Neurodegenerative diseases

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Neurodegenerative diseases is a big part of neuropathology.

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:[1]

  • 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.[2]
    • Alpha-synuclein is implicated in a number of neurodegenerative diseases.[3]

Progressive supranuclear palsy

General

  • AKA Steele-Richardson-Olszewski syndrome.
  • Diagnosis: clinical.[4]

Microscopic

Features:

  • Neurofibrillary tangles.[4][5]

Huntington disease

General

  • Autosomal dominant inheritance.
  • Mutation: unstable CAG repeat.[6]

Gross

  • Missing caudate.[7]

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

See also

References

  1. TN06 PS19
  2. 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.
  3. Uversky, VN. (Oct 2008). "Alpha-synuclein misfolding and neurodegenerative diseases.". Curr Protein Pept Sci 9 (5): 507-40. PMID 18855701.
  4. 4.0 4.1 URL: http://emedicine.medscape.com/article/1151430-overview. Accessed on: 11 November 2010.
  5. 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.
  6. Kumar P, Kalonia H, Kumar A (2010). "Huntington's disease: pathogenesis to animal models". Pharmacol Rep 62 (1): 1–14. PMID 20360611.
  7. URL: http://moon.ouhsc.edu/kfung/jty1/NeuroTest/Q07-Ans.htm. Accessed on: 29 October 2010.