Hirayama's Disease

[wastingaway]

i've recently been diagnosed with Hirayama's Disease and am working on getting my insurance to approve the rest of the appointments i need before surgery..the doctors still want to do one more test before the surgery to make sure it is indeed Hirayama's Disease...i have been trying to get this solved for a year now, longer if you count when i first went to my family doctor about it and he told me it was normal..my hands have slowly been wasting away on me, my left more so than my right. i still have a decent amount of grip in my right hand, but my left hand is that of a small child's. however i am starting to notice my right hand beginning to waste away too. let me tell you, you never realize how important your hands are in everyday life until you cant do things that you use to be able to...

the surgery that my surgeon talked about was more or less to rotor router my spinal cavity in my neck to make room for everything. i was just wondering if anyone has ever had this surgery and if they were ever able to recover their grip and hand functions, my doctors would not give me a straight answer when i asked them. i think they are afraid to incase of a lawsuite, but all i want is hope...i am only 18 years old am quite nervous about the surgery and life after the surgery...i was hoping to be a pilot in the air force but i am afraid with my grip the way it is now there is little chance of that happening. if anyone could give me insight that would be great! thanks

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[Kath Webb]

I am a 60 year old female who has just been diagnosed with Hirayama's disease (although for the past 3 years they have been calling it monomelic spinal atrophy). I have wasting in the left hand and arm but with exercise am beginning to build muscle up again.

The main point of this note though is to ask if there are any other women out there with this. It appears to be male orientated (and much younger as well) and I cannot believe I am the only woman!

[musman100]

Hi to all I'm musman.. i found this great forum in sites and i knew this is a right place for me.

[billshakes]

Hello I'm a 36yr old English guy based in Barcelona and it looks as though I have Hirayama's disease which is causing wasting in my left hand. The official diagnosis is still a few months away but I've had MRI and Electromyography (EMG) tests and symptoms suggest this disease.

Just wondering if anyone has some advice on physical therapy that I can do myself to improve general mobility of the hand? What part of my hand should I exercise and how?

As far as I understand it from my Spanish health care, with a typical MRI result ie there is nothing out of the ordinary, there is no treatment or solution so if anyone knows otherwise then please let me know. I would also like to know what sports are considered ok to do as under doctors advice I have given up anything that strains the neck e.g. tennis, yoga and swimming (front) but am still running.

Also wondering to what degree the wasting could continue, it's been gradually getting worse over a 2 year period and hasn't reached a plateau yet but I'm obviously hoping it will and stop at the hand... Anyone else with experience of the condition I welcome your input be it positive or not, how far has it progressed with you and where.

Good luck all

[allergic2dying]

hey guys,
really felt kinda optimistic afta goin thru all ur stories which i can realy relate to as i'm goin thru the same situation....its been abt a three years....recently my doctor suggestd it as juvenile motor neuron disease...bt seein symptoms i feel they both are same......and 1 thing i wud lyk to share is that weneva i scratch my lil finget wid sum sharp object...i get a strange sensatn that runs ryt thry my finger to the right part of my neck(spinal chord)...reply if u feel the same.....

[Confused/Afraid]

Hello Everyone,
My name is vicky and i am a transgender female,my life has been very difficult as it is. I struggled to get were I was for it all to come crashing down on me.
I was diagnosed with Hirayamas Disease and have all the symptoms.It started with my right arm and now my left but this started when i was 30 not in my teens nor my 20`s like most. I`m 36 now. Is that normal, also they say it happens most to Asians or Japaneese or one`s from India I am latin, is that normal. I was a cosmetologist and a Dental assistant for about 20 years or so, my right is totally non functionable and my left is now getting weaker.I had applied for Disability cuz I can`t do much any more and I have been denied twice. I am now going in front of a judge for an appeal. What I want to know is if there are any of you who have applied for disability, or is there any one who is recieving disability. Should I just stop trying. There is no one out there who will hire some one who can not use there hands and arms. I need to know that there is hope for me and if there is any one who would help me thank you all for your support.

[obama549]

i couldnt find the actual link that i was looking for, but i came up with this. i remember when i DID find the website talking about somethng that sounded similar to what your brother has i was looking up ways to reverse atrophy.

What is Spinal Muscular Atrophy?

here's the link. it's mostly about sma, which is similar to what were going througha nd possibly your brother. i will continue to try to find the link to the site i originally thought sounded a lot like your brother, however the more i thought about it i really hope the site i meant to post is not what your brother has. in it, it talked about how the lower and upper extremities atrophied and after a while it progressed to more vital muscles like the lung and heart. it was quite scary to read about because after i read it i started looking for more signs of wasting on my own body.

i am going to get a flight physical soon to see if i will be able to fly afterall. the more the atrophy progesses the more worried i get that i am going to college for nothing. i keep trying to do stuff to build the muscle back but nothing seems to work. my body does not want to cooperate, its very frustrating. i wonder if when it stops like they say it does, or IF it ever stops, if i will then be able to rebuild the muscle. i think the worst part is juST waiting to see what will happen. i'm still trying to live life to the fullest, but whenever it hink about the future its full of questions. thats why its better to just live for NOW and think about the rest later.

also has anyone found any good excercises to do to help? my forearms are looking scraggly as well ive noticed, but nothing i do seems to help. i think this winter did a number on me. riding season is coming up, but sadly i think it will turn out to be my last. i will have to try to make the best of it i guess.

