Dx: Mononeuritis w/multiplex secondary to Vascultis correct?

[mea]

Male,now 46,onset 4/01.Healthy pipewelder 25+yrs,no tobacco,alcohol,caffeine,rec drugs.Prior hlth problems:sciatica(episode every 2-3 yr)affected L leg & sinus problems(1-2 episodes/year)6’0",260lbs(226 normal)2nd milder attack 10/01,& recently 7/02 admit w/renal failure due low bp,dehydration due to meds.

ER admit 2 CCU w/acute systemic illness of 36hrs,earliest test show wbc 17.4,NA 139,K 4.2,CL 103,CO2 27(normal),serum19:50,GLU 127,BUN 38,CREA 2.4,AST 739,ALT 344(high),ALB 3.6(low).RBC,HGB normal d 1,<normal d3(day8 AST 115,ALT 209)Had focal encephalitis/multi foci of cerebritis,bilateral hippocampus,bilateral inferior basa ganglia. Cerebral arterio normal w/o evidence arteritis,narrowing,no blockages,aneurysm,stenosis.  

Neg western blot,+ reaction E.chaffeensis antibody w/IgG titer 1:512,IgM was <1:20,dismissed w/earlier infection,1993-thought 2b brown recluse @the time.No known labs involving heavy metals.Would mangnanese poisoning present w/sudden on-set?

EMG abnormal 4/01 & 10/01 involving L forearm,wrist,hand and leg.Some involvement n ulnar,suggested brachial plexopathy on left.EMG showed only 1+ fibrillation potentials n area innervated by radial,median,femoral nerve. Screening uninvolved nerves were normal,including r/l sural nerves,r/l peroneal,posterior tibial.Sural nerve biopsy 12/01,evidence of inflammation,not conclusive probably due to prior steroid treatment.Cytoxan added to meds 4/02 w/steroid.Does vasculitis seem like the obvious dx(what kind)?Does vasculitis typically present w/sudden on-set?Would u recommend follow-up @CCF confirming Dx?mea

[CCF-Neuro-M.D.-JT]

This is an extremely complex case that requires careful review of the clinical history and diagnostic workup as well as a personally performed neurological examination for a well-formed professional opinion.  What is most concerning is the lesions on the MRI, one of which was ?ring enhancing at one time. CNS vasculitis can do this, but there are other things such as tumor such as lymphoma, infections, and other types of stroke like syndromes that can cause a similar clinical picture. Doubt this is manganese.  It would be unusual for someone who was completely normal to present out of the blue with vasculitis  affecting the brain in such a dramatic manner as well as the same process going on peripherally, meaning all the brachial plexus/arm nerve abnormalities at the exact same time. Usually, it's one or the other. Infection, inflammation, and tumor need to be considered as I'm sure it has been  with all the specialists on board. THe next step may be a brain biopsy if no one can figure this out.

With all that's gone on in the last year and a half, and the progressive nature of the illness I would seek a second opinion at a major academic institution sooner rather than later. CCF has outstanding ID, neuro, and rheum departments. Also, when there's a tough case like this, we have the advantage of stopping our colleagues in the hall from our own department as well as other specialities and asking for their opinion.  If you do decide to come up, start with neuro and BRING THE FILMS, not just the reports. The EMG will likely need to be repeated, but the brain abnormalities should be dealt with first. Good luck.

[lil sunshine]

Hi,
This is really interesting as I am undergoing patch testing next week for Cobalt, chromium, titanium and other metals. The new theory is that I'm allergic to my limb salvage prosthesis which weighs about 10 -12 lbs. But the thing is I have been having some major neurological problems. A lot of mine are predominantly on the left sde like eye twitching/pain, left sided pararylisis{sp and I'm too lazy to look up spelling} to include my face, and other little fun things. I have 3 lesions on my brain but no MS diagnosis. Vasculitus was one they were looking at , one of my brothers is having neuro problems too. I am eager to hear if you have had any testing as heavy metal posioning is supposedly an etiology of Ms, and other demylinating diseases.
Bonnie

[mea]

Additional tests that were completed on my husband at time of 1st attack (16day hosp/10day at acute care facility for OT/PT).

Lyme disease PCR neg, Borrelia DNA, ANA neg and meningitis/encephalitis panel no detectable antibodies. Screened for coccidian virus, echovirus and mumps.  Cryoglobulin was measured,none detectable.

MRI had diffuse enhancing inflammatory changes in hippocampus,genu of internal capsule and globus pallidus with 1.5 cm diffuse ring-enhancing inflammatory foci @ ea globus pallidus,no other lesions in white matter or cerebrum. CSF on day1 showed 5wbc,0 rbc, differential 89% lymphs ,11% monos, protein 71(norm12-60),gluc 79, gram stain neg, fungal prep neg for enc yeast, AFB neg, cryptococcal antigen eg, CSF immunoglobulin G, quantified at 4.2 (norm 0-8.6), VDRL non-reactive.  Toxic elements improved, regained alertness, became interactive but had profound memory impairment. No aphasia or apraxia. Fever gone d2.  He was put on solu-medrol on day 4 (1000 mg for 5 days) and oral after that. Inflammation rapidly improved and was near normal by day 7. Mononeuritis was evident early and remained in his left arm, hand (wrist drop)and leg.  

