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'''Neuropathology''' is the bane of many anatomical pathologists in teaching hospitals... 'cause they have to fill in for the neuropathologist when he or she is on vacation.
[[Image:MCA-Stroke-Brain-Human-2.JPG|thumb|right|Gross image showing changes of a [[stroke]]. (WC/Marvin 101)]]
This article is an introduction to '''neuropathology'''. There are separate articles for [[brain tumours]], the [[pituitary gland]], the [[spine]], the [[eye]], [[muscle pathologies]], [[neurohistology]] and [[neuroanatomy]].


This article is an introduction to neuropathology.  There are separate articles for [[brain tumours]], the [[pituitary gland]], the [[spine]], the [[eye]], [[muscle pathologies]], [[neurohistology]] and [[neuroanatomy]].
Neuropathology is the bane of many anatomical pathologists in teaching hospitals... 'cause they have to fill in for the neuropathologist when he or she is on vacation.


==Neuroanatomy==
==Neuroanatomy==
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==Neuroradiology==
==Neuroradiology==
Enhancing vs. non-enhancing:
Key factors to consider in evaluation:
*If it is tumour... enhancing usu. high grade, non-enhancing usu. low grade.
# Location.
# Number of lesions - single versus multiple.
# Cystic versus solid lesion.
# Enhancement.
 
==Lesion location==
In neuroradiology and neuropathology, real estate is crucial. Lesion location can often narrow your differential.
 
Cortical lesions (gray matter):
* [[Oligodendroglioma]].
* [[DNET]].
* [[Ganglioglioma]].
* [[Pleomorphic xanthoastrocytoma]].
* Extraventricular [[ependymoma]].
 
Cortical-subcortical junction:
* [[brain metastasis|Metastases]].
* Abscesses (hematogenous spread).
 
Subcortical lesions (white matter):
* [[Glioblastoma]].
* Diffuse gliomas.
* Demyelinating plaques.
 
Deep gray matter lesions (e.g. basal ganglia):
* Gliomas.
* [[Hypertensive hemorrhage]]
 
Cerebellar lesions:
* [[Medulloblastoma]].
* [[Pilocytic astrocytoma]].
* [[AT/RT]].
 
Intraventricular lesions:
* [[Ependymoma]].
* [[Subependymoma]].
* [[Pilocytic astrocytoma]].
* [[Central neurocytoma]].
* Rosette forming glioneuronal tumour of the fourth ventricle.
 
Suprasellar (above the pituitary):
* [[Craniopharyngioma]].
* [[Germinoma]].
* [[Pilomyxoid astrocytoma]].
 
==Number of lesions==
If ''single'' lesion = think primary, neoplastic
If ''multiple'' lesions = think metastatic, neoplastic or infectious
'''NB: glioblastoma can be multifocal''' (and the foci can be quite far apart)
 
==Cystic vs. solid lesions==
Some tumours are classically cystic with a small solid component (so-called cyst with a mural nodule) -- e.g. pilocytic astrocytoma, ganglioglioma, hemangioblastoma
 
==Enhancing vs. non-enhancing:==
*In adults, enhancing generally = high grade.
*In pediatrics, it often depends on the pattern.
Two main patterns to be mindful of -- ring enhancing lesions, and cystic lesions with a mural nodule.


===Ring enhancing lesions===
===Ring enhancing lesions===
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*[[Toxoplasmosis]] - most common.<ref>MUN. Feb 3, 2009.</ref>
*[[Toxoplasmosis]] - most common.<ref>MUN. Feb 3, 2009.</ref>


Ring enhancing lesion (DDx) - mnemonic ''MAGICAL DR'':<ref>TN2005 NS7.</ref>
Ring enhancing lesion (DDx) - mnemonic ''MAGICAL DR'':<ref>{{Ref TN2005 |NS7}}</ref>
*Metstasis.
*Metstasis.
*Abscess.
*Abscess.
*Glioblastoma.
*[[Glioblastoma]].
*Infarct.
*[[Infarct]].
*Contusion.
*Contusion.
*AIDS-related.
*AIDS-related.
Line 25: Line 82:
*Demyelination (e.g. [[multiple sclerosis]]).
*Demyelination (e.g. [[multiple sclerosis]]).
*Resolving hematoma.
*Resolving hematoma.
===Cyst with enhancing mural nodule===
*hemangioblastoma (#1 in adults)
*pilocytic astrocytoma (#1 in peds)
*pleomorphic xanthoastrocytoma
*ganglioglioma


==Grossing==
==Grossing==
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*Pyknosis = nuclear shrinkage + darker staining.  
*Pyknosis = nuclear shrinkage + darker staining.  


Images:
=====Images=====
*[http://www.neuropathologyweb.org/chapter2/images2/2-1HIE.jpg Anoxic neurons (neuropathologyweb.org)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Alzheimer_type_II_astrocyte_high_mag_cropped.jpg Anoxic neurons (WC)].
Image:Alzheimer_type_II_astrocyte_high_mag_cropped.jpg | Anoxic neurons (WC)
Image:AcuteStroke_HE400x.jpg | Neurons in an acute stroke. (WC)
</gallery>
www:
*[http://neuropathology-web.org/chapter2/images2/2-anoxic.png Anoxic neurons (neuropathologyweb.org)].<ref>URL: [http://neuropathology-web.org/chapter2/chapter2aHIE.html http://neuropathology-web.org/chapter2/chapter2aHIE.html]. Accessed on: 10 December 2014.</ref>
*[http://moon.ouhsc.edu/kfung/iacp-olp/APAQ-Images/N1-MS-01-16.gif Anoxic neurons (ouhsc.edu)].<ref>URL: [http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/N1-MS-01-16-Ans.htm http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/N1-MS-01-16-Ans.htm] and [http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/n1-ms-01.htm http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/n1-ms-01.htm]. Accessed on: 31 October 2010.</ref>
*[http://moon.ouhsc.edu/kfung/iacp-olp/APAQ-Images/N1-MS-01-16.gif Anoxic neurons (ouhsc.edu)].<ref>URL: [http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/N1-MS-01-16-Ans.htm http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/N1-MS-01-16-Ans.htm] and [http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/n1-ms-01.htm http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/n1-ms-01.htm]. Accessed on: 31 October 2010.</ref>


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*Vitamin deficiency ([[pellagra]]).<ref name=pmid15577526>{{cite journal |author=Piercecchi-Marti MD, Pélissier-Alicot AL, Leonetti G, Tervé JP, Cianfarani F, Pellissier JF |title=Pellagra: a rare disease observed in a victim of mental and physical abuse |journal=Am J Forensic Med Pathol |volume=25 |issue=4 |pages=342–4 |year=2004 |month=December |pmid=15577526 |doi= |url=}}</ref>
*Vitamin deficiency ([[pellagra]]).<ref name=pmid15577526>{{cite journal |author=Piercecchi-Marti MD, Pélissier-Alicot AL, Leonetti G, Tervé JP, Cianfarani F, Pellissier JF |title=Pellagra: a rare disease observed in a victim of mental and physical abuse |journal=Am J Forensic Med Pathol |volume=25 |issue=4 |pages=342–4 |year=2004 |month=December |pmid=15577526 |doi= |url=}}</ref>


Images:
=====Images=====
*[http://commons.wikimedia.org/wiki/File:Central_chromatolysis_-_intermed_mag_-_cropped.jpg Central chromatolysis - intermed. mag. (WC)].
<gallery>
*[http://en.wikipedia.org/wiki/File:Central_chromatolysis_-_nf_-_very_high_mag.jpg Central chromatolysis - NF stain - very high mag. (WC)].
Image:Central_chromatolysis_-_intermed_mag_-_cropped.jpg | Central chromatolysis - intermed. mag. (WC)
 
Image:Central_chromatolysis_-_nf_-_very_high_mag.jpg | Central chromatolysis - NF stain - very high mag. (WC)
</gallery>
====Axonal swellings====
====Axonal swellings====
H&E:
H&E:
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**Classically described as "funnel-shaped" in benign astrocytes.<ref>MUN. 15 November 2010.</ref>
**Classically described as "funnel-shaped" in benign astrocytes.<ref>MUN. 15 November 2010.</ref>
*Peripheral nucleus.
*Peripheral nucleus.
*Image: [http://commons.wikimedia.org/wiki/File:Reactive_astrocytes_-_lfb_-_high_mag.jpg Reactive astrocytes - high mag. (WC)].
<gallery>
Image:Reactive_astrocytes_-_lfb_-_high_mag.jpg | Reactive astrocytes - high mag. (WC)
</gallery>


Alzheimer type II astrocyte:<ref>URL: [http://www.neuropathologyweb.org/chapter1/chapter1bAstrocytes.html http://www.neuropathologyweb.org/chapter1/chapter1bAstrocytes.html]. Accessed on: 2 July 2010.</ref>
Alzheimer type II astrocyte:<ref>URL: [http://www.neuropathologyweb.org/chapter1/chapter1bAstrocytes.html http://www.neuropathologyweb.org/chapter1/chapter1bAstrocytes.html]. Accessed on: 2 July 2010.</ref>
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*Indistinct cytoplasm.
*Indistinct cytoplasm.
*Found in the context of ''hepatic encephalopathy'' in basal ganglia and lower layers of cortex.<ref name=Ref_Klatt202>{{Ref Klatt|202}}</ref>
*Found in the context of ''hepatic encephalopathy'' in basal ganglia and lower layers of cortex.<ref name=Ref_Klatt202>{{Ref Klatt|202}}</ref>
*Images:  
*Images:
**[http://commons.wikimedia.org/wiki/File:Alzheimer_type_II_astrocyte_high_mag.jpg Alzheimer type II astrocytes (WC)].
**[http://neuroquiz.com/?page=image&topic=1&qid=2714 Alzheimer type II astrocytes (neuroquiz.com)].
**[http://neuroquiz.com/?page=image&topic=1&qid=2714 Alzheimer type II astrocytes (neuroquiz.com)].
**[http://www.neuropathologyweb.org/chapter10/images10/10-AlzIIl.jpg Alzheimer type II astrocytes (neuropathologyweb.org)] .
**[http://www.neuropathologyweb.org/chapter10/images10/10-AlzIIl.jpg Alzheimer type II astrocytes (neuropathologyweb.org)] .
<gallery>
Image:Alzheimer_type_II_astrocyte_high_mag.jpg| Alzheimer type II astrocytes. (WC)
</gallery>


Creutzfeldt cell:<ref name=Ref_PSNP18>{{Ref PSNP|18}}</ref>  
Creutzfeldt cell:<ref name=Ref_PSNP18>{{Ref PSNP|18}}</ref>  
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Bergmann gliosis (in the cerebellum):<ref name=Ref_PSNP18>{{Ref PSNP|18}}</ref>  
Bergmann gliosis (in the cerebellum):<ref name=Ref_PSNP18>{{Ref PSNP|18}}</ref>  
*Thin layer of cells (2-3 cells) with open nuclei that are larger than granular cell layer nuclei; seen with Purkinje cell loss.
*Thin layer of cells (2-3 cells) with open nuclei that are larger than granular cell layer nuclei; seen with Purkinje cell loss.
**Images:
 
***[http://commons.wikimedia.org/wiki/File:Bergmann_gliosis_-_intermed_mag.jpg Bergmann gliosis - intermed. mag. (WC)].
<gallery>
***[http://commons.wikimedia.org/wiki/File:Bergmann_gliosis_-_high_mag.jpg Bergmann gliosis - high mag. (WC)].
Image:Bergmann_gliosis_-_intermed_mag.jpg | Bergmann gliosis - intermed. mag. (WC)
***[http://commons.wikimedia.org/wiki/File:Metastatic_adenocarcinoma_-_cerebellum_-_intermed_mag.jpg Bergmann gliosis due to compression by metastasis - intermed mag. (WC)].
Image:Bergmann_gliosis_-_high_mag.jpg | Bergmann gliosis - high mag. (WC)
***[http://tpx.sagepub.com/content/35/5/676/F5.expansion.html Bergmann gliosis (sagepub.com)].
Image:Metastatic_adenocarcinoma_-_cerebellum_-_intermed_mag.jpg | Bergmann gliosis due to compression by metastasis - intermed mag. (WC)
</gallery>
Image:
*[http://tpx.sagepub.com/content/35/5/676/F5.expansion.html Bergmann gliosis (sagepub.com)].


