Neurosyphilis: Recurrence?

[Shark2009]

Contracted syphilis 1969, tx'd w/ IM PCN. 1984 severe neurological/systemic sxs. Dx'd & tx'd for neurosyphilis w/ IM PCN (IM 1x/dayx10days; developed PCN allergy). Sxs totally resolved. Few yrs later, sxs re-emerged, progressing to v.severe w/ nasal septal perforation a biopsy of which showed early granuloma and necrosis which along w/ previous blood tests specific for syphilis provided enough info for dx of neurosyphilis. Given hx allergy to PCN, was desensitized first then given 14 day course of IV PCN q 4 hrs followed by 10 days IM PCN. Symptoms totally disappeared, reflexes returned, no more numbness, coordination returned, etc. (Nasal perforation healed although tended to get inflamed, more so recently.) 2 1/2 yrs ago, started having back problems, foot numbness, vague perineal numbness which I assumed was due to somewhat minor back/buttocks injury. CT's, MRI's (lumbar, thoracic, cervical, abdominal) done with nothing evident that would explain sxs. Over past yrs sxs have progressed. In part due to foot numbness, slipped and fell on stairs 1 month ago. Immediately thereafter, symptoms have become dramatically worse; intolerable, at times excruciating. No ankle reflexes, patellar reflexes present w/ reinforcement. Sxs less intense in am, progress during day, worse at night. Burning, numbness feet/legs. Perineal area severe burning (my gynecologist checked-nothing), spreading pain is also squeezing, pressure, stinging; speads across inside cheeks of buttocks, right where I sit. VDRL neg all dilutions. Neurologist just did FTA-ABS; said "one lab was neg, one was pos" and wants to do empirical tx with IM PCN 1x/wk x 3 wks. I'm desperate to try anything. However, does FTA-ABS really tell us anything since it apparently remains + even after successful tx? And if he thinks it may be a recurrence or re-emergence of neurosyphilis, wouldn't another course of IV PCN seem the more helpful route to go? Your thoughts, suggestions, etc. would be very much appreciated.

[Edward W Hook, MD]

You are now asking questions which I think really should be answered by your own doctors, not me.  An on-line interchange, without the abiltiy to examine you does not provide sufficient information for me to make these judgements.  I must admit however that whn I heard your history and problems I did wonder about vasculitis-type diseases related to polyarteritis.  With respect to your specific questions:

1.  3 weekly injections of benzathine penicillin was used to treat neurosyphilis for years.  Then, in the 1990's, becasue of theoretical concerns, the practice came to be use of higher, IV doses.  Whether they are needed has not beeen studied.  IF this is NS, and given the chronicity of what has gone on, it is reasonable to try the 3 injections of long-acting penicillin over 3 weeks first.

2.  As I said, the best evidence for neurosyphilis would be derrived from a lumbar puncture.

3.  No

4.  See above.  I think you need to discuss this with your doctor

EWH

[Edward W Hook, MD]

Welcome to our Forum.  You history is a complex one and you have already had two very rare complications of syphilis, neurosyphilis and the nasal perforation.  You have also received excellent therapy.  I presume there are no other factors which might modify your situation such as HIV or other illnesses, am I correct?

There are no studies to provide data on precisely what to o in this situation and, as a result, there must be a certain amount of trial and error involved in managing this situation.  It sounds as though your doctors are well informed and taking a reasonable course of action.  I have several comments which may be helpful to you in clarifying some of the things you mention.

Literature from a study published over 30 years ago inidcate4s that a modest proportion of persons with neurosyphilis may have some progression of their symptoms of infection which can occur years after successful therapy. This is thought to be due to the healing (scarring) process which can sometimes follow successful therapy.  Whether this explains your neurological findings (perineal pain, absent reflexes, etc) or not is hard to say.

With respect to your lab tests,  it sounds as though your RPR or VDRL test is now negative but your FTA-ABS remains positive.  This is not at all unusual if your therapy was successful.  Following successful therapy for syphilis the FTA-ABS can remain positive for life.  Other lab tests and specifically a repeat lumbar puncture (spinal tap) would be something I would want to do in the process of trying to work out the origin of your current symptoms.  

Regarding therapy, a small proportion of persons with neurosyphilis do fail therapy. Personally, before I gave a trial course of therapy I would do all that I could to make sure of the diagnosis. then I might consider a therapeutic trial to see if it helped and if that was the course I planned to follow, I personally would favor IV penicillin over 3 weekly injections although the latter may well be effective.  As I mentioned, at this time it is hard to know.  Your doctor may be trying to save you the inconvenience of the IV therapy.

Finally, given the complexity of your situation, I would want to also be sure that there are not other causes of these problems.  That you ha syphilis before oes not mean that your current symptoms could not be due to something else which is completely unrelated.  Certain types of vasculitis, (diseases characterized by inflammation on blood vessels) can cause the sorts of symptoms you describe.  I presume your doctor has already done this.

I realize I have not given you solid direction or advice. This is on purpose.  I have tried to provide you with information but the best way to deal with a complex issue such as you have is to go to a doctor you have faith in and work with him/her to deal with this.  

I hope these comments are helpful.  Feel free to discuss them with your doctors.  EWH

[Edward W Hook, MD]

Glad to help.  Take care.  EWH

[Shark2009]

I can't tell you how comforting it was to read your explanation that 3 wkly IM's of PCN had been tx of choice up until 1990's. Inspires confidence in the direction my neurologist is taking. Sometimes having questions and not having your MD immediately available to answer can be overwhelming and anxiety-provoking. Now I don't have to be on the edge of panic for the next few days til I can talk to my doc. Thank you very much again.

[Shark2009]

I do respect and understand limitations of online communications. Very much appreciate further clarifications. You have been very helpful. Thank you!

[Shark2009]

Thanks very much for your reply. Yes, have had all sorts of labs done over the years which are all neg. Have hypopituitary hypothyroidism since 1970 tx'd with 130mg Nature-throid. I don't recall any tests for vasculitis. Mid-1980's I asked if I could have Wegener's Granuloma-was told no. Last yr I had an EMG which was "perfect" and a NCS showing small fiber neuropathy. Don't have DM, don't drink alcohol. The nasal septal perforation was not inflamed and silented down immediately after (re-emergence of sxs @1992-1998) IV PCN in 1998. However, it has been more inflamed over past 2.5 yrs as sxs have become worse. 1) Would IM PCN (rather than IV) be adequate to improve sxs and thus provide presumptive evidence of re-emergence of neurosyphilis? (If so and if sxs improve, I would push to follow w/ IV. Believe me, IV would be NO inconvenience given what I've been going through!). 2) Are there any labs specific to neurosyphilis that you might recommend? 3) Are there tx's for progressive sxs (that you mentioned could follow even tx cure)? 4) Any tests for vasculitis you might suggest? Thanks again so much!