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Dr. Saghafi, I Have Been Diagnosed With CNS Vasculitis And

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Posted on Thu, 21 Mar 2019
Question: Dr. Saghafi, I have been diagnosed with CNS Vasculitis and prescribed Cytoxan. I want to get a second opinion at the XXXXXXX Clinic by XXXXXXX Calabrese, DO who is the Vice Chair of the Rheumatologic and Immunologic Disease department. I read about his work diagnosing the disease and was impressed at how thorough he is, far more than my current doctors. He has listed tests that must be positive to get the diagnosis. I am not convinced that my tests meet the criteria. Would you please look at them and tell me what you think?

1. Inflammatory spinal fluid. There must be modest lymphocytic pleocytosis and elevated protein level. My CSF lymphocytes, manual was 48 and my CSF protein was 54.
2. IR Cerebral Angiogram must suggest CNS Vasculitis. The IR radiologist said “ CNS vasculitis is not completely excluded. Clinical correlation is recommended.“
3. The MRI must be abnormal, with punctate infarctions and parenchymal destruction. Mine said I had multiple chronic lacunar strokes, small vessel ischemic changes, and brain volume loss.
4. A brain biopsy, SPECT and PET must be done. I can’t get anyone to do those.
5. CNS infection and malignancy must be ruled out. I haven’t been tested for those.

Do my test results qualify. His article said pristine MRIs and spinal fluid rule out CNS Vasculitis. Are mine considered pristine?

Here is the article:
https://www.mdedge.com/clinicalneurologynews/article/133549/lupus-connective-tissue-diseases/seven-shortcuts-help-diagnosis

Attached is the list of materials I have to submit to get the second opinion.
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Follow up: Dr. Dariush Saghafi (0 minute later)
Dr. Saghafi, I have been diagnosed with CNS Vasculitis and prescribed Cytoxan. I want to get a second opinion at the XXXXXXX Clinic by XXXXXXX Calabrese, DO who is the Vice Chair of the Rheumatologic and Immunologic Disease department. I read about his work diagnosing the disease and was impressed at how thorough he is, far more than my current doctors. He has listed tests that must be positive to get the diagnosis. I am not convinced that my tests meet the criteria. Would you please look at them and tell me what you think?

1. Inflammatory spinal fluid. There must be modest lymphocytic pleocytosis and elevated protein level. My CSF lymphocytes, manual was 48 and my CSF protein was 54.
2. IR Cerebral Angiogram must suggest CNS Vasculitis. The IR radiologist said “ CNS vasculitis is not completely excluded. Clinical correlation is recommended.“
3. The MRI must be abnormal, with punctate infarctions and parenchymal destruction. Mine said I had multiple chronic lacunar strokes, small vessel ischemic changes, and brain volume loss.
4. A brain biopsy, SPECT and PET must be done. I can’t get anyone to do those.
5. CNS infection and malignancy must be ruled out. I haven’t been tested for those.

Do my test results qualify. His article said pristine MRIs and spinal fluid rule out CNS Vasculitis. Are mine considered pristine?

Here is the article:
https://www.mdedge.com/clinicalneurologynews/article/133549/lupus-connective-tissue-diseases/seven-shortcuts-help-diagnosis

Attached is the list of materials I have to submit to get the second opinion.
doctor
Answered by Dr. Dariush Saghafi (25 hours later)
Brief Answer:
CSF not pristine....but is it VASCULITIS??? Good question.

Detailed Answer:
Many thanks for once again reaching out for another opinion on your case. And thank you for bringing to my attention once again the short report that Dr. Calabrese published in CLINICAL NEUROLOGY NEWS. I actually read articles and pick the "good" ones regularly to catch up on a few things. And since I do have several patients in the practice who do require treatment for a variety of vasculitides (includes CNS Lupus, etc.) I am always happy to read opinions from Dr. Calabrese. He is a recognized author in this field. I did read his article and am in agreement with his opinions on how diagnosed a vasculitic disease of the CNS should be approached. Let me say the following before going on to your points on your spinal fluid and other things:

Whenever I read results and make interpretations of remote data I PREFER to be more conservative with opinions unless patients provide actual TEST DOCUMENTATION as opposed to making judgements on specific numbers. There may be some exceptions to this rule...but essentially numbers can be tricky when isolated since lab reference values change and normative ranges found in text books or even other publications are not necessarily always accurate with different subpopulations.

