My partner has a diagnosis of hiryama disease starting 6 years ago which stabilised for a period after surgery and was believed to have plateaued. However he is starting get weaker in the hands again. I'm looking for any case studies on hiryama disease recurring after stopping, and ideally anything on if it stopped again afterwards and how long that too. I already have a couple case studies. Anecdotal experience is also welcome.

Diagnosed when I was 16 or 17. 30 now and truly felt alone when it comes to this disease. Especially because of its rarity there isn’t much info out there about it. But now I found 70+ people with the same condition that I can speak to about it and it’s great thank you Reddit!

Hello everybody. I don’t usually post on reddit but I feel like I need to share my story.

I first started showing symptoms a little over a year ago when I lost the motor function in both my ring finger and middle finger (I may have misspoke in past comments here). I went to an Orthopedist believing it was an issue with my tendons only for them to refer me to a neurologist after a couple visits. Apparently I got lucky and the neurologist I saw was the head of the department at his specific hospital. He was able to immediately give me a diagnosis of Hiroyama and told me he’s only had 1 other patient with it. This was crushing news because it meant that I would no longer be able to participate in sports at school which I had recently become very passionate about. After testing and MRIs to confirm his diagnosis, he recommended a cervical collar but emphasized that there is not much medical evidence for the cervical collar helping. He also told my parents and I about the fusion surgery but my parents said no because of the fact that I am still growing. My parents and I discussed what we were going to do since there is so little information about hiroyama and came to the touch decision that I would wear a cervical collar 24/7. I saw the doctor again 3 months later and the tests showed that the condition was progressing in both arms.

Roughly 6 months after my diagnosis I slightly lost function in my index finger and that is where it has remained. I go back to see the doctor in about a month and we will see if there has been any progression since the last test.

I also have to say wearing that cervical collar around school made me feel like a dog with a cone on and I have stopped wearing it for mental health reasons.

Hey all! Just found out about the community about a week ago.

Im from Buenos Aires, Argentina. Data Scientist of Profession working for US companies.

I played all kind of sports all my life competitively, Rugby, Football (soccer), specially Basketball and lived normally until at age 16-17 I started noticing that when the temperature was cold my left hand did not respond correctly. I could not put all the fingers together, specially little and ring fingers.

As many of you I imagine I started my Medical Adventure visiting lots of different doctors and specialties. Without success the years passed, until I started to see that my left hand was each month losing more and more muscle. Im left handed so it was getting more difficult to write, to play basketball , everything.

I started to quite panic, specially because it has similar syntomps to other Diseases that are no joke like ALS or wose, so I decided to do full weeks of research by myself, specially the googling the syntomps:

-Difficulty to move the hand in Cold weathers. I really hate cold days, it makes controlling the hand waaay harder.

-Specific muscles of the Left hand and arm. Muscular atrophy

-Progression of getting worse

I read a paper on Hirayama and it immediately clicked that it was that, it was exactly what I had. I printed the papers and went to see several Neurologist from Argentina. They had absolutely no idea. In fact the MRI were always positive.

Until I Reached a Neurologist that specialized in Motor neuron diseases which decided to do an MRI with the Neck Flexed as the Paper Suggested. It was super clear, that there was a damage in the nerves. Conclusive.

Now was time for the Treatment.

Many suggest to do nothing, some recommend surgery, some cervic collar (which was an absolutely no for me and my life).

After a lot of consideration I decided to take the chance and have a Surgery which of course had some risk. The idea was to Fix C2 and C3 to limit the flexing on my neck to better protect the damage to the nerves.

After 10 years of the surgery, my hand has no sign of improvement and it has stabilized. The damage is up to a bit below my elbow.

I have never tried to recover the muscles, I stopped doing sports due to hurting my neck a lot and to avoid impact to the nerves there. My life is pretty much normal, I use my left hand on my day to day basis. I tried Muscle Stimulation, going to the Gym and it does not work.

I know it sucks but overall it does not affect life a lot. Hopefully we can help each other and share information as much as we can.

First of all, hello, I caught Hirayama disease when I was 18, sorry for my English, I don't know English very well, I get help from translation. The disease started in my hand, which I actively use. I am now 20 years old. I have muscle atrophy and weakness in my hand. I have cramps and tremors from time to time. To take precautions, my doctor told me not to use a neck brace or to do movements that will strengthen my hand, but I do. What he is wondering is how this disease will affect me in the future, will I be able to drive a car, will I be able to continue using the computer, my writing has changed but will I be able to continue writing? I saw that there are people more experienced than me and I just wanted to write, thank you.

and I'm so glad I discovered this place

Hi guys, I'm a 24-year-old male. I recently got diagnosed with Hirayama disease. I was having hand tremors in my left hand for months, and it got worsened, so I went to the doctor, who conducted an MRI test and confirmed HD. There's muscle wasting in my hand as well; I never noticed it before, as my left hand was always weaker than my right, and my right hand was the dominating one.

I just wanted to know if it is possible to regain the lost muscle and if the tremors ever stop.