[gripit]

Hi everyone!

Im 26 and diagnosed since I was 17.
I have never thought about searching a forum like that, when I saw it for the first time (this week) I was thrilled that there are people like me with the same disease.
The Hirayama got my left arm and its getting pretty stable around the last years.

I have read most of the posts in this forum and I think that it's wonderful that we are sharing our problems in life by using the web, I find peace in knowing that.

During my reading in this forum I notice that (maybe) no one wrote about how he/she is sharing his private "drawback", and I mean how you handle your life's with flaw?
That issue bothered me ever since and I still not over it.
It's hard for me to talk about it with me girlfriend when she is asking question, my best friends don’t really know about that exact disease…

It's been a Hell.. but now im pleased, I found you!

I want to know the people here better and to make this forum more alive, if possible

I will post soon more about myself, but for now that will do.

Thanks for the support!

[SusanH]

Wondering if there are any parents out there looking for other parents of children diagnosed with this disease? My son was diagnosed several months ago after two years of seeing doctor after doctor, tests, and even surgery thinking that nerve compresion was the cause of his left hand wasting away. His right hand has now started showing signs of atrophy. He is a very active athletic 18-year-old who was planning on playing hockey in college but has now had to give up all activities and even changed colleges due to not being able to play hockey. I want to be a positive support system for him and need help from others going through a similar situation. The doctors have told him to wear a soft collar neck brace 24x7 and do not activities that cause flexing of the neck. I was so happy to find this forum. Please let me know if there are other parents out there looking for support/info.

[gripit]

Hi Susan!
Im glad to tell you that you have reached the right place.
Im diagnosed with that desease since age 17.
i have never wore a neck brace, nobody can assure me that it will stop the progress of the desease.

please read my post, its right above yours. i was a sporty guy myself but you have to understand that you must focus on your boys mind and spirit. you get to live with it, you study yoursef everyday and the body and mind are adapting automaticlly to the new state.
im 26 and over it, you can say im beyond my peak. from now on its atable and static.
i can help, if you nead help getting through those years im willing to help out.

i sent you private massage with my skype info, contact me if you like.
gripit

[Jacob91]

Hello to everyone. First of all I would like to express utmost empathy to those of you in the same position as me. Until just recently I felt as if I was the only one in the world with this issue, as doctors had made me feel. I would like to iterate that I was never formally diagnosed with Hirayama's disease or monomelic amyotrophy. However, after doing independent research I feel that the symptoms are uncannily parallel to Hirayama's disease in particular so I don't think it's a leap of faith to self-diagnose myself when doctors may simply be unaware of the condition due to its rarity.

I am a 19 year old male with a past including a lot of risk factors that may have caused or spurred on the progression of this syndrome, I remember seeing signs of it when I was around 16 years old I was a competitive gamer and picked up guitar hero. Although I was able to become very skilled at the game I was not able to reach that level without spending an absurd amount of time playing the game. Looking back my disease probably started its onset during the period of maybe a year and a half that I played. However, I believe that it truly helped my distal strength and was so effective a means of physical therapy my physical therapist and I would eventually begin playing it as a part of my exercise routine.

Now that I understand some of the risk factors for the disease I can draw a lot of conclusions about my past that may have contributed to my proclivity to develop it. At around 17 years I broke my left radius and ulna in an accident at school. Prior to this incident, the weakness was only notable in my left hand during the cold and I determined my lack of distal strength and dexterity could be attributed to the fact that I am right-dominant. A year prior to this, I had shattered my right Scaphoid and neglected medical attention for fear of putting my parents at financial risk. When I finally received medical attention I was told that the scaphoid is instrumental in regulating blood flow. I had to wear a cast for 6 months, using my weakened left hand during this time solely. I began to notice difficulty in clipping fingernails and squeezing bottles. This was still circumvented at the time with the application of heat, but now my hand functions rather consistently between room temperature and warmth. However, it is so extremely sensitive to cold that I'd say five minutes of exposure to zero degrees celsius weather renders my hand fairly useless. It may not be worth mentioning but for the sake of completion I'd like to point out that as a younger boy I was able to dislocate the thumb of my affected hand voluntarily. If anyone else was able to do the same that may well be an early indication of susceptibility.

