Dysarthria w/negative EMG, Nerve Conduction Study, and MRI

[danaka]

My husband started having speech difficulty August of 06.  In December it had progressed where it was much more noticable.  We were concerned about a possible stroke.  He had an MRI which was negative.  We continued on to see a Neurologist which did labs that included Mysthenia Gravis, B12 and Thyroid Panel along with the regular ones, all were negative.  He sent us on the a ALS Specialist at Emory.  There he had an EMG and Nerve Conduction Study.  Both were inconclusive.  We then went back to our PCP which did another MRI which again was negative.

He still has difficulty articulating and seems to get worse at times.  He has no other symptoms.  He seems to clear his throat a lot and has the occasional headache. Do you have any suggestions?

[jean926]

Hi Danaka,
I just came across these postings and found your husband's case puzzling. I know it's been a while but I'm curious to know if you found a diagnosis and if your husband is doing well. Based on all the readings, it sounds to me like it may have been MG. Hope all is well!
jean

[danaka]

Thanks so much for all of your imput!  This is just the information I was looking for.  We are really just at a stand still right now.  I truly am not convinced it is ALS and actually pretty sure it is not.  He was tested for MG but I'm not sure if it was the same test as mentioned by margaretx.  We are scheduled to go back to Emory in June for a follow up.  This time I will be armed with more information.  Thanks again....  :)

[BobbHilton]

Hi Danaka
     Do you read french? because there are few excellent review atrticle in Rev Neurol (Paris) June, 2006 states clearly that the diagnosis of ALS related syndromes REQUIRES the elimination of alternate diagnoses especially if the patient presenting with an isolated unusual symptomes (isolated dysarthria in 9 months) and also these paper reviews the main characteristics of diseases mimicking ALS and the atypical subsets of ALS......So No harm in asking me or any body around beside your doctors who would appreciate a help for sure,  before waiting and  accepting a deadly disease like ALS especially if a treatble condition like Wilson disease is a possibility!!

   No one said it better than Belsh in  Neurology J. 1999;53(8 Suppl 5):S26-30; discussion S35-6  "Although the diagnosis of ALS remains a clinical one, laboratory testing can be used to exclude other diseases "

   Hope this is helpful

   Bob  Hilton

[Margaretx]

The differentials for bulbar ALS are few.  Once stroke, brain tumor and MG have been ruled out, and assuming there are no other obvious symptoms you haven't mentioned, ALS is the most likely diagnosis--which is, of course, why you were sent to an ALS center.  The doctor there, who has examined your husband, tested his reflexes and performed his EMG, is in the best position to offer a diagnosis and alternative.  Your best answer will come from him/her.  The clear MRI's make tumor or stroke very unlikely but unless a single-fiber EMG has been performed I would think MG is still a possibility (sero-negative MG is not uncommon). The SFEMG is the gold standard in diagnosing MG and I'm a little surprised that Emory didn't perform one, but maybe there's something else about your husband's condition or prior testing that ruled it out for this doc.  You might want to ask him about it.  At any rate, I assume the EMG will be repeated in six months or so.  Often it's just a waiting game.

[BobbHilton]

Its somwhat uncommon to have normal swallowing with dysarthria but its not unheard of.
So a structural lesion/stroke are excluded by neuroimaging , you are left with a possibility of a biochemical abnormalities  (I assume he was not on any meds as u did not mention that) so he  should be tested for
1- Vitamine E level
2- Wilson disease (Dysarthria is extremely common)
3- Neuroacanthocytosis (needs a special handling of a blood sample to do a peripheral smear)

other possibilities
1- Early amyloid deposits
2- Lingual dystonia (is there any thing he could do to make his speech better like tuching his chin..ext)
3- Lambert-Eaton syndrome (they might have checked it while doing EMG for the myasthenia),
4- In kids or very younge adult if they had a throat infection by Group A beta-hemolytic streptococci they may get  dysarthria (common),

        I Hope this is helpful

        Bob  Hilton

[danaka]

They didn't give a specific type.  I will have to check with his doctors to see if there was one given.  Yes, he did have contrast with both MRIs.  The one that was done in the ER was an open MRI and the 2nd one he had was a closed.  Both w/ and w/o contrast.  They were clear.  The problem seems to be specifically involving his tongue.  He has never had a loud voice, somewhat soft and airy.  He was tested by a speech pathologist and his swallowing was good, enough that they didn't think he needed a Barium Swallow.

[BobbHilton]

Hi,
  Dysarthria is several types!! have you given a name of a spcific type? e.g flaccid, spastic, hypokinetic, hyperkinetic, ataxic..ext? each one could lead you to a different direction esp no other feature at this pont....Was the MRI done with GD (contrast)..any input from a speech pathologist ?

  Bob Hilton