I'm new here. try to find out more info about PLS

[paul318]

Hi Everyone,

My name is Paul,I am 49 years old and live in the UK. I don't have a diagnosis yet so I am trying to find out as much as possible about PLS. I have been unable to work for three and a half years. I have seen 4 neurologists and have been told that my problems are 'functional'. Functional is another way of saying 'conversion disorder'. I have no history of psychiatric illness and have suffered no major trauma that would cause my problems, so I do not accept this diagnosis. I know that the first neuro I saw put my illness down to stress, this was very early in my illness, and I have a suspicion that all the others have just followed his lead and haven't looked at me objectively. My search for answers continues. I have been on a number of different forums trying to make sense of all the symptoms I have. Here is what has happened so far. Be warned - it is a long story.

As a child I had problems with weak ankles and being clumsy. I saw a consultant for a number of years as a child. At one time they wanted to put my legs in irons to give support. But my mother refused the treatment as she didn't want me to suffer being bullied. I had my shoes adapted to try and correct the problem. I have always had falls and been unsteady on my feet, but have been able to get on with my life. In fact it affected me very little, apart from the embarrassment of the occasional fall. I am not sure if this all has any relevance. In fact it is something that I never mentioned when I saw the first neuro as it was just the way things are.

In late 2004, I started to have problems with my voice. I would have trouble with being heard. My voice was weak and hoarse sounding. This would last a few days and then go only to re-occur again later. My GP put it down to the inhaler I use for asthma. He didn't bother to examine my throat, and just said I should be careful to wash out my mouth after using my inhaler. I did this but the problems with my voice persisted.

During the early part of 2005 I was finding that my energy levels seemed lower. I had a couple of instances when I could see the muscles in my arms moving in a wave like manner. On one occasion I went to pick up a shopping basket at the supermarket when my arm went limp. It didn't last so I ignored it. during this period I noticed a number of things happening, including occasional dizziness, but ignored them as I hate going to the doctor.

Things didn't really get bad until December 2005. I went for a flu jab. The nurse who gave it to me took my blood pressure and found it was really high. I had been diagnosed with hypertension early in 2005. My drugs were changed and I was told to rest. Within a few hours I was experiencing dizziness when standing. I assumed that the problem was caused by the change in medication. It persisted so I saw my GP and I was put back on my original meds. I then noticed I had a strange sensation down the left side of my face. Sort of numbed, but it also felt as if my face had dropped but it wasn't noticeable to anyone else. My speech problems had also reoccurred but this time I was having problems with articulation. My speech was slightly stammering, It sometimes felt as if my I couldn't engage my mouth. my walking was becoming worse. My legs would become stiff after walking a short distance. I saw a locum doctor at my doctors practice. She examined me. Found things she was concerned with and I saw a neurologist within 2 weeks.

This is where it all gets complicated. The first neuro examined me and put it down to stress. I was under a bit of stress, but no more than most people suffer. stress is just a fact of life. He told me to go back to work! I tried but was sent home after a few hours as I was having problems with balance and concentration. I was working at that time in a residential school for profoundly disabled children. I was a deputy manager of one of the residential bungalows. I went back to my doctor. I saw another locum. This was one of the problems I had for the first year I saw a different doctor on just about every visit as my doctor was unwell himself. This inconsistency hasn't helped. This locum sent me for a second opinion.

The next neurologist I saw did a neurological examination, took blood tests and send my for a MRI of my head. I had to wait three months for the results. During this time my walking had deteriorated. I was walking everyday, but was finding that my legs were increasingly stiff towards the end of my walk. My right side was worse than my left, which has always been the case. I sometimes looked drunk when I walked. I resisted using a stick until one day a group of kids were laughing at me as I looked drunk. I also develop strong myoclonic movements, my head would be thrown back very violently. There is no warning when this happens, so there is no way of controlling them. I saw the neuro for the results in July 2006. He saw that my walking was worse. I told him about the movements, and he witnessed some. He said that my MRI was clear. He examined me and said there was nothing neurologically wrong and discharged me.

