Non-Alzheimer's and Atypical Dementia
Multidisciplinary approach to diagnosis and management Allows development of management and care plan strategies Practical approach including case studies Written by a world-renowned editorial team
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 Parkinson's disease and related disorders, and how DBS affects cognition and mood.
Non-Alzheimer's and Atypical Dementia
Michael D. Geschwind and Caroline Racine Belkoura
University of California, San Francisco, San Francisco, CA, USA
This book was developed in order to provide a clinically relevant review of non-Alzheimer's and atypical dementia syndromes. Specifically, we felt there was a need for a broad but comprehensive overview of the differential diagnoses for atypical dementia that could be utilized by health-care providers who encounter these patients in their clinical practice, including neurologists, primary care providers, psychiatrists, neuropsychologists, nurses, social workers, etc. Where relevant, we have included clinical case studies in each chapter to help illustrate key or unique diagnostic features of each disorder and to provide a "real-world" view of how each disorder might present in the clinic.
Multidisciplinary evaluation of the atypical dementia patient
In this chapter, the editors review a framework for the clinical evaluation of the patient with a suspected atypical dementia syndrome. In particular, we focus on the benefits of a multidisciplinary evaluation with a team that includes a combination of a neurologist, neuropsychologist, psychiatrist, nurse, and social worker. Each team member brings a unique set of skills to the evaluation, which enables an in-depth and comprehensive assessment of a variety of domains, including relevant history, neurological function, cognitive abilities, mood and behavior, and daily function. Obtaining information from both the patient and a close family member or friend is essential as many atypical syndromes lead to loss of insight, and thus, more accurate reporting might come from someone other than the patient themselves. We have found that a case conference approach, where all team members meet after seeing the patient to review all relevant findings and discuss the case in detail, leads to a more accurate differential diagnosis, which can then be relayed to the patient and their family members in a timely fashion.
Atypical Alzheimer's disease
In this chapter, Sharon Sha and Gil Rabinovici review the atypical presentations of Alzheimer's disease (AD), which by definition present with symptoms other than memory loss and therefore might not meet most standard diagnostic criteria for AD. These patients tend to be younger than "typical" AD cases and might present with visuospatial complaints, executive dysfunction, behavioral changes, or language impairment. Additionally, often, patients meet diagnostic criteria for posterior cortical atrophy (PCA, a visual dysfunction syndrome), corticobasal syndrome (CBS, executive dysfunction or behavioral syndrome), and/or primary progressive aphasia (PPA, language syndrome) disorders that have not historically associated with AD pathology; however, recent research has demonstrated that a significant portion of these clinical syndromes are ultimately found to have AD pathology at autopsy. Neuropsychological testing and atrophy patterns on MRI often are very helpful in the differential diagnosis of the clinical syndrome. PET imaging with amyloid binding agents such as Pittsburgh compound B (PiB) or florbetapir F18 might provide additional, if not even more convincing, evidence of underlying AD pathology. The recognition of AD pathology as a causative factor in these atypical syndromes is important because of available symptomatic treatments and ongoing clinical trials for AD. Future diagnostic criteria for AD will need to incorporate the possibility of atypical presentations in order to increase sensitivity.
Vascular cognitive impairment: Diagnosis and treatment
In this comprehensive chapter, Helena Chui and Liliana Ramirez Gomez first review the complex history and terminology of vascular contributions to cognitive impairment. They postulate that the physiological effects of vascular brain impa