Wednesday, April 16, 2008
Wednesday: My last day at the AAN annual meeting
I'm currently at an evening conference entitled: "Case Studies in Dementia". This will be my last engagement at the meeting. My family and I leave tomorrow morning for a 3+ hour train ride back to Springfield, IL. Tonight's program features: Dr. Richard Caselli of the Mayo Clinic Scottsdale; Dr. Jody Corey-Bloom from UCSD; and Dr. Ronald Petersen of the Mayo Clinic Rochester. Cory-Bloom started off with a case of a 33 yo female with a one year history of cognitive difficulty. Her boyfriend described reduced speech alternating at other times with incessant speech. The Boston Naming Test showed difficulty with word finding. There was no deficit in memory. Brain MRI showed mild frontal atrophy. Two years later, the patient is now almost mute, with echolalia. She is very oral, and very disinhibited, and paces incessantly. She is also compulsive about routines. Dr. Corey-Bloom feels that the patient has frontotemporal lobar degeneration (the behavioral variant). The other two types of frontotemporal lobar degeneration, as described by Neary, are progressive nonfluent aphasia and semantic dementia. (Semantic dementia is where the patient has fluent speech with loss of meaning. They can also have mental rigidity and a cold affect.) Corey-Bloom went on to talk about FTDP-17, and the recent discovery of the two genetic subtypes: MAPT mutation and Progranulin mutation. Corey-Bloom says that the only treatment for FTLD is aimed at the behavioral symptoms (SSRIs and anti-psychotics).
Dr. Caselli presented a case of a 46 yo woman with a five-month personality change with increased libido, increased wine consumption, chain smoking, erratic sleep patterns, and a fixation on consuming candy mints. She had five seizures witnessed by her physician husband. MRI showed no frontal atrophy and was essentially normal. I raised my hand when there was a call for differential diagnosis, I suggested a low-grade brain tumor. Other people suggested various encephalitides. Brain biopsy showed Nonvasculitic Autoimmune Inflammatory Meningoencephalitis (Caselli doesn't like the term Hashimoto's Encephalitis since these patients don't have serum anti-thyroid antibodies). What was unusual here was that the diffuse process caused a focal (frontal) clinical presentation.
Dr. Petersen presented a case of a patient with a clinical picture of FTLD who, upon imaging for amyloid using PET scanning for Pittsburgh compound B (PiB) caused Petersen to decide that the diagnosis was more consistent with Alzheimer disease.