At this time I started to look into ME/CFS and asked for a referral to the National ME Center. ME is the original name for Chronic Fatigue Syndrome There is much I could writ about this but this post is already too long. You can find out more at http://www.ahummingbirdsguide.com/. The local Health Authority refused to fund the referral and recommended yet another neurologist.

Neurologist number 3 was very thorough. I had a EEG, lumbar puncture, various blood tests, MRI of my head and spine and saw the neuro psychiatrist, who incidently couldn't find any evidence of a psychiatric condition. Non of the tests showed anything. I was discharged with a diagnosis of ME/CFS. I knew i didn't completely fit this diagnosis as fatigue was a minor problem. I hadn't has a day in bed even since becoming ill.

I was sent to the local ME/CFS service. I had to fill out a form before I went. It was all about how sleepy I was. The problem was I wasn't sleepy! The doctor there saw me for a longer time than any of the neuros. He gave me a neurological examination, found spasticity in my legs, a spastic gait, positive Romberg test and fine persistent tremor. He said it looked nothing like ME/CFS. He was the first to mention PLS, but I didn't really take it in at the time. He said i should be referred to the best neurological hospital in the country, The National Neurological Hospital in Queen Square, London. I was hopeful as he said that there was bound to be someone there who would know what is wrong.

My experience at the National wasn't good. The first neurologist I was greeted me with the words, 'You have seen 3 neurologists, 2 I know personally, what on earth are you doing here?' He then preceded to accuse me of going from neuro to neuro wasting there time. My wife and I were shocked. He did a cursory neurological examination. by this time I was so stressed that I am sure it affected how I behaved and only added to his suspicion that I had a conversion disorder. He went to see his boss and came back and said they would admit me.

In January 2008 I was admitted to the National I was greeted with the same disbeleif I suffered at my first visit, which put me under a great deal of stress. They did a startle study, which was positive, a few blood tests and another lumbar puncture. I saw 2 movement disorder specialists on the ward, neither for more than 5 minutes and never in private. They relied on what their registrars had reported to them. That was it! One week in hospital for that. They had me back for more tests in the april. This time i saw a Speech Therapist, had a EMG on right leg, a combined EMG/EEG and neuropsychological testing. Anyhow, they decided that it is all in my head! What they call 'functional, but which means psychiatric.

My symptoms have continued to get worse. At this time I have speech problems, intermittent problems with swallowing and aspirating, stiff neck, involuntary movements of my upper body, fingers and toes that more on their own, muscle twitched in my eye lids and occasional involuntary shutting of my eyes, Muscle spasms in my lower back, occasional muscle spasm in my neck that feels like I am being throttled and my gait has continued to worsen. I also have problems with writing and fine movement in my fingers and some intermittent bladder and bowel problems.

I have been given 6 weeks of physiotherapy, which I have just started. The National would like to admit me into their neuropsychiatric unit for 6 weeks intensive therapy, which would consist of Graded Exercise Therapy, CBT to deal with my abnormal illness beliefs, and physiotherapy. I would have refused it as I have heard enough bad things about the treatment . They decided not to offer it to me as I lost my eldest son last September and I am still grieving.

So that is me. Well and truly in limboland. Lots of symptoms but no answers. The problem, I am told, is that my symptoms do not fit any known neurological disease. There are some that look like parkinsonism, but others that don't fit. I am not even sure they fit PLS, but someone else mentioned it appart from the ME/CFS doctro, which is why I am here. Sorry for a very long first post.