After my left arm got out of the cast my muscular atrophy was very apparent. In fact, I consider this the undeniable onset of my condition. I tried to return to guitar hero and could no longer pass "Through the Fire and Flames" on expert when I once was able to score 95% or better consistently. I kept failing at the "twin solo" and regardless of how much time and energy I dedicated although I felt I possessed talent for it I was unable to return to even a shadow of my previous level. I sought medical attention for my weakness and went through the same series of tests I have read others go through. The EMG test with the electrodes in the muscle with the varying shock intensity was one of them, quite a surprising test for those unprepared (Not that it was awful, but certainly uncomfortable). I had an MRI done that noted a pinched nerve in my C7 vertebrae. I've read a lot about this being implicated as very common among those with Hirayama's disease, but was never formally diagnosed. I underwent ulnar nerve decompression surgery that had very little immediate effect. Physical therapy followed and restored my grip strength but never affected my distal strength. I think all medical professionals had seen me as an unstable teen who would not know how to react if told that my muscle would never return. I became severely depressed over my condition when my girlfriend at the time (the only one I have ever had before and after) became to grow distant and eventually break up with me. I was suicidal to the point I self-mutilated quite often and my left arm was a target of this habit more than any other part of my body. This aggravated the symptoms as you can imagine which were not ameliorated until I had begun to take a more optimistic outlook.

As far as other risk factors go I once considered two that may have contributed in some way to my disposition towards this syndrome. One is that as I was growing up I would walk with my head down looking towards the ground all the time. I had to put forth effort to lift my neck up. This continued to be less and less of a problem over the years though people still to this day point out that I still often stare at the ground, though I consciously try not to. Another possibility is that when I was 14 I was a typical teenager looking for every and any cheap thrill and thought it would improve my reputation somewhat to do a stunt where I would be shot with a hopper of paintballs from 15 feet away without protection. While most of these hit my chest, one or two hit my left arm around my funny bone and caused severe pain. Also, the shrapnel of the paintballs left scars along my bicep. My Humerus protrudes quite prominently (actually moreso than anyone I have met or seen) and was considered by doctors to have aggravated the nerve additionally. When I underwent ulnar nerve decompression my left humerus was actually shaved down. I remember early in my life I would "freak out" my friends and family by lifting my shoulders and dropping my arms. You could clearly see and feel the ulnar nerve below the humerus visibly beneath the skin and I could touch it for a unique tingling sensation.

The pros of the situation and my current outlook are perhaps the redeeming parts of my struggle with this disorder. My right arm is fortunately unaffected and my physical therapist even suggested that it has become stronger to compensate for the other hand. I may not have a normal hand to compare it to, but my right arm does feel as if it is very strong still and I am thankful for that. My left pinky and ring finger I cannot straighten out, though I find that Sam-E and heat therapy have allowed me to function day to day without many people noticing how the disease affects me. My atrophy is so severe on my left arm that there is a deep valley on my forearm between the radius and ulna. It is noticeable about halfway down to my elbow. Also, the muscle between my thumb and pointing finger is very small and atrophied, despite constant conditioning on my end for over a year now. The good news is that I feel the condition has self-regulated and will not continue to degrade. My right arm is probably at risk now for developing weakness because it has to do more than its share of activities, but I have yet to see any signs of that happening yet.

A couple of things I might mention to wrap this up with my experience is that I experience muscle cramps that vary in intensity maybe a couple of times a week. The cramp is very sudden and often debilitating until I manually straighten my fingers out with my other hand. Sometimes I experience chest cramps as well and Tricep cramps, but these are not nearly as severe or even painful. My conditioned has somewhat interfered with others' perception of me and with my goals and ambitions. It makes simpler tasks at work take more effort, as I am a cage cashier at a casino and my hand will cramp often when counting money. I am able to continue playing many games at a competitive level and am even world class at a few games. My coordination is unaffected and I feel as if the condition is at the very least stable. I would like to add that within the past year I have noticed my grip has improved tremendously. I am almost able to pass captains of crush grippers level 1 (60 lbs resistance). When I began physical therapy my grip strength was less than a quarter of that. However, I attribute this primarily to the lack of use and conditioning of unaffected muscles when my arm was broken and subsequently another period of no use following surgery during recovery.

Thank you for reading my battles with this disorder and I am here to empathize for anyone who has to deal with this on a daily basis. It is hard, often easy to lose hope.

[nishantpatil]

Hello all, I m 27 now[ I guess, this problem started when I was 15-16]... I hv visited Neurologist today and as per him I have Hirayama's disease and there is no cure for it ..

It would be great if you have any idea to cure this problem.

Thank you very much!!

[JOSEBA]

Hi everybody, I am from Spain, father of a son that last Christmas was diagnosed Hirayama´s disease. My son is 20 years old and at these moments he is a little nervous; it seems he will need surgery, the doctors say. We have been in two hospitals and have asked to some others doctors about the issue, and we have decided to do that, nevertheless my son is worried at that moment.
He has the typical sinthoms and he has one leg affected as well.

I will be here with you from now on, thank you and take care

[nishantpatil]

Hello Joseba, hope you are doing well and nice to hear that "your son was diagnosed Hirayama's disease".

It would be really great, if you can let me know which processes you followed to diagnose this disease [ my personal email id is [email protected]].

