Horner's syndrome-BobbHilton could you answer this please?

[Annie62]

I have really wanted to ask you this question but have been reluctant because you are so busy answering others, but I'm afraid you will have left this board in disgust by the time I ask it.  It's kind of complicated (and I don't know if you will know the answer) but I will try to be clear.

I was just dxd a few months ago with autoimmune autonomic neuropathy (after 13 years of trying to get a dx) but no cause was identified (blood work, brain/C-spine MRI and LP all normal).  I have some mild intermittent motor/sensory symptoms that don't fit with this dx, so I have my doubts about it being a primary AAN.

In 1990 I developed what was left-sided ptosis, smaller pupil on left side, and severe hypohidrosis/anhidrosis) of my head, neck and chest.  It might have extended below that but I wasn't aware of it because I don't sweat much on my stomach/legs anyway.  It basically lasted two years, but I had hypohidrosis of left leg on QSART test a few months ago (possibly residual from past Horner's?)

My understanding is there are three kinds of Horner's-1st order/central, 2nd order/preganglionic, and 3rd-order postganglionic, and that 3rd order either involves no anhidrosis or only limited to forhead/nose; 2nd order involves supraclavicular anhidrosis; 1st order involves hemibody.  I have read that when anhidrosis associated with Horner's involves the entire side of the body/hemibody (found in 1st order), that is indicative of a CNS problem, unlike 2nd and 3rd order Horner's.  From my sweating pattern I do believe I had a 1st order Horner's (hence, a CNS problem) and not the peripheral neuropathy/primary autonomic neuropathy I have been assigned.  But since the neurologist that I saw back in 1991 never did any testing other than a chest x-ray, and then the Horner's disappeared, it was never localized.  Do you think from my description of the anhidrosis it was due to a CNS lesion/first order problem?  Thank you much if you do answer this.

[Annie62]

Bob,

If you are still reading this thread, thank you for all the info on MSA.  That is the answer I wanted to hear-that it would be highly unlikely in my case.  The reason I asked was because they could not find a definite cause for my autonomic (and other neuro) symptoms at Mayo.  MSA was mentioned as one of the causes of autonomic neuropathy in the neuro's note that I just received, though she did NOT say/never mentioned to me personally that she thought I had it and thinks I probably have an autoimmune auton. neuropathy (whether secondary or primary).  It eases my mind to know that it's very unlikely to have a negative brain MRI after 17 years of symptoms with MSA.  Also, If I ever get in to see a neuro I will mention the resting supine norephinephine level to see if they think it is worth doing in my case and will read more on it myself.  I never had any evoked potentials done and would like to have the VEP especially done in spite of my negative MRIs and LP.

Regarding anhidrosis in postganglioinic Horner's, I know I have the article saved in my files, but I searched and can't find it and also did a search online.  I did come up with this, though-describing anhidrosis confined to the forehead/nose-don't know if it's describing a postganglionic lesion or not (because I don't understand the anatomy terminology).  It was an article on WebMD/E-Medicine by Malvinder Parmar, MD, stating "With lesions distal to the carotid bifurcation, the lack of sweating is confined to the medial aspect of the forehead and the side of the nose."--don't know if that is postganglionic or not.  If I ever find the other article I saved I will post it.

Thank you again for your time.  It's much appreciated.

[BobbHilton]

So one can have mild sensory and motor problems with autoimmune autonomic neuropathy? yes

I see that , few of your symptomes are unusual e.g I also had blurred vision in right eye that lasted six months back in '98-'99........but I think a visual evoked potential could give some answere
or the worsoning during/after warm shower?Uhthoff's  seen in Demyelination

Also, Is there any way I could have something like multisystem atrophy? Would there have to be abnormalities on brain MRI with that? yes especially after 16 years!!
1-Routine MRI will show Iron deposition in the putamen (part of the basal ganglia) which occurs in over 50% of cases of MSA   and even  a higher proportion of cases of the striatonigral degeneration type (the MSA-P).
there is a recent article out of Germany published in the movement disorder journal (Mov Disord. 2006 Oct;21(10):1674-81)  by using 3D-based volumetry (a simple 3d way of looking at a picture) confirmed that in MSA-C there is significant atrophy in the medulla and pons, vermis and cerebellar hemispheres, caudate nucleus and putamen compared with controls, so it suggested 3D-based MRI volumetry to be  a tool  to follow the progression of MSA  over 2 years (measure disease progression so if you are worried then you could have it done and repeated in 2 years) this article supported an earlier one in Brain. 2004 Jan;127:175-81

if you want to know about MCA-P (though you dont have the symptomes to suggest it) then the best article is in Neuroimage. 2006 May 15;31(1):240-5 using deffusion weighted MRI (DWI used usually for stroke) showed abnormal diffusivity in the putamen (part of the basal ganglia) of MSA-P patients
The rest maybe helpful too
3- Nerve conduction and EMG studies may show subclinical polyneuropathy in multiple system atrophy.
4-External urethral sphincter EMG shows denervation in almost all MSA.
5- As I mentioned earlier , In MSA, dysautonomia is the result of central neuronal loss, therefore, the resting supine norepinephrine level is normal or slightly elevated. While In pure autonomic failure the levels are decreased. {In neither case does the level show its normal increase on standing}