Paul

My Vlog

 

[olly]

hello paul

firstly i am so sorry for what you have been through and for the loss of your son.

i can not say if it could be pls or not,you do have some symptoms like the stiffness,myoclonus and balance issues.
my mum has "me" and gets chronic fatigue but no neurological symptoms per say.

something did make me think in your post,about your childhood problems.
i have been doing my latest research in mitochondrial deseases.
these are metabolic disorders that can cause neurological symptoms among other things and can mimic other neurological deseases.
in fact mito dysfunction is thought to be one of the factors of developing neurological deseases like ms and parkinsons.

anyway,it is a genetic condition were symptoms are present from birth or early childhood but some don,t develop or notice symptoms till adulthood.
you can have genetic testing but its not 100% accurate as there are so many various types of this desease.
a muscle biopsy to look for ragged red fibres is also a means of diognosing it.
but sadly some do get labled with me or fybro and other things because this desease is missed all to ofton in adults who develop symptoms.

the reason i have been studying this despite my diagnosed of pls and having this 10yrs is that i think theres a chance i have it.
i had a baby brother who died aged 2yrs when i was 4yrs old. he was born with feeding difficulties and failed to thrive,he never learned to talk,walk or sit up and still looked like a baby when he died. this i have learnt is classic in babies born with severe mito desease but it was 1971/2 and there was no real way of knowing what was wrong with him.
i had unexplained seizures as a baby and a few during childhood plus two as an adult.
my son has been complaining of symptoms similar to mine,he jokes that i had better not have given him my desease. i hope to god not.

you sound very level headed to me,hell even the psycho doc thinks your ok.
sometimes even if a neuro does notice something off unless your unable to function to a certain degree or look like your dying they will say your ok.
you just have to love there "i am god","up my own backside" wonderfull manner:wink::lol:

i can,t think of what else to tell you , try the physio and see how things go for a while.
whatever is wrong will show itself eventually and those neuro,s will have to eat there words.
i do have lots of info on file for pls and mito,i will try and post some for you tomorrow when i have time.

we are always here to listen if you need to vent or to give support and answer any questions.
take good care paul

 

[olly]

hi paul.
just found a few things for you.

Primary lateral sclerosis - Wikipedia, the free encyclopedia

http://www.ninds.nih.gov/disorders/primary_lateral_sclerosis/primary_lateral_sclerosis.htm

these should give you some info i hope is helpfull.

 

[paul318]

Hi Caroline,

Thank you for all of the info. There is a lot of evidence to suggest that mitochondria are involved in ME/CFS too. Dr Sarah Myhill has just published a paper about this http://www.ijcem.com/files/IJCEM812001.pdf. Originally ME was called atypical polio mylitis, as it resembled polio in it's effects and was often seen in epidemic form alongside polio. ME is classified by WHO as a neurological condition. Unfortunately, when there was an outbreak of it in the USA in the '80s, the CDC got involved, along with a large contingent of psychiatrists, and it got renamed Chronic Fatigue Syndrome and given new criteria, the main one being fatigue. A large list of symptoms can be present with ME http://www.ahummingbirdsguide.com/themesymptomlist.htm.

I feel pretty sure that I do not have ME, as I have met many people online who have it and I don't really fit their description. One thing I forgot to mention in my first post is that the ME/CFS doctor I saw also said I have hype reflexes. I'll have look at the links you have posted about PLS and Mitochondrial diseases.

The biggest challenge I face is getting anyone from the medical profession to take me seriously. After all, the best Neurological hospital in the UK has said I have a psychiatric not a neurological disorder! I am hoping that the observations from my physio will hold some weight. Although the speech therapist wrote to the National and my GP about my swallowing difficulties, which she observed, and they totally ignored what she said.

Best Wishes,

Paul

 

[Geo]

Paul ,not to worry if they dont have an answer they'll just say your a little nutty. Its in your head ,their right in a way HMMMM Yah its in my head so now you have a start ,this is what you tellem . Im a lot like you all tests are somewhat normal ,no history of it in my family . When the Idiots have no answers they have to blame you . MY beliefs are its a Virus and once it has established itself in your Brain even a spinal
tap wont show anything abnormal . Our Medical Society has no Clue and you think their going to admit anything NO CHANCE . All i can tell you is Find a way to learn how to Cope ,because dont plan on a doctor being any help Sir . Your Friend Geo

 

[Zaphoon]

Regarding PLS

There are a lot of websites you can go to that will tell you about PLS. Some say that it is extremely rare - 1 in 10 million people may have it. Other sources may say it is a bit more prevalent.