====Reactive change vs. malignancy====
====Reactive change vs. malignancy====
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====Encephalitis====
====Encephalitis====
see also:
* [[Viruses]]
* [[Microorganisms]]
=====General=====
=====General=====
DDx:
DDx:
*Viral encephalitis.
*Viral encephalitis (Neurotrophic viruses):<ref>{{Cite journal  | last1 = Ludlow | first1 = M. | last2 = Kortekaas | first2 = J. | last3 = Herden | first3 = C. | last4 = Hoffmann | first4 = B. | last5 = Tappe | first5 = D. | last6 = Trebst | first6 = C. | last7 = Griffin | first7 = DE. | last8 = Brindle | first8 = HE. | last9 = Solomon | first9 = T. | title = Neurotropic virus infections as the cause of immediate and delayed neuropathology. | journal = Acta Neuropathol | volume = 131 | issue = 2 | pages = 159-84 | month = Feb | year = 2016 | doi = 10.1007/s00401-015-1511-3 | PMID = 26659576 }}</ref>
** Eteroviruses are the most common cause of aseptic meningitis.
***Coxackie Virus.
***Enteric cytopathic human orphan (ECHO) virus.
** Human Herpesviruses (HSV1, HSV2, VZV, CMV, EBV, Roseola)
*** HSV encephalitis has high mortality without acyclovir treatment.
*** Childhood cerebellitis mainly associated with varicella.
*** VZV is the second most common viral meningitis after enterovirus.
** Measles virus(worldwide more than 100.000 deaths annually).
***Is linked to [[acute demyelinating encephalomyelitis]] (ADEM) and Subacute sclerosing encephalitis (SSPE).
** Seasonal influenza A virus (highest patogenic potential: avian influenza H5N1).
** Polio and Non-Polio Enterovirus (mostly children).
***Although massive eradication: Polio still existent in Pakistan, Afghanistan, Nigeria.
** Rabies virus
** Tick-borne encephalitis virus (Europe, Siberia, Russian far-east).
** West-Nile virus (US, Southern europe).
** St. Louis encephalitis virus (US).
** Japanese encephalitis virus (South, south-east asia, high disability rate).
** La Crosse virus (esp. children, midwest & eastern US).
** Borna disease virus (VSBV-1).
** Equine encephalitis viruses (EEEV, VEEV, WEEV, CHIKV).
*Paraneoplastic syndromes.
*Paraneoplastic syndromes.
*Autoimmune antibody-mediated limbic encephalitis (NMDAR).
*Purulent bacterial encephalitis
**Streptococcus, [[Actinomyces]] ....
*Septic metastatic encephalitis
**microabscesses, local astrogliosis, two or more granulocytic infiltrates without relation to vessel.<ref>{{Cite journal  | last1 = Tauber | first1 = SC. | last2 = Bunkowski | first2 = S. | last3 = Brück | first3 = W. | last4 = Nau | first4 = R. | title = Septic metastatic encephalitis: coexistence of brain damage and repair. | journal = Neuropathol Appl Neurobiol | volume = 37 | issue = 7 | pages = 768-76 | month = Dec | year = 2011 | doi = 10.1111/j.1365-2990.2011.01196.x | PMID = 21696418 }}</ref>
*Septic embolic encephalitis
**Embolic endocarditis, Stroke-like lesions.<ref>{{Cite journal  | last1 = Bitsch | first1 = A. | last2 = Nau | first2 = R. | last3 = Hilgers | first3 = RA. | last4 = Verheggen | first4 = R. | last5 = Werner | first5 = G. | last6 = Prange | first6 = HW. | title = Focal neurologic deficits in infective endocarditis and other septic diseases. | journal = Acta Neurol Scand | volume = 94 | issue = 4 | pages = 279-86 | month = Oct | year = 1996 | doi =  | PMID = 8937541 }}</ref>
*Non-purulent bacterial encephalitis
** [[Tuberculosis]]...
=====Gross=====
*Frontal and temporal lobe - most common for HSV encephalitis.<ref>{{Ref APBR|416 Q47}}</ref>


=====Microscopic=====
=====Microscopic=====
Features:
Features:<ref name=pmid20051019>{{Cite journal  | last1 = Takei | first1 = H. | last2 = Wilfong | first2 = A. | last3 = Malphrus | first3 = A. | last4 = Yoshor | first4 = D. | last5 = Hunter | first5 = JV. | last6 = Armstrong | first6 = DL. | last7 = Bhattacharjee | first7 = MB. | title = Dual pathology in Rasmussen's encephalitis: a study of seven cases and review of the literature. | journal = Neuropathology | volume = 30 | issue = 4 | pages = 381-91 | month = Aug | year = 2010 | doi = 10.1111/j.1440-1789.2009.01079.x | PMID = 20051019 }}</ref>
*Perivascular and perineuronal inflammation.
*Perivascular inflammation.
*Microglia.
*+/-Neuronophagia.
**Phagocytosis of neurons.<ref>URL: [http://medical-dictionary.thefreedictionary.com/neuronophagia http://medical-dictionary.thefreedictionary.com/neuronophagia]. Accessed on: 11 April 2012.</ref>
*+/-Viral cytopathic changes.
*+/-Perineuronal inflammation.
 
Notes:
*Hemorrhage<ref name=pmid18246335>{{Cite journal  | last1 = Vossough | first1 = A. | last2 = Zimmerman | first2 = RA. | last3 = Bilaniuk | first3 = LT. | last4 = Schwartz | first4 = EM. | title = Imaging findings of neonatal herpes simplex virus type 2 encephalitis. | journal = Neuroradiology | volume = 50 | issue = 4 | pages = 355-66 | month = Apr | year = 2008 | doi = 10.1007/s00234-007-0349-3 | PMID = 18246335 }}</ref> and necrosis - characteristic of HSV encephalitis.
 
Image:
*[http://neuropathology-web.org/chapter5/images5/5-21l.jpg HSV encephalitis (neuropathology-web.org)].<ref>URL: [http://neuropathology-web.org/chapter5/chapter5dViruses.html http://neuropathology-web.org/chapter5/chapter5dViruses.html]. Accessed on: 27 January 2012.</ref>
 
=====IHC=====
IHC stains for:
*Viral etiologies, e.g. [[HSV]], [[CMV]].
*Parasites, e.g. [[toxoplasma]].
*[[Fungi]], e.g. PASD.
 
<gallery>
File:CNS_lymphocytic_encephalitis_frozen_section.jpg | Intraoperative appearance of a lymphocytic encephalitis (WC/jensflorian)
File:CNS_lymphocytic_encephalitis_FFPE_section.jpg | Perivascular inflammation in a lymphocytic encephalitis (WC/jensflorian)
File:HSV_hemorrhagic_encephalitis.jpg | Hemorrhage in HSV encephalitis (WC/jensflorian)
File:HSV_necrotizing_encephalitis.jpg | HSV encephalitis, higher magnification (WC/jensflorian)
File:Cmv_status_verrucosus.jpg | Neonatal brain with migration disturbances due to CMV infection (WC/jensflorian)
File:Cmv_neuronal_inclusions.jpg | Neuronal nuclear inclusions in a neonatal CMV infection (WC/jensflorian)
File:Rabies encephalitis PHIL 3368 lores.jpg | Rabies encephalitis (CDC.gov)
File:Rabies negri bodies brain.jpg | Negri bodies in Purkinje cells (CDC.gov)
</gallery>
 
====Vasculitis====
DDx Cerebral vasculitis / angiitis:
*[[Systemic lupus erythematosus]] (SLE)
*[[Rheumatoid arthritis]].
*Medications and drugs (amphetamine, cocaine and heroin).
*Paraneoplastic(lymphomas, leukemia and lung cancer).
*[[Granulomatosis_with_polyangiitis]]
*[[Giant cell arteritis]]
*[[Takayasu's arteritis]]
*[[Polyarteritis nodosa]]
*Beta-amyloid-related angiitis (ABRA)
 
<gallery>
File:ABRA_HE_40x.jpg | Beta-amyloid related angitis, HE (WC)
File:ABRA_beta-amyloid_40x.jpg |  Beta-amyloid related angiitis, abeta IHC (WC)
File:Giant cell arteritis -- low mag.jpg | Giant cell arteritis, HE (WC)
</gallery>


===Architecture===
===Architecture===
====All things rosette====
====Rosettes====
*Rosette = circular/flower-like arrangement of cells.<ref name=pmid16551982>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref>
*Rosette = circular/flower-like arrangement of cells.<ref name=pmid16551982>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref>


*Perivascular pseudorosette = circular/flower-like arrangement of cells with blood vessel at the centre.<ref name=pmid16551982>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref>
*Perivascular pseudorosette = circular/flower-like arrangement of cells with blood vessel at the centre.<ref name=pmid16551982>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref>
**Ependymoma.
**[[Ependymoma]].
**[[Medulloblastoma]], PNET.  
**[[Medulloblastoma]], PNET.  
**Central neurocytoma.
**[[Central neurocytoma]].
**[[Glioblastoma]]s.
**[[Glioblastoma]]s.


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***Image: [http://moon.ouhsc.edu/kfung/IACP-OLP/APAQ-Images/N1-TU-01-17.gif Medulloblastoma (ouhsc.edu)].<ref>URL: [http://moon.ouhsc.edu/kfung/IACP-OLP/APAQ-Text/N1-TU-01.htm#17 http://moon.ouhsc.edu/kfung/IACP-OLP/APAQ-Text/N1-TU-01.htm#17]. Accessed on: 3 December 2010.</ref>
***Image: [http://moon.ouhsc.edu/kfung/IACP-OLP/APAQ-Images/N1-TU-01-17.gif Medulloblastoma (ouhsc.edu)].<ref>URL: [http://moon.ouhsc.edu/kfung/IACP-OLP/APAQ-Text/N1-TU-01.htm#17 http://moon.ouhsc.edu/kfung/IACP-OLP/APAQ-Text/N1-TU-01.htm#17]. Accessed on: 3 December 2010.</ref>
**PNET (can be thought of as a supratentorial medulloblastoma) .
**PNET (can be thought of as a supratentorial medulloblastoma) .
**Neuroblastoma


*Flexner-Wintersteiner rosette = rosette with empty centre (donut hole).<ref name=pmid16551982/>
*Flexner-Wintersteiner rosette = rosette with empty centre (donut hole).<ref name=pmid16551982/>
**[[Retinoblastoma]]s.
**[[Retinoblastoma]]s.
**Pineoblastomas.
**[[Pineoblastomas]].
**Medulloepitheliomas.
**[[Medulloepithelioma]]s.


