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→Multiple system atrophy: update
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==Multiple system atrophy== | ==Multiple system atrophy== | ||
Multiple system atrophy is a neurodegenerative disease of the parkinsonism-plus disorder group. | |||
===General=== | ===General=== | ||
Clinical findings variable: | Clinical findings variable: | ||
*Parkinsonism (stiatonigral degeneration). | *Parkinsonism (stiatonigral degeneration, MSA-P). | ||
*Ataxia (olivo- | *Ataxia (olivo-ponto-cerebellar degeneration, MSA-C). | ||
*Autonomic dysfunction (Shy-Drager syndrome, depreceated). | |||
*Clinical onset between 40-60 years. | |||
* Progedient tremor, atxia, laryngeal paresis, wakness, cognitive decline. | |||
* Patients usually succumb after 6 years from aspiration pneumonia. | |||
DDx: | |||
* [[Spinocerebellar ataxia]]. | |||
* [[Parkinson disease]]. | |||
* Motor-neuron disease. | |||
* [[Lewy-Body disease]]. | |||
===Macroscopy=== | |||
* Cerebral (mild) & cerebellar atrophy. | |||
* greenish [[putamen]]. | |||
* Discoloration [[Substantia nigra]] and [[Locus coeruleus]] | |||
===Microscopic=== | ===Microscopic=== | ||
Features: | Features: | ||
*Alpha-synuclein-rich glial cytoplasmic inclusions (finding at autopsy).<ref name=pmid18825660>{{Cite journal | last1 = Wenning | first1 = GK. | last2 = Stefanova | first2 = N. | last3 = Jellinger | first3 = KA. | last4 = Poewe | first4 = W. | last5 = Schlossmacher | first5 = MG. | title = Multiple system atrophy: a primary oligodendrogliopathy. | journal = Ann Neurol | volume = 64 | issue = 3 | pages = 239-46 | month = Sep | year = 2008 | doi = 10.1002/ana.21465 | PMID = 18825660 }}</ref> | *Inclusions cerebral, subcortical white matter, cerebellar. | ||
*Neuronal loss and gliosis. | |||
*Alpha-synuclein-rich glial and neuronal cytoplasmic inclusions (finding at autopsy).<ref name=pmid18825660>{{Cite journal | last1 = Wenning | first1 = GK. | last2 = Stefanova | first2 = N. | last3 = Jellinger | first3 = KA. | last4 = Poewe | first4 = W. | last5 = Schlossmacher | first5 = MG. | title = Multiple system atrophy: a primary oligodendrogliopathy. | journal = Ann Neurol | volume = 64 | issue = 3 | pages = 239-46 | month = Sep | year = 2008 | doi = 10.1002/ana.21465 | PMID = 18825660 }}</ref> | |||
**Inclusions in oligodendrocytes.<ref>MUN. 16 November 2010.</ref> | **Inclusions in oligodendrocytes.<ref>MUN. 16 November 2010.</ref> | ||
*Pons and Putamen: | |||
** Nuclear inclusions (sparse in most cases). | |||
** Neuropil threads (alpha-synuclein). | |||
*Loss of myelinated fibers from external capsule, striatum and pallidum. | |||
===Images=== | |||
<gallery> | |||
File:Msa_macroscopy_putamen_discoloration.jpg | Gray-brown discoloration in putamen of a striatonigral-type MSA (WC/jensflorian). | |||
File:MSA Gallays Papp Lantos inclusions.jpg | Gallays silver stain showing MSA-typic inclusions (WC/jensflorian). | |||
File:MSA aSynuclein.jpg | a-synuclein IHC showing Glial and neuronal cytoplasmic inclusion in the pons of a MSA case (WC/jensflorian). | |||
</gallery> | |||
===Molecular=== | |||
* No known alpha-synuclein mutation. | |||
* Genetic variants of SNCA gene assoicated with MSA. <ref name=PMID2743996>{{Cite journal | last1 = Pimenta | first1 = PF. | last2 = da Silva | first2 = RP. | last3 = Sacks | first3 = DL. | last4 = da Silva | first4 = PP. | title = Cell surface nanoanatomy of Leishmania major as revealed by fracture-flip. A surface meshwork of 44 nm fusiform filaments identifies infective developmental stage promastigotes. | journal = Eur J Cell Biol | volume = 48 | issue = 2 | pages = 180-90 | month = Apr | year = 1989 | doi = | PMID = 2743996 }}</ref> | |||
=Tauopathies= | =Tauopathies= | ||