Difference between revisions of "Neurodegenerative diseases"

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'''Neurodegenerative diseases''' is a big part of [[neuropathology]].
'''Neurodegenerative diseases''' is a big part of [[neuropathology]].
 
==Overview==
They are essentially progressive and selective neuron loss.  Clinically, they are not unique.  They are defined by molecular pathology.<ref name=pmid19918325>{{cite journal |author=Dickson DW |title=Neuropathology of non-Alzheimer degenerative disorders |journal=Int J Clin Exp Pathol |volume=3 |issue=1 |pages=1–23 |year=2009 |pmid=19918325 |pmc=2776269 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2776269/?tool=pubmed}}</ref>
 
Molecular schema of neurodegenerative disorders:<ref name=pmid19918325/>
{{familytree/start}}                     
{{familytree  | | | | | | | A01 | | | | | | | | A01=Neurodegenerative<br>disorders}}
{{familytree  | |,|-|-|-|v|-|^|-|v|-|-|-|.| | |}}
{{familytree  | B01 | | B02 | | B03 | | B04 | |B01=Amyloidoses|B02=Tauopathies|B03=α-synucleinopathies|B04=TDP-43}}
{{familytree/end}}


==General DDx of dementia==
==General DDx of dementia==

Revision as of 13:50, 12 November 2010

Neurodegenerative diseases is a big part of neuropathology.

Overview

They are essentially progressive and selective neuron loss. Clinically, they are not unique. They are defined by molecular pathology.[1]

Molecular schema of neurodegenerative disorders:[1]

 
 
 
 
 
 
Neurodegenerative
disorders
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Amyloidoses
 
Tauopathies
 
α-synucleinopathies
 
TDP-43
 

General DDx of dementia

  • Alzheimer's dementia.
  • Vascular.
    • Multi-infarct dementia.
  • Parkinson's associated dementia.
  • Lewy body dementia.
  • Alcohol-related dementia.
  • Fronto-temporal dementia (Pick disease).
  • Multisystem atrophy.

Mnemonic VITAMIN D VEST:[2]

  • Vitamin deficiency (B12, folate, thiamine).
  • Infection (HIV).
  • Trauma.
  • Anoxia.
  • Metabolic (Diabetes).
  • Intracranial tumour.
  • Normal pressure hydrocephalus.
  • Degenerative (Alzheimer's, Huntington's, CJD).
  • Vascular.
  • Endocrine.
  • Space occupying lesion (chronic subdural hematoma).
  • Toxins (alcohol).

Lewy body dementia

  • Parkinsonian features.
  • Hallucinations (visual).
  • Progressive cog. decline with fluctuations.

Multiple system atrophy

  • Alpha-synuclein-rich glial cytoplasmic inclusions - finding at autopsy.[3]
    • Alpha-synuclein is implicated in a number of neurodegenerative diseases.[4]

Progressive supranuclear palsy

General

  • AKA Steele-Richardson-Olszewski syndrome.
  • Diagnosis: clinical.[5]

Microscopic

Features:

  • Neurofibrillary tangles.[5][6]

Huntington disease

General

  • Autosomal dominant inheritance.
  • Mutation: unstable CAG repeat.[7]

Gross

  • Missing caudate.[8]

Image: Huntington's disease (ouhsc.edu).

See also

References

  1. 1.0 1.1 Dickson DW (2009). "Neuropathology of non-Alzheimer degenerative disorders". Int J Clin Exp Pathol 3 (1): 1–23. PMC 2776269. PMID 19918325. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2776269/?tool=pubmed.
  2. TN06 PS19
  3. Wenning, GK.; Stefanova, N.; Jellinger, KA.; Poewe, W.; Schlossmacher, MG. (Sep 2008). "Multiple system atrophy: a primary oligodendrogliopathy.". Ann Neurol 64 (3): 239-46. doi:10.1002/ana.21465. PMID 18825660.
  4. Uversky, VN. (Oct 2008). "Alpha-synuclein misfolding and neurodegenerative diseases.". Curr Protein Pept Sci 9 (5): 507-40. PMID 18855701.
  5. 5.0 5.1 URL: http://emedicine.medscape.com/article/1151430-overview. Accessed on: 11 November 2010.
  6. Williams DR, Lees AJ (March 2009). "Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges". Lancet Neurol 8 (3): 270–9. doi:10.1016/S1474-4422(09)70042-0. PMID 19233037.
  7. Kumar P, Kalonia H, Kumar A (2010). "Huntington's disease: pathogenesis to animal models". Pharmacol Rep 62 (1): 1–14. PMID 20360611.
  8. URL: http://moon.ouhsc.edu/kfung/jty1/NeuroTest/Q07-Ans.htm. Accessed on: 29 October 2010.