Another Question RE: Bulbar onset

[sunnygal]

First, I want to thank you for your response to my previous post.  I have a little more information.  I had an esophageal manometry, showed peristalsis, but 4/10 swallows hypotensive.  UES was had a resting pressure of 40, a little low.  Is this a sign of weakness?  I am scheduled to have a modified barium swallow on Tuesday.  Does this help dx and/or rule out the possibility of MND?

I went to a neuro last Monday, was told that I have BRISK end of normal of all reflexes (What does that mean?), and bilateral hoffman's sign, no babinski.  Could these be signs?  Both of my hands hurt, thumb area and between thumb and forefinger and don't work quite right.  My arms (just below the elbow towards the wrist) are stiff, and sometimes spasm.

I had an EMG on my rt arm in March and rt leg in June done by a physiatrist, which were normal, except for cold leg conduction problems.

Is a more thorough EMG needed to rule out ALS?  Thanks.

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

The manometry study does not seem to be consistent with a rip-roaring MND. As you have a history of bulimia, you may have some esophageal dysfunction from chronic exposure to the gastric acids. But this is something you should discuss with a GI specialist.

It is quite normal for young patients (you mentioned you were 25 in th last post) to have brisk reflexes. We score reflexes on a scale of 1-4 with 2-3 being normal and 3 a little brisk. 4 is pathological with sustained jerking (clonus) on examination, which your doctor would have mentioned if it was present. Hoffman's can be sign of upper motor neuron disease, but keep in mind that it is present in about a quarter of the normal population (I have a few friends with Hoffman's). And no babinski's is good as this would be abnormal.

We usually also check paraspinal muscles for ALS.  But the need for repeat EMG really depends on the clinical suspicion of your neurologist for true possibility of the disease. If everything has been normal and the story is not typical of ALS, then it is unlikely that another EMG is needed. Good luck.

[sunnygal]

Sorry, I forgot to mention that a BIG sx is a tingling sensation in the tip of my tongue that is constant.  I also feel that I have difficulty speaking, like my tongue won't work right.  I have even limited the amount I speak in fear that it won't come out right, bc usually it doesn't.  It seems some of this may have begun with the feeling of a lump in my throat in September of 2000, and progressed from there.

[Craig]

Hi mommaof3babes. You're OK, kid, trust me.
Frodo

[sunnygal]

Hey Frodo!! You caught me.  I guess I made my identity quite obvious to anyone from neuro by the content of my question.  Yes, I am still worried, trying not to post as much on the neuro forum.  Don't like irritating people.  How are you doing?