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Jensflorian (talk | contribs) (→Degenerative changes: Axonal degeneration) |
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===General=== | ===General=== | ||
Etiology:<ref>{{Cite journal | last1 = Prieto-González | first1 = S. | last2 = Grau | first2 = JM. | title = Diagnosis and classification of granulomatous myositis. | journal = Autoimmun Rev | volume = 13 | issue = 4-5 | pages = 372-4 | month = | year = | doi = 10.1016/j.autrev.2014.01.017 | PMID = 24424169 }}</ref> | Etiology:<ref>{{Cite journal | last1 = Prieto-González | first1 = S. | last2 = Grau | first2 = JM. | title = Diagnosis and classification of granulomatous myositis. | journal = Autoimmun Rev | volume = 13 | issue = 4-5 | pages = 372-4 | month = | year = | doi = 10.1016/j.autrev.2014.01.017 | PMID = 24424169 }}</ref> | ||
* [[Sarcoidosis]] | * [[Sarcoidosis]]. | ||
* Idiopathic | * Idiopathic. | ||
* Infectious ([[Tuberculosis]], Syphillis, Brucellosis | * Infectious ([[Tuberculosis]], Syphillis, Brucellosis. | ||
* Foreign-body reaction | * Foreign-body reaction. | ||
* [[Thymoma]] - myasthenia gravis | * [[Thymoma]] - myasthenia gravis. | ||
* [[Lymphoma]] - paraneoplastic | * [[Lymphoma]] - paraneoplastic. | ||
* [[Primary biliary | * [[Primary biliary cholangitis]]. | ||
<gallery> | <gallery> | ||
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*Axon = green. | *Axon = green. | ||
*Myelin = red. | *Myelin = red. | ||
Toluidine blue staion: | |||
*Plastic embedded semithin sections (1µm). | |||
===Artifacts=== | |||
*Myelin splits: stretching. | |||
*Neurokeratin: Formalin fixation (longitudinal: "herringbone", cross section: "wagon-wheels"). | |||
*Dark staining myelin: crushing. | |||
*Pale expanding myelin sheets: delayed fixation. | |||
*Uneven myelin staining: osmication problems. | |||
*Shrunken crescentic fascicles: Hyperosmolarity. | |||
===Reactive changes=== | ===Reactive changes=== | ||
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*Axonal swelling. | *Axonal swelling. | ||
*Intra-axonal filamentous aggregates. | *Intra-axonal filamentous aggregates. | ||
*Mitochondrial abnormalities. | |||
*Aggregation of organelles and dense bodies. | |||
====Wallerian degeneration==== | ====Wallerian degeneration==== | ||
*Watery axon and granular disintegration (distal). | |||
*Macrophage accumulation (3-4d after transsection). | |||
*Many lysosomes (CD68+ve). | |||
*Endoneurial proliferation. | |||
*Digestion chambers - '''key feature'''. | *Digestion chambers - '''key feature'''. | ||
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File:Onion bulb formation HMSN.jpg | Onion bulbs in HSMN type I. | File:Onion bulb formation HMSN.jpg | Onion bulbs in HSMN type I. | ||
</gallery> | </gallery> | ||
===Regeneration=== | |||
*Axon sprouts (regenerating clusters): Three or more closely apposed myelinated axons. | |||
*Thin myelin sheaths. | |||
===Inflammation=== | ===Inflammation=== | ||
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*[[Leprosy]] (Leprous neuropathy). | *[[Leprosy]] (Leprous neuropathy). | ||
*[[Sarcoidosis]]. | *[[Sarcoidosis]]. | ||
*CMV neuritis in immuncompromised patients. | *CMV neuritis in immuncompromised patients. | ||
*[[Vasculitis]]. | *[[Vasculitis]]. | ||
*Paraprotein-associated neuropathy. | |||
*Neuropathy with macrophage-induced demyelination (CIDP, GBS). | |||
====Guillain–Barré syndrome==== | |||
*Acute inflammatory demyelinating polyneuropathy (AIDP) | |||
*Preceding infection (RSV, EBV, CMV, HIV, Mycoplasma). | |||
