Difference between revisions of "Neuropathology"

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Neuropathology (view source)

Revision as of 07:07, 14 November 2010

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*Characteristic MRI findings - present in asymptomatic individuals with mutation.
*Characteristic MRI findings - present in asymptomatic individuals with mutation.


===Etiology===
====Etiology====
*Mutation of ''Notch 3'' gene.<ref name=pmid15537516>{{Cite journal  | last1 = Kalaria | first1 = RN. | last2 = Viitanen | first2 = M. | last3 = Kalimo | first3 = H. | last4 = Dichgans | first4 = M. | last5 = Tabira | first5 = T. | title = The pathogenesis of CADASIL: an update. | journal = J Neurol Sci | volume = 226 | issue = 1-2 | pages = 35-9 | month = Nov | year = 2004 | doi = 10.1016/j.jns.2004.09.008 | PMID = 15537516 }}</ref>
*Mutation of ''Notch 3'' gene.<ref name=pmid15537516>{{Cite journal  | last1 = Kalaria | first1 = RN. | last2 = Viitanen | first2 = M. | last3 = Kalimo | first3 = H. | last4 = Dichgans | first4 = M. | last5 = Tabira | first5 = T. | title = The pathogenesis of CADASIL: an update. | journal = J Neurol Sci | volume = 226 | issue = 1-2 | pages = 35-9 | month = Nov | year = 2004 | doi = 10.1016/j.jns.2004.09.008 | PMID = 15537516 }}</ref>
**Diagnosis: proven ''Notch 3'' mutation.


===Diagnosis===
===Microscopic===
*Proven ''Notch 3'' mutation.
*Can be diagnosed on a skin biopsy.
*IHC for ''Notch 3'' -- +ve staining in Notch 3 mutants.
 
===Histology===
Features:
Features:
*Subcortical infarcts.
*Subcortical infarcts.
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*Binswanger's disease - multi-infarct dementia affecting subcortical white matter.
*Binswanger's disease - multi-infarct dementia affecting subcortical white matter.
**Often diagnosed as ''Alzheimer's disease'' in the past.
**Often diagnosed as ''Alzheimer's disease'' in the past.
====Skin biopsy diagnosis====
*Can be diagnosed on a skin biopsy.
**IHC for ''Notch 3''; +ve granular staining in small vessels in ''Notch 3'' mutants.


===Electron microscopy===
===Electron microscopy===
*Granular osmiophilic material (GOM).
*Granular osmiophilic material (GOM).
==Binswanger's disease==
===General===
*Multi-infarct dementia affecting subcortical white matter.
*Waste-basket diagnosis; diagnosed if CADASIL and amyloidosis have been excluded.
*Diagnosis has been controversial -- most with this entity (in the past) were diagnosed with ''Alzheimer's disease''.
===Microscopic===
Features:
*Subcortical lesions that replace the myelin consisting of macrophages.
==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=
=See also=
Michael
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