief from the moaning, we even tryed botox on the vocal cords but did not help.. He is fed with a feeding for the last 3 years and now won t even open his mouth, I need some encouragement from anyone

[JanetCurry]

Just went through this over the long term with a lover/friend of the same age. I used to massage him on a regular basis while visiting him in his nursing home and that seemed to help -- especially foot and hand massage. He was also on pain meds round-the-clock to ease his discomfort. Although he was not able to speak or swallow at all at the end, he could use hand signals and foot signals to answer "yes" and "no" questions. He could also tell us he loved us by stroking us with his feet and holding tightly onto our hands. The really painful part about this disease is that we know our loved ones are trapped inside bodies that do not work and that their ability to communicate with us is greatly impaired. Make sure to keep telling each other you love each other. It's the MOST important thing.

[BarbieL]

How do you determine when PSP patients are in final stages