Thanks & Regards,
Nishant Patil

Hi everybody, I am from Spain, father of a son that last Christmas was diagnosed Hirayama´s disease. My son is 20 years old and at these moments he is a little nervous; it seems he will need surgery, the doctors say. We have been in two hospitals and have asked to some others doctors about the issue, and we have decided to do that, nevertheless my son is worried at that moment.
He has the typical sinthoms and he has one leg affected as well.

I will be here with you from now on, thank you and take care

[wastingaway]

Hello everyone,

I apologize for neglecting this thread. Since this is such a rare disease it only gets activity every couple of months, and I honestly haven't checked it until today after receiving a private message from a gentleman.

I feel everyone's pain and now that many years have passed I feel I should provide an update. I understand everyone's concern about being diagnosed with this disease, and I too will admit when I first started suffering symptoms I was in a somewhat panicked state of mind. The important thing, is finding a way to stay positive. In the beginning, I let it consume me. I quit working out, being active, and being as social as I once was. I withdrew from people and only hung around my best friends and family. I remember my parents use to tell me things like, "You have a bad attitude. It's not that bad. People adapt. There are people who use their feet to do things. If it comes to that point, you will adapt." While that is not something anyone wants to hear, in a way they were right. After several years of my withdrawn state of mind, I finally just accepted it for what it was. It was not going to go away, and I still needed to live my life. The important thing to remember is, life does go on. Life is what you make it and people's perception of you is correlated to how you perceive yourself.

I underwent surgery in May of 2007. Looking back, I agree with some of the prior comments. I too (now) feel like my surgeon did not have the heart to tell me my muscles may never come back. After my surgery, I did regain muscle strength. I think it was partially from the muscle stimulator and from me wearing the neckbrace. After the neckbrace came off though, it slowly started to deteriorate again. After that, I think the muscle simulator only slowed the process down. Eventually my insurance quit approving my occupational therapy visits after about 2 years. I can't blame them, two years with little to no progress. The downside to that was I was unable to get pads for my muscle stimulator after that. Eventually once the pads I had went bad I just quit using it. I was still in a bad state of mind, and just didn't want to deal with it anymore. I am not sure if the surgery really helped or not. My hands use to get tired during strenuous activities which would force me to quit. After the surgery the tired feeling subsided, however it didn't stop the deterioration.

Once I moved out of my parent's house I finally started to become more positive and confident. I thank this largely to a co-worker who befriended me. He was always so happy and so positive, even when something was bothering him. His mentality rubbed off on me. People don't want to be around somebody who is miserable, and once I realized this and started making positive changes I noticed a change in people's perception of me. Sure, I can't do things like I once could however I adapted. I can still lift heavy things (although not solely using my finger strength), I could still ride my motorcycle, I could still get a date, I could still live. Dating is still pretty easy. Even with my boney hands I can still lure girls in with my personality -- it may take a little longer but I think it's probably a better relationship in the end because of it. I do not lift weights any more because I worry about my own safety, however I usually just tell people it's because of my back. I have a pretty big scar down my neck and upper back so they generally don't ask many questions. I still work out and make an effort to keep my body as healthy as possible but by doing push-ups, curls, and other exercises.

I will admit, you do lose a few first impression points when people notice something is different. When people shake my hand I usually just squeeze as hard as I possibly can to try achieve a 'normal' hand shake. Sometimes I get over zealous, dont leave my hand open long enough, and get my pinky trapped inside the hand shake since it is always slightly bent. People notice that haha. I am still very conscious of my hands and I generally try to keep my hands as relaxed as I can with as few hand gestures as possible during any sort of presentation. The look you get when people notice is always heart wrenching, but the important thing is to not let it effect you and still radiate confidence. There are, of course, certain jobs I can not get and family members did at one time suggest applying for Social Security. My father looked into it and it seems rather difficult to get this condition recognized and to actually be rewarded Social Security for it. I myself, am strongly against it and probably wouldn't have applied because I'd like to maintain any sort of normalcy that I can. I don't want to be labled, and I can still do many things very well. If anything it motivates me to try harder. I did give up on my dream to become a pilot, however I picked up my second love, IT, and have excelled with it.

The important thing that I would encourage everyone to do is seek medical assistance, accept treatment, and continue to live your life. Everyone has some sort of problem with them, some more severe than others but it's how you address your problems that will define you. In the beginning, I took the wrong route. Once I accepted it, I was able to enjoy life and focus on achieving life goals for myself. I know it's scary at first, but stay positive. You will find a way to make it through this!

[drumett]

My daughter was diagnosed with this illness 2 months ago. She was 13. It's a long story starting when she was 5. She had a back pain; which thinking now, it is possible that it was her neck and not back. She lost mobilization in both hands, arms and legs were weak. After 3 days, she began to gain movement back starting in her fingers. Long story short...she realized 3 years ago that her left hand was smaller than her right. Her left thumb began to 'jump' uncontrollably. We started with dr's again to find out that all of her left side has muscle atrophy. About a year ago now, her right fingers began to jump and then both her hands and arms. I don't think it's as bad now. The dr is trying to figure out why her left leg and foot also has atrophy since it doesn't go with hirayama illness.

