I discuss issues pertaining to the practice of neuropathology -- including nervous system tumors, neuroanatomy, neurodegenerative disease, muscle and nerve disorders, ophthalmologic pathology, neuro trivia, neuropathology gossip, job listings and anything else that might be of interest to a blue-collar neuropathologist.
Tuesday, August 12, 2008
A 48-year-old man with mental confusion
We had an interesting case here last week of a 48-year-old gentleman with a clinical diagnosis of exacerbation of "some kind of an autoimmune encephalitis". The clinicians were wondering about CNS vasculitis and multiple sclerosis but really didn't know what the cause of the patient's problem was. An MRI showed multifocal edema and infarcts. He had been receiving weekly steroids with some improvement, but was now getting more symptoms of encephalopathy (confusion, weakness, etc.). A brain biopsy was performed, which showed the small vessels stuffed with neoplastic B-cell lymphocytes. The diagnosis based on brain biopsy was intravascular (angiotrophic) large B-cell lymphoma, a rare lymphoma wherein the neoplastic cells lack the capacity for diapedesis, remaining captured within the vascular lumen (pictured) and causing secondary brain infarction.
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So is he going to be OK?
Don't know yet. Probably not. I will keep you updated.
He is stable now. Consulting hem-onc. Rituximab is a promising treatment, but this patient has hepatitis C, so has risk of flare up.
Thanks for the update, Brajesh. Please keep us informed about the patient's course.
As soon as I saw the photo, I knew the diagnosis. Piece o' cake!
Yes, it is an easy diagnosis from a surgical pathology point of view. But the neurologists were scratching their heads for a long time regarding this case. Could it be primary CNS angiitis? MS? They were perplexed until a biopsy was performed.
Yes, it can be hard from a clinical standpoint (which I know from my days as a neurologist).
Its been a long time since I have seen such a clear image. Its always such a shame when what I would consider quite a 'young' person is affected.
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