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Difference between revisions of "Neurodegenerative diseases"
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Neurodegenerative diseases (view source)
Revision as of 22:33, 16 November 2010
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==Overview== | ==Overview== | ||
They are essentially progressive and selective neuron loss. Clinically, they are not unique. They are defined by molecular pathology.<ref name=pmid19918325>{{cite journal |author=Dickson DW |title=Neuropathology of non-Alzheimer degenerative disorders |journal=Int J Clin Exp Pathol |volume=3 |issue=1 |pages=1–23 |year=2009 |pmid=19918325 |pmc=2776269 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2776269/?tool=pubmed}}</ref> | *They are essentially progressive and selective neuron loss. | ||
*Clinically, they are not unique, e.g. dementia can be caused by several diseases (with different molecular etiologies). | |||
*They are defined by molecular pathology.<ref name=pmid19918325>{{cite journal |author=Dickson DW |title=Neuropathology of non-Alzheimer degenerative disorders |journal=Int J Clin Exp Pathol |volume=3 |issue=1 |pages=1–23 |year=2009 |pmid=19918325 |pmc=2776269 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2776269/?tool=pubmed}}</ref> | |||
**The diseases are due to the accumulation of abnormal protein. | |||
***The amino acid sequence of the protein may be completely normal. The problem may just be folding/protein conformation. | |||
Molecular schema of neurodegenerative disorders:<ref name=pmid19918325/> | Molecular schema of neurodegenerative disorders:<ref name=pmid19918325/> | ||
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*Pick's disease. | *Pick's disease. | ||
Synucleinopathies: | Synucleinopathies:<ref name=pmid18855701>{{Cite journal | last1 = Uversky | first1 = VN. | title = Alpha-synuclein misfolding and neurodegenerative diseases. | journal = Curr Protein Pept Sci | volume = 9 | issue = 5 | pages = 507-40 | month = Oct | year = 2008 | doi = | PMID = 18855701 }}</ref> | ||
*Parkinson disease. | *Parkinson disease. | ||
*Dementia with Lewy bodies. | *Dementia with Lewy bodies. | ||
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*TDP-43. | *TDP-43. | ||
==General DDx | ==Clinical perspective (becoming obsolete)== | ||
*Alzheimer's dementia. | ===General (mostly useless) DDx=== | ||
*Alzheimer's dementia - most common. | |||
*Vascular. | *Vascular. | ||
**Multi-infarct dementia. | **Multi-infarct dementia. | ||
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*Multisystem atrophy. | *Multisystem atrophy. | ||
===Mnemonic=== | |||
Mnemonic ''VITAMIN D VEST'':<ref>TN06 PS19</ref> | Mnemonic ''VITAMIN D VEST'':<ref>TN06 PS19</ref> | ||
*Vitamin deficiency (B12, folate, thiamine). | *Vitamin deficiency (B12, folate, thiamine). | ||
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*Toxins (alcohol). | *Toxins (alcohol). | ||
==Lewy body | ===Functional anatomy of dementia=== | ||
*Hippocampus (essential for forming new memories). | |||
*Frontal lobe (essential for retrieval of memories). | |||
==Lewy body diseases== | |||
DDx: | |||
*Parkinson's disease. | |||
*Dementia with Lewy bodies. | |||
Etiology: | |||
*Alpha-synuclein. | |||
Clinical features of Dementia with Lewy bodies: | |||
*Parkinsonian features. | *Parkinsonian features. | ||
*Hallucinations (visual). | *Hallucinations (visual). | ||
*Progressive | *Progressive cognitive decline with fluctuations. | ||
==Multiple system atrophy== | ==Multiple system atrophy== | ||
*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> | Clinical findings variable: | ||
** | *Parkinsonism (stiatonigral degeneration). | ||
*Ataxia (olivo-panto-cerebellar degeneration). | |||
Etiology: | |||
*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 in oligodendrocytes.<ref>MUN. 16 November 2010.</ref> | |||
==Progressive supranuclear palsy== | ==Progressive supranuclear palsy== | ||
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*[http://commons.wikimedia.org/wiki/File:SpongiformChangeCJD.jpg CJD (WC)]. | *[http://commons.wikimedia.org/wiki/File:SpongiformChangeCJD.jpg CJD (WC)]. | ||
*[http://commons.wikimedia.org/wiki/File:VCJD_Tonsil.jpg vCJD - IHC (WC)]. | *[http://commons.wikimedia.org/wiki/File:VCJD_Tonsil.jpg vCJD - IHC (WC)]. | ||
==See also== | ==See also== | ||