Rare disease removes ability to use words

Dear Dr. Roach: I am an 84-year-old man diagnosed with primary progressive aphasia. The neurologist says nothing can be done for it. What can you tell me?

J.M.M. Primary progressive aphasia is a progressive inability to use words and language. It is not related to other neurological diseases. It is a rare disease, at least compared with Alzheimer’s disease. The condition may begin as early as a person’s 40s and is caused by degeneration in nerve cells. In about 30 per cent of people, PPA is caused by the same process as Alzheimer’s disease, but in the common form of Alzheimer’s, there are many neurologic problems besides language.

While there is not a specific treatment, there are ways to improve function and quality of life. The most important thing is to improve ability to communicate, using speech therapy (a speech and language pathologist is an essential part of your treatment team) and sometimes through assistive technology.

Dr. Roach Writes: A column on apparent temporomandibular joint pain generated many letters. One periodontist wrote me that his TMJ had been misdiagnosed and he found relief when operated on for trigeminal neuralgia. Other conditions masquerading as TMJ that readers wrote to me about included migraine, blocked Eustachian tubes, food allergies and Eagle syndrome (compression of a cranial nerve).

A TMJ specialist, Dr. Carol Cunningham, wrote: “I instruct patients to sleep on the edge of the pillow, and to make sure that the pillow ends even with their ear. They should have nothing touching (no pressure) on their face past the point of their ear.

“The facial muscles want to be neutral and relaxed during sleep. If pressure is exerted on the muscles by a pillow, hand or arm, then the muscle will try to get back to a neutral position. Most often this is done by clenching and grinding. Pressure can be exerted on the muscles and joint, and this results in pain.

“We see a lot of [TMJ] problems in people who are stomach sleepers, as this results in the jaw being torqued throughout the night from lateral pressure on the face. I also tell patients to be aware of what they are doing with their teeth during the day. The only time teeth should touch is when you eat (and very briefly during certain words). During all other times, lips may be together, but teeth should not touch. Often, we will fabricate splints to relieve pain in the TMJ and muscles of mastication. After splint fabrication, we will see the patient on a weekly basis to ensure that the splint is well-adjusted. Closing on the splint will change as the muscles relax and the joint seats to its normal position. We often complete therapy by adjusting the bite so that the teeth fit together as well with the splint as without. Sometimes, bite problems may need to be treated by restorative work, such as crowns or orthodontic treatment. In rare cases, surgery may be indicated to correct severe bite problems caused by skeletal deformities.”