*True ependymal rosette = surrounds a space.<ref name=pmid16551982/>
*True ependymal rosette = surrounds a space.<ref name=pmid16551982/>
Line 179: Line 337:
**[[Pineocytoma]].
**[[Pineocytoma]].
**[[Neurocytoma]].
**[[Neurocytoma]].
**[[RGNT]] - Rosette forming glioneuronal tumor of the IVth ventricle.
*Radial (cartwheel) profiles = neoplastic cells anchoring to stromal vessels, shorter processes than in ependymal pseudorosettes
**[[Astroblastoma]].
**[[Glioblastoma]].
*Multilayered rosettes
**[[Ependymomblastoma]]s.
**[[Medulloepithelioma]]s.
*Meningeothelial rosettes
**[[Meningioma]] - a rare pattern.<ref>{{Cite journal  | last1 = Liverman | first1 = C. | last2 = Mafra | first2 = M. | last3 = Chuang | first3 = SS. | last4 = Shivane | first4 = A. | last5 = Chakrabarty | first5 = A. | last6 = Highley | first6 = R. | last7 = Hilton | first7 = DA. | last8 = Byrne | first8 = NP. | last9 = Wesseling | first9 = P. | title = A clinicopathologic study of 11 rosette-forming meningiomas: a rare and potentially confusing pattern. | journal = Acta Neuropathol | volume = 130 | issue = 2 | pages = 311-3 | month = Aug | year = 2015 | doi = 10.1007/s00401-015-1456-6 | PMID = 26106026 }}</ref>
<gallery>
Image:Neuroblastoma Homer Wright rosettes HE.jpg | Homer-Wright rosettes (WC).
Image:RGNT HE 2.jpg | Neurocytic rosettes (WC).
Image:Ependymoma H&E.jpg | Perivascular pseudorosette (WC).
Image:Ependymoblastoma ETMRjpg.jpg | Multilayered rosettes (WC).
Image:Ependymoblastomatous Rosette.jpg | Ependymoblastous rosettes (WC/Marvin101).
Image:Ependymoma true ependymal rosettes and pseudorosettes.jpg | True ependymal rosettes and pseudorosettes (WC).
Image:Astroblastoma HE Specimen.jpg | Radial (cartwheel) profiles (WC).
</gallery>


====Other====
====Other important histological features====
*Rosenthal fibres = worm-like or corkscrew-like (brightly) eosinophilic bodies; 10-40 micrometers.
*Rosenthal fibres = worm-like or corkscrew-like (brightly) eosinophilic bodies; 10-40 micrometers.
**Key feature: variable thickness; helps separate from RBCs.
**Key feature: variable thickness; helps separate from RBCs.
**Well-seen on trichrome stains.
**Well-seen on trichrome stains.  
**Images: [http://en.wikipedia.org/wiki/File:Rosenthal_HE_40x.jpg Rosenthal fibres (WP)], [http://commons.wikimedia.org/wiki/File:Rosenthal_fibers.jpg Rosenthal fibres - smear (WC/AFIP)].
<gallery>
*Eosinophilic granular bodies = related to Rosenthal fibres; round cytoplasmic hyaline droplets in astrocytes.<ref>{{Ref MBNP|11}}</ref>
Image:Rosenthal_HE_40x.jpg | Rosenthal fibres. (WP)
**Image: [http://commons.wikimedia.org/wiki/File:Pilocytic_Micro.jpg EGBs (WC/AFIP)].
Image:Rosenthal_fibers.jpg | Rosenthal fibres - smear (WC/AFIP)
</gallery>
*Eosinophilic granular bodies = related to Rosenthal fibres; round cytoplasmic hyaline droplets in [[astrocyte]]s.<ref>{{Ref MBNP|11}}</ref>
<gallery>
File:Pilocytic Micro.jpg | Eosinophilic granular bodies in pilocytic astrocytoma smear (WC/AFIP)
File:PXA HE x20.jpg | Eosinophilic granular body in a pleomorphic xanthoastrocytoma (WC/jensflorian)
</gallery>
 
*Pseudopalisading - picket fence-like alignment of cells; long axis of cells perpendicular to interface with other structures/cells.
*Pseudopalisading - picket fence-like alignment of cells; long axis of cells perpendicular to interface with other structures/cells.
**Pseudopalisading of tumour cells (around necrotic regions) is seen in glioblastoma.  
**Pseudopalisading of tumour cells (around necrotic regions) is seen in [[glioblastoma]].  
<gallery>
File:GBM pseudopalisading necrosis.jpg | Pseudopalisading necrosis in a glioblastoma (WC/jensflorian)
</gallery>
 
*Perivascular lymphocytic cuffing - Lymphocytes surrounding vessels.
** Seen in many inflammatory conditions including MS.
** Often seen in [[ganglioglioma]] and [[pleomorphic xanthoastrocytoma]].
** Less common in some gemistocytic [[astrocytoma]].
*** No association with survival. <ref>{{Cite journal  | last1 = Rossi | first1 = ML. | last2 = Jones | first2 = NR. | last3 = Candy | first3 = E. | last4 = Nicoll | first4 = JA. | last5 = Compton | first5 = JS. | last6 = Hughes | first6 = JT. | last7 = Esiri | first7 = MM. | last8 = Moss | first8 = TH. | last9 = Cruz-Sanchez | first9 = FF. | title = The mononuclear cell infiltrate compared with survival in high-grade astrocytomas. | journal = Acta Neuropathol | volume = 78 | issue = 2 | pages = 189-93 | month =  | year = 1989 | doi =  | PMID = 2750489 }}</ref>
<gallery>
File:Ganglioglioma lymphocytic cuffing PAS.jpg | Lymphocytic cuffing in [[ganglioglioma]] (WC/jensflorian)
</gallery>


Note:  
Note:  
Line 196: Line 395:
*Negri bodies.
*Negri bodies.
**Cytoplasmic inclusions; classically in Purkinje cells of the cerebellum, pyramidal cells of Ammon's horn.
**Cytoplasmic inclusions; classically in Purkinje cells of the cerebellum, pyramidal cells of Ammon's horn.
**Rabies.
**[[Rabies]].  
**Image: [http://commons.wikimedia.org/wiki/File:Rabies_encephalitis_Negri_bodies_PHIL_3377_lores.jpg Negri bodies (WC/CDC)].
<gallery>
Image:Rabies_encephalitis_Negri_bodies_PHIL_3377_lores.jpg | Negri bodies. (WC/CDC)
</gallery>
*Owl eye inclusions.
**Basiopilic neuronal inclusions in enlarged cells, typically seen in CMV encephalitis
<gallery>
Image:CMV encephalitis owl eye inclusions HE stain.jpg | Owl eye inclusions
</gallery>


*Lewy bodies.
*Lewy bodies.
**Eosinophilic cytoplasmic inclusion - composed mostly of alpha-synuclein.<ref name=pmid15235805>{{cite journal |author=Marui W, Iseki E, Kato M, Akatsu H, Kosaka K |title=Pathological entity of dementia with Lewy bodies and its differentiation from Alzheimer's disease |journal=Acta Neuropathol. |volume=108 |issue=2 |pages=121–8 |year=2004 |month=August |pmid=15235805 |doi=10.1007/s00401-004-0869-4 |url=}}</ref>
**Eosinophilic cytoplasmic inclusion - composed mostly of alpha-synuclein.<ref name=pmid15235805>{{cite journal |author=Marui W, Iseki E, Kato M, Akatsu H, Kosaka K |title=Pathological entity of dementia with Lewy bodies and its differentiation from Alzheimer's disease |journal=Acta Neuropathol. |volume=108 |issue=2 |pages=121–8 |year=2004 |month=August |pmid=15235805 |doi=10.1007/s00401-004-0869-4 |url=}}</ref>
**Image: [http://commons.wikimedia.org/wiki/File:Lewy_Koerperchen.JPG Lewy body (WC)].
<gallery>
 
Image:Lewy_Koerperchen.JPG | Lewy body. (WC)
</gallery>
====Table of inclusions====
====Table of inclusions====
{| class="wikitable"  
{| class="wikitable sortable"  
| Feature  
! Feature  
| Appearance
! Appearance
| Associated disease
! Associated disease
| Comment
! Comment
| Image
! Image
|-
|-
| Grumous bodies<br>[[AKA]] granular bodies
| Grumose bodies<br>[[AKA]] granular bodies
| granular and eosinophilic ~50 micrometers
| granular and eosinophilic ~50 micrometers
| neurodegenerative disease, neuroaxonal dystrophies, aging
| neurodegenerative disease, neuroaxonal dystrophies, aging
Line 227: Line 434:
| [[Parkinson disease]], dementia with Lewy bodies
| [[Parkinson disease]], dementia with Lewy bodies
| morphology dependent on <br>location in brain; +ve for alpha-synuclein, <br>alpha-B crystallin, ubiquitin
| morphology dependent on <br>location in brain; +ve for alpha-synuclein, <br>alpha-B crystallin, ubiquitin
| [http://commons.wikimedia.org/wiki/File:Lewy_Koerperchen.JPG], [http://firstaidteam.com/usmlerximages/v/USMLERxLewy+bodies.gif.html]
| [[Image:Lewy_Koerperchen.JPG |thumb|center|150px|]], [http://firstaidteam.com/usmlerximages/v/USMLERxLewy+bodies.gif.html]
|-  
|-  
| Lafora body
| Lafora body
Line 245: Line 452:
| rabies
| rabies
| found in hippocampal neurons and Purkinje cells
| found in hippocampal neurons and Purkinje cells
| [http://commons.wikimedia.org/wiki/File:Rabies_encephalitis_Negri_bodies_PHIL_3377_lores.jpg]
| [[Image:Rabies_encephalitis_Negri_bodies_PHIL_3377_lores.jpg|thumb|center|150px|]]
|-  
|-  
| Hirano body
| Hirano body
Line 299: Line 506:
Standard work-up:
Standard work-up:
*GFAP.
*GFAP.
*p53.
*MAP2C. <ref>{{Cite journal  | last1 = Blümcke | first1 = I. | last2 = Müller | first2 = S. | last3 = Buslei | first3 = R. | last4 = Riederer | first4 = BM. | last5 = Wiestler | first5 = OD. | title = Microtubule-associated protein-2 immunoreactivity: a useful tool in the differential diagnosis of low-grade neuroepithelial tumors. | journal = Acta Neuropathol | volume = 108 | issue = 2 | pages = 89-96 | month = Aug | year = 2004 | doi = 10.1007/s00401-004-0873-8 | PMID = 15146346 }}</ref>
*Ki-67.
*Ki-67 (MIB-1).
 
