Myelopathy workup case

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Timeoutofmind

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Pt has new onset RLE pain, with neuropathic descriptors, anteriorly down the leg, all the way to the sole of the foot. History of neotofibromatosis type 1.

Had L spine MRI before seeing me, unremarkable.

When I examine, strength and sensory intact. Babinski normal. All reflexes normal, except the right patellar is strongly hyper-reflexic.

Obviously I am concerned about myelopathy.

Here is my question. The issue could be in the T or the C spine. I was thinking that given his normal upper extremity exam, would start with T spine imaging. Or would you just order both right away? Anything else you would order off the bat?

Thanks!
 
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Normal strength and sensation. So you are going after pain and an abnormal reflex in a patient with neurofibromas. Lyrica free card while you get MRI T-spine, C-spine. If disc, PT. If tumor, Neurosurgery.
 
Pt has new onset RLE pain, with neuropathic descriptors, anteriorly down the leg, all the way to the sole of the foot. History of neotofibromatosis type 1.

Had L spine MRI before seeing me, unremarkable.

When I examine, strength and sensory intact. Babinski normal. All reflexes normal, except the right patellar is strongly hyper-reflexic.

Obviously I am concerned about myelopathy.

Here is my question. The issue could be in the T or the C spine. I was thinking that given his normal upper extremity exam, would start with T spine imaging. Or would you just order both right away? Anything else you would order off the bat?

Thanks!

If the patient had a myelopathy, you would have BILATERAL hyper-reflexia. The chances of cervical/thoracic myelopathy in the absence of bilateral hyper-reflexia is 1/10,000. Certainly, patients with neurofibromatosis can have neurofibromas in the canal. Did the patient have a positive Hoffman's sign? That is even more sensitive than hyper-reflexia for cervical myelopathy. The finger escape sign is another useful examination maneuver which is rarely performed, but is helpful with bilateral hyper-reflexia. Weakness is more common in the upper leg muscles than lower leg muscles.

Some patients are just hyper-reflexic. However, I can't tell you how many patients I have found with cervical stenosis who were sent for lumbar epidurals for lower extremity pain that turned out to be secondary to their cervical myelopathy.

If there is BILATERAL hyper-reflexia in a patient with neurofibromatosis, even in the absence of Babinski sign and/or clonus, I would image them. A CT is adequate of cervical/thoracic in most cases, however, with a potential neurofibroma you are looking for a potential soft tissue mass which may be only visualized via MRI. A positive Hoffman's sign, of course, should demand a cervical MRI. I have found that few physicians, outside of neurosurgeons or neurologists, even do this anymore, which is somewhat sad.
 
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If someone has typical myelopathy (presents with cervical radiculopathy symptoms but also has gait issues, brisk DTRs), I will just order cervical MRI. If they present with more wacky symptoms, like the ones you're discussing, I'd get MRI cervical and thoracic together. DTRs can be tricky. If someone has decreased or even absent DTRs, they can still be myelopathic if there is an additional lower motor neuron pathology going on at the same time (radiculopathy, peripheral neuropathy, etc). LMN pathology can mask UMN pathology.
 
I had a guy referred to me for RLE weakness, no pain. He was hyper-reflexic in the right leg. I ended up getting a brain MRI and low and behold he had had a stroke. Asymmetric hyper-reflexia makes me think of the brain.

And speaking of long tract signs, does a Hoffman always warrant further workup? I see patients not infrequently that may have a Hoffman on one side. I’ll ask them about neck pain, gait imbalance and difficulty with fine motor movements such as buttoning their shirt. If they say no to all the above I usually don’t go fishing. Should I be?
 
I had a guy referred to me for RLE weakness, no pain. He was hyper-reflexic in the right leg. I ended up getting a brain MRI and low and behold he had had a stroke. Asymmetric hyper-reflexia makes me think of the brain.

