Several years ago I blogged about my experience with an autoimmune condition that was only then being identified by the Mayo Clinic, where I had ended up after I could find no one in Chicago to help me. I let this site, and the subject, languish for several years, but was reminded both when I received an email notification of a response to that old blog post, apparently made by one of the few others (only seven have been identified by Mayo) who had this affliction and had been helped by Mayo. So when I received some interesting news from my recent Mayo checkup, I decided to post it here.
At that visit to Mayo, upon questioning my doctor as to whether there had been any new occurances of the condition, I was told that In October of this year, a Mayo team, including my neurologist, had finally presented a paper on my disease at the 2012 Annual Meeting of the American Neurological Association. Thus the disease’s name, chronic microglial encephalomyelitis (CME), is now official.
A summary of the presentation’s contents can be found on line in the ANA’s 2012 Abstract Book, the relevant text of which I offer here:
2012 Annual Meeting of the American Neurological Association in partnership with Association of British Neurologists
October 7–9, 2012, Marriott Copley Place Boston
T1802. Chronic Microglial Encephalomyelitis (CME)
Allen J. Aksamit, Brian G. Weinshenker and Joseph E. Parisi; Rochester, MN
Six patients presented with a unique corticosteroid responsive disabling subacute encephalomyelitis evolving to chronic cognitive and behavioral dysfunction, tremor, myoclonus, and optic nerve swelling. Five patients also had myelopathy with longitudinally extensive T2 hyper-intensity on MRI of the spinal cord. MRI head was characteristic with periventricular diffuse T2 white matter abnormalities with a prominent pattern of radially oriented, linear perivascular increased signal, that enhanced after gadolinium. All had spinal fluid pleocytosis. All had a poorly defined systemic autoimmune illnesses. Brain biopsy in each case revealed prominent perivascular microglial activation and lymphocytic cuffing without demyelination or other specific features. The initial outcome after high dose corticosteroid therapy was favorable. Chronic immunosuppressive therapy was required to prevent relapse. We propose to call this syndrome chronic microglial encephalomyelitis (CME).
Whether this condition represents a single disease entity or a common syndrome of many causes is unknown. However, it can be recognized and differentiated from CNS sarcoidosis, lymphoma, vasculitis, and multiple sclerosis. It can be treated effectively, but requires long term immunomodulatory therapy to prevent relapse.
Study supported by: NONE.