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Jensflorian (talk | contribs) (→Overview: FUSopathies added) |
Jensflorian (talk | contribs) (→Other: NIFID, BIBD) |
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*[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)= | |||
* Clinical manifestations depend on the distribution of the pathologic alterations in the CNS | |||
* Currently 3 disorders among the FTLD-FUS subgroup. | |||
* In contrast to ALS-FUS, no genetic alterations of FUS have been reported to date for cases within the FTLD-FUS group. | |||
DDx (also FUS+ve): | |||
*Spinocerebellar Ataxia (SCA) | |||
*Huntington Disease (SD) | |||
==Basophilic inclusion body disease== | |||
* AKA: BIBD. | |||
* Variable clinic (behavioral, cognitive alterations, parkinsonism, motor neuron diseases, ALS-like). | |||
* Age of onset: 35-70 years. | |||
* Intraneuronal cytoplasmic basophilic inclusion bodies. | |||
* FUS+ve (universally). | |||
* TAF15+ve | |||
* alpha-Internexin+ve. | |||
==Neuronal Intermediate Filament Inclusion Disease== | |||
* AKA: NIFID. | |||
* Hyaline conglomerates (brightly eosinophilic branching fibrillar structures embedded in a round, well-delineated, glassy vacuole). | |||
* FUS+ve (heterogenous). | |||
* Ubiquitin +/-ve. | |||
* NF +ve (some subunits). | |||
* p62 +/-ve. | |||
* TDP43-ve. | |||
* Tau-ve. | |||
* α-synuclein-ve. | |||
=Other= | =Other= |