It takes a long time to come to the conclusion of a diagnosis of PLS because like ALS, all other diseases/disorders/dystrophies and syndromes have to be ruled out first. That, my friend, is a long list of stuff!

Usually, the neurologist will say he/she suspects it to be PLS and will monitor you for a period of time before any kind of final diagnosis. Some sources say the monitoring period is 3 - 5 years. This period of time is necessary to make sure you are not developing lower motor neuron trouble. In which case, the diagnosed would be changed to something like upper motor neuron dominant ALS or just ALS.

Good luck! Hope you don't have it!

Zaphoon

 

[paul318]

Hi Geo,

I have come to the same conclusion. I have times when I try to ignore it and get on with my life, but it has a horrible way of showing itself. Having a very disabling illness with no real explanation or name for it isn't easy as it makes you feel alone. That is my problem. I don't 'fit'. In fact that's the problem I have with the neuros - my symptoms do not fit their neat set of criteria for any one disease - at least the diseases they are aware of. I have had the occasional neuro who has admitted there is much they still do not know, but the ones who make the decisions about your treatment tend to prefer to blame the patient than to recognise their own lack of knowledge and diagnostic weaknesses.

I did read a article about PLS that said extra pyramidal system of the brain are not affected in PLS. I have many involuntary movements and involuntary movements tend to come from extra pyramidal system. I have been doing some more research and have found that the complicated version of Hereditary Spastic Paraparesis can show signs of extra pyramidal damage. Spasms and stiff legs are a symptom of that condition too. Actually, known of this makes much difference in a way as neither PLS or HSP can be cured. Having a diagnosis that makes logical sense would really help me. Also having an idea of the prognosis would also help me to plan for the future. At the moment I must plan in the dark. The likelihood of being referred to another neuro in the near future is small. Even if I was to get another referral my notes will follow me, and the neuro is likely to accept what has already been said about my condition without objectively looking at me.

Paul

 

[olly]

oh paul,i just got a chance to look at your video and i wanted to cry.

i noticed the myoclonic jerks and drooping of one of your eyes.

have they tested for mg?
have you had genetic testing?

you also mentioned speech problems and stroke like episodes,these are symptoms of mito along with eye problems,spasms,myoclonus.
i think if you followed any other route mito desease should be investigated.

here is some more info..............
http://www.umdf.org/site/c.dnJEKLNqFoG/b.3042169/k.7A8C/About_Mitochondrial_Disease.htm
http://biochemgen.ucsd.edu/mmdc/brochure.htm
http://www.umdf.org/site/c.dnJEKLNqFoG/b.3558759/k.8256/Frequently_Asked_Questions.htm

they have found now that those with me do have some abnormal findings in the cns and on imaging and its not all in the head but a real neurological illness.
sadly it was badly labled in the begining and that has stuck with some doctors.

at my very first neuro appointment after having spasms,stiffness,clonus,myoclonus and foot drop plus clinical weakness in my left lower leg............after 5minutes the neuro said it was me.
i swore and called him an idiot as my mum had me so i know what its like to live with.
i was shown into the next room were i saw another neuro who said no its not me,he is a well respected proffessor in neurology.
i had several years of testing and repeated mri,s,ms was top of the list then later multi system atrophy.
i had genetic testing(tests done in london)
every test came back ok.
my symptoms and progression(leg onset but spasms progressed up the body to bulbar)also progressive muscle weakness indicate pls.
with my family history i can,t help but think mito desease could be playing a part in causing the pls.

can you identify anything in your family medical history like your symptoms..........also deafness,respitory desease and gastric disorders are classic mito problems.
my mum has deafness and gastric problems,sister has gastric problems and my son has a type of asthma due to congenital weakness of the respitory muscles.
the one thing i never had was a muscle biopsy and may consider this.

but as you said in your video it all gets too much searching for answers,in the end they can only treat the symptoms.
my neuro said they will know for definate on autopsy,in some of us this is the case.
pls is a clinical diagnosed and can only be confirmed on autopsy.

hope some of this helps. you are in my thoughts.

