Re: Hirayama's Disease - consulted with Dr Hirayama
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.
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
Quote:
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.
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
Re: Hirayama's Disease - consulted with Dr Hirayama
Thank you for sharing. This has been really helpful.