And speaking of long tract signs, does a Hoffman always warrant further workup? I see patients not infrequently that may have a Hoffman on one side. I’ll ask them about neck pain, gait imbalance and difficulty with fine motor movements such as buttoning their shirt. If they say no to all the above I usually don’t go fishing. Should I be?

That is true- unilateral hyper-reflexia is going to be caused by compressive, rather than systemic, sources of myelopathy. Instead of cervical stenosis, think of vascular or compressive sources of pathology other than spondylitic sources. Keep in mind that a Hoffman's sign is usually going to be indicative of pathology at C4/C5 or above. C5/C6 and C6/C7 are going to be more common in younger patients, whereas C4/C5 and C3/C4 are going to be more common in older patients.

Review the mechanism behind myelopathies. We have a reduction in the descending inhibitory neural activity, which results in an unrestricted reflex arc. Something up north is causing the descending fibers to no longer hold the reflex in check, allowing the reflex arc to go on uninhibited.

Break it up broadly into compressive and non-compressive sources of myelopathy. We are always looking for compressive pathology only and ignore the other systemic factors (of which there are many) that can result in myelopathy. Keep in mind the number 12mm in your back pocket- this is the cervical canal diameter below which you can have central compressive myelopathy in the cervical canal. This is why it is important for the radiologists to communicate canal diameter so that there is something objective that we can all identify with, rather than severe, moderate, or minimal in descriptions.

Do not disregard systemic and inflammatory causes of myelopathy, as well as electrolyte, infectious, and rheumatologic sources. The incidence of MS presenting in a pain clinic setting is 1/300, so be aware of this. As pain docs, we are conditioned to look for compressive pathology and sometimes disregard vascular, inflammatory, and systemic causes of myelopathy. It is somewhat of a large work-up. I created a monster in my NPs after giving them a morning lecture on myelopathies; they saw hyper-reflexia everywhere after than and I had to show them a few examples of real hyper-reflexia and a real Hoffman's sign so they would stop ordering all the excessive imaging and labs.
 
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That is true- unilateral hyper-reflexia is going to be caused by compressive, rather than systemic, sources of myelopathy. Instead of cervical stenosis, think of vascular or compressive sources of pathology other than spondylitic sources. Keep in mind that a Hoffman's sign is usually going to be indicative of pathology at C4/C5 or above. C5/C6 and C6/C7 are going to be more common in younger patients, whereas C4/C5 and C3/C4 are going to be more common in older patients.

Review the mechanism behind myelopathies. We have a reduction in the descending inhibitory neural activity, which results in an unrestricted reflex arc. Something up north is causing the descending fibers to no longer hold the reflex in check, allowing the reflex arc to go on uninhibited.

Break it up broadly into compressive and non-compressive sources of myelopathy. We are always looking for compressive pathology only and ignore the other systemic factors (of which there are many) that can result in myelopathy. Keep in mind the number 12mm in your back pocket- this is the cervical canal diameter below which you can have central compressive myelopathy in the cervical canal. This is why it is important for the radiologists to communicate canal diameter so that there is something objective that we can all identify with, rather than severe, moderate, or minimal in descriptions.

Do not disregard systemic and inflammatory causes of myelopathy, as well as electrolyte, infectious, and rheumatologic sources. The incidence of MS presenting in a pain clinic setting is 1/300, so be aware of this. As pain docs, we are conditioned to look for compressive pathology and sometimes disregard vascular, inflammatory, and systemic causes of myelopathy. It is somewhat of a large work-up. I created a monster in my NPs after giving them a morning lecture on myelopathies; they saw hyper-reflexia everywhere after than and I had to show them a few examples of real hyper-reflexia and a real Hoffman's sign so they would stop ordering all the excessive imaging and labs.

Which electrolytes abnormalities should we screen for?
 
Which electrolytes abnormalities should we screen for?


Calcium, magnesium, phosphate, and sodium. Although not a defined source of hyper-reflexia, hyper or hypokalemia can cause muscle weakness, so I throw that in there.
 
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