Hirayama's Disease

[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.

[Hirayama Mum]

Thank you for sharing. This has been really helpful.