Difference between revisions of "Neurodegenerative diseases"

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→‎FUSopathies (FTLD-FUS): now termed FTLD-FET
m (→‎Common diseases: FTLD-TDP)
(→‎FUSopathies (FTLD-FUS): now termed FTLD-FET)
Line 593: Line 593:
*[http://www.nature.com/nrneurol/journal/v6/n2/fig_tab/nrneurol.2009.216_F1.html Pick body (nature.com)].<ref name=pmid20139998>{{Cite journal  | last1 = Grossman | first1 = M. | title = Primary progressive aphasia: clinicopathological correlations. | journal = Nat Rev Neurol | volume = 6 | issue = 2 | pages = 88-97 | month = Feb | year = 2010 | doi = 10.1038/nrneurol.2009.216 | PMID = 20139998 }}</ref>
*[http://www.nature.com/nrneurol/journal/v6/n2/fig_tab/nrneurol.2009.216_F1.html Pick body (nature.com)].<ref name=pmid20139998>{{Cite journal  | last1 = Grossman | first1 = M. | title = Primary progressive aphasia: clinicopathological correlations. | journal = Nat Rev Neurol | volume = 6 | issue = 2 | pages = 88-97 | month = Feb | year = 2010 | doi = 10.1038/nrneurol.2009.216 | PMID = 20139998 }}</ref>


=FUSopathies (FTLD-FUS)=
=TDP Proteinopathies=
 
=FTLD-FET=
* Clinical manifestations depend on the distribution of the pathologic alterations in the CNS
* Clinical manifestations depend on the distribution of the pathologic alterations in the CNS
* Currently 3 disorders among the FTLD-FUS subgroup.
* Currently 3 disorders among the FTLD-FET subgroup.
* In contrast to ALS-FUS, no genetic alterations of FUS have been reported to date for cases within the FTLD-FUS group.
* In contrast to ALS-FUS, no genetic alterations of FUS have been reported to date for cases within the FTLD-FUS group.
* 5–10% of all FTLD cases
* Deposited Proteins: FUS, EWS, TAF-15.
* FUS‐positive inclusions in FTLD cases show co‐aggregation of TAF15 and EWS
**(Different from ALS-FUS)


DDx (also FUS+ve):
DDx (also FUS+ve):
*Spinocerebellar Ataxia (SCA)
*Spinocerebellar Ataxia (SCA)
*Huntington Disease (SD)
*Huntington Disease (SD)
==Atypical FTLD‐U==
* Neuronal cytoplasmic inclusions in hippocampus and frontotemporal lobes.


==Basophilic inclusion body disease==
==Basophilic inclusion body disease==
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