Non-Alzheimer's and Atypical Dementia

Michael Geschwind, MD PhD; Dr. Geschwind received his MD and PhD in neuroscience through the National Institutes of Health-sponsored Medical Scientist Training Program at the Albert Einstein College of Medicine in New York. He completed his internship in internal medicine at the University of California, Los Angeles Medical Center, his neurology residency at the Johns Hopkins University School of Medicine in Baltimore and his fellowship in behavioral neurology at the UCSF Memory and Aging Center (MAC). He joined the Memory and Aging Center faculty in 2003 and is an Associate Professor of Neurology. Dr. Geschwind evaluates patients in the MAC new patient clinic and participates in the management and care for these patients in the MAC continuity clinic. He is active in the training of medical students and residents at UCSF. Dr. Geschwind teaches a national course and lectures, both nationally and internationally, on the assessment of rapidly progressive dementias, including human prion diseases. Dr. Geschwind's primary research interest is the assessment and treatment of rapidly progressive dementias, including prion diseases such as Creutzfeldt-Jakob disease (CJD). Dr. Geschwind helped establish an inpatient hospital program for the assessment of rapidly progressive dementias at UCSF, one of the first of its kind in the country. He ran the first ever US treatment study for CJD. He also has an active research interest in cognitive dysfunction in movement disorders, such as Huntington's disease, corticobasal degeneration (CBD), progressive supranuclear palsy (PSP) and other Parkinsonian dementias.

Caroline Racine Belkoura, PhD; Caroline Racine Belkoura received her BA in Psychology from Boston University, where she completed an honors thesis exploring visual-perceptual deficits in patients with stroke and traumatic brain injury. From 1997-1999 she worked as a research assistant with Dr. Dan Schacter at Harvard University on studies examining false memories in healthy aging. Dr. Racine Belkoura went on to obtain an MA and PhD in Clinical Psychology at Washington University in St. Louis, specializing in Neuropsychology and Aging. Her research at Washington University examined changes in frontal lobe function during healthy aging using both behavioral and neuroimaging methods (e.g., fMRI). She completed her clinical internship in Neuropsychology at Duke University in 2005 and afterward completed a two-year postdoctoral fellowship in Neuropsychology at the UCSF Memory and Aging Center. Since 2009, she has been an Assistant Professor in Neurological Surgery at UCSF. Currently, she evaluates patients with movement disorders who are undergoing workup for deep brain stimulation (DBS), as well as patients who are suspected of having atypical parkinsonian disorders. Her research focuses on cognitive and behavioural changes in the context of Parkinsons disease and related disorders, and how DBS affects cognition and mood.

Notes on contributors, vi

1 Introduction, 1
Michael D. Geschwind and Caroline Racine Belkoura

2 The multidisciplinary evaluation of the atypical dementia patient, 6
Michael D. Geschwind and Caroline Racine Belkoura

3 Atypical Alzheimer's disease, 17
Sharon J. Sha and Gil D. Rabinovici

4 Vascular cognitive impairment: Diagnosis and treatment, 30
Helena C. Chui and Liliana Ramirez-Gomez

5 Frontotemporal dementia, 49
David C. Perry and Howard J. Rosen

6 Lewy body dementias (DLB/PDD), 64
Carol F. Lippa and Katherine L. Possin

7 Corticobasal degeneration and progressive supranuclear palsy, 77
Suzee E. Lee and Bruce L. Miller

8 Repeat expansion diseases and dementia, 90
Praveen Dayalu, Roger L. Albin and Henry Paulson

9 Prion diseases and rapidly progressive dementias, 103
Leonel T. Takada and Michael D. Geschwind

10 Autoimmune dementias, 123
Andrew McKeon and Sean J. Pittock

11 Toxic and metabolic dementias, 134
Michelle Mattingly, Katie Osborn and Leon Prockop

12 Leukoencephalopathies/leukodystrophies, 150
Gregory M. Pastores and Swati A. Sathe

13 Infectious causes of dementia, 170
Cheryl A. Jay, Emily L. Ho and John Halperin

14 Rheumatologic and other autoimmune dementias, 186
Laura J. Julian and Christopher M. Filley

15 Comprehensive management of the patient with an atypical dementia, 202
Jennifer Merrilees, Cynthia Barton, Amy Kuo and Robin Ketelle

Index, 215