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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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</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 | *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: | ||
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=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== | ||
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==Epilepsy== | ==Epilepsy== | ||
{{Main|Epilepsy}} | {{Main|Epilepsy}} | ||
== | ==Epilepsy== | ||
* | {{Main|Epilepsy}} | ||
* | ===Focal cortical dysplasia (FCD)=== | ||
{{ | *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): | |||
*Type I FCD (focal) | |||
* | **Ia: Abnormal radial cortical lamination. | ||
**Ib: Abnormal tangential cortical lamination. | |||
**Ic: Abnormal radial and tangential cortical lamination. | |||
*Type II FCD (focal) | |||
* | **IIa: Presence of dysmorphic neurons. | ||
**IIb: Presence of dysmorphic neurons and balloon cells. | |||
*Type III FCD (associated with other lesion) | |||
**IIIa: FCD associated with [[Epilepsy#Hippocampal_sclerosis|hippocampal sclerosis]]. | |||
* | **IIIb: FCD adjacent to a brain tumor. | ||
** | **IIIc: FCD adjacent to vascular malformation. | ||
**IIIc: FCD associated with previous injury (trauma, inflammation...). | |||
<gallery> | <gallery> | ||
File:FCDIIa dysmorphic neurons HE.jpg|Dysmorphic neurons in FCD (HE) | |||
File:FCDIIa neuronal heterotopia neun.jpg|Heterotopic neurons (NeuN) | |||
</gallery> | </gallery> | ||
=== | ===Hamartia=== | ||
* | * 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''. | |||
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: | Clinical - preferentially: | ||
*Eye (optic neuritis). | |||
* | *Spinal cord (myelitis). | ||
* | |||
Microscopic: | |||
*Inflammation - lymphocytes, macrophages. | |||
* | *Reactive astrocytes. | ||
* | |||
Images: | Images: | ||
*[http:// | *[http://path.upmc.edu/cases/case637.html Neuromyelitis optica - several images (upmc.edu)]. | ||
* | |||
IHC: | |||
*Mixed lymphocyte population with CD3 > CD20. | |||
*Aquaporin-4 loss. | |||
===Progressive multifocal leukoencephalopathy=== | |||
*Abbreviated ''PML''. | |||
{{Main|Progressive multifocal leukoencephalopathy}} | |||
=Cysts= | =Cysts= | ||
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===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/> | ||
=See also= | =See also= |