UCSF Memory and Aging Center | New Clinical Approaches to the Assessment of Rapidly Progressive Dementias and Neurologic Conditions

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New Clinical Approaches to the Assessment of Rapidly Progressive Dementias and Neurologic Conditions

Study Director	Michael Geschwind, MD, PhD
Sponsor	National Institutes of Health, the John Douglas French Foundation for Alzheimer’s Research
Recruiting?	Yes
Official Study Title	New Clinical Approaches to the Assessment of Rapidly Progressive Dementias and Neurologic Conditions
Conditions Studied	Transmissible Spongiform Encephalopathies, Rapidly Progressive Dementias
Purpose	The primary goal of this study is to develop and test methods for differentiating CJD from other diseases and to learn how to diagnose CJD as early as possible. A secondary goal of this study is to learn more about how CJD and other rapid neurological conditions progress by studying these diseases in each patient over time. Because CJD is a rare condition and it is very rapidly progressive, diagnosis of the disease often is made very late in the illness. If any future treatments for CJD are to be successful, they likely will need to be given to patients as early as possible, before the disease progresses too far. It is therefore important that we learn how to make an accurate diagnosis earlier.
Eligibility
Inclusion Criteria	Patients who are 18 years of age or older. Must fulfill WHO Revised Criteria for CJD, have a family history of familial human prion disease, or have a history of progressive neurologic decline resulting in dementia or significant functional impairment from patient's baseline over two years or less from symptoms onset. Must have an informant who has frequent contact with the subject and is available to provide information about the subject.
Exclusion Criteria	Unable to undergo MRI due contraindication to this procedure (e.g., pacemaker, pregnant women). Known HIV positive or immunosuppresed patients.
What is Involved?
Testing	History and physical neurological examination, lumbar puncture, neuropsychological testing, MRI of the brain, EEG, MEG, nerve conduction study, blood draw. (For deceased and patients who cannot come to UCSF for a visit, we are only requesting the medical records for review and research purposes.)
Frequency of Visits	Will return for repeat MRI of the brain, EEG and MEG testing depending on the severity and progression of the specific neurologic conditions.
Contact Information
Coordinator	Aissa Haman, MD and Amy Kuo, RN
Phone	(415) 476-2905 and (415) 476-2907
Materials Needed Prior to Evaluation	Diagnosis of a rapidly progressive dementia
Costs	None


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