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. Other Frontotemporal Dementia   There are other types of frontal dementia that are not marked simply by language or behavioral presentations. ALS dementia is now recognized as a frontal dementia in which there is motor-neuron disease. Afflicted persons usually are first symptomatic with dementia. They may not present to a neurologist until they have ALS, but families usually report that personality changes preceded weakness, fasciculations, and motor findings. Progressive supranuclear palsy (PSP) is a Parkinsonian disorder that can also be considered a frontotemporal dementia, although frontal functions are affected much more than temporal. Patients usually present with prominent axial rigidity and eye movement abnormalities, but very frequently also have cognitive disturbance, particularly in language and subcortical executive functions. Cortico-basal degeneration is another disorder that, while not strictly frontotemporal dementia, is more broadly classified as one of the frontal dementias. It is characterized by abnormality of praxis, unlike frontotemporal dementia per se, in which praxis is not affected. In corticobasal degeneration, people cannot carry out their organized, sequential, and programmed movements of ordinary life. They have difficulty putting on clothes, writing, driving , or doing many simple tasks.         ALS Dementia frontal dementia loss of drive, anhedonia, apathy, anorexia subsequent lower motor neuron (±UMN) signs fasciculations, muscle atrophy, weakness gait disorder, dysarthria, dysphagia EMG/NCV-confirmed motor neuronopathy   ALS dementia is now recognized as a frontal dementia in which there is motor-neuron disease. Courtesy of Dr. Lawrence Honig           Progressive Supranuclear Palsy Steele-Richardson-Olszewski Syndrome supranuclear gaze deficits: vertical > horizontal Parkinsonism: bradykinesia and rigidity (axial) frequent falls pseudobulbar palsy subcortical dementia language impairment (echolalia, palilalia)   Progressive Supranuclear Palsy (PSP) is a Parkinsonian disorder and a frontal type of dementia. Courtesy of Dr. Lawrence Honig           Corticobasal Degeneration subcortical dementia aphasia, obsessiveness apraxias: ideomotor and ideational asymmetric sensory and motor findings Parkinsonism: rigidity, clumsiness, gait abnormality cortical sensory loss, alien limb phenomenon   Corticobasal degeneration is not strictly a frontotemporal dementia but may still be considered one of the frontal dementias. Courtesy of Dr. Lawrence Honig           Huntington's Disease personality change (disinhibition, substance/ETOH use, promiscuity) psychosis (delusions, but rarely hallucinations) subcortical dementia (decreased drive, executive function) verbal output decline (spontaneous speech) chorea (UE > LE) gait change   Huntington's disease is not strictly a frontotemporal dementia but may still be considered one of the frontal dementias. Courtesy of Dr. Lawrence Honig     Functional imaging studies may reveal distinct archetypal appearances for frontotemporal dementia, in which the frontal lobes show reduced metabolism or perfusion, and for Alzheimer's dementia, in which parietal and temporal lobes show such reductions. In cortico-basal degeneration, it is the central area of the brain, the motor-sensory strip, that seems most prominently affected, and this is often strikingly asymmetric. The motor-sensory area involvement may correlate with the severe disorders of praxis noted in this disorder. Huntington's disease is also within the spectrum of frontal-like dementias. It is principally a subcortical dementia in which psychosis and personality changes are the common presenting and prominent features. Chorea and gait change are the features most commonly appreciated in Huntington's disease patients. Indeed, motor findings are common in almost all of the atypical dementias except frontotemporal dementia in its purest forms. In the brains of Huntington's-disease patients, the caudate nucleus is usually atrophic. Even though it is a fairly primitive area of the brain, the caudate may have an effect on control of delusions and hallucinations.         Huntington's Disease   Huntington's disease is principally a subcortical dementia. Courtesy of Dr. Lawrence Honig       PREVIOUS | NEXT