Difference between revisions of "Neurodegenerative diseases"

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Neurodegenerative diseases (view source)

Revision as of 03:29, 31 March 2011

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'''Neurodegenerative diseases''' is a big part of [[neuropathology]].   
'''Neurodegenerative diseases''' is a big part of [[neuropathology]].   


==Overview==
=Overview=
*Neurodegenerative disease = essentially progressive and selective neuron loss.   
*Neurodegenerative disease = essentially progressive and selective neuron loss.   
*Clinically, they are not unique, e.g. dementia can be caused by several diseases (with different molecular etiologies).
*Clinically, they are not unique, e.g. dementia can be caused by several diseases (with different molecular etiologies).
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==IHC==
=Immunohistochemistry=
===Alpha-synuclein===
===Alpha-synuclein===
Look for:
Look for:
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*Lewy bodies. (???)
*Lewy bodies. (???)


==Clinical perspective==
=Clinical perspective=
===General (mostly useless) DDx===
===General (mostly useless) DDx===
*Alzheimer's dementia - most common.
*Alzheimer's dementia - most common.
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*Frontal lobe (essential for retrieval of memories).
*Frontal lobe (essential for retrieval of memories).


=Amyloidoses=
==Alzheimer disease==
==Alzheimer disease==
===General===
===General===
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**Important in microtubule assembly.
**Important in microtubule assembly.


==Lewy body diseases==
==Prion diseases==
Etiology:<ref name=pmid16609731>{{cite journal |author=Watts JC, Balachandran A, Westaway D |title=The expanding universe of prion diseases |journal=PLoS Pathog. |volume=2 |issue=3 |pages=e26 |year=2006 |month=March |pmid=16609731 |pmc=1434791 |doi=10.1371/journal.ppat.0020026 |url=}}</ref>
*Misfolded cell-surface protein called PrP(C).
 
Includes:<ref name=pmid16609731>{{cite journal |author=Watts JC, Balachandran A, Westaway D |title=The expanding universe of prion diseases |journal=PLoS Pathog. |volume=2 |issue=3 |pages=e26 |year=2006 |month=March |pmid=16609731 |pmc=1434791 |doi=10.1371/journal.ppat.0020026 |url=}}</ref>
*Creutzfeldt-Jakob disease (CJD).
*Sporadic fatal insomnia (sFI).
 
==Creutzfeldt-Jakob disease==
===General===
*Commonly abbreviated as ''CJD''.
*Rare.
*Incurable disease.
 
*Usually diagnosed clinically.
**Characteristic findings:
***Very rapid decline (3-4 months).
***Characteristic (cortex findings on) neuroradiology.
 
====Variant Creutzfeldt-Jakob disease (vCJD)====
*Associated with bovine spongiform encephalopathy.
*Should sample: spleen, lymph nodes, tonsils.<ref name=Ref_HospAuto83>{{Ref HospAuto|83}}</ref>
 
===Microscopic===
Features:
*Spongy appearance (cytoplasmic vacuolization<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/N0I002-PQ01-M.htm http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/N0I002-PQ01-M.htm]. Accessed on: 19 October 2010.</ref>).
 
Images:
*[http://commons.wikimedia.org/wiki/File:SpongiformChangeCJD.jpg CJD (WC)].
*[http://commons.wikimedia.org/wiki/File:VCJD_Tonsil.jpg vCJD - IHC (WC)].
 
=Lewy body diseases=
DDx:
DDx:
*Parkinson's disease.
*Parkinson's disease.
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*Progressive cognitive decline with fluctuations.
*Progressive cognitive decline with fluctuations.


==Parkinson disease==
===General===
*Common.
Clinical ''TRAP'':<ref>URL: [http://www.nysslha.org/i4a/pages/index.cfm?pageid=3519 http://www.nysslha.org/i4a/pages/index.cfm?pageid=3519]. Accessed on: 30 March 2011.</ref>
*Tremor.
*Rigidity.
*Akinesia.
*Postural instability.
===Gross===
Features:<ref name=Ref_PBoD8_1319>{{Ref PBoD8|1319}}</ref>
*Abnormally pale substantia nigra.
**Pigmentation increases with age.
Notes:
*Substantia nigra is a midbrain structure.
**Image: [http://commons.wikimedia.org/wiki/File:Midbraincrosssection.png Midbrain - schematic (WC)].
===Microscopic===
Features:<ref name=Ref_PBoD8_1319>{{Ref PBoD8|1319}}</ref>
*Loss of pigmented (catecholaminergic) neurons in the substantia nigra.
*Gliosis - due to neuron loss.
*Lewy bodies (in remaining neurons) - '''key feature'''.
**Eosinophilic cytoplasmic inclusion with "dense" (darker) core and pale (surrounding) halo.
***Consist of filaments composed of alpha-synuclein.
===IHC===
*Alpha-synuclein +ve.
=Alpha-synucleinopathies=
==Multiple system atrophy==
==Multiple system atrophy==
===General===
===General===
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**Granular eosinophilic material adjacent to nuclei; once thought to be pathognomonic for PSP.<ref>URL: [http://neuropathologyblog.blogspot.com/2008/03/grumose-degeneration-in-cerebellar.html http://neuropathologyblog.blogspot.com/2008/03/grumose-degeneration-in-cerebellar.html]. Accessed on: 4 December 2010.</ref><ref>{{cite journal |author=Yamanouchi H, Yokoo H, Yuhara Y, ''et al.'' |title=An autopsy case of ornithine transcarbamylase deficiency |journal=Brain Dev. |volume=24 |issue=2 |pages=91–4 |year=2002 |month=March |pmid=11891099 |doi= |url=}}</ref>
**Granular eosinophilic material adjacent to nuclei; once thought to be pathognomonic for PSP.<ref>URL: [http://neuropathologyblog.blogspot.com/2008/03/grumose-degeneration-in-cerebellar.html http://neuropathologyblog.blogspot.com/2008/03/grumose-degeneration-in-cerebellar.html]. Accessed on: 4 December 2010.</ref><ref>{{cite journal |author=Yamanouchi H, Yokoo H, Yuhara Y, ''et al.'' |title=An autopsy case of ornithine transcarbamylase deficiency |journal=Brain Dev. |volume=24 |issue=2 |pages=91–4 |year=2002 |month=March |pmid=11891099 |doi= |url=}}</ref>


