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What Are The Signs And Symptoms Of Hirayama Disease?
Question: Dr. Spaho, I e-mailed you a few months ago about my 17 year old son. The Mayo Clinic in Minnesota did confirm (October 2015) my belief that he has Hirayama Disease. The pediatric neurologist at Mayo is 90% certain that it is HD, though she was unable to definitely confirm through the MRIs. She has seen one other case in her career at Mayo. She recommends we take a conservative approach by wearing the neck brace for several years, but this is not feasible. The neck brace is causing my son to have neck and shoulder pain he never had before. We consulted with a cervical neurosurgeon at the University of Kansas and he has offered to do a posterior cervical laminectomy with spinal fusion (using rods) on my son. He is said to be a fantastic neurosurgeon. He said the risk of paralysis is about 1%. He has never had an HD patient, or even heard of HD, but he hopes to arrest the disease with the surgery, and I hope that my son can regain some of his hand strength. Both arms are affected -- the right is the worst. Both of his legs have begun twitching infrequently and sometimes his right knee is very painful. The KU neurosurgeon also took his own MRIs. He said he "thinks" he can see the compression is between C5 to C7, and that is what he would open up in surgery. My son wants the surgery. Please, what is your opinion?
Brief Answer:
Cervical fusion and cervical brace aim the same thing.
Detailed Answer:
Welcome back and thanks for asking me directly.
The role of fusion spine surgery in treating Hirayama disease is not well studied, so, it is difficult to predict if the surgery will succeed or not.
However, in my opinion, basically neck brace and cervical spinal fusion both aim to stabilize the cervical spine and to stop further damage of the spinal cord.
It is true, and in this point I agree with the above mentioned Neurosurgeon's opinion that fusion of cervical spine offers definite stabilization, while posterior laminectomy makes more room for the compressed cord, since he sees some compression.
Since the risk of complications is minimal and cervical brace is not feasible, I think your son should have the fusion done.
About the level of fusion, both distribution of symptoms (nerve roots affected) and MRI findings are needed to make the decision.
Hope you found the answer helpful.
I remain at your disposal for further clarifications.
Wishing you and your son good health.
Cervical fusion and cervical brace aim the same thing.
Detailed Answer:
Welcome back and thanks for asking me directly.
The role of fusion spine surgery in treating Hirayama disease is not well studied, so, it is difficult to predict if the surgery will succeed or not.
However, in my opinion, basically neck brace and cervical spinal fusion both aim to stabilize the cervical spine and to stop further damage of the spinal cord.
It is true, and in this point I agree with the above mentioned Neurosurgeon's opinion that fusion of cervical spine offers definite stabilization, while posterior laminectomy makes more room for the compressed cord, since he sees some compression.
Since the risk of complications is minimal and cervical brace is not feasible, I think your son should have the fusion done.
About the level of fusion, both distribution of symptoms (nerve roots affected) and MRI findings are needed to make the decision.
Hope you found the answer helpful.
I remain at your disposal for further clarifications.
Wishing you and your son good health.
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Above answer was peer-reviewed by :
Dr. Arnab Banerjee
Answered by
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