A 25 year old man presented with an acute spinal cord syndrome. Symptoms progressed within hours starting with fluctuating hypaethesia and paresis of the right arm. After 4-5 hours patient had tetraplegia and respiratory insufficiency. After initial imaging of brain and spinal cord as well as lumbar puncture were without any pathological findings, repeated imaging confirmed a myelitis from C1 to Th1. Lumbar puncture showed 80 lymphos. We found no evidence of a (para)infectious cause, only Ana/Anti-Ro was elevated as hint for systemic autoimmune disease. Anti-AQP was negative, visual evoked potentials on intensive care unit were not conclusive.
Patient was treated with high dose methylprednisolone and plasma exchange. Which additional chemotherapeutic treatment would you suggest?
The diagnosis is transverse myelitis?
I'll propose the differential diagnosis of spinal cord infarction, because the disease clinical course is very acute. If the patient showed no response to mPSL or plasma exchange, you should reevaluate this patient's diagnosis.
The patient had diagnosis criteria?
Presence of spinal cord inflammation and CSF pleocytosis, elevated CSF IgG index, or gadolinium enhancement on a spinal MRI
Absence of an identified CNS infection.
Exclusion of acute myelopathy secondary to a known underlying disease, spinal cord infarct and compressive myelopathies
It seems to an acute form of autoimmune myelitis. I I propose to add 6 monthly IV pulses of cyclophosphamide then azathioprine for at least 18 months
Thanks for the answers so long:
@Yoshiyuki: spinal infarction was unlikely due to the very high spinal chord syndrome, but furthermore Imaging was highly atypical, as well as CSF analysis would not fit. Nevertheless we ruled out aortic dissection.
@Marta:
- Presence of spinal cord inflammation YES
- CSF pleocytosis YES (but rather high)
- elevated CSF IgG index YES
- gadolinium enhancement on a spinal MRI YES
- Absence of an identified CNS infection YES
- Exclusion of acute myelopathy secondary to a known underlying disease YES (only Ana/Anti-Ro was positive)
- ...spinal cord infarct and compressive myelopathies YES
@M. Houman: good answer this was one of the options we considered...
This is what J.Birnbaum from J. Hopkins answered:
...my preference for a patient who is so severely ill and has eminent jeopardy of diaphragmatic function is to treat with IV Cytoxan initially at 750mg/m2, and with monthly doses continued for 3-6 months with judicious tapering and adjustments depending on side-effects. Subsequently, oral steroid-sparing therapy wtih cellcept, 2 to 3 grams per day.
In our SLE patients, risk for relapse is Ro antibodies and NMO IgG antibodies. The presence of background rheumatic disease is an overall risk, and if the patient has anti-Ro/SS-A antibodies should ideally also undergo a lip biopsy to confirm Sjogren'ns syndrome .
The question was asked years ago and we do not know how it went with the patient.
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