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07-SEPTEMBER-2008 03:17:44 - Frontotemporal dementia This article or section includes a list of references or external links, but its sources remain unclear because it lacks in-text citations. You can improve this article by introducing more precise citations where appropriate. Frontotemporal dementia Classification and external resources A human brain showing frontotemporal lobar degeneration causing frontotemporal dementia. ICD-9 331.19 OMIM 600274 DiseasesDB 10034 MeSH D003704 Frontotemporal dementia FTD is a clinical syndrome caused by degeneration of the frontal lobe of the brain and may extend back to the temporal lobe. It is one of three syndromes caused by frontotemporal lobar degeneration. Contents 1 Signs and symptoms 2 Pathology 3 Imaging 4 Genetics 5 Management 6 See also 7 References 8 Further reading 9 External links Signs and symptoms Symptoms can be classified roughly into two groups which underlie the functions of the frontal lobe: behavioural symptoms and/or personality change and symptoms related to problems with executive function. Behavioural symptoms include lethargy and aspontaneity or oppositely disinhibition. Apathetic patients may become socially withdrawn and stay in bed all day or no longer take care of themselves. Disinhibited patients can make inappropriate sometimes sexual comments or perform inappropriate acts. Patients with FTD can sometimes get into trouble with the police because of inappropriate behaviour such as stealing. Recently, findings indicate that psychotic symptoms are rare in FTD, possibly due to limited temporal-limbic involvement in this disorder. Among the FTD patients, only 2 2.3% had delusions, 1 of whom had paranoid ideation; no FTD patient had hallucinations. This was significantly less than the AD patients, 4 17.4% of whom had delusions and paranoia. See ref. Psychotic symptoms. Executive function is the cognitive skill of planning and organizing. Patients become unable to perform skills that require complex planning or sequencing. Language skills can be affected in a number of ways with two broad patterns. Some patients remain fluent with normal phonology and syntax but increasing difficulty with naming and word comprehension, known as semantic dementia in which there is atrophy of the anterior temporal lobes, typically with a asymmetric pattern. Other patients, by contrast, present with a breakdown in speech fluency due to articulation difficulty, phonological and/or syntactic errors but preservation of word comprehension, referred to as progressive nonfluent aphasia. In addition to the characteristic cognitive dysfunction, a number of primitive reflexes known as frontal release signs are often able to be elicited. Usually the first of these frontal release signs to appear is the palmomental reflex which appears relatively early in the disease course whereas the palmar grasp reflex and rooting reflex appear late in the disease course. FTD can occur in patients with motor neurone disease also known in the US as Lou Gehrig's disease or amyotrophic lateral sclerosis in a small number of cases. The prognosis for people with MND is worse when combined with FTD, shortening survival by about a year.1 Pathology A number of case series have now been published looking at the pathological basis of frontotemporal dementia. As with other syndromes associated with FTLD, a number of different pathologies are associated with FTD: Pick's disease 3-repeat Tau inclusions Other tau-positive pathology including FTDP-17, corticobasal degeneration, progressive supranuclear palsy FTLD with ubiquitin positive, tau- and alpha-synuclein negative inclusions with and without motor neuron degeneration recently characterized by nuclear and cytoplasmic staining of TDP-43 protein Dementia lacking distinctive histology In rare cases, patients with clinical FTD were found to have changes consistent with Alzheimer's disease on autopsy Evidence suggests that FTD selectively