=Other=
==Huntington disease==
==Huntington disease==
===General===
===General===
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*Weakness.
*Weakness.


==Parkinson disease==
=See also=
===General===
*Common.
 
Clinical ''TRAP'':<ref>URL: [http://www.nysslha.org/i4a/pages/index.cfm?pageid=3519 http://www.nysslha.org/i4a/pages/index.cfm?pageid=3519]. Accessed on: 30 March 2011.</ref>
*Tremor.
*Rigidity.
*Akinesia.
*Postural instability.
 
===Gross===
Features:<ref name=Ref_PBoD8_1319>{{Ref PBoD8|1319}}</ref>
*Abnormally pale substantia nigra.
**Pigmentation increases with age.
 
Notes:
*Substantia nigra is a midbrain structure.
**Image: [http://commons.wikimedia.org/wiki/File:Midbraincrosssection.png Midbrain - schematic (WC)].
 
===Microscopic===
Features:<ref name=Ref_PBoD8_1319>{{Ref PBoD8|1319}}</ref>
*Loss of pigmented (catecholaminergic) neurons in the substantia nigra.
*Gliosis - due to neuron loss.
*Lewy bodies (in remaining neurons) - '''key feature'''.
**Eosinophilic cytoplasmic inclusion with "dense" (darker) core and pale (surrounding) halo.
***Consist of filaments composed of alpha-synuclein.
 
===IHC===
*Alpha-synuclein +ve.
 
==Prion diseases==
Etiology:<ref name=pmid16609731>{{cite journal |author=Watts JC, Balachandran A, Westaway D |title=The expanding universe of prion diseases |journal=PLoS Pathog. |volume=2 |issue=3 |pages=e26 |year=2006 |month=March |pmid=16609731 |pmc=1434791 |doi=10.1371/journal.ppat.0020026 |url=}}</ref>
*Misfolded cell-surface protein called PrP(C).
 
Includes:<ref name=pmid16609731>{{cite journal |author=Watts JC, Balachandran A, Westaway D |title=The expanding universe of prion diseases |journal=PLoS Pathog. |volume=2 |issue=3 |pages=e26 |year=2006 |month=March |pmid=16609731 |pmc=1434791 |doi=10.1371/journal.ppat.0020026 |url=}}</ref>
*Creutzfeldt-Jakob disease (CJD).
*Sporadic fatal insomnia (sFI).
 
==Creutzfeldt-Jakob disease==
===General===
*Commonly abbreviated as ''CJD''.
*Rare.
*Incurable disease.
 
*Usually diagnosed clinically.
**Characteristic findings:
***Very rapid decline (3-4 months).
***Characteristic (cortex findings on) neuroradiology.
 
====Variant Creutzfeldt-Jakob disease (vCJD)====
*Associated with bovine spongiform encephalopathy.
*Should sample: spleen, lymph nodes, tonsils.<ref name=Ref_HospAuto83>{{Ref HospAuto|83}}</ref>
 
===Microscopic===
Features:
*Spongy appearance (cytoplasmic vacuolization<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/N0I002-PQ01-M.htm http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/N0I002-PQ01-M.htm]. Accessed on: 19 October 2010.</ref>).
 
Images:
*[http://commons.wikimedia.org/wiki/File:SpongiformChangeCJD.jpg CJD (WC)].
*[http://commons.wikimedia.org/wiki/File:VCJD_Tonsil.jpg vCJD - IHC (WC)].
 
==See also==
*[[Neuropathology]].
*[[Neuropathology]].
*[[Neurohistology]].
*[[Neurohistology]].


==References==
=References=
{{Reflist|2}}
{{Reflist|2}}


[[Category:Neuropathology]]
[[Category:Neuropathology]]
Michael
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