I am a 60 year old female who has just been diagnosed with Hirayama's disease (although for the past 3 years they have been calling it monomelic spinal atrophy). I have wasting in the left hand and arm but with exercise am beginning to build muscle up again.

The main point of this note though is to ask if there are any other women out there with this. It appears to be male orientated (and much younger as well) and I cannot believe I am the only woman!

[davisjone]

Hello all, I m 27 now[ I guess, this problem started when I was 15-16]... I hv visited Neurologist today and as per him I have Hirayama's disease and there is no cure for it ..

It would be great if you have any idea to cure this problem.

Thank you very much!!

Unfortunately there is no cure for this disease.. but you can go for the treatment that consists of muscle strengthening exercises and training in hand coordination.

[Hope_1996]

Hey guys and hello Kath Webb!
I'm a 16 year old GIRL from Germany and I've been diagnosted with Hirayama 2-3 months ago.

It's 3 weeks after my surgery now and I'm trying to live with that stupid neck brace I've got to wear. Does anyone have advice for me? Showering and writing etc is pretty hard ( I still go to school). And of course I'm trying to deal with the fact that my hand will surely not be getting better....

I've had problems with my left hand and arm for about a year now. Earlier I only had problems in winter or when my hands were cold. But then my muscles startet to waste away and I could not correctly use my left hand anymore. I went to a lot of doctors and nobody knew..... ( I think you know what I mean )
I first had to wear a neck brace, than I had a surgery and now I've got to wear another neck brace for 3 months. After this I should be able to live without one and do sports like everyone else. My hand should not be getting worse anymore but the doctors don't think it will get better either . Nevertheless I've got to do a lot off physiotherapy to try to improve my strenght and hand coordination.

Kath Webb, you're not alone!

I am so happy I found this forum

[HD mom]

My 17 year old son started having tremors in his left hand, we initially thought it was thyroid or lack of vitamin B and started him on supplements (he is generally underweight and has been very thin from young). Later when the right hand started trembling and he dropped some cutlery, we took him to 2 different GPs. Only one noticed muscle wastage (5 months after he noticed the trembling). We went to see several neurologists, who initially diagnosed it as Multifocal motor neuropathy (MMN). Based on our own Internet search we felt that his symptoms were more Hirayama's. Although the specialists felt it was unnecessary, we got him to start using a neck brace (7 months after the trembling started). Finally the doctors concurred it was probably Hirayama’s Disease after a series of tests for MMN and an attempt at a flexion MRI. Through a Japanese friend, we learnt that Dr Hirayama himself provides concultation once a week at a clinic in Tokyo which sees such patients from all over Japan and the world. We managed to get an appointment through the friend and also enlisted the services of translation agency. The address/details of clinic and agency are provided below. At the clinic, a flexion MRI was done (they did not do any nerve conduction/blood tests) and confirmed it was Hirayama’s disease. According to Dr Hirayama:


⦁ This is a self arresting condition. Symptoms usually start about 1.5-2 years after a growth spurt. Generally affects boys more than girls. The problem is cause by the dura matter around the spinal column. Spinal column basically has grown faster than the matter (casing) around the column. So it is too tight, causing the spine to be squeezed everytime the neck is bent forward. This will cause the blood supply to be cut and overtime the nerve cells in that area are affected. (Imagine bending your toes wearing very tight sock)
⦁ The only thing that can help stop or reduce the progress of the disease is wearing a neck brace. It does not have to be a hard brace. However, he said the one my son was wearing was too soft and recommended something which was slightly harder and which was available for sale at the clinic. He also said that you did not have to wear the collar all the time. For instance, you do not have to wear the collar when sleeping. Also during the day – if you are not planning to flex or bend your neck forward – then you need not – but since this is difficult to stop/predict – it is best to wear it during the day.
⦁ He advised against surgery on the spine (basically done to prevent neck flexing). No long term studies were done on patients who underwent these surgeries. Surgery on the hands were more for cosmetic reasons.
⦁ He said normal exercise/movement of the hand would help – need not do any special exercises (though we are still going to a physiotherapist on our own in our country for regular sessions). Lost muscle is unlikely to be recovered as it is due to nerve damage.He said, the nerve cells which are still working - may try to connect in some way- and so that it is useful to keep trying to do things normally. He said on the other hand, one should not do exercises to such an extent that it damages the muscles further.
⦁ B12 vitamins supplements may help to by encouraging renervation.
⦁ Key thing here is to try and prevent further loss by avoiding bending the neck down for more than 5 minutes at a time.
⦁ The life span of the disease progression could be anyway between 5 to 7 years. The earlier the intervention the better. (After the growth stage, the dura matter around the spine needs to catch up with the spine's growth - hence 2 more years after growth before the condition is arrested)
⦁ During winter – the symptoms may be worse – or a person may sense the loss more – but that does not mean the disease is actually getting worse.
⦁ The whole session lasted about half a day and cost about US$1500 excluding airfare/hotel accommodation/translation fees.
My son's condition seems to have stabilized as of now (almost 11 months since the trembling symptoms started).
⦁ The respective details are as follows:
Kita Hirayama Memorial Clinic (It is Dr Kita's clinic, Dr Hirayama sees patients every wednesday afternoon only). Dr Hirayama looks very sharp for his age (late 70s?), came across as a very gentle humble person. Dr Hirayama and Dr Kita speak a little english, no one else does. You will need a Japanese person to contact the clinic. No email address. The medical interpreter is a necessity.
Clinic Location - https://www.google.com.sg/maps/place...9.702524&hl=en
Kohei Kita, M.D., Ph.D.
Hirayama Memorial
Kita Neurologic Clinic
5-16-16 Shimomeguro, Meguro-ku
Tokyo 153-0064
JAPAN
Phone: 81-3-5768-1235