Now to your questions:

1. Inflammatory spinal fluid. The commonly held upper limit of normal protein in CSF is taken to be 45 therefore, 54 would appear to be slightly elevated and merit further analysis. In my practice I would interpret this protein level in the face of the TOTAL CELL COUNT which does include lymphocytes. In your case you've mentioned 48 lymphocytes, however, I do not have a total cell count nor the volume in which these cells were counted. Typically, the unit of measure is the MICROLITER. If they did a manual cell count then, someone may have seen the need to go through and check numbers that are typically obtained through automated means. However, I can't say how elevated that is over normal without the full rest of the cell differential in place. My guess is that with the slightly elevated protein levels the actual % count to total WHITE CELLS with a count of 48 lymphs in some standard volume could be elevated...but it's not extraordinarily high in terms of what I've seen in true and present vasculitic CNS disease.

2. The angiogram is also not entirely conclusive on the presence of a vasculitic PICTURE...but as you know...pictures do not always TELL the truth and sometimes they can MISLEAD people into thinking things are opposite from how they are. Therefore, the radiologist's read in this case would be UNHELPFUL to me if I had to make a call of VASCULITIS or NO VASCULITIS.

3. The concept of PUNCTATE infarcts is tricky and even good rheumatologists use the term without being entirely sensitive to spectrum of radiographic abnormalities that would be acceptable to neurologists and not just acceptable but confirmatory enough to fit the criteria necessary for vasculitic changes to be recognized. Multiple chronic lacunar infarcts are not typically looked at by neurologists as PUNCTATE infarcts but again, this is also why when I see patients with possible vasculitic presentations I READ THE MRI myself and if necessary call the radiologist to discuss things with them. In your case I would say that CHRONIC MULTIPLE LACUNAR INFARCTS, SMALL VESSEL ISCHEMIC CHANGES (here are what WE more commonly refer to as punctate infarcts), and volume loss would fit the criteria for an abnormal MRI.

4. With all due respect to the good Dr. Calabrese stating the underutilization of brain biopsies I disagree that it is necessary to be that invasive in all but the most controversial or urgent of cases when neither the clinical presentation nor laboratory can help sway things in one way or another. I am definitely trained by mentors who never reached for brain biopsy anymore frequently in locking in a diagnosis of CNS vasculitis any more frequently than they did when diagnosing PARKINSON'S DISEASE, ALZHEIMER'S, or MULTIPLE SCLEROSIS. Make sense. I don't know your case any more than you've presented and do not have the privilege of knowing your neurological history first hand but simply based on given data I would not want to make a brain biopsy a priority in this case.

5. I certainly agree that CNS infection and malignancy absolutely should be ruled out but since I do not have copies of all lab work etc. I don't know that it wasn't done but that you're simply not aware of the NEGATIVE RESULTS. Certainly, you would agree that your MRI likely is negative for anything such as a solid tumor or even lymphoma since the radiologist would've called that...but if you're referring to "hidden cancers" such as occurs in PARANEOPLASTIC settings then, of course, those require other levels of testing so...perhaps, those have not been completed. By the same rationale, CNS infection is likely ruled out since the clinical presentation of a meningitis (bacterial or viral) would've been picked up first and certainly a CSF cell count would've demonstrated clear evidence of such infection. The same could be said for other common infections of the CNS such as toxoplasma, cysticercosis, and many others...so we know those probably don't exist. But something like Lyme's? Has that been possibly tested for or occult cryptococcal infection which is not always ROUTINELY looked at in CSF? I don't know about those entities.

So to finally answer the final question of yours in terms of your CSF being pristine? Well, again, since I don't have ALL THE RESULTS at my disposal to make a best judgement I would say, it's hard to say with certainty...but the protein levels appear to be slightly elevated over normal and there may be a lymphocytic infiltration or pleocytosis.

