Difference between revisions of "Neuromuscular pathology"

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re-arr., sub-divide
(re-arr., sub-divide)
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Muscle pathology is dealt together with neurologic disease as, at the presentation, they are not infrequently impossible to definitely distinguish.
Muscle pathology is dealt together with neurologic disease as, at the presentation, they are not infrequently impossible to definitely distinguish.


==Work-up==
=Work-up=
===General===
===General===
#Clinical history, including family history.
#Clinical history, including family history.
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#Myopathy - something is wrong with the muscle fibres.
#Myopathy - something is wrong with the muscle fibres.


==Stains for muscle biopsies==
=Stains for muscle biopsies=
===Standard===
===Standard===
{| class="wikitable"
{| class="wikitable"
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***Same protein that that in implicated in [[ALS]] and [[frontotemporal dementia]].
***Same protein that that in implicated in [[ALS]] and [[frontotemporal dementia]].


==Inflammatory myopathy==
=Inflammatory myopathy=
DDx:
DDx:
#Polymyositis.
#Polymyositis.
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*Periodic paralysis.
*Periodic paralysis.


=Specific entities=
==Amyotrophic lateral sclerosis==
==Amyotrophic lateral sclerosis==
===General===
===General===
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*A type of congenital myopathy.
*A type of congenital myopathy.
*Paediatric thingy.
*Paediatric thingy.
==Drug-induced rhabdomyolysis==
*AKA ''drug-induced acute necrotizing myopathy''.
===General===
Clinical:<ref name=pmid15021204>{{Cite journal  | last1 = Coco | first1 = TJ. | last2 = Klasner | first2 = AE. | title = Drug-induced rhabdomyolysis. | journal = Curr Opin Pediatr | volume = 16 | issue = 2 | pages = 206-10 | month = Apr | year = 2004 | doi =  | PMID = 15021204 }}</ref>
*Myalgias.
*Myoglobinuria.
*Increased elevated serum creatine kinase (CK).
Causes:
*Ecstasy (MDMA).
*Statins.
===Microscopic===
Features:
*Muscle [[necrosis]].
**Fibre collapse = increased staining on [[H&E stain|H&E]], [[HPS stain|HPS]].
**Karyolysis - loss of nuclei.
**Macrophage (phagocytosis) clean-up = pale moth-eaten appearance (seen well on [[PAS stain|PAS]]).
*No inflammation.
*No perifascicular atrophy.
Images:
*[http://path.upmc.edu/cases/case184/micro.html Drug-induced rhabdomyolysis - several images (upmc.edu)].
===Stains===
*PAS +ve fibres (macrophages).
===IHC===
*CD45 -ve (no lymphocytes).
===EM===
*Negative for [[tubuloreticular inclusions]].


==Limb-girdle muscular dystrophy==
==Limb-girdle muscular dystrophy==
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*Inflammatory myopathies.
*Inflammatory myopathies.


=Groups of disorders=
==Mitochondrial disorders==
==Mitochondrial disorders==
===General===
===General===
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*[http://commons.wikimedia.org/wiki/File:Denervation_atrophy_-_atp94_-_high_mag.jpg Type 2 fibre atrophy - ATPase pH 9.4 - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Denervation_atrophy_-_atp94_-_high_mag.jpg Type 2 fibre atrophy - ATPase pH 9.4 - high mag. (WC)].


==Drug-induced rhabdomyolysis==
=Nerve stuff=
*AKA ''drug-induced acute necrotizing myopathy''.
===General===
Clinical:<ref name=pmid15021204>{{Cite journal  | last1 = Coco | first1 = TJ. | last2 = Klasner | first2 = AE. | title = Drug-induced rhabdomyolysis. | journal = Curr Opin Pediatr | volume = 16 | issue = 2 | pages = 206-10 | month = Apr | year = 2004 | doi =  | PMID = 15021204 }}</ref>
*Myalgias.
*Myoglobinuria.
*Increased elevated serum creatine kinase (CK).
 
Causes:
*Ecstasy (MDMA).
*Statins.
 
===Microscopic===
Features:
*Muscle [[necrosis]].
**Fibre collapse = increased staining on [[H&E stain|H&E]], [[HPS stain|HPS]].
**Karyolysis - loss of nuclei.
**Macrophage (phagocytosis) clean-up = pale moth-eaten appearance (seen well on [[PAS stain|PAS]]).
*No inflammation.
*No perifascicular atrophy.
 
Images:
*[http://path.upmc.edu/cases/case184/micro.html Drug-induced rhabdomyolysis - several images (upmc.edu)].
 
===Stains===
*PAS +ve fibres (macrophages).
 
===IHC===
*CD45 -ve (no lymphocytes).
 
===EM===
*Negative for [[tubuloreticular inclusions]].
 
==Nerve stuff==
===General===
===General===
*Most common biopsy: sural nerve.
*Most common biopsy: sural nerve.
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*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>


==See also==
=See also=
*[[Neuropathology]].
*[[Neuropathology]].


==References==
=References=
{{reflist|2}}
{{reflist|2}}


==External links==
=External links=
*[http://moon.ouhsc.edu/kfung/jty1/NeuroHelp/ZNEWWU10.htm How to work up a muscle biopsy (ouhsc.edu)].
*[http://moon.ouhsc.edu/kfung/jty1/NeuroHelp/ZNEWWU10.htm How to work up a muscle biopsy (ouhsc.edu)].
*[http://neuromuscular.wustl.edu/lab/mbiopsy.htm Muscle biopsies (wustl.edu)].
*[http://neuromuscular.wustl.edu/lab/mbiopsy.htm Muscle biopsies (wustl.edu)].


[[Category:Neuropathology]]
[[Category:Neuropathology]]
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