Two years ago today I had ten hours of neurosurgery at the University of Pittsburg Medical Center. For two years prior I had numbness and tingling on the right side of my lip and chin. The symptoms were perfectly asymmetrical, and as the numbness progressed down my chin an imaginary line separate the left side, which was fine, from the right side which grew increasingly more numb. When a MRI diagnosed a "schwannoma," a fatty benign tumor, on my right trigeminal nerve I choose UPMC because of their pioneering surgeon, Dr. Amin Kassam, for the endoscopic (via the nose and sinuses) approach to the Meckel's Cave region of lower brain -- perhaps the most difficult area of the body to access given its interior location and juncture of spinal chord and brain stem.
Ten days later pathologists discovered that instead of a schwannoma the biopsied tissue revealed amyloid. Usually enthusiastic, Dr. Kassam presented this information to me in a somber tone. I had never heard of such a thing and asked him to spell it! He then suddenly headed for the door and said, "Go home." "To my apartment?" I asked, the one we had taken in Pittsburgh for a three week recovery period. "No, Ithaca."
That night I search the Internet for this strange term. Alzheimer disease rose to the top of the searches. Over and over again I read about proteins unfolding, burrowing into the brain, forming plaque and destroying the tissue. Common knowledge taught me that doctors diagnose the disorder from symptoms not tissue because no one has a brain biopsy. Except me, almost sort of. It turns out that the amyloid on my nerve consists of a different type of protein, a lambda light chain, produced by plasma cells and which lead to another protein-related disorder, amyloidosis. I have since learned that there are numerous disorders for which proteins are implicated.
In light of President Obama's pledge to find a "cure" for Alzheimer's it may be important to recognize that this disease is not alone in the constellation of protein disorders. Medical researchers already recognize Lewy Body Demetia in which plague affects the lower brain steam. That disorder may have a close relationship to Parkinson's Disease given the similarity of symptoms involving motor control. Owen Thomas's tragic death has also thrown a spotlight on amyloid deposition believed to be related to traumatic head injuries. The entire National Football League grabbles with the consequences of head trauma on its most distinguished players. I could not authoritatively say how many other recognized diseases exist directly involving proteins, but simple Internet searches reveal many strange and unusual names.
Protein disorders in general require the kind of public support and attention that we have given cancer and AIDs in the past and now, as us baby-boomers grow older, Alzheimer's. Not only do we need financial support for the research and clinical trials, but a coordination of these efforts in such a way to connect the dots of these various disorders to create new diagnostic tools and treatment protocols. That the need exists is clear to me. Months after the diagnosis in Pittsburg I went to the Boston University Amyloid Center for a follow up, which I now do annually. When I was there this past August a fellow patient died in clinic's waiting room.
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