Introduction     Diagnosis of Mild Cognitive Impairment and Alzheimer's Disease Karen L. Bell, M.D.     Treatment Strategies for Dementia and Mild Cognitive Impairment Mary Sano, Ph.D.     Treatment of Depression, Agitation, and Psychosis in Dementia Davangere P. Devanand, M.D.     Recognition of Vascular Dementia, Dementia with Lewy Bodies, and Frontotemporal Dementia Lawrence S. Honig, M.D., Ph.D.     Categorization of Dementias       Prevalence of Non-Alzheimer's Dementias       The Spectrum of Lewy-Body Disease       Frontotemporal Dementia       Other Frontotemporal Dementia       Vascular Dementia       Other Non-Alzheimer's Dementias         Neuropsychology of Mild Cognitive Impairment, Alzheimer's Disease, Dementia with Lewy Bodies, and Frontotemporal Dementia Penne Sims, Ph.D.     Neuroimaging in Dementia Scott A. Small, M.D.     Genetics of Neurodegenerative Disease: Alzheimer's Disease, Frontotemporal Dementia Jennifer Williamson-Catania, M.S.     Legal and Ethical Issues for Patients with Dementia Daniel G. Fish, Esq.     Posttest         Accreditation     Reference List     Acknowledgements		Recognition of Vascular Dementia, Dementia with Lewy Bodies, and Frontotemporal Dementia Lawrence S. Honig, M.D., Ph.D. Frontotemporal Dementia   In the past, a large variety of conditions of frontotemporal cerebral degeneration have been reported. However, these are all now generally grouped under the greater umbrella term of frontotemporal dementia. Some the subtypes of frontotemporal dementia have a pathological description, while other have more clinical descriptors.         Frontotemporal Dementias Pick's Disease—subtypes(PiD) frontal-lobe degeneration dementia lacking distinctive histopathological features (DLDH) progressive subcortical gliosis (PSG) long-duration Creutzfeldt-Jakob disease dysphasic dementia aphasia with predominantly subcortical lesion sites atypical presenile dementia non-Alzheimer's lobar atrophy ALS-dementia or MND-dementia disinhibition-dementia-Parkinsonism-amyotrophy   (DDPAC;17q21)     Recent efforts have led to the development of organized criteria for frontotemporal dementia. These recognize the various clinical features of this disorder that distinguish it from Alzheimer's disease and other dementing disorders. There are two broadly different presentations: one involving behavioral and personality change, and the other involving language impairment.         Frontotemporal Dementia: Criteria prominent/early language impairment echolalia, stereotypy, progressive reduction of speech prominent/early behavioral (frontal) deficits loss of drive and interests, mental rigidity, social withdrawal social impropriety, disinhibition, impulsivity, distractibility hyperorality, stereotyped behaviors (verbal, ritual, motoric) early affective symptoms depression, anxiety, somatization, aspontaneity, indifference relative preservation of praxis and visuospatial skills   Criteria exist to distinguish the clinical features of frontotemporal dementia from Alzheimer's disease and other dementias. Courtesy of Dr. Lawrence Honig     A host of behavioral abnormalities can occur in frontotemporal dementia; many of these overlap with known primary psychiatric syndromes. It is not uncommon for people with frontotemporal dementia, who ultimately develop a very evident dementing course, to first be diagnosed with "pure" psychiatric disease. Unlike Alzheimer's disease, nonspecific agitation is not common, nor are hallucinations and delusions. Rather more frontal-type symptoms such as loss of interest, obsessional behaviors, loss of social inhibitions leading to social improprieties may be prominent. There may also be mood disturbances in frontotemporal dementia; depression, anxiety, and somatization are not uncommon. Those individuals presenting with language involvement usually have fewer behavioral symptoms, but often have striking impairment of ability to express themselves (primary progressive aphasia) or less often impaired understanding of language (semantic dementia). One key feature that may differentiate frontotemporal dementia from Alzheimer's disease is relative preservation of memory. That is not to say that memory is not affected in these disorders. However, memory is usually not as affected as executive function or disorders of language. Also, persons with frontotemporal dementia typically have relative preservation of visuospatial skills and of praxis, their ability to carry out motor performance activities. If a patient is a plumber, he or she may be able to plumb very well. There may be all sorts of personality problems and difficulties on the job, but manual dexterity remains good and the person does not get lost or wander off.         Frontotemporal Dementia   The brain of a person with frontotemporal dementia shows a shrunken and sometimes asymmetric frontal lobe and a normal parietal lobe. Courtesy of Dr. Lawrence Honig           Frontotemporal Dementia: Sagittal Sections   In sagittal sections, the front part of the brain of a person with frontotemporal dementia has less perfusion, which appears as a darker, redder color on a pseudocolor-coded SPECT scan. Courtesy of Dr. Lawrence Honig     The brain of a person with frontotemporal dementia often shows shrunken and sometimes asymmetric frontal lobes, with relatively normal parietal lobes. Functional imaging such as SPECT or PET may show in sagittal sections that the front part of the brain has less perfusion, which appears as a darker, redder color on a pseudocolor-coded SPECT scan.     PREVIOUS | NEXT