 

[paul318]

Hi Caroline,

They have tested for MG - or at least I think they did. I had an EMG in April 2008. I don't know the results but I assume they were normal. I am not aware of having any genetic testing done. I have been thinking about having Mito testing done. Dr MyHill offers this and an interpretation of the tests on her website. But at £250 (over $400), I can't afford to have them done. They are not easily available on the NHS unless I can get a consultant to believe there is actually something wrong.

I have been down the route of having tests for Lyme disease. This cost a fortune too and came back negative. I did have a private doctor who diagnosed Lyme on a clinical basis. I was on very high doses of antibiotics for about 4 months, but it was having no effect so he said I should stop taking them. The problem with Lyme (which can affect the brain) is the tests are not accurate. And although I have been to places where Lyme is endemic, I have no recollection of being bitten by a tick.

The problem with the whole ME/CFS thing is that it has become a waste bin diagnosis. Any one who is 'tired' for 3 months or more, and whom they cannot easily find anything wrong with, is given the CFS label. In fact neurological symptoms will rule you out of any of the current CFS criteria used in the UK. The original diagnosis of ME was on the basis of muscle fatiguability not general tiredness. Numerous neurological symptoms have been documented, and studies have shown every body system is affected in some way, in particular the heart.

Some neurologists you see will know about the neurological effects of ME, overs only know the psychiatrically influenced CFS diagnostic criteria. I think the third neurologist I saw knew about the neurological complications of ME, in fact he called it Myalgic Encephalomyelitis , rather than the usual Chronic Fatigue Syndrome. He actually said that my symptoms 'could not be explained by somatisation or conversion disorder'. The CFS doctor I saw would have used the Oxford Criteria for CFS/ME which rules out neurological symptoms as part of the diagnosis.


Both my mother and father had heart problems. No history of deafness, respiratory problems that I am aware of. My father had polio as a child. I saw Dr Byron Hyde a few years ago. He is an expert in ME based in Canada. He was in the UK for a conference and offered to see me. He thought it was significant that my father had polio, as he thinks there is a genetic suseptibility to the virus (which may be an entrovirus like Polio) he believes causes ME. He wouldn't say whether he thought I have ME though, as he couldn't examine me because he wasn't insured to practice medicine in the UK, and he will only diagnosis it on the basis of a SPECT scan. He and others have found that people with true ME, as opposed to CFS, have hypoprofusion of blood to the brain stem. He was going to take on my case, but it is impossible to get tests like SPECT scans done unless a consultant orders them. So unfortunately he couldn't help me.

It is strange - whenever I write about this I find myself almost talking myself into the ME diagnosis! Perhaps that is what I have and I am chasing for another explanation because so many I meet who have ME don't have the severe neurological version I have, and simply have fatigue along some cognitive difficulties. The problem with ME, and for people who have it, is that it is so controversial. Patients have to wade through a quagmire of often conflicting opinions.

This forum may well not be the place for me! Perhaps I should just accept the third neurologists verdict, forget all about what the National said (who incidentally think ME/CFS is a conversion disorder even though there are over 2000 published papers that show physical abnormalities), and get on with things the best I can. Like PLS, ME has no cure either!

Paul

 

[olly]

hi again.
i had to answer the door at the end of my last post(the asda van delivering my shopping).

the frequent myoclonic jerking i can see you have is not a me symptom.
you do get myoclonus in pls but if yours is persistant(every so any minutes as seen in your video)this would indicate a movement disorder and from some personal descriptions of ones with mito desease persistant myoclonus is a symptom.

i think i read that about half the population has the defective mito gene but only a small number develop the desease and symptoms.
the fact it can effect any organ and could be one of the underlying causes for many neurological deseases and cancers means it is very poorly recognised,especially in adults.

the neuro(proff) i saw after the idiot one has been my neuro for 10yrs.
from the begining he believed me even when tests were coming back ok,yes at times he frustrates me when i ask him something and he wont or can,t give me a straight answer.

