Re: Undiagnosed degenerative brain process

Posted By CCF Neuro[P] MD, RPS on December 23, 1998 at 12:36:03:

In Reply to: Undiagnosed degenerative brain process posted by mcastles on December 23, 1998 at 08:01:15:






I wrote some time ago re my 55 year old husband's condition.
In brief:  he suffered rapid memory loss, confusion, disorientation, dilerium, longer term apathy, unwellness.  Has had bloodshot eye/s and severe headaches for a year. CT scan is normal. Lumbar puncture shows increased white cells butnot significantly. Spect scan showed "slow brain waves", IMPORTANTLY, MRE scan shows clear areas of white patches on the white matter -after dye injected also showed some bleeding around those areas.    A frontal biopsy found nothing - normal, no vasculitis.   A stereotaptic biopsy was done today hopefully getting tissue from an affected area (lesion).  Treatment with massive infusion of steroids (prednesilone_ seems to be dampening symptoms of confusion and memory is a little clearer (after 5 days) and bloodshot eyes are gone.  However right eye looks up when he focusses to the left and left eye is getting a tremor.  ALso one side of soft palate is now rigid.  Doctors say this indicates brain stem affected (hence the urgent stereotaptic biopsy). Have suggested:rare form of MS, slow virus (PML??) various types of cancer(lymphoma), infection, virus, fungus, localised vasculitis, but do not know. He has been in hospital fo 2 months and was alot worse in the first few weeks - seems to be better now but by no means "well".  Is very weepy and distressed as greater insight into problems gained follwoing steroid infusion. A raft of specialists here do not know what is wrong, so I've suggested trying internet  - especially getting the MRI scans on the internet to see if any other practitioner sees asimilarity in pattern with a known case. If you have any ideas attall I'd be most interested to hear them. I would also like to know if you have access to an existing medical website or list of teaching hospitals etc, in the States or the world  - in early Jan i am seeing a neuroligist who is interested in puttin gsomething on the net so I need to get some information ready re addresses and sites. Appreciate any assistance or ideas that you can offer.The case has the experts stumped here. Thanks.
Dear Mcastles:
Sorry to hear about your husband.  Sounds like he has gone through alot.  Without the benefit of examining your husband and all the lab tests and imaging studies it is impossible to make other than an educated guess.  The rapid course of his conditon brings out a couple of the diseases you have mentioned.  The first is PML that is a rapidly progressing disorder caused by the JC virus.  It usually occurs in the immunocompromised patient but can occur in the competent host.  The second of these diseases is lymphoma.  It can be very rapid in progression and give the look of PML.  The biopsy should prove very helpful if it is either of these two entities.  The thrid thing that comes to mind in prion disease.  One can send some CSF and have it analyzed for this entity.  In all but the latter disease, steroids would help.  Vasculitis would be evident by the CSF and angiography.  However, we have seen if fool very good radiologist, again the brain biopsy will be telling with this disorder.  Neurolupus is another
entity that is in the diffeential.  However, here the CSF would show a positive ANA.  I would go on the internet, starting with these diseases and use them as a starting point.  I hope the the etiology of your husbands problem becomes clear soon.  Let us know what happens.
Sincerely,
CCF Neuro[P] MD, RPS