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The lawyer who could not swallow his own saliva

August 8th, 2006

uhmc.sunysb.edu
A 52 yo lawyer was referred to your office because of difficulty swallowing his own saliva. He had been in his usual state of health until 3 months ago when he began to have difficulty swallowing. He first noticed the problem with liquids and was still able to eat solids and semi soft foods, however the problem had progressed to the point where he could not swallow any liquids including his own saliva. He carried a handkerchief with him and often sat with it just below his mouth catching the dripping saliva. He denied any other complaints, but when queried admitted that he tended to slur his words in the last few weeks. His physical examination was normal, but he could not swallow. He also had trouble carrying out rapid alternating movements of his tongue (”Say la, la, la,la…”) and palate (”Say.k, k, k, k, …”). His tongue appeared atrophied and had numerous obvious fasiculations on both sides. His deep tendon reflexes were 3+ throughout and his ankle reflexes were 4+ with unsustained clonus. A right Babinski sign was present. Plantar stimulation on the left was equivocal.

Questions:
 
1)
What is the differential diagnoses and what test would you carry out to make the diagnosis?
2)
Name this syndrome. What group of diseases include it?
3)
Give at least two theories of the origin of this group of diseases. What is the evidence for each?
4)
What should be done for the patient and his family?

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