Odd case, Sequela to intramedullary infarct

[crs2006]

My sister is 22. Last year she underwent corrective surgery for idiopathic degenerative scoliosis (pedicle screw/rod/fusion). During the procedure, MEPs were lost and SSEPs were substantially diminished. Post-op, she demonstrated symptoms consistent with spinal cord infarction at the superior endplate of T4, and spinal shock. Post op she demonstrated areflexive paralysis, however recovered approximately 80% of pre op neurological function in the subsequent 4 months. Prior to the most recent incident she demonstrated the following:

Negative Babinski and Romberg signs.
Minor gait ataxia
Fecal incontinence
Diminished nocioception in the left leg.

There has been one occurance of Autonomic Dysreflexia originating from UTI. UTI was treated and AD resolved.

At the end of September and early October, she demonstrated the following:

Positive Babinski and Romberg signs
increased gait ataxia
Stabbing, non pulsating pain at the T4 dermatome local to the Posterior midline. (rate 8/10 in pain, this has not diminished or resolved)
Ascending paresthesia halting at T4 dermatome
Ascending motor deficit halting at T4 dermatome, severity is in the distal musculature.
Spasticity/clonus (demonstrated after reflexive testing)
Intermittent urinary incontinence with bladder retention (negative for UTI)

Blood cultures and Brain MRI (MS protocol) rule out autoimmune. EMG rules out GBS. CSF biopsy shows WBC within normal levels and no Myelin elements, ruling out infectious, demyelinating, or neoplastic etiology. RBC was normal ruling out hemorrhagic etiology.

Upon review of diagnostic imaging, there does not appear to be a cystic myelopathy. Caudal to the T4 glial scar, a minor T2 hyperintense area is demonstrated in the central aspect of the cord, with a T2 hypointense area in the posterior aspect extending caudally approximately 2 spinal levels. This is possibly a demonstration of post-traumatic myelomalacia or artifact. The cervical, thoracic, and lumbar imaging is inconsistent with a symptomatic syringohydromyelia. some minor hyperintense abnormalities are observed in sagittal imaging, however cannot be corroborated in the axial plane.

I'm not a medical professional, but I was thinking that given the new or enhanced involvement of the posterior column and corticospinal tracts, in conjunction with a lack of MBP in CSF and enhancement of the initial scar ruling out infectious or ischemic factors, a structural defect is to be considered. The cord demonstrates atrophy two spinal segments caudal to the glial scar, and there are two areas in the thoracic region where the cord occupies a highly eccentric lateral area of the spinal column. In these areas on the imaging, the epidural space appears to be obliterated. The two areas are local to T4, and in the scoliotic concavity of the thoracolumar region.

I was thinking that The scoliosis, leads to iatrogenic infarct, which leads to post-traumatic edema, leading to focal arachnoiditis/adhesion. This adhesive insult works in conjunction with a tethering in the thoracolumbar region causing a compressive elongation of the cord resulting in the acute neurological deficit observed today.

We've sent this case out to multiple centers including Craig and The Miami Project. Craig initially agrees with Tethered cord theory, and we are yet to hear from Miami. Weve also sent this to Mayo and to Ohio State.

Any thoughts would be appreciated

[SCI-Nurse]

This is a very complex case. I will ask Dr. Young to comment when he has a chance.

Where is your sister now? Is she getting any rehabilitation therapies?? Appropriate management of her bowel/bladder issues?

(KLD)

[crs2006]

She is currently discharged from the Rehabilitation Institute of Chicago on an outpatient therapy plan.

The Bowel is managed via suppository as it was prior to this injury, the bladder is simply being monitored. The severity of retention/incontinence does not warrant catheterization as of yet.

Any infectious symptoms she has shown have resolved over the last few weeks and were generally attributed to allergic reaction to pharmacologic strategy. I have advised her of the hallmark symptoms of AD and to seek professional help immediately if they are observed.

[crs2006]

Definitely appreciate you passing it on to Dr. Young. It could be very helpful to us.

[SCI-Nurse]

So, it sounds like surgery post op issues/massive swelling issues but Dr Young will discuss that. and other issues as you mentioned.
If she experiences asymptoms of AD she needs to relieve her bladder ( would haave to see the full urodynamics report to really make a comment on that) or do bowel program. Gas can cause AD or any irritation so she needs to look for the irritation and not just seek medical help. I am sure she learned this at RIC.
Is she a year out from injury?
But no matter what these scans show- the body is an amazing thing and I am sure your sister is an amzing person and with hard work she will get as much return function as she can. with her continued outaatient therapy.
CWO

[crs2006]

Yeah, she is over a year and a half post injury. They have her on narcotic painkillers for the pain right now, but she says its pretty unbearable pain

[Wise Young]

crs2006,

Sorry for the long delay in answering your very careful and comprehensive post of your sister's situation. Our server was down for a long time after Sandy. Even though you are not a medical professional, you are clearly very detailed in both your thinking and medical vocabulary.