the neuro,s you have seen have failed you,you are not to blame for any of this.
i was lucky but ofton it can take trying several neuro,s to find one that is willing to listen and dig further.
i think you need to find that "good" neuro.
see how things go with your therapy for a while.
please feel free to stay with us,its good to have you here.
you do have spasms,stiffness and myoclonus ect like in pls so.............i think we can make you fit in here:grin:

 

[Geo]

You know with Odds like that (1-10 Mil.) we should all play the Lotto . But i think its more because alot of folks feel like freeks so they just stay home and deal with it best they can ,but i think its good to reach out to others if you can . Good to get it off the shoulders , venting is good if it helps relieve stress . For some . Your Friend Geo

 

[olly]

paul,i forgot to ask have they given you anything for the myoclonus?
they use anti seizure drugs to treat chronic persistant myoclonus.

when i first got ill the myoclonus was really bad,i had vilolent myoclonic jerks someimes like someone was picking me up and throwing me.
i was offered anti seizure drugs but i have hypotention and blackouts(probably automic dysfunction)so declined.
i have been on baclofen for about 9yrs now and long term it has calmed them down.

i suspect its the myoclonus the reason you are no longer able to drive?
if it is effecting day to day living its only reasonable that they try to treat it.
i have heard of people with myoclonus as a desease,if they concentrate on a certain thing(i think one lady it was house work and a man playing a musical instrument)there myoclonus stopped briefly during that time.............wierd.

if your not on any meds ask your gp to try you on some,its worth a shot.

geo.
how are you and your lovely wife doing?
can you send us some of that florida sunshine?
we must be made of "some "good stuff my friend...............you can,t keep a good man or woman down:lol:
as zaphoons quote says "we just pick ourselves up and get back in the race.............thats life":lol:

 

[paul318]

I am on clonazepam, Caroline. I only take one at night which helps me to sleep. I have tried taking more but they make me feel too spaced out. I'd rather have the myoclonus than feel constantly sedated. I did try baclofen a couple of years ago, but I don't think I gave it long enough. At that time my symptoms were not so severe and my GP only let me have it to try at my request.

The myoclonus is one reason I don't drive, but I also have some problems with concentration and with my eyes closing involuntarily. I do very occasionally drive locally, but only if I am having a really good day.

Paul

 

[paul318]

You know with Odds like that (1-10 Mil.) we should all play the Lotto . But i think its more because alot of folks feel like freeks so they just stay home and deal with it best they can ,but i think its good to reach out to others if you can . Good to get it off the shoulders , venting is good if it helps relieve stress . For some . Your Friend Geo

Hi Geo. Fortunately I am not confined to the house at the moment and do have contact with people. The problem is I find long conversations difficult and tiring, due to my speech problems. Walking is also difficult as I am sure it with everyone on this forum. I agree it is good to vent and get things off your chest, especially with those who understand.

Best Wishes,

Paul

 

[drvcolombo]

Hi Paul,

I am new to this site also. My Mom was just diagnosed with PLS last week. She has seen several neurologist as well. The doctor who finally diagnosed PLS said he "most likely" has this. He is monitoring her and will re-evaluate in 3 months. It is frustrating and upsetting. Her problems started with her walking. SHe said she felt like her legs were lead, and coudn't lift them up to walk. Now her hands and arms are affected. She gets physical therapy 3x week and we are looking into accupunture. Someone suggested tonic water. I don't know about that, but others have rec. it.

My Mom is 67 and was extremelyl active and healthy. This came out of nowhere. This is a good site and we are all exchanging info. We are looking for another doctor, a "specialist" if one exists on this PLS. We live on Long Island, NY, and Mom is seeing Dr. Posner in Manhattan. Is there a PLS specialist?

Mom has had numerous MRIs, EMGs, neuro-psych exmas, and one lumbar puncture. It took almost 6 months to come up with this diagnosis. Good luck to you, and hopefully we can all help each other. We must keep the faith and keep praying.

Victoria