Meri
Forum Newbie
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CNS Vasculitis as a complication of myelosysplasia:
I am looking for help on this topic as a result of my husband's recent death and not as a student. Please forgive any intrusion. The reason I am here is because a keyword search has brought me to your forum. I have not been able to isolate the thread that brought me here, so I will post my question with the hope that someone here can give me a clue...
My husband suffered from an undiagnosed neurodegenerative disorder for 2-3 years before his death on April 1st. A diagnosis has evaded several top neurologists, even after his autopsy. Without going into the whole story of his symptoms, I have information taken from his neuropathology report. The Final Neuropatholigical Diagnosis was this:
1. Small vessel chronic vasculitis, widespread through central nervous system (see comment)
2. Bilateral damage to globus pallidus, remote
3. Cerebral laminar necrosis, recent
4. Vascular congestion with atypical myeloid cells consistent with myelodysplasia
5. Granulomatous inflammation involving lung section, non-caseating, special stains for fungus and mycobacteria negative.
I only have a vague idea of what it all means because I am not a medical person. I only know what I have learned as an advocate for my husband when my HMO didn't believe he was sick for the first 18 months of his illness. I did know he was diagnosed with myelodysplasia at one point and that he had a very low platelet count three years ago. He also suffered from smoking-related interstitial lung disease. Tests ruled out just about every known neurodegenerative disease. Huntington's, Wilson's disease, Parkinson's... at first I thought he suffered from ALS. The symptoms were very similar. But his muscles were OK. He just eventually lost control over where they moved... he fell down a lot before he was finally bedridden.
The comment says that his diagnosis was reviewed by several neuropathologists. It goes beyond their experience and, as far as they can tell is only the second example of central nervous system vasculitis as a complication of myelodysplasia (Incalzi RA, ArenaV, Capilli A, Gambassi G. Isolated PACNS-like presentation of a systemic vasculitis complicating a myelodysplastic syndrome, Journal of Internal Medicine 2004;255:674-679) The difference I see in my husband from that case was that his vasculitis was isolated to his brain and that he suffered no dimentia. He was clear as a bell until the end, although he could no longer speak.
I don't know whether any of this makes sense, but as I mentioned, my keyword search brought me to your forum. If anyone out there has any knowledge of a case that has any similarities besides the one mentioned in our neuropathology autopsy report, please let me know.
Some great minds have been pondering this question, however, it may be a connection made by the layperson that ultimately brings about an answer. At least that is my hope in this case. Somewhere, somebody has died the same way as my husband... If I can find out who, perhaps I can find the answer to why. What caused this isolated vasculitis? Was it a complication of myelodysplasia...or was it something unknown... and was it ultimately the cause of the isolated pallidal damage and cerebral laminar necrosis that ultimately ended his life?
Thank you for reading this to the end. Any help you can offer is greatly appreciated.
Meri in Washington State
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| BellKicker
Forum Junior
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Posts: 49
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I'm sorry for your loss.
The description you gave us certainly goes beyond anything I could comment on with any sort of authority. I doubt any users of this forum could. Most users are still medical students or fresh out of medical school so it's unlikely we can be of any help.
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| Meri
Forum Newbie
Topics: 1
Posts: 2
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Thanks for the response. I understand that the forum members here are mostly students. I probably wasn't very clear when I posted last night. It was late... Really what I'm after is a clue, not an answer. Maybe someone here has heard of something similar or even experienced something like this within their family... or know of a friend... I'm hoping to stumble onto a clue that the doctors may not have seen because they know better than to be looking in the places that a layperson like me is looking. A clue that I can share with his doctors or the pathology department for investigation. I'm hoping there are some dots in existance that just need connecting. A keyword search brought me here and I don't know why.
I know I'm grasping at straws, but my options are limited. I'm not ready to call it quits. If you or anyone has suggestions of where else I might pose these questions, please let me know.
Many thanks,
Meri
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| usmledoctor1
Forum Junior
Topics: 4
Posts: 77
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sorry for ur loss. i m spellbound as i am just a student and cant comment on the complexity of the disease .
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