impairs spindle neurons, a type of neuron which has only been found in the brains of humans, great apes, and whales Imaging Structural MRI scans often reveal frontal lobe and/or anterior temporal lobe atrophy but in early cases the scan may seem normal. Atrophy is often be asymmetric. Registration of images at different time points e.g. one year apart can show evidence of atrophy in two cross-sectional images that may be reported as normal. This is a useful diagnostic technique. However, many research groups are currently looking at ways of making an early diagnosis of FTD using other techniques magnetic resonance spectroscopy, functional imaging, cortical thickness measurements etc.. FDG-PET scans classically show frontal and/or anterior temporal hypometabolism, which helps differentiate from Alzheimer's disease. The PET scan in Alzheimer's disease classically shows biparietal hypometabolism. Genetics A higher proportion of FTD cases seem to have a familial component perhaps more so than Alzheimer's disease. Two known mutations are associated with familial FTD: tau-positive frontotemporal dementia with parkinsonism FTDP-17 with mutations in the MAPT gene on chromosome 17, and tau-negative frontotemporal lobar degeneration with ubiquintin-positive inclusions FTLD-U; positive for TDP-43 with progranulin mutations also on chromosome 17. However, it is estimated that each of these two genes only accounts for about 5-10% of all cases of FTD, thus other genes or heritable components are likely responsible for the high degree of heritability in FTD. Management There is no known curative treatment for FTD. Supportive care is essential. Management of behavioural symptoms may be necessary e.g. SSRIs for depression; atypical neuroleptics etc.. Because FTD often occurs in younger people i.e. in their 40's or 50's, it can severely affect families. Patients often still have children living in the home. Financially, it can be devastating as the disease strikes at the time of life that are often the top wage earning years. See also Frontotemporal lobar degeneration Pick's disease Semantic dementia Progressive nonfluent aphasia Alzheimer's disease Corticobasal degeneration References ^ Olney et al.. The effects of executive and behavioral dysfunction on the course of ALS 177. Retrieved on 2008-04-09. Neary D, Snowden JS, Gustafson L, et al 1998. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51 6: 1546-54. PMID 9855500. Hodges JR, Patterson K, Oxbury S, Funnell E 1992. Semantic dementia: progressive fluent aphasia with temporal lobe atrophy. Brain 115 6: 1783-806. PMID 1486461. Neary D, Snowden JS, Mann DM 2000. Classification and description of frontotemporal dementias. Ann. N. Y. Acad. Sci. 920: 46-51. PMID 11193176. Kramer JH, Jurik J, Sha SJ, et al 2003. Distinctive neuropsychological patterns in frontotemporal dementia, semantic dementia, and Alzheimer disease. Cogn Behav Neurol 16 4: 211-8. PMID 14665820. Rosen HJ, Gorno-Tempini ML, Goldman WP, et al 2002. Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology 58 2: 198-208. PMID 11805245. Miller BL, Seeley WW, Mychack P, Rosen HJ, Mena I, Boone K 2001. Neuroanatomy of the self: evidence from patients with frontotemporal dementia. Neurology 57 5: 817-21. PMID 11552010. Diehl J, Ernst J, Krapp S, Förstl H, Nedopil N, Kurz A 2006. Misdemeanor in frontotemporal dementia in German. Fortschr Neurol Psychiatr 74 4: 203-10. PMID 16671160. Seeley WW, Carlin DA, Allman JM, et al 2006. Early frontotemporal dementia targets neurons unique to apes and humans. Ann. Neurol. 60 6: 660-7. doi:10.1002/ana.21055. PMID 17187353. Hodges JR, Davies R, Xuereb J, Kril J, Halliday G 2003. Survival in frontotemporal dementia. Neurology 61 6: 349-354. PMID 12913196. Hodges JR, Davies R, Xuereb J, Casey B, Broe M, Bak T, Kril J, Halliday G 2004. Clinicopathological correlates in frontotemporal dementia. Ann. Neurol. 