      Not to forget the monthly  e.mail alert I recieved this month from  Archieve of Neurol. 2007 Apr;64(4):pages 545-51 has an article describes the clinical features of 4  families with MSA (I have not have the time yet to review it in details)

    I hope this is helpful
    Bob
    

[BobbHilton]

Sorry I missed your reply yesterday..I'm not sure why?..but I have to rush to do something in the city, I will answer you the minute I'm back


   Bob

[Annie62]

Thank you for answering.  So one can have mild sensory and motor problems with autoimmune autonomic neuropathy?   (Also, I will try to find the article I read in the past regarding 3rd order/postganglionic Horner's that mentioned the anhidrosis of the forehead and one side of the nose.)  Also, Is there any way I could have something like multisystem atrophy?   Would there have to be abnormalities on brain MRI with that?  

Even though I was told I probably have an autoimmune autonomic neuropathy at Mayo, I wasn't given a definite dx (ALL blood work was normal including the neuronal nicotinic acetylcholine receptor) and i think the doctor I saw up there does acknowledge that I have some symptoms that don't fit with that diagnosis (I don't want to put words in her mouth, but that's what it sounds like by what she wrote)--and I agree.  Even though my MRIs and LP were normal she said she couldn't 100% rule out MS.  And I believe from what I know of my past Horner's (anhidrosis down to waist) that it was a 1st order/CNS thing.  It happened so long ago but I have a good memory and also made reference to the sweating pattern in my journals back then.  

This is what I have/have had:  Drooping of right side of mouth associated (in the past) with slurred speech, mild intermittent tingling in left hand/foot and heaviness (like blood is being cut off/tourniquet-like feeling)  in left arm/leg (once with significant weakness/dragging of leg that came on suddenly during ovulation).  I also had blurred vision in right eye that lasted six months back in '98-'99.  I have a thumping on the left side of my throat when I swallow (since '99) and have had some intermittent trouble swallowing.  I also have had decreased sensation in my perineal area/occasional urge incontinence since about 2002 (worse during/after warm shower.  No urinary retention (test normal at Mayo).  I was dxd last August with severe gastroparesis on gastric emptying scan.  I have a slow pulse (in 50s) but don't have any problem with orthostatic hypotension (tilt test normal at Mayo).  SNIF test normal at Mayo.  QSART abnormal with hypohidrosis of left leg.  I also had something happen with my chest after an RTI 13 years ago where I got severe chest pain that eventually resolved after over a year, but I was left with a weak feeling in my chest and slowed respirations.  I also feel very faint at times (not dizzy from low blood pressure, but just ill/faint.  My heart pounds on the left side (but is slow, not fast).  All my symptoms were exacerbated in the extreme by monthly ovulation including the chest symptoms (no longer an issue, just went through early menopause at 43).    Also, infections preceded both the onset of chest symptoms in '94 and exacerbation of chest symptoms and onset of new symptoms in '99.  I think my illness sounds like an autoimmune disease (infections/hormonal influence), and autoimm. diseases do run in my mom's family including MS.  But I don't see how one could have all these symptoms with autoimmune auton. neuropathy.  

I appreciate any insight at all you can give me.  I'm still trying to find a local neurologist who will take me as a patient, so I still have lots of questions regarding my recent (probable, thought no certain) diagnosis.  Thanks again.

[BobbHilton]

Hi Annie
     We use (AAN) for  American Academy of Neurology..
Although my main experience is in general neurology esp the central diseases, and only few centers in the state could give detailed answers to cases like yours, but I know that there is a degree of somatic nerve involvement , so you cant doupt the diagnosis of a primary AAN due to some mild intermittent motor/sensory symptoms.
About the  hypohidrosis on QSART test, I may agree with you as it could a residual of a previous HS because its only on one side , otherwise it could be seen in AAN on both legs , called signs of distal sympathetic failure e.g, difficulty keeping feet warm, increased perspiration (early), anhidrosis (late), acrocyanosis, peripheral pain/edema...the best thing was to investigate fully  then (in 1991) as you did have decreased sweating beyond the 2nd order neuron HS

But for a central lesion, I never needed the sweating issue , as the central failure  declare itself by other association symptomes at presentation or later especially in 16 years ( since 1991), or more accurately in less than  than 10 years.
    I know QSART may not help in differentiating both but a supine plasma norepinephrine may help (not reduced in central causes)

   About the nose? I dont think its a 3rd order neuron HS..where did you read that?

   I  Hope this is helpful

   Bob Hilton