Useful additional markers:
*IDH1(R132H) in Astrocytic/Oligodendroglial tumors. <ref>{{Cite journal  | last1 = Paulus | first1 = W. | title = GFAP, Ki67 and IDH1: perhaps the golden triad of glioma immunohistochemistry. | journal = Acta Neuropathol | volume = 118 | issue = 5 | pages = 603-4 | month = Nov | year = 2009 | doi = 10.1007/s00401-009-0600-6 | PMID = 19847448 }}</ref>
*ATRX in mixed gliomas. <ref>{{Cite journal  | last1 = Reuss | first1 = DE. | last2 = Sahm | first2 = F. | last3 = Schrimpf | first3 = D. | last4 = Wiestler | first4 = B. | last5 = Capper | first5 = D. | last6 = Koelsche | first6 = C. | last7 = Schweizer | first7 = L. | last8 = Korshunov | first8 = A. | last9 = Jones | first9 = DT. | title = ATRX and IDH1-R132H immunohistochemistry with subsequent copy number analysis and IDH sequencing as a basis for an "integrated" diagnostic approach for adult astrocytoma, oligodendroglioma and glioblastoma. | journal = Acta Neuropathol | volume = 129 | issue = 1 | pages = 133-46 | month = Jan | year = 2015 | doi = 10.1007/s00401-014-1370-3 | PMID = 25427834 }}</ref>
*EMA in Ependymal tumors. <ref>{{Cite journal  | last1 = Hasselblatt | first1 = M. | last2 = Paulus | first2 = W. | title = Sensitivity and specificity of epithelial membrane antigen staining patterns in ependymomas. | journal = Acta Neuropathol | volume = 106 | issue = 4 | pages = 385-8 | month = Oct | year = 2003 | doi = 10.1007/s00401-003-0752-8 | PMID = 12898159 }}</ref>
*OLIG-2 usually -ve in Ependymomas. <ref>{{Cite journal  | last1 = Ishizawa | first1 = K. | last2 = Komori | first2 = T. | last3 = Shimada | first3 = S. | last4 = Hirose | first4 = T. | title = Olig2 and CD99 are useful negative markers for the diagnosis of brain tumors. | journal = Clin Neuropathol | volume = 27 | issue = 3 | pages = 118-28 | month =  | year =  | doi =  | PMID = 18552083 }}</ref><ref>{{Cite journal  | last1 = Otero | first1 = JJ. | last2 = Rowitch | first2 = D. | last3 = Vandenberg | first3 = S. | title = OLIG2 is differentially expressed in pediatric astrocytic and in ependymal neoplasms. | journal = J Neurooncol | volume = 104 | issue = 2 | pages = 423-38 | month = Sep | year = 2011 | doi = 10.1007/s11060-010-0509-x | PMID = 21193945 }}</ref>
===Neuronal===
===Neuronal===
*Synaptophysin.
*Synaptophysin.
Line 315: Line 526:
=Brain tumours=
=Brain tumours=
{{main|Neuropathology tumours}}
{{main|Neuropathology tumours}}
Tumours are a big part of neuropathology.  The most common brain tumour is a metastasis.  The most common primary tumour (in adults) is glioblastoma which has a horrible prognosis.
Tumours are a big part of neuropathology.  The most common brain tumour (in adults) is a metastasis.  The most common primary tumours originating in the brain (in adults) are [[glioma]]s. More than 50% of these are classified as [[glioblastoma]] which has a horrible prognosis.


=Non-tumour=
=Non-tumour=
==Cerebral hemorrhage==
==Vascular disorders==
===Cerebral hemorrhage===
:See: ''[[Intracranial hematoma]]'' for intracranial bleeds
:See: ''[[Intracranial hematoma]]'' for intracranial bleeds


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*[[Intracerebral hematoma]]s.
*[[Intracerebral hematoma]]s.


==Duret hematoma==
===Duret hematoma===
*[[AKA]] Duret hemorrhage.
*[[AKA]] Duret hemorrhage.
===General===
====General====
*Bleed in the upper brainstem (midbrain and pons).
*Bleed in the upper brainstem (midbrain and pons).
**Thought to be due to transtentorial herniation secondary to supratentorial mass effect (e.g. supratentorial tumour, [[intracranial hemorrhage]]).<ref name=pmid11819006>{{Cite journal  | last1 = Parizel | first1 = PM. | last2 = Makkat | first2 = S. | last3 = Jorens | first3 = PG. | last4 = Ozsarlak | first4 = O. | last5 = Cras | first5 = P. | last6 = Van Goethem | first6 = JW. | last7 = van den Hauwe | first7 = L. | last8 = Verlooy | first8 = J. | last9 = De Schepper | first9 = AM. | title = Brainstem hemorrhage in descending transtentorial herniation (Duret hemorrhage). | journal = Intensive Care Med | volume = 28 | issue = 1 | pages = 85-8 | month = Jan | year = 2002 | doi = 10.1007/s00134-001-1160-y | PMID = 11819006 }}</ref>
**Thought to be due to transtentorial herniation secondary to supratentorial mass effect (e.g. supratentorial tumour, [[intracranial hemorrhage]]).<ref name=pmid11819006>{{Cite journal  | last1 = Parizel | first1 = PM. | last2 = Makkat | first2 = S. | last3 = Jorens | first3 = PG. | last4 = Ozsarlak | first4 = O. | last5 = Cras | first5 = P. | last6 = Van Goethem | first6 = JW. | last7 = van den Hauwe | first7 = L. | last8 = Verlooy | first8 = J. | last9 = De Schepper | first9 = AM. | title = Brainstem hemorrhage in descending transtentorial herniation (Duret hemorrhage). | journal = Intensive Care Med | volume = 28 | issue = 1 | pages = 85-8 | month = Jan | year = 2002 | doi = 10.1007/s00134-001-1160-y | PMID = 11819006 }}</ref>
*Often fatal.<ref name=pmid11098635>{{Cite journal  | last1 = Fujimoto | first1 = Y. | last2 = Aguiar | first2 = PH. | last3 = Freitas | first3 = AB. | last4 = de Andrade | first4 = AF. | last5 = Marino Júnior | first5 = R. | title = Recovery from Duret hemorrhage: a rare complication after craniotomy--case report. | journal = Neurol Med Chir (Tokyo) | volume = 40 | issue = 10 | pages = 508-10 | month = Oct | year = 2000 | doi =  | PMID = 11098635 }}</ref>
*Often fatal.<ref name=pmid11098635>{{Cite journal  | last1 = Fujimoto | first1 = Y. | last2 = Aguiar | first2 = PH. | last3 = Freitas | first3 = AB. | last4 = de Andrade | first4 = AF. | last5 = Marino Júnior | first5 = R. | title = Recovery from Duret hemorrhage: a rare complication after craniotomy--case report. | journal = Neurol Med Chir (Tokyo) | volume = 40 | issue = 10 | pages = 508-10 | month = Oct | year = 2000 | doi =  | PMID = 11098635 }}</ref>
===Gross===
====Gross====
*Extravasated blood in midbrain and pons - usu. ventral (anterior) and paramedian (adjacent to the midline).<ref name=pmid11819006/>
*Extravasated blood in midbrain and pons - usu. ventral (anterior) and paramedian (adjacent to the midline).<ref name=pmid11819006/>


Line 339: Line 551:
*[http://library.med.utah.edu/WebPath/jpeg5/CNS037.jpg Duret hemorrhage (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/npfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/npfrm.html]. Accessed on: 4 December 2011.</ref>
*[http://library.med.utah.edu/WebPath/jpeg5/CNS037.jpg Duret hemorrhage (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/npfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/npfrm.html]. Accessed on: 4 December 2011.</ref>


===Microscopic===
====Microscopic====
Features:
Features:
*RBC extravasation.  
*RBC extravasation.  
*+/-Hemosiderin-laden macrophages.
*+/-Hemosiderin-laden macrophages.
*+/-Ischemic neurons.
*+/-Ischemic neurons.
===Cerebral amyloid angiopathy===
====General====
*Abbreviated ''CAA''.
*Disease of the old.
*Strong association with ''[[lobar haemorrhage]]'' (bleeds of the cerebellar cortex and cerebral cortex).<ref name=pmid16982664>{{cite journal |author=Thanvi B, Robinson T |title=Sporadic cerebral amyloid angiopathy--an important cause of cerebral haemorrhage in older people |journal=Age Ageing |volume=35 |issue=6 |pages=565–71 |year=2006 |month=November |pmid=16982664 |doi=10.1093/ageing/afl108 |url=}}</ref>
Etiology:
*[[Amyloid]] deposition in the basal lamina of smooth muscle (in the cerebellar cortex and cerebral cortex).
====Gross====
*Bleeds typically superficial (cortex and subcortical white matter) and in the frontal lobe or parietal lobe.<ref name=pmid17297004>{{Cite journal  | last1 = Haacke | first1 = EM. | last2 = DelProposto | first2 = ZS. | last3 = Chaturvedi | first3 = S. | last4 = Sehgal | first4 = V. | last5 = Tenzer | first5 = M. | last6 = Neelavalli | first6 = J. | last7 = Kido | first7 = D. | title = Imaging cerebral amyloid angiopathy with susceptibility-weighted imaging. | journal = AJNR Am J Neuroradiol | volume = 28 | issue = 2 | pages = 316-7 | month = Feb | year = 2007 | doi =  | PMID = 17297004 | URL = http://www.ajnr.org/content/28/2/316.long }}</ref>
====Microscopic====
Features:
*Amorphous, acellular eosinophilic material within walls of small arteries.
**This is a high power diagnosis with congo red staining.
Notes:
*Amyloidosis is seen in all individuals with [[Alzheimer's disease]]; the amount of amyloid is what differs -- in CAA it is lots and lots.
*The white matter is typically spared by CAA.<ref name=pmid19225408>{{Cite journal  | last1 = Schröder | first1 = R. | last2 = Deckert | first2 = M. | last3 = Linke | first3 = RP. | title = Novel isolated cerebral ALlambda amyloid angiopathy with widespread subcortical distribution and leukoencephalopathy due to atypical monoclonal plasma cell proliferation, and terminal systemic gammopathy. | journal = J Neuropathol Exp Neurol | volume = 68 | issue = 3 | pages = 286-99 | month = Mar | year = 2009 | doi = 10.1097/NEN.0b013e31819a87f9 | PMID = 19225408 }}
</ref>
====Images====
<gallery>
Image:Cerebral_amyloid_angiopathy_-_very_high_mag.jpg |CAA - congo red - very high mag. (WC)
Image:Cerebral_amyloid_angiopathy_-_low_mag.jpg |CAA - congo red - low mag. (WC)
Image:Cerebral_amyloid_angiopathy_-2b-_amyloid_beta_-_high_mag.jpg |CAA - beta-amyloid - high mag. (WC)
</gallery>
====Stains====
*[[Congo red]].
====IHC====
*Abeta-amyloid (AKA beta-amyloid).
===Cerebral amyloid angiopathy associated with inflammation (I-CAA)===
* Cognitive decline.
* Microbleedings in MRI.
* Responsive to steroids.
* Abeta deposits in vessels.
* Perivascular lymphocytic infiltrate (but no vasculitis!).
* Giant cells may be present.
===Vascular malformations===
{{Main|Vascular malformations}}
Types:<ref name=pmid17076525>{{cite journal |author=Prayson RA, Kleinschmidt-DeMasters BK |title=An algorithmic approach to the brain biopsy--part II |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=11 |pages=1639–48 |year=2006 |month=November |pmid=17076525 |doi= |url=}}</ref>
*[[Vascular_malformations#Arteriovenous_malformation|Arteriovenous malformation]].
*Varix.
*Venous angioma.
*[[Vascular_malformations#Cavernous_hemangioma|Cavernous hemangioma]]  (Cavernoma).
*Capillary teleangiectasia.
Also see: ''[[Sturge-Weber syndrome]]''.
===Atherosclerosis===
{{Main|Vascular_disease#Atherosclerosis}}
*Intracranial atherosclerosis most common at circle of Willis.
*Macroscopic yellow discoloration.
*Luminal stenosis and eccentric intimal thickening.
<gallery>
File:Carotid Plaque (121061911).jpg|Plaque of the carotid bifurcation (Ed Uthman).
</gallery>
===Other large arterial diseases===
*[[Vascular_disease#Fibromuscular_dysplasia|Fibromuscular dysplasia]].
*Moyamoya disease.
**Progressive stenosis of basal intracranial arteries and abnormal vascularization.
*[[Aortic dissection|Arterial dissection]].
*[[Giant cell arteritis]].
*[[Takayasu's arteritis]].
*[[Antiphospholipid antibody syndrome|Antiphospholipid antibody]] mediated thrombosis.
===Microangiopathy===
*Defined as Small vessel disease (<300µm in transverse section).
*Includes atherosclerosis and cerebral amyloid angiopathy.
Other causes:
*Primary angitis of the CNS (PACNS).
*[[Polyarteritis nodosa]].
*[[Granulomatosis with polyangiitis]] (Wegener's granulomatosis).
*[[Lymphomatoid granulomatosis]].
*Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (commonly abbreviated ''CADASIL'').
{{Main|Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy}}
===Hypoxic-ischemic encephalopathy===
*Abbreviated ''HIE''.
{{Main|Hypoxic-ischemic encephalopathy}}
**Hypoxia: reduction in oxygen supply or utilization.
**Ischemia: reduction in blood supply.
===Cerebrovascular accident===
*Abbreviated ''CVA''.
*[[AKA]] ''stroke''.
{{Main|Cerebrovascular accident}}
*Stroke includes:
**Infarction (ischemia in defined vascular distribution persisting for at least 24hrs).
**Intracrebral hemorrhage (focal blood accumulation in the brain parenchyma).
**Subarachnoid hemorrhage (SAH).
**Cerebral venous thrombosis (CVT).


