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Dementia classifications-- rapid FTD's, by abnormal protein

Posted Dec 25 2009 7:39pm
Frontotemporal dementia subtypes and genetics-- rapidly progressive subtypes
1. FTD and parkinsonism due to mutation on chromosome 17 on gene encoding microtubule associated protein tau (MAPT)= FTD17MAPT
2. FTD and parkinsonism due to mutation on chromosome 17 on gene encoding progranulin (PGN)=FTD17PRGN
3. FTD and frontal lobar degeneration due with ubiquitin and TDP-43 positive inclusions=FTDUTDP43
4. FTD and frontal lobar degeneration due with ubiquitin and TDP-43 negative inclusions=FTDU-TDP43
5. Neuronal inclusion body dementia
6. Dementia lacking distinctive histopathology (DLDH) (except gliosis and neuronal loss)-- may also be included in rubric of FTD
By protein
Amyloidopathies--
1.Alzheimer's disease,
2. Down's syndrome
Tau-opathies-
1.-Picks disease (sporadic and familial);
2. corticobasal degeneration (CBD) (sporadic and familial);
3. progressive supranuclear palsy PSP (sporadic and familial);
4. Argyrophilic grain disease (AGD) (sporadic and familial);
5. multisystem tau-opathy (sporadic and familial);
6. FTD-17-MAPT;
7. Alzheimer's disease
Synucleinopathies
1.Lewy body disease * (also PDD, DLB, PD) sporadic and familial
2.Multiple system atrophy (rarely associated with dementia)
3. Pure autonomic failure
Huntingtin
1. Huntington's disease
Alpha internexin
1. Neurofilament inclusion body disease (NIBD)* =neuronal intermediate filament inclusion disease (NIFID)
TAR 43 binding protein (TDP 43)
1. FTLD-u-TDP43 * aka a) FTD with ITSNU (inclusions, tau and synuclein negative, with ubiquitin) b) FTLD with MND (motor neuron disease inclusions) c) MNDID motor neuron disease inclusion dementia
2. FTD 17 PRGN
Protein unknown
1. FTLD-u (TDP43 negative inclusions)*
2. DLDH*
* evolve to death within 3 years*
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