So as I shove off to the hospital this morning to do my best to stamp out neurological disease wherever it rears its unwelcome head in our veterans in XXXXXXX ..I bid thee....a solid Cheers my dear!

As always I'm most appreciative of your complimentary comments and hope I've provided useful or helpful information to your question. I would be interested in getting some updated information on how things are going in the near future...and if you were planning on coming and visiting with Dr. Calabrese....you're only a couple of highways and bridges away from Parma Neurology! HA!

You can always reach me here at: www.bit.ly/drdariushsaghafi for this or other questions. Be well

This query has utilized a total of 41 minutes of professional time in research, review, and synthesis for the purpose of formulating a response.


Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
doctor
Answered by Dr. Dariush Saghafi (0 minute later)
Brief Answer:
CSF not pristine....but is it VASCULITIS??? Good question.

Detailed Answer:
Many thanks for once again reaching out for another opinion on your case. And thank you for bringing to my attention once again the short report that Dr. Calabrese published in CLINICAL NEUROLOGY NEWS. I actually read articles and pick the "good" ones regularly to catch up on a few things. And since I do have several patients in the practice who do require treatment for a variety of vasculitides (includes CNS Lupus, etc.) I am always happy to read opinions from Dr. Calabrese. He is a recognized author in this field. I did read his article and am in agreement with his opinions on how diagnosed a vasculitic disease of the CNS should be approached. Let me say the following before going on to your points on your spinal fluid and other things:

Whenever I read results and make interpretations of remote data I PREFER to be more conservative with opinions unless patients provide actual TEST DOCUMENTATION as opposed to making judgements on specific numbers. There may be some exceptions to this rule...but essentially numbers can be tricky when isolated since lab reference values change and normative ranges found in text books or even other publications are not necessarily always accurate with different subpopulations.

Now to your questions:

1. Inflammatory spinal fluid. The commonly held upper limit of normal protein in CSF is taken to be 45 therefore, 54 would appear to be slightly elevated and merit further analysis. In my practice I would interpret this protein level in the face of the TOTAL CELL COUNT which does include lymphocytes. In your case you've mentioned 48 lymphocytes, however, I do not have a total cell count nor the volume in which these cells were counted. Typically, the unit of measure is the MICROLITER. If they did a manual cell count then, someone may have seen the need to go through and check numbers that are typically obtained through automated means. However, I can't say how elevated that is over normal without the full rest of the cell differential in place. My guess is that with the slightly elevated protein levels the actual % count to total WHITE CELLS with a count of 48 lymphs in some standard volume could be elevated...but it's not extraordinarily high in terms of what I've seen in true and present vasculitic CNS disease.

2. The angiogram is also not entirely conclusive on the presence of a vasculitic PICTURE...but as you know...pictures do not always TELL the truth and sometimes they can MISLEAD people into thinking things are opposite from how they are. Therefore, the radiologist's read in this case would be UNHELPFUL to me if I had to make a call of VASCULITIS or NO VASCULITIS.

3. The concept of PUNCTATE infarcts is tricky and even good rheumatologists use the term without being entirely sensitive to spectrum of radiographic abnormalities that would be acceptable to neurologists and not just acceptable but confirmatory enough to fit the criteria necessary for vasculitic changes to be recognized. Multiple chronic lacunar infarcts are not typically looked at by neurologists as PUNCTATE infarcts but again, this is also why when I see patients with possible vasculitic presentations I READ THE MRI myself and if necessary call the radiologist to discuss things with them. In your case I would say that CHRONIC MULTIPLE LACUNAR INFARCTS, SMALL VESSEL ISCHEMIC CHANGES (here are what WE more commonly refer to as punctate infarcts), and volume loss would fit the criteria for an abnormal MRI.