The medical interpretation service:
EMAIL：[email protected]
TEL:03-3811-8600
FAX:03-3811-8650
Med in Japan
(One session is 4 hours, after that there is an hourly charge)




Hope it helps.

[KelcieLe]

From the information I was able to gather on web, the medical science has not found a cure for Hirayama’s disease. But the form of treatment approach that can be used, include muscle strengthening exercise, as well as training related to the person’s hand coordination.

[Courtney_Meier]

Hello to everyone. First of all I would like to express utmost empathy to those of you in the same position as me. Until just recently I felt as if I was the only one in the world with this issue, as doctors had made me feel. I would like to iterate that I was never formally diagnosed with Hirayama's disease or monomelic amyotrophy. However, after doing independent research I feel that the symptoms are uncannily parallel to Hirayama's disease in particular so I don't think it's a leap of faith to self-diagnose myself when doctors may simply be unaware of the condition due to its rarity.

I am a 19 year old male with a past including a lot of risk factors that may have caused or spurred on the progression of this syndrome, I remember seeing signs of it when I was around 16 years old I was a competitive gamer and picked up guitar hero. Although I was able to become very skilled at the game I was not able to reach that level without spending an absurd amount of time playing the game. Looking back my disease probably started its onset during the period of maybe a year and a half that I played. However, I believe that it truly helped my distal strength and was so effective a means of physical therapy my physical therapist and I would eventually begin playing it as a part of my exercise routine.

Now that I understand some of the risk factors for the disease I can draw a lot of conclusions about my past that may have contributed to my proclivity to develop it. At around 17 years I broke my left radius and ulna in an accident at school. Prior to this incident, the weakness was only notable in my left hand during the cold and I determined my lack of distal strength and dexterity could be attributed to the fact that I am right-dominant. A year prior to this, I had shattered my right Scaphoid and neglected medical attention for fear of putting my parents at financial risk. When I finally received medical attention I was told that the scaphoid is instrumental in regulating blood flow. I had to wear a cast for 6 months, using my weakened left hand during this time solely. I began to notice difficulty in clipping fingernails and squeezing bottles. This was still circumvented at the time with the application of heat, but now my hand functions rather consistently between room temperature and warmth. However, it is so extremely sensitive to cold that I'd say five minutes of exposure to zero degrees celsius weather renders my hand fairly useless. It may not be worth mentioning but for the sake of completion I'd like to point out that as a younger boy I was able to dislocate the thumb of my affected hand voluntarily. If anyone else was able to do the same that may well be an early indication of susceptibility.

After my left arm got out of the cast my muscular atrophy was very apparent. In fact, I consider this the undeniable onset of my condition. I tried to return to guitar hero and could no longer pass "Through the Fire and Flames" on expert when I once was able to score 95% or better consistently. I kept failing at the "twin solo" and regardless of how much time and energy I dedicated although I felt I possessed talent for it I was unable to return to even a shadow of my previous level. I sought medical attention for my weakness and went through the same series of tests I have read others go through. The EMG test with the electrodes in the muscle with the varying shock intensity was one of them, quite a surprising test for those unprepared (Not that it was awful, but certainly uncomfortable). I had an MRI done that noted a pinched nerve in my C7 vertebrae. I've read a lot about this being implicated as very common among those with Hirayama's disease, but was never formally diagnosed. I underwent ulnar nerve decompression surgery that had very little immediate effect. Physical therapy followed and restored my grip strength but never affected my distal strength. I think all medical professionals had seen me as an unstable teen who would not know how to react if told that my muscle would never return. I became severely depressed over my condition when my girlfriend at the time (the only one I have ever had before and after) became to grow distant and eventually break up with me. I was suicidal to the point I self-mutilated quite often and my left arm was a target of this habit more than any other part of my body. This aggravated the symptoms as you can imagine which were not ameliorated until I had begun to take a more optimistic outlook.

