The Cognitive Neuroscience Section of the National Institute of Neurological Disorders and Stroke proposes to continue its cross-sectional and longitudinal studies of cerebral metabolism in frontal lobe dementias and atypical basal ganglia disorders. These studies include repeated assessments of neuropsychological and brain anatomical and metabolic function in subjects with these important and possibly related brain disorders.

Objective

To continue the cross-sectional and longitudinal studies performed by the Cognitive Neuroscience Section of NINDS on the neuroanatomy, cerebral metabolism, neuropsychology, and genetics of frontotemporal dementia, corticobasal syndrome, and related brain disorders.

Study population

Frontal lobe dementias and atypical basal ganglia disorders including frontotemporal dementia, corticobasal syndrome, and related brain disorders.

Design

A cross-sectional assessment of neuropsychological, brain anatomical and metabolic function, and genetic measures in subjects with these disorders.

Outcome measures

MRI, FDG-PET, neuropsychological and clinical tests, genetics, and neuropathology.

• Alzheimer's Disease
• Dementia
• Down's Syndrome

• Schwartz M, Creasey H, Grady CL, DeLeo JM, Frederickson HA, Cutler NR, Rapoport SI. Computed tomographic analysis of brain morphometrics in 30 healthy men, aged 21 to 81 years. Ann Neurol. 1985 Feb;17(2):146-57.
• Schlageter NL, Horwitz B, Creasey H, Carson R, Duara R, Berg GW, Rapoport SI. Relation of measured brain glucose utilisation and cerebral atrophy in man. J Neurol Neurosurg Psychiatry. 1987 Jun;50(6):779-85.
• Duara R, Grady C, Haxby J, Ingvar D, Sokoloff L, Margolin RA, Manning RG, Cutler NR, Rapoport SI. Human brain glucose utilization and cognitive function in relation to age. Ann Neurol. 1984 Dec;16(6):703-13.

• Subjects will be recruited from the NIH referral center, neurological and psychiatric clinics, from self or family referral in response to advertisements or from private physicians. Healthy volunteers will include subjects who are considered aT-risk for the development of FTD or CBS and subjects who serve as normal controls matched for key characteristics with the patients.

INCLUSION CRITERIA FOR PATIENTS:

• Diagnosis of possible / probable FTD or CBS
• Caregiver willing and able to accept the responsibilities involved in the study

EXCLUSION CRITERIA:

• Pregnant women. Women of childbearing potential will be screened by history for the possibility of pregnancy and undergo a urine pregnancy test. These women will be excluded from the imaging portions of the protocol.
• Behavioral symptoms that would preclude the gathering of data for the study
• Other medical or social condition that would preclude participation in the opinion of the investigators