56 3: 349-354. doi:10.1002/ana.20203. PMID 15349867. Mioshi E, Kipps C, Dawson K. Mitchell J, Graham A, Hodges JR 2007. Activities of daily living in frontotemporal dementia. Neurology 68: 2077-2084. doi:10.1212/01.wnl.0000264897.13722.53. PMID 17562828. Mendez MF, Shapira JS, Woods RJ, Licht EA, Saul RE Jr 2008. Psychotic symptoms in frontotemporal dementia: prevalence and review. Dement Geriatr Cogn Disord. 25 3: 206-211. Further reading Radin, Lisa. What If It's Not Alzheimer's: A Caregiver's Guide to Dementia. Prometheus Books. 2003. Hodges, John R. The Frontotemporal Dementia Syndromes. Cambridge University Press. 2007 ISBN 978-0-521-85477-1 External links The Association for Frontotemporal Dementias Columbia University Update A collection of articles about Frontotemporal dementia in the journal Neurology FTD Info at the UCSF Memory and Aging Center Conference 6th International Conference on Frontotemporal Dementia in Rotterdam, September 2008 New York Times article 4/8/2008 suggesting that scientist Anne Adams and composer Maurice Ravel had frontotemporal dementia FRONTIER - Frontotemporal Dementia Research Group - at POWMRI, Sydney, Australia v d e WHO ICD-10 mental and behavioral disorders F · 290-319 Neurological/symptomatic Dementia Alzheimer's disease, multi-infarct dementia, Pick's disease, Creutzfeldt-Jakob disease, Huntington's disease, Parkinson's disease, AIDS dementia complex, Frontotemporal dementia, Elopement, Sundowning, Wandering · Delirium · Post-concussion syndrome · Organic brain syndrome Psychoactive substance alcohol drunkenness, alcohol dependence, alcoholic hallucinosis, Alcohol withdrawal, delirium tremens, Korsakoff's syndrome, alcohol abuse · opioids opioid dependency · sedative/hypnotic benzodiazepine withdrawal · cocaine cocaine dependence · general Intoxication, Drug abuse, Physical dependence, Withdrawal Psychotic disorder Schizophrenia disorganized schizophrenia · Schizophreniform disorder · Schizotypal personality disorder · Delusional disorder · Folie à deux · Schizoaffective disorder Mood affective Mania · Bipolar disorder · Clinical depression · Cyclothymia · Dysthymia Neurotic, stress-related and somatoform Anxiety disorder Agoraphobia, Panic disorder, Panic attack, Generalized anxiety disorder, Social anxiety, Social phobia · OCD · Acute stress reaction · PTSD · Adjustment disorder · Conversion disorder Ganser syndrome · Somatoform disorder Somatization disorder, Body dysmorphic disorder, Hypochondriasis, Nosophobia, Da Costa's syndrome, Psychalgia · Neurasthenia Physiological/physical behavioral Eating disorder: Anorexia nervosa · Bulimia nervosa Sleep disorder: Dyssomnia Hypersomnia, Insomnia · Parasomnia REM behavior disorder, Night terror · Nightmare Sexual dysfunction: Erectile dysfunction · Premature ejaculation · Vaginismus · Dyspareunia · Hypersexuality · Female sexual arousal disorder Postpartum depression · Postnatal psychosis Adult personality and behavior Personality disorder · Passive-aggressive behavior · Kleptomania · Trichotillomania · Voyeurism · Factitious disorder · Munchausen syndrome · Ego-dystonic sexual orientation · Fetishism Mental retardation Mental retardation Psychological development developmental disorder Specific: speech and language expressive language disorder, aphasia, expressive aphasia, receptive aphasia, Landau-Kleffner syndrome, lisp · Scholastic skills dyslexia, dysgraphia, Gerstmann syndrome · Motor function developmental dyspraxia Pervasive: Autism · Rett syndrome · Asperger syndrome Behavioral and emotional, childhood and adolescence onset ADHD · Conduct disorder · Oppositional defiant disorder · Separation anxiety disorder · Selective mutism · Reactive attachment disorder · Tic disorder · Tourette syndrome · Speech stuttering · cluttering Retrieved from http://en..org/wiki/Frontotemporal_dementia Categories: Neurology | Cognitive disordersHidden category: Articles lacking in-text citations Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Français Nederlands Svenska This page was last modified on 27 August 2008, at 15:13
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