*Monophasic course of motor / sensory deficits. | |||
*Hours to 4 weeks. | |||
*Elevated CSF protein but normal cell count. | |||
*Mononuclear ednoneurial perivascular inflammatory infiltrate (mostly CD4+ve). | |||
*Destructive myelin stripping by macrophages. | |||
*Reduced fiber density. | |||
*Uncompacted myelin / Widely spaced myelin. | |||
====Chronic inflammatory demyelinating polyneuropathy (CIDP)==== | |||
*Progredient course longer than 8 weeks.<ref>URL: [http://path.upmc.edu/cases/case426.html http://path.upmc.edu/cases/case426.html]. Accessed on: 14 | |||
November 2010.</ref> | |||
*Progressive or relapsing and remitting course. | |||
*Multifocal affections of proximal nerves (motor and sensory symptoms). | |||
*Responsive to steroids. | |||
*Enlargement of affected nerve. | |||
*Variation of fiber density between fascicles / reduced axon numbers. | |||
*CD4+ve/CD8+ve inflammatory infiltrates(approx. 65% cases). | |||
*Demyelination (thinly myelinated axons, macrophages). | |||
*Onion-bulb formations (15-40%, chronic recurrent demyelination and remyelination). | |||
DDx: Familial hypertrophic neuropathy. | |||
====Neurosarcoidosis==== | |||
*Neurological symptoms in 5% of sarcoidosis cases. | |||
*Granulomas may be endoneurial or epineurial. | |||
*Compact mass of epitheloid cells. | |||
*Perilesional fibrosis and lymphocytic infiltrates. | |||
*Axonal loss and regenerating fibers. | |||
*Segmental demyelination and remyelination. | |||
====Vasculitic neuropathy==== | |||
*Endoneurial and epineurial mircrovessels, arterioles and venules. | |||
*Ischemia of nerve: thrombosis and fibrinoid necrosis. | |||
*Signs of previous vasculitis: Vessel narrowing, fragmentation of elastica, fibrous obliteration and recanalization. | |||
*Often nerve involvement in systemic vasculitis: | |||
**Medium-sized epineurial vessels: mostly classic polyarteritis nodosa. | |||
**Small and medium-sized vessels and eosinophilia: Churg-Strauss angitis. | |||
**Small vessels and necrotizing: ANCA-associated microscopic polyangitis. | |||
<gallery> | <gallery> | ||
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===Other Diseases=== | ===Other Diseases=== | ||
*Amyloid neuropathy: Amorphic endoneurial deposits. | |||
**TTR amyloidosis is of specific interest, because treatment options exist.<ref>{{Cite journal | last1 = Adams | first1 = D. | last2 = Koike | first2 = H. | last3 = Slama | first3 = M. | last4 = Coelho | first4 = T. | title = Hereditary transthyretin amyloidosis: a model of medical progress for a fatal disease. | journal = Nat Rev Neurol | volume = 15 | issue = 7 | pages = 387-404 | month = Jul | year = 2019 | doi = 10.1038/s41582-019-0210-4 | PMID = 31209302 }}</ref> | |||
**Example of amyloid deposits [https://www.nature.com/articles/s41582-019-0210-4/figures/3 here] | |||
*Neuropathy associated with paraproteinemia: Alterations in myelin periodicity, nerve fiber loss. | |||
**[[MGUS]] - Monoclonal gammopathy of unknown significance. | |||
** Multiple myeloma. | |||
**[[POEMS]] syndrome. | |||
**[[LCDD]] - light chain deposition diesease. | |||
*Toxic polyneuropathy (drug toxicity).<ref>URL: [http://path.upmc.edu/cases/case173.html http://path.upmc.edu/cases/case173.html]. Accessed on: 8 January 2012.</ref> | *Toxic polyneuropathy (drug toxicity).<ref>URL: [http://path.upmc.edu/cases/case173.html http://path.upmc.edu/cases/case173.html]. Accessed on: 8 January 2012.</ref> | ||
*Polyglucosan body disease. | |||
===Neoplasms=== | ===Neoplasms=== |
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