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20-September-2008 10:08:30 - Frontotemporal lobar degeneration Frontotemporal lobar degeneration Classification and external resources A human brain showing frontotemporal lobar degeneration causing frontotemporal dementia. OMIM 600274 DiseasesDB 10034 MeSH D003704 Frontotemporal lobar degeneration FTLD is the name for a group of clinically, pathologically and genetically heterogeneous disorders associated with atrophy in the frontal lobe and temporal lobe of the brain, with sparing of the parietal and occipital lobes. In the over 65 age group, FTLD is probably the fourth most common cause of dementia after Alzheimer's disease, Dementia with Lewy bodies and vascular dementia. In the below 65 age group, it is the second most common cause after Alzheimer's disease. There are three clinical subtypes described: frontotemporal dementia semantic dementia progressive nonfluent aphasia. Contents 1 Histology 2 Genetics 3 Examples 4 External links 5 References 6 See also Histology There are 3 main histological subtypes found at post-mortem: tau inclusions Pick's disease, MAPT mutations, corticobasal degeneration, progressive supranuclear palsy ubiquitin positive tau-negative inclusions - in the majority of cases that have this type of pathology the ubiquitinated inclusions contain a protein called TDP-43. There are three subtypes of this type of pathology described in the recent consensus criteria by Cairns et al: type 1 with neurites predominantly, type 2 with cytoplasmic inclusions predominantly; and type 3 with intranuclear inclusions. It should be noted that not all ubiquitin-positive, tau negative cases stain for TDP-43 e.g. the CHMP2B cases but also other cases. Dementia lacking distinctive histology DLDH - a rare and controversial entity - new analyses have allowed many cases to be reclassified into one of the positively-defined subgroups. Genetics Many cases possibly up to 50% of FTLD are genetic rather than sporadic. There are 2 major genes in which mutations cause FTLD: Mutations in the Tau gene on chromosome 17q21 - known as MAPT or Microtubule Associated Protein Tau can cause FTLD and there are over 40 known mutations at present. A series of new mutations associated with FTLD has been recently described in the progranulin gene which is remarkably also on chromosome 17q21. Patients with progranulin mutations have type 3 ubiquitin-positive, TDP-43 positive, tau-negative pathology at post-mortem. Progranulin is associated with tumorgenesis when overproduced, whereas the mutations seen in the progranulin gene associated with FTLD suggests a deficit in progranulin may be the problem. There are currently 2 other known genes that can cause FTLD: CHMP2B on chromosome 3 which is associated with a behavioural syndrome mainly in a large Jutland cohort VCP valosin-containing protein, on chromosome 9 which is associated with the IBMPFD syndrome inclusion body myopathy, Paget's disease and frontotemporal dementia. These 2 genes only account for a tiny proportion of cases. A locus on chromosome 9 is associated with FTD-MND or FTD-ALS i.e. frontotemporal dementia associated with motor neurone disease or amyotrophic lateral sclerosis - the hunt for this gene is currently the focus of a number of research labs around the world. Examples United States Senator Pete Domenici R-NM is a known sufferer of FTLD, and the illness is the main reason behind his October 4, 2007 announcement of retirement at the end of his term. External links FTD Support Forum The Association for Frontotemporal Dementias UCSF Memory and Aging Center - FTD Resources and FTD Info Mayo Clinic - FTD Info Pick's Disease Support Group Online Frontotemporal Dementia Caregiver Support Center References Mackenzie IR, Baborie A, Pickering-Brown S, Plessis DD, Jaros E, Perry RH, Neary D, Snowden JS, Mann DM. Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotype. Acta Neuropathol Berl. 2006 Nov;1125:539-49. Epub 2006 Sep 26. Davidson Y, Kelley T, Mackenzie IR, Pickering-Brown S, Du Plessis D, Neary D, Snowden JS, Mann DM. Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43. Acta Neuropathol Berl. 2007 Jan 12; Epub ahead of print Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. 'Neurology' 1998 516:1546-54. Available: 1 Pickering-Brown SM. The complex aetiology of frontotemporal lobar degeneration. Exp Neurol. 2007 Jul;2061:1-10. Epub 2007 Mar 24. See also Corticobasal degeneration v d e Pathology of the nervous system, primarily CNS G00-G47, 320-349 Inflammatory Meningitis Arachnoiditis - Encephalitis - Myelitis - Encephalomyelitis Acute disseminated - Tropical spastic paraparesis - Cavernous sinus thrombosis Systemic atrophies Huntington's disease - Spinocerebellar ataxia Friedreich's ataxia, Ataxia telangiectasia, Herary spastic paraplegia - Spinal muscular atrophy: Werdnig-Hoffman - Kugelberg-Welander - Fazio-Londe - MND ALS, PMA, PBP, PP, PLS Extrapyramidal and movement disorders Parkinson's disease - Neuroleptic malignant syndrome - Postencephalitic parkinsonism - Pantothenate kinase-associated neurodegeneration - Progressive supranuclear palsy - Striatonigral degeneration - Dystonia/Dyskinesia Spasmodic torticollis, Meige's, Blepharospasm - Essential tremor - Myoclonus - Lafora - Chorea Choreoathetosis - Restless legs - Stiff person Other degenerative/ demyelinating diseases dementia: Alzheimer's - Pick's - Dementia with Lewy bodies - Frontotemporal lobar degeneration mitochondrial disease: Leigh's demyelinating: Multiple sclerosis - Devic's - Central pontine myelinolysis - Transverse myelitis - Marchiafava-Bignami disease - CAMFAK syndrome - Alpers' Seizure/epilepsy Focal - Generalised - Status epilepticus - Myoclonic epilepsy Headache Migraine Familial hemiplegic - Cluster - Vascular - Tension Vascular Transient ischemic attack Amaurosis fugax, Transient global amnesia Cerebrovascular disease MCA, ACA, PCA, Foville's, Millard-Gubler, Lateral medullary, Weber's, Lacunar stroke Sleep disorders Insomnia - Hypersomnia - Sleep apnea Obstructive, Ondine's curse - Narcolepsy - Cataplexy - Kleine-Levin - Circadian rhythm sleep - Delayed sleep phase - Advanced sleep phase Intracranial hypertension Hydrocephalus Normal pressure - Idiopathic intracranial hypertension Other encephalopathy Brain herniation - Cerebral edema - Reye's Other spinal cord disease Syringomyelia - Syringobulbia - Morvan's syndrome - Spinal cord compression Retrieved from http://en..org/wiki/Frontotemporal_lobar_degeneration Categories: Neurology | Cognitive disorders | Anatomical pathology 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 Polski Svenska This page was last modified on 19 June 2008, at 20:42
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