==Alcohol & CNS==
==Alcohol & CNS==
Line 372: Line 682:
General:
General:
*Due to thiamine deficiency.
*Due to thiamine deficiency.
**Malnourishment often accompanies [[alcoholism]].


Features:
Features:<ref name=pmid3929155>{{Cite journal  | last1 = Torvik | first1 = A. | title = Two types of brain lesions in Wernicke's encephalopathy. | journal = Neuropathol Appl Neurobiol | volume = 11 | issue = 3 | pages = 179-90 | month =  | year =  | doi =  | PMID = 3929155 }}</ref>
*Neurons preserved - '''key'''.
*Neurons of mammillary bodies preserved - '''key'''.
*Loss of myelin.
*Loss of myelin.
*Hemorrhage.
*Hemorrhage.
*Spongiosis.
*Edema.
*Reactive blood vessels.
*Reactive blood vessels.


Note:
*The thalamus and inferior olives show neuronal loss.<ref name=pmid3929155/>


====Common non-specific findings====
====Common non-specific findings====
Line 394: Line 707:
*+/-Decreased level of consciousness.
*+/-Decreased level of consciousness.


Etiology:
CSF findings:
{| class="wikitable sortable"
! Type
! Glucose
! Protein
! Cells
! Cytopathology
|-
| Bacterial, acute
| low
| high
| neutrophils
| [[File:Purulent_CSF.jpg|100px|center|]][[CSF_cytopathology#Acute_bacterial_meningitis|Cytophathology]]
|-
| Viral
| normal
| slight elevation
| lymphocytes
| [[CSF_cytopathology#Viral_meningitis|Cytophathology]]
|}
 
 
 
====Etiology====
*Infectious.
*Infectious.
**Bacterial.
**Bacterial.
Line 401: Line 737:
*Autoimmune.
*Autoimmune.
*Toxic.
*Toxic.
*Aseptic - see ''[[Mollaret's meningitis]]''.
Bacterial meningitis - most probably cause by age:<ref>{{Ref PCPBoD8|666-7}}</ref>
{| class="wikitable sortable"
! Age
! Organism
|-
| Neonate
| ''Escherichia coli'', ''Group B Streptococcus''
|-
| Infants, children
| ''Streptococcus pneumoniae''
|-
| Adolescents, young adults
| ''Neisseria meningitidis''
|-
| Elderly
| ''Streptococcus pneumoniae'', ''Listeria monocytogenes''
|}


===Gross===
===Gross===
Line 409: Line 764:
*+/-Cerebral edema.
*+/-Cerebral edema.


Image:
====Image====
*[http://en.wikipedia.org/wiki/File:Streptococcus_pneumoniae_meningitis,_gross_pathology_33_lores.jpg Meningitis (WP)].
<gallery>
Image:Streptococcus_pneumoniae_meningitis,_gross_pathology_33_lores.jpg | Streptococcus Meningitis. (WC/Dr. Edwin P. Ewing, Jr.)
File:Haemophilus influenzae meningitis 5003 lores.jpg | Hemophilus influenza Meningitis. (WC/CDC)
File:Pneumococcal meningitis.jpg | Pneumococcus Meningitis. (WC/Dr. Yale Rosen)
File:Meningitis-MRI.JPG | Bacterial Meningitis MRI (WC/MBq)
</gallery>


===Microscopic===
===Microscopic===
Features:
Features:
*Inflammation of the meninges.
*Inflammation of the meninges:
*Infectious meningitis:
**+/-[[Neutrophil]]s.
**Microorganisms (bacteria, [[fungi]]).
**+/-Lymphocytes.
**+/-[[Plasma cell]]s.
*+/-Microorganisms (infectious meningitis):  
**Bacteria.
**[[Fungi]], e.g. [[aspergillosis]] (may be intravascular).
 
Main DDx:
*[[Lymphoma]].
 
====Image====
<gallery>
Image:Meningitis_Histopathology.jpg | Bacterial meningitis. (WC/Marvin101)
</gallery>


==Cerebral abscess==
==Cerebral abscess==
Line 433: Line 805:
==Neurodegenerative diseases==
==Neurodegenerative diseases==
{{Main|Neurodegenerative diseases}}
{{Main|Neurodegenerative diseases}}
This is a hueueuge topic.  It is covered its own article and includes a general discussion of dementia.
This is a hueueuge topic.  It is covered in its own article and includes a general discussion of dementia.
 
==Malformation of cortical development(MCD) ==
===Lissencephaly===
* Greek: ‘lissos': smooth and ‘enkephalos': brain.
* Absent (agyria) or decreased (pachygyria) convolutions.
* Cortical thickening
* Smooth cerebral surface
* Subtypes with different layering: 2-layered, 3-layered, and 4-layered forms.
* Heterotopic neurons in a pattern suggestive of laminar organization.
* 14 LIS mutations account for 90% of all cases.<ref name="pmid27781032">{{cite journal |authors=Parrini E, Conti V, Dobyns WB, Guerrini R |title=Genetic Basis of Brain Malformations |journal=Mol Syndromol |volume=7 |issue=4 |pages=220–233 |date=September 2016 |pmid=27781032 |pmc=5073505 |doi=10.1159/000448639 |url=}}</ref>
 
===Polymicrogyria===
* Abnormal cortical lamination.
* Abnormally small and partly fused gyri.
* Can be unilateral, bilateral and symmetrical.
* Intellectual disability.
* Sometimes severe encephalopathy.
* Pharmacoresistant epilepsy
* Cortical lamination can be unlayered or four-layered.
** Unlayered:  Unorganized radial distribution of neurons.
** Four-layered:  Molecular layer, outer neuronal layer, nerve fiber layer, and inner neuronal layer.
* 1q trisomy in unilateral cases.<ref name="pmid32979071">{{cite journal |authors=Kobow K, Jabari S, Pieper T, Kudernatsch M, Polster T, Woermann FG, Kalbhenn T, Hamer H, Rössler K, Mühlebner A, Spliet WGM, Feucht M, Hou Y, Stichel D, Korshunov A, Sahm F, Coras R, Blümcke I, von Deimling A |title=Mosaic trisomy of chromosome 1q in human brain tissue associates with unilateral polymicrogyria, very early-onset focal epilepsy, and severe developmental delay |journal=Acta Neuropathol |volume=140 |issue=6 |pages=881–891 |date=December 2020 |pmid=32979071 |doi=10.1007/s00401-020-02228-5 |url=}}</ref>


==Epilepsy==
==Epilepsy==
===General===
{{Main|Epilepsy}}
*Seizure that are "idiopathic", i.e. no brain tumour, no mass lesion, no brain injury.
===Focal cortical dysplasia (FCD)===
*Most common form: ''temporal lobe epilepsy''.<ref>URL: [http://emedicine.medscape.com/article/342150-overview http://emedicine.medscape.com/article/342150-overview]. Accessed on: 20 November 2010.</ref>
*Localized malformations of the cortex.
*Frequently associated with epilepsy in children.
*Includes cortical dyslamination, cytoarchitectural changes and white matter abnormalities.
*Current consensus: ILAE classification scheme 2011 <ref>{{Cite journal  | last1 = Blümcke | first1 = I. | last2 = Aronica | first2 = E. | last3 = Miyata | first3 = H. | last4 = Sarnat | first4 = HB. | last5 = Thom | first5 = M. | last6 = Roessler | first6 = K. | last7 = Rydenhag | first7 = B. | last8 = Jehi | first8 = L. | last9 = Krsek | first9 = P. | title = International recommendation for a comprehensive neuropathologic workup of epilepsy surgery brain tissue: A consensus Task Force report from the ILAE Commission on Diagnostic Methods. | journal = Epilepsia | volume = 57 | issue = 3 | pages = 348-58 | month = Mar | year = 2016 | doi = 10.1111/epi.13319 | PMID = 26839983 }}
</ref>(based on previous classification by Palmini 2004):


===Microscopic===
Features:<ref>MUN. 15 November 2010.</ref>
*Mesial temporal sclerosis = scarring of the medial temporal lobe.
**Involves: hippocampus, parahippocampal gyrus and amygdala.
***Hippocampus: CA1 and CA4 affected.