Cerebral arterio on d11 was normal w/o evidence of arteritis and narrowing, no blockages, aneurysm or stenosis. 3rd MRI on d14 showed improvement w/continued evidence of inflammatory signal change in hippocampi and medial basal ganglia but had diminished dye enhancement. Surface echocardiogram w/M-mode and 2-D echo and color flow – normal.

HIS ILLNESS REALLY STUMPED ALL THE DRS WHO SAW HIM DURING INITIAL HOSP STAY (rheumotologist,cardiologist,infectious disease,gastro)

2nd attack, 10/01, which affected R hand involving ulnar and median nerve and possible seizure. No loss of motion, only numbness,tingling from fingers to wrist. Admitted for 5 day solu-medrol treatment and additional tests. CSF = wbc 1, rbc 1, glucose 96, protein 62, polys 0, lymps 11, monos 14, eos 0. CT w/wo contrast abdomen, pelvis and chest all normal. Abnormal EMG w/severe axonal injury or complete neuropraxis in l median innervated muscles. MRI w/wo contrast. T2 signal hyperintensities involving the basal ganglia bilaterally. Small T2 signal hyperintensities involving the subcortical deep white matter in r frontal lobe vs mucous retension cyst involving l maxillary sinus.Nasal inflammatory disease involving the nasal cavity.

At time of biopsy, 12/01,had Neg HNPP blood study (has bro w/dx epilepsy, sister w/dx MS, bro w/facial numbness, muscle spasms, bro w/carpal problems in both feet) and new EMG study which was abnormal, severe radial study. Could HNPP test be right w/this kind of family history?

Current meds are oxycontin for pain,atenelol,ambien,40 mg prednisone (30 mg on 11/1, 20 mg 12/1) 150/200 mg cytoxan on odd/even days,protonix,celexa. Has experienced normal side effects of prednisone and cytoxan (weight gain, round face, increase b sugar and hair loss-wbc is staying between 3.5-3.9.)After 18 months he still has significant pain (even w/medication),numbness, tingling in L forearm and hand or no feeling at all.L leg is weak,sometimes gives out,uses cane.No change n R hand. R leg only limb not affected. Still does not drive and not able to work.  His cognitive is better, but still has some short-term memory, but improved concentration
He has improved wrist rotation/flexion and can almost make complete fist minus thumb...doesn't wear wrist splint anymore.
Has developed a resting/exertion tremor in left hand

I (we) have been satisfied with our neurologist and have received excellent care and attention and feel fortunate to have him, coming to him from an ER experience.  I’m just thinking that with such a serious illness, should we be content with what we’ve been told so far? I have never dealt with a serious illness before and can’t imagine what the next 10, 20, 30 yrs will be like when looking back at the last 18 months. Thanks for your help! mea

[mea]

I haven't read anything about the absorption of metals into the body...what I've read is more related to inhalation of fumes, particles...let me know how your test(s) come out.  

Since my husband was not tested for metals, wondering if something could have been missed early on,since he has always been a welder and rods have quite a bit of manganese. He had change job locations abt 18mo prior to illness and was working more in an enclosed area(shop)vs working in "field" locations where it was more open. Not sure if testing 18 mo after the fact would even help, that's why I was asking if manganese would present suddenly. Our neuro thought it would have appeared more slowly if it was the cause...which leads to my next comments/theories.....

1)I've had friends since he became sick tell me that his memory had not been so good in the 2-3 mo prior to illness, I had noticed it a little but thought it was more of a husband/wife thing and he just wasn't listening.  ex., I would tell him something important abt kids that he should know and 2-3 days later when it was brought up again he would deny that I had ever said anything and ? me abt why I hadn't. He began a stressful leadership position (not related to employment)6 mo prior to illness...I've thought it could also be related, which would also explain why I wasn't noticing the memory problem, thinking that he just had too much on his mind.  

2)When the sciatic episode started (which was related to moving furniture,painting and cutting trees) he was taking percocet for the pain, my thinking is could the tylenol level have become too high for kidney to handle, which would cause his back to hurt and put more pressure on "sciatica" and that in turn made L leg hurt. The untreated infection, which was huge by time he went to hospital, could have led to inflammation in the joints which would have led to the demylinating condition of nerves. What makes this not hold up...is the encephalopathy could not have been caused by the bladder/kidney infection because of the blood/brain barrier.  

If my "non-medical background thinking" is to hold up on what caused the demylinating condition in his arm and leg, there would have to be a totally separate issues going on at the same time and that's what scares me.  mea

[lil sunshine]

Hi Mea,
I was on up to 1800 mgs a day spread out over a 24 hour period, I don't remember the sequence now. Remember I said I think I am allergic to my prosthesis? well I have learned that Neurontin has Titanium in it, in the form of Titanium Dioxide. I think  that I was helping along my "posioning". I have researched other drugs I have had reactions to and they either contain Titanium or magnesium and a few have Chromium. I am taking no medications now as they tended to make me worse. Now I think I know why. Of course none of the doctors will admit this to me.
Bonnie

[tadams_43]

Does your husband have any morning stiffness in his hands? Short-term memory loss? Rash? Scalp Numbness? Feelings of increased intracranial pressure? Vision problems?

[tadams_43]

Does your husband have any morning stiffness in his hands? Short-term memory loss? Rash? Scalp Numbness? Feelings of increased intracranial pressure? Vision problems?

[tadams_43]

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[tadams_43]

Does your husband have any morning stiffness in his hands? Short-term memory loss? Rash? Scalp Numbness? Feelings of increased intracranial pressure? Vision problems?