Hirayama's Disease

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Originally Posted by 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!!

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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.

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 :)

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.

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.

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Originally Posted by 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.

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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

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.

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.

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

Thank you for sharing. This has been really helpful.

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

Hey Guys,
I'm a law student who was, diagnosed with Hirayama's Disease when I was 19. I'm 21 now, I wear a neck collar and I exercise frequently. Hirayama's Disease affected my life to a major extent, I was a basketball player and I used to play the guitar. Now all I can do is write or type, but that too is slowly being affected. I'm looking for other people with the same disease who have lived with this and have over come the drastic changes that it entails. I feel that considering the fact that there's no awareness about Hirayama's, we should have a community or at least a Facebook group wherein we can contact and share information along with the mental support and hope that it'll bring.

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Originally Posted by LawAlucard

Hey Guys,
I'm a law student who was, diagnosed with Hirayama's Disease when I was 19. I'm 21 now, I wear a neck collar and I exercise frequently. Hirayama's Disease affected my life to a major extent, I was a basketball player and I used to play the guitar. Now all I can do is write or type, but that too is slowly being affected. I'm looking for other people with the same disease who have lived with this and have over come the drastic changes that it entails. I feel that considering the fact that there's no awareness about Hirayama's, we should have a community or at least a Facebook group wherein we can contact and share information along with the mental support and hope that it'll bring.

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Hey there i have symptoms as yours and same age and things u may contact me

[email protected]
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I want to share info with you thanks

Hello everybody my name is Blake I’m from Ontario Canada I have lived with this since I was 15 it was mainly in my left hand gives that claw effect but I can still lift a lot with that hand I lose grip fairly fast but I’m 28 now I’m thinking it might be spreading to other hand up to this point I have done good but recently I have had a lot of anxiety about it never wore collar or seen specialist after I was diagnosed at 15 but I always stayed very athletic my question for someone who may no im thinking about taking steroids to see what effects may happen also Germany is huge into stem cell research I question if some how this could help but I’m going to fight this in my right side just wondering if anybody would no what would happen if I did try steroids or would I be the first to attempt just got excepted for teaching at York university don’t want this to take over my mind this has been on my mind now for a month straight thank god fo this forum because I was starting to get very scared I never really new it could spread witch does suck I hate the tremors but as terrible as this sounds it totally could be worst I started looking up als and that is scared the heck out of me because of my right hand I would really like to no if anybody else done steroids to see what would happen even if it brought back mass or stopped muscle wasting in the right hand

I'm Also suffering from hirayama

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I want to talk to you I'm also surfing from this hirayama

I am also suffering from this disease since 2015.....you can talk to me by mailing ([email protected])

Hi! Are any members still active?

My son (16) has recently been diagnosed with HD and I’m interested to find out more from others about their journey.