Many people with scoliosis have tethering of the spinal cord and surgery to correct the curvature frequently causes spinal cord injury. That is the main reason why intraoperative monitoring was started in the 1970's, to avoid such complications due to over-correction. So, it is very likely that your sister had tethering of the spinal cord before and then arachnoiditis after the spinal cord injury that she suffered during the surgery, worsening the arachnoid adhesions and tethering. This would explain the development of syringomyelia and her worsening symptoms. I believe that she should have surgery to untether the spinal cord and to remove adhesions to restore her cerebral spinal fluid flow in the spinal cord.

The question is which surgeon should do this. Obviously, it should be somebody who is very experienced.

Wise.

My sister is 22. Last year she underwent corrective surgery for idiopathic degenerative scoliosis (pedicle screw/rod/fusion). During the procedure, MEPs were lost and SSEPs were substantially diminished. Post-op, she demonstrated symptoms consistent with spinal cord infarction at the superior endplate of T4, and spinal shock. Post op she demonstrated areflexive paralysis, however recovered approximately 80% of pre op neurological function in the subsequent 4 months. Prior to the most recent incident she demonstrated the following:

Negative Babinski and Romberg signs.
Minor gait ataxia
Fecal incontinence
Diminished nocioception in the left leg.

There has been one occurance of Autonomic Dysreflexia originating from UTI. UTI was treated and AD resolved.

At the end of September and early October, she demonstrated the following:

Positive Babinski and Romberg signs
increased gait ataxia
Stabbing, non pulsating pain at the T4 dermatome local to the Posterior midline. (rate 8/10 in pain, this has not diminished or resolved)
Ascending paresthesia halting at T4 dermatome
Ascending motor deficit halting at T4 dermatome, severity is in the distal musculature.
Spasticity/clonus (demonstrated after reflexive testing)
Intermittent urinary incontinence with bladder retention (negative for UTI)

Blood cultures and Brain MRI (MS protocol) rule out autoimmune. EMG rules out GBS. CSF biopsy shows WBC within normal levels and no Myelin elements, ruling out infectious, demyelinating, or neoplastic etiology. RBC was normal ruling out hemorrhagic etiology.

Upon review of diagnostic imaging, there does not appear to be a cystic myelopathy. Caudal to the T4 glial scar, a minor T2 hyperintense area is demonstrated in the central aspect of the cord, with a T2 hypointense area in the posterior aspect extending caudally approximately 2 spinal levels. This is possibly a demonstration of post-traumatic myelomalacia or artifact. The cervical, thoracic, and lumbar imaging is inconsistent with a symptomatic syringohydromyelia. some minor hyperintense abnormalities are observed in sagittal imaging, however cannot be corroborated in the axial plane.

I'm not a medical professional, but I was thinking that given the new or enhanced involvement of the posterior column and corticospinal tracts, in conjunction with a lack of MBP in CSF and enhancement of the initial scar ruling out infectious or ischemic factors, a structural defect is to be considered. The cord demonstrates atrophy two spinal segments caudal to the glial scar, and there are two areas in the thoracic region where the cord occupies a highly eccentric lateral area of the spinal column. In these areas on the imaging, the epidural space appears to be obliterated. The two areas are local to T4, and in the scoliotic concavity of the thoracolumar region.

I was thinking that The scoliosis, leads to iatrogenic infarct, which leads to post-traumatic edema, leading to focal arachnoiditis/adhesion. This adhesive insult works in conjunction with a tethering in the thoracolumbar region causing a compressive elongation of the cord resulting in the acute neurological deficit observed today.

We've sent this case out to multiple centers including Craig and The Miami Project. Craig initially agrees with Tethered cord theory, and we are yet to hear from Miami. Weve also sent this to Mayo and to Ohio State.

Any thoughts would be appreciated

[crs2006]

Thank you for your response Dr. Young. I'm happy to see you are back in business after that storm.

She has a consult with Craig Hospital in December. They are pretty sure that tethering is the root cause here, its simply a matter of designing a surgical strategy. We really appreciate your insight in this matter, as it has offered some clarity and direction in a situation where both have been hard to find.


We've gotten a lot of vague diagnoses or "wait and see" kind of responses, so it's good that we have a lot of eyes on this, investigating it from every angle.

I will definitely update this thread with any new developments in the interest of sharing knowledge of this more "rare" sequence of spinal cord injury.

[crs2006]

The consult with Craig Hospital was today. Dr. Falci has concluded that there is a syrinx present at T4 and there is definitely adhesions and tethering. He is recommending surgical intervention.

Would that be arachnoidolysis, untethering, and shunting of the syrinx?