4. With all due respect to the good Dr. Calabrese stating the underutilization of brain biopsies I disagree that it is necessary to be that invasive in all but the most controversial or urgent of cases when neither the clinical presentation nor laboratory can help sway things in one way or another. I am definitely trained by mentors who never reached for brain biopsy anymore frequently in locking in a diagnosis of CNS vasculitis any more frequently than they did when diagnosing PARKINSON'S DISEASE, ALZHEIMER'S, or MULTIPLE SCLEROSIS. Make sense. I don't know your case any more than you've presented and do not have the privilege of knowing your neurological history first hand but simply based on given data I would not want to make a brain biopsy a priority in this case.

5. I certainly agree that CNS infection and malignancy absolutely should be ruled out but since I do not have copies of all lab work etc. I don't know that it wasn't done but that you're simply not aware of the NEGATIVE RESULTS. Certainly, you would agree that your MRI likely is negative for anything such as a solid tumor or even lymphoma since the radiologist would've called that...but if you're referring to "hidden cancers" such as occurs in PARANEOPLASTIC settings then, of course, those require other levels of testing so...perhaps, those have not been completed. By the same rationale, CNS infection is likely ruled out since the clinical presentation of a meningitis (bacterial or viral) would've been picked up first and certainly a CSF cell count would've demonstrated clear evidence of such infection. The same could be said for other common infections of the CNS such as toxoplasma, cysticercosis, and many others...so we know those probably don't exist. But something like Lyme's? Has that been possibly tested for or occult cryptococcal infection which is not always ROUTINELY looked at in CSF? I don't know about those entities.

So to finally answer the final question of yours in terms of your CSF being pristine? Well, again, since I don't have ALL THE RESULTS at my disposal to make a best judgement I would say, it's hard to say with certainty...but the protein levels appear to be slightly elevated over normal and there may be a lymphocytic infiltration or pleocytosis.

So as I shove off to the hospital this morning to do my best to stamp out neurological disease wherever it rears its unwelcome head in our veterans in XXXXXXX ..I bid thee....a solid Cheers my dear!

As always I'm most appreciative of your complimentary comments and hope I've provided useful or helpful information to your question. I would be interested in getting some updated information on how things are going in the near future...and if you were planning on coming and visiting with Dr. Calabrese....you're only a couple of highways and bridges away from Parma Neurology! HA!

You can always reach me here at: www.bit.ly/drdariushsaghafi for this or other questions. Be well

This query has utilized a total of 41 minutes of professional time in research, review, and synthesis for the purpose of formulating a response.

Note: For further follow up on related General & Family Physician Click here.

Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
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Dr. Dariush Saghafi

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Practicing since :1988

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Dr. Saghafi, I Have Been Diagnosed With CNS Vasculitis And

Dr. Saghafi, I have been diagnosed with CNS Vasculitis and prescribed Cytoxan. I want to get a second opinion at the XXXXXXX Clinic by XXXXXXX Calabrese, DO who is the Vice Chair of the Rheumatologic and Immunologic Disease department. I read about his work diagnosing the disease and was impressed at how thorough he is, far more than my current doctors. He has listed tests that must be positive to get the diagnosis. I am not convinced that my tests meet the criteria. Would you please look at them and tell me what you think? 1. Inflammatory spinal fluid. There must be modest lymphocytic pleocytosis and elevated protein level. My CSF lymphocytes, manual was 48 and my CSF protein was 54. 2. IR Cerebral Angiogram must suggest CNS Vasculitis. The IR radiologist said “ CNS vasculitis is not completely excluded. Clinical correlation is recommended.“ 3. The MRI must be abnormal, with punctate infarctions and parenchymal destruction. Mine said I had multiple chronic lacunar strokes, small vessel ischemic changes, and brain volume loss. 4. A brain biopsy, SPECT and PET must be done. I can’t get anyone to do those. 5. CNS infection and malignancy must be ruled out. I haven’t been tested for those. Do my test results qualify. His article said pristine MRIs and spinal fluid rule out CNS Vasculitis. Are mine considered pristine? Here is the article: https://www.mdedge.com/clinicalneurologynews/article/133549/lupus-connective-tissue-diseases/seven-shortcuts-help-diagnosis Attached is the list of materials I have to submit to get the second opinion.