Notes:
*Type I FCD (focal)
*Changes in CA1 & CA4 of the hippocampus - DDx:
**Ia: Abnormal radial cortical lamination.
**Epilpsy.
**Ib: Abnormal tangential cortical lamination.
**Dementia.
**Ic: Abnormal radial and tangential cortical lamination.


==Cysts==
===General===
*All are "benign", but some may be fatal due to spatial constraints.


===List of cysts===
*Type II FCD (focal)
*Colloid cyst.<ref>MUN. 11 Mar 2009.</ref>
**IIa: Presence of dysmorphic neurons.
**Columnar epithelium.
**IIb: Presence of dysmorphic neurons and balloon cells.
*Arachnoid cyst - considered precursor of meningioma.
**[[Psammoma bodies]].
**Clumps of cells.
**Whorled pattern.
*Dermoid cyst.
**Skin + adnexal structures.
**... think of ovarian dermoid.
*Epidermoid.
*Choriod cyst.
*Neuroenteric cyst.
**Foregut cyst with connection to dura.<ref>URL: [http://bhj.org/journal/2003_4502_april/neurentericcyst_373.htm http://bhj.org/journal/2003_4502_april/neurentericcyst_373.htm]. Accessed on: 19 December 2011.</ref>
***Gastrointestinal tract epithelium.
***Usually seen with vertebral anomalies.
*Epithelial cyst.
*Others.


==Cerebrovascular accident==
*Abbreviated ''CVA''.
*[[AKA]] ''stroke''.


===General===
*Type III FCD (associated with other lesion)
*Very common.
**IIIa: FCD associated with [[Epilepsy#Hippocampal_sclerosis|hippocampal sclerosis]].
*Leading cause of morbidity and mortality.
**IIIb: FCD adjacent to a brain tumor.
**IIIc: FCD adjacent to vascular malformation.
**IIIc: FCD associated with previous injury (trauma, inflammation...).


Clinical classification:
# Hemorrhagic stroke.
# Ischemic stroke.


===Gross===
<gallery>
*Soft/mushy brain.
File:FCDIIa dysmorphic neurons HE.jpg|Dysmorphic neurons in FCD (HE)
*Older infarcts.
File:FCDIIa neuronal heterotopia neun.jpg|Heterotopic neurons (NeuN)
**A "roof" is present - a thin submeningeal layer is preserved by the CSF.<ref>MUN. 16 December 2009.</ref>
</gallery>
***"Roof" is absent in trauma.
**Cavity - in older infarcts.
***''[[Multiple sclerosis]]'' does not cavitate.
*Laminar necrosis = (thin) chalky line replaces grey mater.<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/neurotest/Q03-Ans.htm http://moon.ouhsc.edu/kfung/jty1/neurotest/Q03-Ans.htm]. Accessed on: 26 October 2010.</ref>
**[[AKA]] ''pseudolaminar necrosis'' - as it is not localized to a specific layer of the cortex.<ref>MUN. 26 November 2010.</ref>


===Microscopic===
===Hamartia===
Features:
* Small collection of ectopic glioneuronal cells.
*Ischemic neurons.
**Morpholology resembling oligodendroglial-like cells. <ref>{{Cite journal  | last1 = Kasper | first1 = BS. | last2 = Stefan | first2 = H. | last3 = Buchfelder | first3 = M. | last4 = Paulus | first4 = W. | title = Temporal lobe microdysgenesis in epilepsy versus control brains. | journal = J Neuropathol Exp Neurol | volume = 58 | issue = 1 | pages = 22-8 | month = Jan | year = 1999 | doi =  | PMID = 10068310 }}</ref>
*+/-Neuronal loss.
* Mostly amygdala, less common in hippocampus or temporal lobe.
*+/-Microglial.
* Can coexist with focal cortical dysplasia.
*+/-[[Thrombosis]].
*+/-[[Atherosclerosis]].


==Hypoxic-ischemic encephalopathy==
==Demyelination==
*Abbreviated ''HIE''.
===General===
*Often due to ''cardiac arrest'', i.e. global ischemia.
*Triple watershed area = parieto-occipital cortex, extrastriate occipital cortex.


Note:
===Multiple sclerosis===
*''Hypoxia'' = blood decreased oxygen carrying capacity,<ref name=Ref_PCPBoD8_10>{{Ref PCPBoD8|10}}</ref> e.g. [[anemia]].
*Abbreviated ''MS''.
*''Ischemia'' = decreased blood flow.<ref name=Ref_PCPBoD8_10>{{Ref PCPBoD8|10}}</ref>
{{Main|Multiple sclerosis}}
*Either ''or'' both = less oxygen delivery to tissue.
===Microscopic===
Features:
*Hippocampal ischemic changes (in adults):
**Loss of neurons in CA1, CA3 and CA4 +/- "cavitation".
***Neuronal loss: No blue (nuclei) where there should be some.
***Cavitation: bubbles/clear spaces where there should be none.
**CA2 neurons preserved/resistant.
*Purkinje cell loss in the cerebellum and [[Bergmann gliosis]].
*"Anoxic neurons".<ref>URL: [http://www.neuropathologyweb.org/chapter2/chapter2aHIE.html http://www.neuropathologyweb.org/chapter2/chapter2aHIE.html]. Accessed on: 12 July 2010.</ref>
**Shrunken neurons with intensely eosinophilic cytoplasm and pyknotic (shrunken) nuclei.
*Pseudolaminar necrosis - (uncontrolled) cell death in the cerebral cortex in a band-like pattern,<ref>Hypoxic and Ischemic Encephalopathy. neuropathology.neoucom.edu. Accessed on: 29 December 2010.</ref> with a relative preservation of cells immediately adjacent to the meninges.


Images:
===Osmotic demyelination syndrome===
*Anoxic neurons:
{{Main|Osmotic demyelination syndrome}}
**[http://www.neuropathologyweb.org/chapter2/images2/2-1HIE.jpg Anoxic neurons (neuropathologyweb.org)].
*Previously known as ''central pontine myelinolysis'' (abbreviated ''CPM'').
**[http://commons.wikimedia.org/wiki/File:Alzheimer_type_II_astrocyte_high_mag_cropped.jpg Anoxic neurons (WC)].
**[http://moon.ouhsc.edu/kfung/iacp-olp/APAQ-Images/N1-MS-01-16.gif Anoxic neurons (ouhsc.edu)].<ref>URL: [http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/N1-MS-01-16-Ans.htm http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/N1-MS-01-16-Ans.htm] and [http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/n1-ms-01.htm http://moon.ouhsc.edu/kfung/iacp-olp/apaq-text/n1-ms-01.htm]. Accessed on: 31 October 2010.</ref>
*Hippocampal ischemic changes:
**[http://www.neuropathologyweb.org/chapter2/images2/2-hippoHIE.jpg Hippocampus in HIE (neuropathologyweb.org)].<ref>URL: [http://www.neuropathologyweb.org/chapter2/chapter2aHIE.html http://www.neuropathologyweb.org/chapter2/chapter2aHIE.html]. Accessed on: 14 January 2011.</ref>
*Pseudolaminar necrosis:
**[http://commons.wikimedia.org/wiki/File:Cortical_pseudolaminar_necrosis_-_lfb_-_very_low_mag.jpg Pseudolaminar necrosis - very low mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Cortical_pseudolaminar_necrosis_-_lfb_-_intermed_mag.jpg Pseudolaminar necrosis - intermed. mag. (WC)].


Notes:
===Acute disseminated encephalomyelitis===
*Neurons of ''subiculum'' in adults - usu. normal (as they are resistant to ischemic changes).
*Abbreviated ''ADEM''.
{{Main|Acute disseminated encephalomyelitis}}


==Multiple sclerosis==
===Neuromyelitis optica===
===General===
*Abbreviated ''NMO''.
*A bread 'n butter disease of neurology in Canada.


Clinical:
General:
*CSF: oligoclonal bands of immunoglobulin.<ref>{{Ref PBoD8|1311}}</ref>
*Rare autoimmune disease - once considered a variant of [[multiple sclerosis]].
**Autoantibodies directed at aquaporin-4.<ref name=pmid22087205>{{Cite journal  | last1 = Kim | first1 = W. | last2 = Kim | first2 = SH. | last3 = Kim | first3 = HJ. | title = New insights into neuromyelitis optica. | journal = J Clin Neurol | volume = 7 | issue = 3 | pages = 115-27 | month = Sep | year = 2011 | doi = 10.3988/jcn.2011.7.3.115 | PMID = 22087205 }}</ref>


===Radiologic/Gross===
Diagnosis:
Features:<ref>URL: [http://library.med.utah.edu/kw/ms/path.html http://library.med.utah.edu/kw/ms/path.html]. Accessed on: 12 July 2010.</ref>
*NMO-IgG.
*White matter lesions.
**Cerebrum (classically): periventricular distribution.
**Optic nerves (optic neuritis) - classic presentation.


===Microscopic===
Clinical - preferentially:
Features:<ref>URL: [http://library.med.utah.edu/kw/ms/path.html http://library.med.utah.edu/kw/ms/path.html]. Accessed on: 12 July 2010.</ref>
*Eye (optic neuritis).
*Perivascular inflammation.
*Spinal cord (myelitis).
*Demyelination.
**Subcortical myelinated fibers are often spared.


Classification of MS lesions:
Microscopic:
*Early active.
*Inflammation - lymphocytes, macrophages.
*Inactive.
*Reactive astrocytes.
*Early remyelinating.
*Late remyelinating.


Images:
Images:
*[http://path.upmc.edu/cases/case79.html Muliple sclerosis masquerading as a diffuse astrocytoma (upmc.edu)].
*[http://path.upmc.edu/cases/case637.html Neuromyelitis optica - several images (upmc.edu)].


===IHC===
IHC:
*HAM-56 - macrophages.
*Mixed lymphocyte population with CD3 > CD20.
*CD8 - lymphocytes.
*Aquaporin-4 loss.


==Cerebral amyloid angiopathy==
===Progressive multifocal leukoencephalopathy===
===General===
*Abbreviated ''PML''.
*Abbreviated ''CAA''.
{{Main|Progressive multifocal leukoencephalopathy}}
*Disease of the old.
*Strong association with ''[[lobar haemorrhage]]''.<ref name=pmid16982664>{{cite journal |author=Thanvi B, Robinson T |title=Sporadic cerebral amyloid angiopathy--an important cause of cerebral haemorrhage in older people |journal=Age Ageing |volume=35 |issue=6 |pages=565–71 |year=2006 |month=November |pmid=16982664 |doi=10.1093/ageing/afl108 |url=}}</ref>
**Cerebellar cortex.
**Cerebral cortex.


Etiology:
*[[Amyloid]] deposition in the basal lamina of smooth muscle (in the cerebellar cortex and cerebral cortex).


===Microscopic===
Features:
*Amorphous, acellular eosinophilic material within walls of small arteries.
**This is a high power diagnosis with congo red staining.


