Difference between revisions of "Neuropathology"

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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.
** 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=====
=====Gross=====
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File:Cmv_neuronal_inclusions.jpg | Neuronal nuclear inclusions in a neonatal 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 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>
</gallery>


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**[[Ependymomblastoma]]s.
**[[Ependymomblastoma]]s.
**[[Medulloepithelioma]]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>
<gallery>
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</gallery>
</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.
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Image:Rosenthal_fibers.jpg | Rosenthal fibres - smear (WC/AFIP)
Image:Rosenthal_fibers.jpg | Rosenthal fibres - smear (WC/AFIP)
</gallery>
</gallery>
*Eosinophilic granular bodies = related to Rosenthal fibres; round cytoplasmic hyaline droplets in astrocytes.<ref>{{Ref MBNP|11}}</ref>
*Eosinophilic granular bodies = related to Rosenthal fibres; round cytoplasmic hyaline droplets in [[astrocyte]]s.<ref>{{Ref MBNP|11}}</ref>
**Image: [Image:Pilocytic_Micro.jpg EGBs (WC/AFIP)].
<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:  
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=Brain tumours=
=Brain tumours=
{{main|Neuropathology tumours}}
{{main|Neuropathology tumours}}
Tumours are a big part of neuropathology.  The most common brain tumour (in adults) 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/>


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*[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).
===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 638: Line 798:


==Epilepsy==
==Epilepsy==
{{Main|Epilepsy}}
{{Main|Epilepsy}}  


==Cerebrovascular accident==
==Epilepsy==
*Abbreviated ''CVA''.  
{{Main|Epilepsy}}
*[[AKA]] ''stroke''.
===Focal cortical dysplasia (FCD)===
{{Main|Cerebrovascular accident}}
*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):


==Hypoxic-ischemic encephalopathy==
*Abbreviated ''HIE''.
{{Main|Hypoxic-ischemic encephalopathy}}


==Multiple sclerosis==
*Type I FCD (focal)
*Abbreviated ''MS''.
**Ia: Abnormal radial cortical lamination.
{{Main|Multiple sclerosis}}
**Ib: Abnormal tangential cortical lamination.
**Ic: Abnormal radial and tangential cortical lamination.


==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:
*Type II FCD (focal)
*[[Amyloid]] deposition in the basal lamina of smooth muscle (in the cerebellar cortex and cerebral cortex).
**IIa: Presence of dysmorphic neurons.
**IIb: Presence of dysmorphic neurons and balloon cells.


===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===
*Type III FCD (associated with other lesion)
Features:
**IIIa: FCD associated with [[Epilepsy#Hippocampal_sclerosis|hippocampal sclerosis]].
*Amorphous, acellular eosinophilic material within walls of small arteries.
**IIIb: FCD adjacent to a brain tumor.
**This is a high power diagnosis with congo red staining.
**IIIc: FCD adjacent to vascular malformation.
**IIIc: FCD associated with previous injury (trauma, inflammation...).


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>
<gallery>
Image:Cerebral_amyloid_angiopathy_-_very_high_mag.jpg |CAA - congo red - very high mag. (WC)
File:FCDIIa dysmorphic neurons HE.jpg|Dysmorphic neurons in FCD (HE)
Image:Cerebral_amyloid_angiopathy_-_low_mag.jpg |CAA - congo red - low mag. (WC)
File:FCDIIa neuronal heterotopia neun.jpg|Heterotopic neurons (NeuN)
Image:Cerebral_amyloid_angiopathy_-2b-_amyloid_beta_-_high_mag.jpg |CAA - beta-amyloid - high mag. (WC)
</gallery>
</gallery>
===Stains===
*[[Congo red]].


===IHC===
===Hamartia===
*Abeta-amyloid (AKA beta-amyloid).
* Small collection of ectopic glioneuronal cells.
**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>
* Mostly amygdala, less common in hippocampus or temporal lobe.
* Can coexist with focal cortical dysplasia.
 
==Demyelination==
 
===Multiple sclerosis===
*Abbreviated ''MS''.
{{Main|Multiple sclerosis}}
 
===Osmotic demyelination syndrome===
{{Main|Osmotic demyelination syndrome}}
*Previously known as ''central pontine myelinolysis'' (abbreviated ''CPM'').
 
===Acute disseminated encephalomyelitis===
*Abbreviated ''ADEM''.
{{Main|Acute disseminated encephalomyelitis}}
 
===Neuromyelitis optica===
*Abbreviated ''NMO''.


==Central pontine myelinolysis==
General:
*Abbreviated ''CPM''.
*Rare autoimmune disease - once considered a variant of [[multiple sclerosis]].
*[[AKA]] ''pontine myelinolysis''.
**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>
===General===
*Classically in the pons, ergo "pontine" is in the name.
*Classically midline, ergo "central" is in the name.
**May occur elsewhere -- known as ''extrapontine myelinolysis''.


Etiology:
Diagnosis:
*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>
*NMO-IgG.
*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>
Clinical - preferentially:
*Decreased level of consciousness - most common.
*Eye (optic neuritis).
*Quadriplegia.
*Spinal cord (myelitis).
*Poor prognosis.


===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>
*Inflammation - lymphocytes, macrophages.
*Myelin loss.
*Reactive astrocytes.
*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>
*[http://path.upmc.edu/cases/case637.html Neuromyelitis optica - several images (upmc.edu)].
*[http://dartmed.dartmouth.edu/spring09/html/virtual_microscopy_we/ CPM (dartmouth.edu)].
 
IHC:
*Mixed lymphocyte population with CD3 > CD20.
*Aquaporin-4 loss.
 
===Progressive multifocal leukoencephalopathy===
*Abbreviated ''PML''.
{{Main|Progressive multifocal leukoencephalopathy}}


==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>
#Arteriovenous malformation.
#*Most important clinically - highest risk of bleeding.
#Varix.
#*One large (dilated) vein.
#Venous angioma.
#*Many small veins.
#Cavernous malformation.
#*Vessels are back-to-back (no intervening parenchyma).


Also see: ''[[Sturge-Weber syndrome]]''.


=Cysts=
=Cysts=
Line 806: Line 961:
===Epidemiology===
===Epidemiology===
*Autosomal recessive - mutation in a number of genes including NPHP1, AHI1, and CEP290.<ref name=ninds_joubert/>
*Autosomal recessive - mutation in a number of genes including NPHP1, AHI1, and CEP290.<ref name=ninds_joubert/>
=Weird stuff=
==Acute disseminated encephalomyelitis==
*Abbreviated ''ADEM''.
{{Main|Acute disseminated encephalomyelitis}}
==Neuromyelitis optica==
*Abbreviated ''NMO''.
===General===
*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>
Diagnosis:
*NMO-IgG.
Clinical - preferentially:
*Eye (optic neuritis).
*Spinal cord (myelitis).
===Microscopic===
Features:
*Inflammation - lymphocytes, macrophages.
*Reactive astrocytes.
Images:
*[http://path.upmc.edu/cases/case637.html Neuromyelitis optica - several images (upmc.edu)].
===IHC===
*Mixed lymphocyte population with CD3 > CD20.
*Aquaporin-4 loss.
==Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy==
*Commonly abbreviated ''CADASIL''.
{{Main|Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy}}
==Progressive multifocal leukoencephalopathy==
*Abbreviated ''PML''.
{{Main|Progressive multifocal leukoencephalopathy}}


=See also=
=See also=
Jensflorian
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