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Dr. Andrew Rynne

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Are difficulty in swallowing food, muscle twitching, and hoarseness signs of a motor neuron disease such as ALS?

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Dr. Olsi Taka

Neurologist

Practicing since :2004

Answered : 3633 Questions

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Posted on Thu, 2 Aug 2018 in Brain and Spine
Question: Hello, I have been suffering from dysphagia for a little over a year, with solid food. I’ve had several tests, barium X-ray swallow, gastroscopy, detailed swallow analysis by a speech language pathologist, 2 MRIs, and most recently, esophageal manometry. The MRIs showed a small lesion on the left side of the pons, and “a few scattered nonspecific focus of increased T2/FLAIR signal within the supratentorial periventricular white matter that are stable. These may reflect mild chronic microangiopathic ischemic white matter changes.” I got the manometry report today, and my gastroenterologist was stumped. It showed mid respiratory resting pressure of the XXXXXXX slightly elevated, incomplete relaxation of the lower esophageal sphincter with the median IRP elevated. A pressure zone with rapid fluctuations of pressure is seen just above the XXXXXXX suggestive of cardiac or vascular artifact. 11 liquid swallows were obtained. Four demonstrated abnormal contractions with simultaneous or premature contractions. The other seven demonstrated normal paristalsis. With a challeng of bread swallows there were more abnormal contractions noted. Of the six bread swallows, four demonstrated a pattern of simultaneous or premature contractions. With impedance studies, it looked like there is good exit of the liquid bolus in the majority of swallows. Even though there was residual pressure in the XXXXXXX it could be seen that there is enough relaxation that the liquid bolus does pass easily through the XXXXXXX Upper Esophageal Sphincter: the resting UES pressure is elevated at 184 mmHg. There is a relaxation of the UES but it does appear that the interval of time for the complete relaxation is very short. The clinical significance of this is unknown.” Impression: This is a difficult study to interpret. I hesitated to report it until I had a chance to review it at the McMaster Motility rounds. All reviewers agree that this motility study does not fit into any recognizable pattern. Whereas most of the swallows appeared to be peristaltic and functional, there were clearly a few abnormal esophageal motility demonstrated, especially with the bread challenge, the functionality of the esophagus appears impaired at times also. Because the majority of swallows are peristaltic even with incomplete relaxation of the XXXXXXX it does not fit the criteria for achalasia. Early or evolving achalasia cannot be excluded. Some kind of spastic motility disorder is potentially suspected.” My question is this: because they couldn’t determine a diagnosis, is there a higher probability that I may have a neuro-motor disease? I’m frightened of ALS, and have a few other symptoms: muscle twitching, and vocal changes..loss of vocal range in singing, a bit of hoarseness. The speech language pathologist noted neck muscle straining in my normal speech. But since I’ve had dysphagia for more than a year, with no corresponding muscle weakness, does that point away from ALS? Also, should the MRI results be a cause for concern? I am trying to follow up with my neurologist, but it can take months to get an appointment. I have an anxiety disorder, so please keep this in mind as you respond. Much appreciated.
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Answered by Dr. Olsi Taka 2 hours later
Brief Answer:
ALS remains highly unlikely.

Detailed Answer:
Hello and welcome back to HealthcareMagic.

I wouldn't be afraid of that test providing any indication of motor neuron disease (ALS). First of all in neurological conditions such as motor neuron disease the difficulty is more in swallowing liquids not with solid food as in your case.
In addition to that reading at that account there doesn't seem to be a paralysis, a weakness of the swallowing muscles in the throat due to the neuron damage (which would be the case in ALS), on the contrary it seems to show an increased tonus, an exaggerated activity increasing the pressure and causing the swallowing difficulty.
The time factor as you say yourself points against ALS as well, other areas are usually involved after a year.

As for the MRI, if chronic microangiopathic ischemic white matter changes, the scattered foci should be a sign of some concern in the long term in the sense that you should pay particular attention to vascular risk factors (high blood pressure, diabetes, smoking, high cholesterol, sedentary lifestyle etc) in order for such changes not to progress more rapidly in the future. Some changes are common as we age, but the issues I mentioned affect blood vessels and accelerate these changes. As for the small signal increase in the pons the MRI is unable to distinguish its cause, but still because it's not increased in size and doesn't enhance after contrast administration it means that it is not an active evolving process may have been there for years.
Whether these changes can cause dysphagia, in theory yes, but it's in much more advanced cases, in my opinion the size and quantity of these mild changes doesn't justify dysphagia. Furthermore again it would be more pronounced for liquids than food.

I remain at your disposal for other questions.
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
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Follow up: Dr. Olsi Taka 7 minutes later
Thank you for your answer, I found it very reassuring.
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Answered by Dr. Olsi Taka 7 hours later
Brief Answer:
You're welcome. Glad to have been of help.

Detailed Answer:
You're welcome. Glad to have been of help.
Above answer was peer-reviewed by : Dr. Kampana
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