As far as other risk factors go I once considered two that may have contributed in some way to my disposition towards this syndrome. One is that as I was growing up I would walk with my head down looking towards the ground all the time. I had to put forth effort to lift my neck up. This continued to be less and less of a problem over the years though people still to this day point out that I still often stare at the ground, though I consciously try not to. Another possibility is that when I was 14 I was a typical teenager looking for every and any cheap thrill and thought it would improve my reputation somewhat to do a stunt where I would be shot with a hopper of paintballs from 15 feet away without protection. While most of these hit my chest, one or two hit my left arm around my funny bone and caused severe pain. Also, the shrapnel of the paintballs left scars along my bicep. My Humerus protrudes quite prominently (actually moreso than anyone I have met or seen) and was considered by doctors to have aggravated the nerve additionally. When I underwent ulnar nerve decompression my left humerus was actually shaved down. I remember early in my life I would "freak out" my friends and family by lifting my shoulders and dropping my arms. You could clearly see and feel the ulnar nerve below the humerus visibly beneath the skin and I could touch it for a unique tingling sensation.

The pros of the situation and my current outlook are perhaps the redeeming parts of my struggle with this disorder. My right arm is fortunately unaffected and my physical therapist even suggested that it has become stronger to compensate for the other hand. I may not have a normal hand to compare it to, but my right arm does feel as if it is very strong still and I am thankful for that. My left pinky and ring finger I cannot straighten out, though I find that Sam-E and heat therapy have allowed me to function day to day without many people noticing how the disease affects me. My atrophy is so severe on my left arm that there is a deep valley on my forearm between the radius and ulna. It is noticeable about halfway down to my elbow. Also, the muscle between my thumb and pointing finger is very small and atrophied, despite constant conditioning on my end for over a year now. The good news is that I feel the condition has self-regulated and will not continue to degrade. My right arm is probably at risk now for developing weakness because it has to do more than its share of activities, but I have yet to see any signs of that happening yet.

A couple of things I might mention to wrap this up with my experience is that I experience muscle cramps that vary in intensity maybe a couple of times a week. The cramp is very sudden and often debilitating until I manually straighten my fingers out with my other hand. Sometimes I experience chest cramps as well and Tricep cramps, but these are not nearly as severe or even painful. My conditioned has somewhat interfered with others' perception of me and with my goals and ambitions. It makes simpler tasks at work take more effort, as I am a cage cashier at a casino and my hand will cramp often when counting money. I am able to continue playing many games at a competitive level and am even world class at a few games. My coordination is unaffected and I feel as if the condition is at the very least stable. I would like to add that within the past year I have noticed my grip has improved tremendously. I am almost able to pass captains of crush grippers level 1 (60 lbs resistance). When I began physical therapy my grip strength was less than a quarter of that. However, I attribute this primarily to the lack of use and conditioning of unaffected muscles when my arm was broken and subsequently another period of no use following surgery during recovery.

Thank you for reading my battles with this disorder and I am here to empathize for anyone who has to deal with this on a daily basis. It is hard, often easy to lose hope.

Re: Hirayama's Disease - Thank you for the detailed information. My son (15) is having his 2nd MRI (w/Flexion this time) to verify the dx of Hirayama's. What do you suggest as a parent I do to prepare and help him understand what's happening to him and what the best possible out come is.

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Re: Hirayama's Disease - Thank you all for sharing your stories and the detailed information. I Need parenting advice.

My son (15) is having his 2nd MRI (w/Flexion this time) to verify the dx of Hirayama's. First MRI was normal. If the flex MRI shows the compression, what do you suggest as a parent I do to prepare and help him understand what's happening. I don't want him to get depressed, but I also don't want to give false hope of returning to "normal" if he won't. He's only noticed the symptoms for about 4 months and once he told me we promptly went for EMG/NCS. Nerver test was good, muscle test not so much.

Does anyone feel the neck brace actually help stop the progression? Should I buy a nerve / muscle stimulator? I feel rushed / panicked to move faster than the doctors are moving - as if every moment we do nothing, he's getting worse. He plays tennis on the Varsity tennis team and I don't want to see him have to stop these activities. Is tennis OK to play?

I have a million questions, but will stop here. Any and all advice / opinions welcome. Thanks for your thoughts and time.
Courtney

[Jacob91]

Hi everyone. I posted on this thread a few years ago and unfortunately my hand has not gotten better since. I have been able to adapt well enough to play video games rather well and I do so quite often to keep the strength up in my hand, but it only seems to slow down the progression of the condition getting any worse. It seems to be stable for the last year or so with the odd tremors and sensations and the sensitivity to cold, but thankfully has not been getting worse due to my constant use of my hands.