I am a 19 year old male who was recently diagnosed with hirayama disease. It is in my left hand. I don't mind it actually, but just after a week of gym, the lateral movement of my fingers has stopped. Is it common or not/ The doctor assured me that the disease is limiting.

Hi Everyone,

Can someone that went through surgery share their experience and how did the recovery went? And how long? Can you please share some details like hand strength evolution and hand morphology in geral improved?

Thanks for the support!

Hey guys has anyone gotten surgery? I am getting ACDF a week and I’m pretty nervous for it. Any input would be great

In the coming year, I am planning to test various approaches in the fight against Hirayama. This after
my surgeons have denied me surgery, as my spinal cord is no longer considered too narrow, which is believed to be the cause of our illness.

With this new information, my research has shifted towards healing my nerves and muscle wasting, rather than finding a cure for Hirayama. Through my efforts, I have received confirmation that healing nerves can be a challenging task, but not impossible.

The following are the subjects I am planning to try:

1. Electromyostimulation: This therapy utilizes electrical impulses to stimulate muscles, which may help improve muscle strength and function, and potentially aid in muscle waste recovery.
2. Ultrasound therapy: This non-invasive therapy uses high-frequency sound waves to stimulate tissues, increase blood flow, and promote healing in nerves and muscles.
3. BPC157 and TB500: These are peptide compounds that have shown potential in promoting tissue healing and regeneration, including nerves and muscles.
4. ARA290: A synthetic peptide that possesses anti-inflammatory properties and may help reduce inflammation and promote nerve and muscle healing.
5. Cerebrolysin: A neurotrophic peptide drug that has demonstrated potential neuroprotective and neuroregenerative effects, which may aid in nerve healing.
6. HGH (Human Growth Hormone): HGH is a hormone that plays a role in tissue growth and repair and may have potential benefits for muscle healing.
7. NGF (Nerve Growth Factor): NGF is a protein that promotes the growth and maintenance of nerve cells and may aid in nerve healing and regeneration.
8. Lion's Mane: A medicinal mushroom known for its potential neuroprotective and neuroregenerative properties, which may support nerve healing and function.

Some of these compounds are banned by WADA (World Anti-Doping Agency) due to their performance-enhancing effects, but since I am not a professional athlete, I feel that these are worth trying. The order and combination of these compounds will be determined based on further research. While these effects have mostly been tested on animals in studies found on sources such as PubMed, anecdotal reports from communities like /r/peptides indicate that many individuals have resolved long-standing issues using peptides or similar compounds, particularly BPC157 and TB500, which are likely the first ones I will test. Additionally, I have been researching different diets, such as /r/carnivore, which have been reported to help people with autoimmune disorders, although I have yet to find something specifically related to Hirayama. Nonetheless, I may still try it for further investigation.

As a side note, to provide some hope but also to somewhat support for my findings, there is a case report of a woman with lifelong neuronal dysfunction that was cured using compounds in this case steroids, which can be read in this study.

​

If any of y'all have some aditional information or have already tried something please inform me.

​

Thanks,

I’ve had HD issues since I was about 17, 45 now.

Over the decades I’ve developed increasing chronic pain in my neck and shoulders near where the lesion is. I get stabbing pains in my neck when I move and my shoulders are spasmed and feel like they’re on fire. And some days I get nerve pain going down my shoulders and arms. Originally all of this was on my left side, where the motor deficits are, but the last couple of years it’s spread to my right side. MRIs don’t show anything abnormal besides the HD lesion, and EMG in my neck shows dystonic activity in places.

It is shitty but tolerable most days, but sometimes it becomes difficult to move or even sit up it’s so bad. And it makes exercising difficult — exercise, including my PT exercises, exacerbates the pain even on the normal days.

I know that pain is not a symptom typically described in HD, but most of the published reports seem to focus on people in their teens and 20s going through the initial diagnosis and stabilization process. Not down the road after decades of dealing with it. There are only a few reports I’ve seen of people who’ve had it for a long time, and I have seen a couple that involved spasms.

So, I don’t feel like it’s the HD itself causing the issues, but probably some downstream consequence of the HD — part of a chain reaction.

Does anyone else experience anything like this? So far my docs and I haven’t had much luck in getting the pain under control.

Hello all -- I've created this sub so that there will be a place for those of us who suffer from this rare condition to connect, discuss our experiences, and learn from each other.

This condition is so rare that even many neurologists aren't aware of it. Despite suffering with it since was 17, it took until age 43 for me to get a diagnosis. And this is after seeing some of the top neurologists in the world when I was 17-19. None of the names for this condition were ever even mentioned even over the 20+ years of doctors tracking my spinal lesion.

Once I saw a new neurologist recently, he took my history, looked at my MRI, and immediately said: "Hirayama. Go look it up. Come back to your next appointment and let me know if it sounds right to you."

And shockingly almost every case report I found described exactly what I had been going through since I was a teen.

So, welcome to r/HirayamaDisease, friends!