Notes:
=Cysts=
*Amyloidosis is seen in all individuals with [[Alzheimer's disease]]; the amount of amyloid is what differs -- in CAA it is lots and lots.
===General===
*The white matter is typically spared by CAA.<ref name=pmid19225408>{{Cite journal  | last1 = Schröder | first1 = R. | last2 = Deckert | first2 = M. | last3 = Linke | first3 = RP. | title = Novel isolated cerebral ALlambda amyloid angiopathy with widespread subcortical distribution and leukoencephalopathy due to atypical monoclonal plasma cell proliferation, and terminal systemic gammopathy. | journal = J Neuropathol Exp Neurol | volume = 68 | issue = 3 | pages = 286-99 | month = Mar | year = 2009 | doi = 10.1097/NEN.0b013e31819a87f9 | PMID = 19225408 }}
*All are "benign", but some may be fatal due to spatial constraints.
</ref>


Images:
===List of cysts===
*[http://commons.wikimedia.org/wiki/File:Cerebral_amyloid_angiopathy_-_very_high_mag.jpg CAA - congo red - very high mag. (WC)].
*[[Colloid cyst]].
*[http://commons.wikimedia.org/wiki/File:Cerebral_amyloid_angiopathy_-_low_mag.jpg CAA - congo red - low mag. (WC)].
**Columnar epithelium.
*[http://commons.wikimedia.org/wiki/File:Cerebral_amyloid_angiopathy_-2b-_amyloid_beta_-_high_mag.jpg CAA - beta-amyloid - high mag. (WC)].
*Arachnoid cyst - considered precursor of [[meningioma]].
 
**[[Psammoma bodies]].
===Stains===
**Clumps of cells.
*[[Congo red]].
**Whorled pattern.
 
*[[Dermoid cyst]].
===IHC===
**Skin + adnexal structures.
*Abeta-amyloid (AKA beta-amyloid).
**... think of ovarian dermoid.
*Epidermoid cyst.
*Choroid plexus cyst.
*Neuroenteric cyst.
**Foregut cyst with connection to dura.<ref>URL: [http://bhj.org/journal/2003_4502_april/neurentericcyst_373.htm http://bhj.org/journal/2003_4502_april/neurentericcyst_373.htm]. Accessed on: 19 December 2011.</ref>
***Gastrointestinal tract epithelium.
***Usually seen with vertebral anomalies.  
*Epithelial cyst.
*Cyst with a mural nodule tumor of the brain.
**Commonly seen in: <ref>{{Cite journal  | last1 = Raz | first1 = E. | last2 = Zagzag | first2 = D. | last3 = Saba | first3 = L. | last4 = Mannelli | first4 = L. | last5 = Di Paolo | first5 = PL. | last6 = D'Ambrosio | first6 = F. | last7 = Knopp | first7 = E. | title = Cyst with a mural nodule tumor of the brain. | journal = Cancer Imaging | volume = 12 | issue =  | pages = 237-44 | month = Aug | year = 2012 | doi = 10.1102/1470-7330.2012.0028 | PMID = 22935908 }}</ref>
**[[Hemangioblastoma]]
**[[Craniopharyngioma]]
**[[Ganglioglioma]]
*Others.


==Central pontine myelinolysis==
==Colloid cyst==
*Abbreviated ''CPM''.
*[[AKA]] ''pontine myelinolysis''.
===General===
===General===
*Classically in the pons, ergo "pontine" is in the name.
Classic presentation:<ref name=pmid15228889>{{Cite journal  | last1 = Spears | first1 = RC. | title = Colloid cyst headache. | journal = Curr Pain Headache Rep | volume = 8 | issue = 4 | pages = 297-300 | month = Aug | year = 2004 | doi =  | PMID = 15228889 }}</ref>
*Classically midline, ergo "central" is in the name.
*Headache - may be relieved by lying down.
**May occur elsewhere -- known as ''extrapontine myelinolysis''.
*Can cause [[sudden natural death]].<ref name=pmid14716130>{{Cite journal  | last1 = Kava | first1 = MP. | last2 = Tullu | first2 = MS. | last3 = Deshmukh | first3 = CT. | last4 = Shenoy | first4 = A. | title = Colloid cyst of the third ventricle: a cause of sudden death in a child. | journal = Indian J Cancer | volume = 40 | issue = 1 | pages = 31-3 | month =  | year =  | doi =  | PMID = 14716130 }}</ref>


Etiology:
===Gross===
*Rapid correction of hyponatremia.<ref name=pmid22080394>{{Cite journal  | last1 = Chang | first1 = Y. | last2 = An | first2 = DH. | last3 = Xing | first3 = Y. | last4 = Qi | first4 = X. | title = Central pontine and extrapontine myelinolysis associated with acute hepatic dysfunction. | journal = Neurol Sci | volume =  | issue =  | pages =  | month = Nov | year = 2011 | doi = 10.1007/s10072-011-0838-3 | PMID = 22080394 }}</ref>
*Fluid filled cyst - classically in the third ventricle.
*Tacrolimus post-liver transplant.<ref name=pmid21959523>{{Cite journal  | last1 = Fukazawa | first1 = K. | last2 = Nishida | first2 = S. | last3 = Aguina | first3 = L. | last4 = Pretto | first4 = E. | title = Central pontine myelinolysis (CPM) associated with tacrolimus (FK506) after liver transplantation. | journal = Ann Transplant | volume = 16 | issue = 3 | pages = 139-42 | month = Sep | year = 2011 | doi =  | PMID = 21959523 }}</ref>
*Associated with alcoholism and malnourishment.


Clinical:<ref>{{Cite journal  | last1 = Lai | first1 = CC. | last2 = Tan | first2 = CK. | last3 = Lin | first3 = SH. | last4 = Chen | first4 = HW. | title = Central pontine myelinolysis. | journal = CMAJ | volume = 183 | issue = 9 | pages = E605 | month = Jun | year = 2011 | doi = 10.1503/cmaj.090186 | PMID = 21543311 | PMC = 3114939 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3114939/?tool=pubmed }}</ref>
====Images====
*Decreased level of consciousness - most common.
<gallery>
*Quadriplegia.
Image:Human brain showning a colloid cyst in the third ventricle.jpg| Colloid cyst at autopsy. (Shaktawat ''et al.''<ref name=pmid16867192>{{Cite journal  | last1 = Shaktawat | first1 = SS. | last2 = Salman | first2 = WD. | last3 = Twaij | first3 = Z. | last4 = Al-Dawoud | first4 = A. | title = Unexpected death after headache due to a colloid cyst of the third ventricle. | journal = World J Surg Oncol | volume = 4 | issue = | pages = 47 | month = | year = 2006 | doi = 10.1186/1477-7819-4-47 | PMID = 16867192 }}</ref>)
*Poor prognosis.
</gallery>
www:
*[http://www.ajnr.org/content/21/8/1470/F3.expansion.html Colloid cyst of third ventricle (ajnr.org)].<ref name=pmid11003281/>


===Microscopic===
===Microscopic===
Features:<ref name=npw_ch6>URL: [http://neuropathology-web.org/chapter6/chapter6dCPM.html http://neuropathology-web.org/chapter6/chapter6dCPM.html]. Accessed on: 20 December 2011.</ref>
Features:<ref name=pmid11003281>{{Cite journal  | last1 = Armao | first1 = D. | last2 = Castillo | first2 = M. | last3 = Chen | first3 = H. | last4 = Kwock | first4 = L. | title = Colloid cyst of the third ventricle: imaging-pathologic correlation. | journal = AJNR Am J Neuroradiol | volume = 21 | issue = 8 | pages = 1470-7 | month = Sep | year = 2000 | doi =  | PMID = 11003281 }}</ref>
*Myelin loss.
*Simple epithelium with ciliated cells and goblet cells.
*No inflammation.
*Relative preservation of neurons.


Images:
====Images====
*[http://neuropathology-web.org/chapter6/images6/6-9l.jpg CPM (neuropathology-web.org)].<ref name=npw_ch6>URL: [http://neuropathology-web.org/chapter6/chapter6dCPM.html http://neuropathology-web.org/chapter6/chapter6dCPM.html]. Accessed on: 20 December 2011.</ref>
<gallery>
*[http://dartmed.dartmouth.edu/spring09/html/virtual_microscopy_we/ CPM (dartmouth.edu)].
Image:Colloid_Cyst_HE_40x.jpg | Colloid cyst. (WC)
 
</gallery>
==Vascular malformations==
www:
{{Main|Vascular malformations}}
*[http://www.ajnr.org/content/21/8/1470/F4.expansion.html Colloid cyst (ajnr.org)].<ref name=pmid11003281/>
Types:<ref name=pmid17076525>{{cite journal |author=Prayson RA, Kleinschmidt-DeMasters BK |title=An algorithmic approach to the brain biopsy--part II |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=11 |pages=1639–48 |year=2006 |month=November |pmid=17076525 |doi= |url=}}</ref>
#Arteriovenous malformation.
#*Most important clinically - highest risk of bleeding.
#Varix.
#*One large (dilated) vein.
#Venous angioma.
#*Many small veins.
#Caverous malformation.
#*Vessels are back-to-back (no intervening parenchyma).
 
Also see: ''[[Sturge-Weber syndrome]]''.


=Paediatric pathology=
=Paediatric pathology=
===Joubert syndrome===
==Kernicterus==
*Malformation of the cerebellar vermis.<ref name=ninds_joubert>[http://www.ninds.nih.gov/disorders/joubert/joubert.htm http://www.ninds.nih.gov/disorders/joubert/joubert.htm]</ref>
 
===Epidemiology===
*Autosomal recessive - mutation in a number of genes including NPHP1, AHI1, and CEP290.<ref name=ninds_joubert/>
 
=Weird stuff=
==Acute disseminated encephalomyelitis==
*Abbreviated ''ADEM''.
===General===
===General===
*Thought to be autoimmune; often associated with/preceded by by viral illness.<ref name=pmid17438235>{{cite journal |author=Tenembaum S, Chitnis T, Ness J, Hahn JS |title=Acute disseminated encephalomyelitis |journal=Neurology |volume=68 |issue=16 Suppl 2 |pages=S23–36 |year=2007 |month=April |pmid=17438235 |doi=10.1212/01.wnl.0000259404.51352.7f |url=}}</ref>
*Due to hyperbilirubinemia.<ref name=pmid7063283>{{Cite journal | last1 = Turkel | first1 = SB. | last2 = Miller | first2 = CA. | last3 = Guttenberg | first3 = ME. | last4 = Moynes | first4 = DR. | last5 = Godgman | first5 = JE. | title = A clinical pathologic reappraisal of kernicterus. | journal = Pediatrics | volume = 69 | issue = 3 | pages = 267-72 | month = Mar | year = 1982 | doi = | PMID = 7063283 }}</ref>
*May mimic [[multiple sclerosis]].