However, I wanted to share with everyone that there is hope for those of you suffering from this condition. For many people who suffer from hirayama's disease, the paralysis involved is actually a learned paralysis that is taught following trauma or extension of the nerves stemming from the brachial plexus near the cervix region of the spine. I saw a wonderful tedtalk by a doctor named ramachandran who invented a very affordable and genius solution to treat this type of paralysis. It involves using an illusion to "unlearn" the paralysis associated with this condition. It is very simple and affordable to virtually anyone, and from many case studies appears to work better than physical therapy even after as little as one session. It involves creating a "mirror box" in which you place the affected hand in a box with a mirror facing the unaffected hand, then move the unaffected hand in a way that your affected hand cannot, focusing on the reflection. Some people notice new mobility immediately, while others take a bit more time to make the needed connections to allow for new movement. This technique is often used to alleviate phantom limb pain, but it works in a similar fashion with monomelic amyotrophy. Unfortunately this technique does not work for bimelic sufferers, but great progress has been made on that front medically. I read that a group of doctors in finland have been able to successfully treat both forms of amyotrophy with great success - something that wasn't possible only a couple of years ago. At the rate advances are being made, hopefully those of us who have lived with this condition will be able to get appropriate treatment of one form or another before too long.

Here's a video explaining how one can create and use a "mirror box" to unlearn paralysis by illusion therapy for about US 5$. I recently stumbled upon this and I'm anxious to try it out for myself. I'll keep everyone posted with the results as they happen, it seems that there already have been hundreds of cases successfully treated in this manner. So keep your hopes up everyone! I share your pain and I believe that it will get better before long one way or another.. even if it means we must wait for the advent and implementation of bionic implants. Let me know if this was any use for you!

Link to video - https://www.youtube.com/watch?v=gHFOkVakRkw

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Hi courtney, first of all I would like to say that it must be very difficult being a parent and hearing of such a diagnosis. Being one that has lived with this condition for several years now, I have many things to tell you that should put you at ease. With maintenance and regular use of the hand, the condition will arrest (meaning it stops getting worse) shortly after symptoms appear in many cases. Before I got diagnosed I was a competitive gamer, and I was devastated as I thought that I would be unable to continue doing what I loved. However, thankfully I have been able to remain successful in playing games despite my condition, and I even have dozens of world records that prove that this condition can be overcome if one does not let it affect them negatively. I was at one time quite depressed because of my condition but I have learned to do everything that I would be able to do otherwise without my peers even noticing oftentimes that I have such a condition.

I worked as a cage cashier at a casino for about two years and was able to perform my job quicker and more accurately than many others who I worked with.. even after being around them daily for over a year, no one even noticed that anything was wrong with me - they were quite surprised to hear that I had such a condition. It isn't an effortless thing by any means to overcome, but it will rarely get in the way of a normal life. The most difficult thing I have to deal with anymore is buttoning my pants - but something so minor isn't something that isn't adapted to reflexively. Although there have been times when I was worried that such a condition would affect my love life.. being self-conscious of it has affected it much more than the actual impediment itself. Also, you might look into the very inexpensive mirror box therapy that I mentioned in my previous reply. I have heard many great success stories using this technique and it's so easy to make and use that I highly recommend trying it - it's definitely worth a shot. Also now that we live in an age of ever rapidly advancing technology, bionic implants that mimic natural dexterity are already available and are set to become much more commonplace within five years to a decade, tops. And that may be the worst case scenario, as in just the last couple years successful treatments have been reported much more often than ever before.

I wore a neck brace briefly and unless your son has severe cervical flexion, I wouldn't personally recommend it - though I'm no doctor.. (but I do know a lot more about this subject in particular than many doctors, and am studying to become one someday). Having a neck brace is something that carries a psychological stigma for the wearer that might convince them that they are unable to influence the progression of their condition otherwise, which is only true in the most severe of cases.

I can't give much advice about being a parent, but if I can say anything it's don't coddle him so much for it. I hated having my family treat me and talk down to me as if I was disabled.. I know it's hard not to try to console him and accommodate him, but do so according to his own problems so as to not make him feel more as if he is disabled. If I am any example, I promise that even with severe atrophy, the condition can be overcome and a normal life can be led.

I hope this helps. Feel free to ask as many questions as you want, I'm more than happy to provide any input that I can on the subject.

[Shuntsman]

Hi everyone I would like to talk to you about Hirayma's Disease with you. My doctor says shes 90% sure I have Hirayama's Disease but they can't find anything in my MRI'S every thing is normal. And I have to wear a neck brace. I'm 17 years old and it started when I was 16 so it's been going on for 1-1/2 years. I have weakness in my right hand and it shakes and I have muscle wasting. It is also hard for me to write with a pencil and use my hand. And I was wondering if I can talk to you guys about your experience with this disease because I don't know anyone that has it. If you would like to email me at [email protected] or on this website ( but I really don't know how to use this site) I would really appreciate it. Thanks.

[Shuntsman]

Hi I would like to talk to you about Hirayama disease my email is [email protected] thanks.

[Hirayama Mum]

Thank you for sharing. This has been really helpful.

[qwerta]

Hello all of you thankyou for sharing its the best help about Hirayama
Im supposed to be diagnosed with Hirayama at 2018 on France but actually they dont know what to do with me thats very stressful for me im just 23 years old i have some questions about it
1.do you have pain how where after how many years?
2.do you use medicinal or something so
3.what do you suggest me to do thankyou