ALS Concerns

[DMINOTMAN]

I'm a 43 y.o. male with widespread fasciculations/cramps (legs, arms, back, neck, throat, face) for over 7 months.  Constant pain in center of back between shoulder blades. Occassional burning pain in shoulders/arms/calves. Feel more fatigued, no obvious weakness. EMG in Feb 05 at Mayo Clinic was abnormal (Fasciculation potentials with varying long duration complex MUPs in a right L5, S1 distribution, with fibrillation potentials only seen in the gluteus maximus. Varying MUPs noted in some right upper extremity and left cranial innervated muscles, without abnormal insertional activity. Single fiber EMG of right deltoid, was normal with no increased jitter detected). Physical exam/labs were normal. MRI showed stenosis/disk herniation of lumbar spine. Dx with benign fasciculation's and lumbar stenosis. Repeat EMG in Aug 05. I also have tightness in throat and difficulty swallowing some foods (coconut, grapes, cereal, etc.). Small pieces of food come back up for serveral hours after a meal. No weight loss. ENT exam normal. Barium swallow test normal. Gastro endoscopy/w dil showed narrowing in esophagus. No improvement with Prevacid or with dilation. Gastro wants further testing to r/o motility disorder.
1. If this were ALS wouldn't I have more noticable weakness?
2. Can Bulbar ALS effect throat muscles before effecting the tongue?
3. If this is Bulbar ALS wouldn't it have progressed faster?
4. If this is ALS will my repeat EMG show significant changes?
5. Can benign fasciculation/Cramps effect throat muscles?
6. What other things could this be if it's not ALS?

Thanks!

[sunny67]

I am (almost) 38 years old and a mother of a one and three year old.  Since June (2005) I have noticed muscle twitching in various spots all over my body -- mostly when I am sitting or lying down. I was not particularly concerned until I started having trouble sleeping and would wake up frequently through the night to check if they were still there. The twitches started in my right foot and leg and then spread to the left and upward to my arms and hands. They have subsided somewhat, but I still notice faint and less frequent twitching throughout and mainly in my left hand. I have been to see my doctor and she checked the results of recent blood tests, as well as my reflexes. All were normal. She indicated that ALS is accompanied by muscle weakness and muscle atrophy after that.  I have been in a high state of anxiety since the twitching started and reading up on the possibility of ALS. I guess I am wondering at this point when does muscle weakness in ALS set in? Does ALS normally start with twitching? Does the twitching come and go before other symptoms present themselves? My GP does not seem concerned as she indicated that more than one symptom must be present to warrant any further testing.  This wait and see game is no fun.  It would be great to hear some good news for my birthday!

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

1) yes, fasciculation without objective muscular weakness or wasting is unlikely to be due to ALS, especially over time. Benign fasiculations are more likely to be widespread from the onset

2) both should be affected at the same time especially if present over time, both throat and tongue are involved in swallowing so it would be hard to tell clinically if only one or the other is involved

3) correct

4) yes

5) conceivably yes, but functional disorders of the esophagus like achalasia (spasm of the esophogeal muscles) would be more common cause of your symptoms and may appear as a narrowing on endoscopy.

6) sounds like you have an S1 radiculopathy (pinched nerve root) from the EMG. There may be other pinched nerves in the cervical and thoracic spine causing the other symptoms (and not tested on EMG or MRI). Pinched nerves can also cause fasciculations.Varying MUPS does not mean an abnormality and is usually be just related to variable voluntary movement durign the EMG.