Treatment:
===Gross===
*Steroids.
*Yellow staining:<ref name=npw>URL: [http://neuropathology-web.org/chapter3/chapter3eBilirubinencephalopathy.html http://neuropathology-web.org/chapter3/chapter3eBilirubinencephalopathy.html]. Accessed on: 30 May 2012.</ref>
*Plasmapheresis.
**Basal ganglia.<ref name=pmid10920171>{{Cite journal  | last1 = Hansen | first1 = TW. | last2 = Hervieux | first2 = JF. | last3 = Orth | first3 = J. | last4 = Schmorl | first4 = CG. | last5 = Baumes | first5 = JB. | title = Pioneers in the scientific study of neonatal jaundice and kernicterus. | journal = Pediatrics | volume = 106 | issue = 2 | pages = E15 | month = Aug | year = 2000 | doi =  | PMID = 10920171 }}</ref>
**Hippocampus.<ref name=pmid15091133>{{Cite journal  | last1 = Paksoy | first1 = Y. | last2 = Koç | first2 = H. | last3 = Genç | first3 = BO. | title = Bilateral mesial temporal sclerosis and kernicterus. | journal = J Comput Assist Tomogr | volume = 28 | issue = 2 | pages = 269-72 | month =  | year =  | doi =  | PMID = 15091133 }}</ref>
**Subthalamic nucleus.


Diagnosis:
Note:
*Need to r/o infection (with lumbar puncture).
*May not be specific.<ref name=pmid7063283/>
*No old plaques on imaging (MRI).


An acute form exists known as ''acute hemorrhagic leukoencephalitis''<ref>URL: [http://path.upmc.edu/cases/case102/dx.html http://path.upmc.edu/cases/case102/dx.html]. Accessed on: 2 January 2012.</ref> (AKA ''acute necrotizing hemorrhagic encephalomyelitis'').
Image:
*[http://www.flickr.com/photos/neonatal-box/6275988844/ Kernicterus (flickr.com)].


===Microscopic===
===Microscopic===
Features:<ref>{{Ref PBoD8|1312}}</ref>
Features - similar to [[HIE]]:<ref name=npw/>
*Myelin loss with sparing of axons.
*+/-Red neurons.
*Inflammation:
*+/-Gliosis.
**Early: neutrophils.
**Late: mononuclear cells (lymphocytes, plasma cells).
*Lipid-laden macrophages.


DDx:
==Joubert syndrome==
*[[Multiple sclerosis]].
*Malformation of the cerebellar vermis.<ref name=ninds_joubert>[http://www.ninds.nih.gov/disorders/joubert/joubert.htm http://www.ninds.nih.gov/disorders/joubert/joubert.htm]</ref>
**Age of lesions differs.
*Acute necrotizing hemorrhagic encephalomyelitis (ANHE) - if one considers this a separate entity.


==Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy==
===Epidemiology===
*Commonly abbreviated ''CADASIL''.
*Autosomal recessive - mutation in a number of genes including NPHP1, AHI1, and CEP290.<ref name=ninds_joubert/>
===General===
*Autosomal dominant disorder - as the name implies.<ref name=pmid19174371>{{Cite journal  | last1 = Tikka | first1 = S. | last2 = Mykkänen | first2 = K. | last3 = Ruchoux | first3 = MM. | last4 = Bergholm | first4 = R. | last5 = Junna | first5 = M. | last6 = Pöyhönen | first6 = M. | last7 = Yki-Järvinen | first7 = H. | last8 = Joutel | first8 = A. | last9 = Viitanen | first9 = M. | title = Congruence between NOTCH3 mutations and GOM in 131 CADASIL patients. | journal = Brain | volume = 132 | issue = Pt 4 | pages = 933-9 | month = Apr | year = 2009 | doi = 10.1093/brain/awn364 | PMID = 19174371 }}
</ref>
*Causes strokes in 40-50 year-old.
*Cerebral microbleeds - common.
**Associated with increased risk of [[intracerebral hemorrhage]].<ref name=pmid17135568>{{Cite journal  | last1 = Choi | first1 = JC. | last2 = Kang | first2 = SY. | last3 = Kang | first3 = JH. | last4 = Park | first4 = JK. | title = Intracerebral hemorrhages in CADASIL. | journal = Neurology | volume = 67 | issue = 11 | pages = 2042-4 | month = Dec | year = 2006 | doi = 10.1212/01.wnl.0000246601.70918.06 | PMID = 17135568 }}</ref>
*Characteristic MRI findings - present in asymptomatic individuals with mutation.
*Increased risk of [[heart|myocardial infarction]].<ref name=pmid12861102>{{cite journal |author=Lesnik Oberstein SA, Jukema JW, Van Duinen SG, ''et al.'' |title=Myocardial infarction in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) |journal=Medicine (Baltimore) |volume=82 |issue=4 |pages=251–6 |year=2003 |month=July |pmid=12861102 |doi=10.1097/01.md.0000085054.63483.40 |url=}}</ref>
 
====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>
**Diagnosis: proven ''Notch 3'' mutation.
 
===Microscopic===
Features:
*+/-Subcortical infarcts.
**Patches of (non-myelinated) tissue within the white matter deep to the cortex with abundant macrophages.
*Blood vessels typically have a basophilic granularity.<ref name=pmid17076524>{{cite journal |author=Kleinschmidt-DeMasters BK, Prayson RA |title=An algorithmic approach to the brain biopsy--part I |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=11 |pages=1630–8 |year=2006 |month=November |pmid=17076524 |doi= |url=}}</ref>
 
====IHC====
*Notch 3: smooth muscle and pericytes punctate +ve.<ref name=pmid12861102/>
 
Image: [http://commons.wikimedia.org/wiki/File:CADASIL_-_very_high_mag.jpg Notch 3 staining in CADASIL (WC)].
 
Notes:
*No cortical involvement -- this is unlike ''multiple sclerosis''.
 
DDx:
*[[Amyloidosis]].
*[[Binswanger's disease]] - multi-infarct dementia affecting subcortical white matter.
**Often diagnosed as ''Alzheimer's disease'' in the past.
 
====Skin biopsy diagnosis====
*Can be diagnosed on a skin biopsy.<ref name=pmid11755616 >{{cite journal |author=Joutel A, Favrole P, Labauge P, ''et al.'' |title=Skin biopsy immunostaining with a Notch3 monoclonal antibody for CADASIL diagnosis |journal=Lancet |volume=358 |issue=9298 |pages=2049–51 |year=2001 |month=December |pmid=11755616 |doi=10.1016/S0140-6736(01)07142-2 |url=}}</ref>
 
===Electron microscopy===
*Granular osmiophilic material (GOM).
 
==Progressive multifocal leukoencephalopathy==
*Abbreviated ''PML''.
===General===
*Caused by ''JC virus'' (a type of [[polyomavirus]]<ref name=pmid21499097>{{Cite journal  | last1 = Berger | first1 = JR. | title = The basis for modeling progressive multifocal leukoencephalopathy pathogenesis. | journal = Curr Opin Neurol | volume = 24 | issue = 3 | pages = 262-7 | month = Jun | year = 2011 | doi = 10.1097/WCO.0b013e328346d2a3 | PMID = 21499097 }}</ref>) in the context of immunodeficiency; usu. in the setting of [[HIV]] infection.<ref name=pmid12709870>{{Cite journal  | last1 = Berger | first1 = JR. | title = Progressive multifocal leukoencephalopathy in acquired immunodeficiency syndrome: explaining the high incidence and disproportionate frequency of the illness relative to other immunosuppressive conditions. | journal = J Neurovirol | volume = 9 Suppl 1 | issue =  | pages = 38-41 | month =  | year = 2003 | doi = 10.1080/13550280390195261 | PMID = 12709870 }}</ref>
**Approximately 5% of HIV patients develop PML.<ref name=pmid12709870/>
**Virus destroys oligodendrocytes -> demyelination results.<ref name=pmid21823157>{{Cite journal  | last1 = Mateen | first1 = FJ. | last2 = Muralidharan | first2 = R. | last3 = Carone | first3 = M. | last4 = van de Beek | first4 = D. | last5 = Harrison | first5 = DM. | last6 = Aksamit | first6 = AJ. | last7 = Gould | first7 = MS. | last8 = Clifford | first8 = DB. | last9 = Nath | first9 = A. | title = Progressive multifocal leukoencephalopathy in transplant recipients. | journal = Ann Neurol | volume = 70 | issue = 2 | pages = 305-22 | month = Aug | year = 2011 | doi = 10.1002/ana.22408 | PMID = 21823157 }}
</ref>
*Suspected cases are biopsied - unlike other demyelinating diseases.<ref>URL: [http://path.upmc.edu/cases/case336/dx.html http://path.upmc.edu/cases/case336/dx.html]. Accessed on: 15 January 2012.</ref>
===Gross===
*Multifocal - as the name suggests.
 
===Microscopic===
Features:<ref>URL: [http://path.upmc.edu/cases/case120/dx.html http://path.upmc.edu/cases/case120/dx.html]. Accessed on: 3 January 2012.</ref>
*Perivascular inflammatory cells.
*Foamy histiocytes.
*Abnormal appearing glial cells:<ref name=uscf_pml>URL: [http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Didactic/Pml.htm http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Didactic/Pml.htm]. Accessed on: 3 January 2012.</ref>
**Reactive astrocytes.
**Oligodendrocytes with nuclear enlargement and glassy magenta chromatin - '''key feature'''.
**Atypical mitoses - known as ''[[Creutzfeldt cell]]''.
 
Images:
*[http://path.upmc.edu/cases/case120/micro.html PML (upmc.edu)].
*[http://path.upmc.edu/cases/case120/dx.html PML (upmc.edu)].
*[http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Figures/PMLHandE.jpg PML oligodendrocyte (ucsf.edu)].<ref name=uscf_pml>URL: [http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Didactic/Pml.htm http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Didactic/Pml.htm]. Accessed on: 3 January 2012.</ref>
*[http://neuro.psychiatryonline.org/data/Journals/NP/3923/RA4831F2.jpeg PML oligodendrocyte (psychiatryonline.org)].<ref>{{Cite journal  | last1 = Hurley | first1 = RA. | last2 = Ernst | first2 = T. | last3 = Khalili | first3 = K. | last4 = Del Valle | first4 = L. | last5 = Simone | first5 = IL. | last6 = Taber | first6 = KH. | title = Identification of HIV-associated progressive multifocal leukoencephalopathy: magnetic resonance imaging and spectroscopy. | journal = J Neuropsychiatry Clin Neurosci | volume = 15 | issue = 1 | pages = 1-6 | month =  | year = 2003 | doi =  | PMID = 12556565 }}</ref>
 
===IHC===
*SV40 +ve.<ref name=pmid15581180>{{Cite journal  | last1 = Muñoz-Mármol | first1 = AM. | last2 = Mola | first2 = G. | last3 = Fernández-Vasalo | first3 = A. | last4 = Vela | first4 = E. | last5 = Mate | first5 = JL. | last6 = Ariza | first6 = A. | title = JC virus early protein detection by immunohistochemistry in progressive multifocal leukoencephalopathy: a comparative study with in situ hybridization and polymerase chain reaction. | journal = J Neuropathol Exp Neurol | volume = 63 | issue = 11 | pages = 1124-30 | month = Nov | year = 2004 | doi =  | PMID = 15581180 }}</ref>


=See also=
=See also=
Michael
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