Brief Answer:
Information about BULBAR ALS

Detailed Answer:
Good evening. My name is Dr. Saghafi. I am a neurologist and deal with a variety of forms of ALS at the VA Medical Center in XXXXXXX OH so I've seen a number of cases of the bulbar onset. This is a different variation of the typical onset of classical type of ALS which occurs in the limbs and strikes 75% of people who contract ALS. 25% have the bulbar form which typically is noted as affecting clarity of speech, swallowing function, affectation of respiratory function, and loss of tongue mobility.

The rate at which symptoms progress is highly variable but a similar pattern seems to exist among patients. Usually speech and ability to communicate clearly begin to be affected followed by swallowing which becomes more difficult and finally respiratory status becomes compromised. Respiratory weakness and complications are the usual cause of death in the majority of bulbar onset patients. Other signs and symptoms of the classic form of ALS also affect bulbar patients. Therefore, we'd expect to see weakness in the axial muscles with loss of muscle mass and weight loss as time progresses. About 30% of bulbar onset patients also go through a phase of what is referred to as pseudobulbar affect which means that there is the rapid cycling between extremes of emotions so that one moment the patient may be laughing while the next moment they may be crying.

Furthermore, there is no rhyme or reason to the emotional "lability" or swings. Most people mistakenly believe that patients are depressed or to the contrary extremely happy and even OVERLY happy without cause. However, in reality this lability of emotion is part of the natural course of this disease at least in this population of patients.

I can appreciate why doctors are hesitant to fix the cervical radiculopathy. The problem there is that you would have to be anesthetized with general anesthesia and with the bulbar onset form that affects respiration it would be a huge risk to do that and hope that the respiratory component not be prematurely triggered. Therefore, the surgeons likely believe that the benefits of the surgery do not outweigh the risks of the procedure which could potentially reduce the likelihood of a successful surgical outcome due to respiratory complications.

I am not entirely sure why your upper right abdomen bothers you with pain unless this is in fact your diaphragm or intercostal muscles between your ribs used for respiration that are being affected by the disease. Your doctors taking care of you should be able to help answer this question.

I hope the above information has given you more of a bird's eye view of the bulbar onset form of ALS. If you consider this to be the type of information you were looking for would you please respond with a brief bit of feedback and a STAR RATING for the question. Also, I'd be very appreciative if you'd close the query on your end if there are no further questions or comments on what I've written as this will help expedite and signal the computer to process and archive this response for future use by colleagues as necessary.

You can also send me questions in the future on this or any other topic of medicine by writing me through my webpage at:

bit.ly/drdariushsaghafi

This query required 60 minutes of physician directed review, research, and final response compilation for patient envoy.

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