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Fahr´s disease

GC: n

CT: Although the term Fahr's disease is often used to designate either familial or sporadic basal ganglia calcification, it is unknown whether the non-familial cases represent the same disease. The term Fahr's disease is ambiguous and should be avoided.
S: NCBI - https://www.ncbi.nlm.nih.gov/books/NBK1421/(external link) (last access: 11 June 2017)

N: 1. - Fahr (pn): Karl Theodor Fahr (1877 – 1945) was a German pathologist.
- disease (n): Early 14c., from Old French desaise (‘discomfort, inconvenience’), des- (‘dis = without, away’) + -aise (ease = physical comfort, undisturbed state of the body; tranquility, peace of mind’).
2. Basal ganglia calcification is also known as Fahr’s disease or Fahr’s syndrome. It is a rare inherited or sporadic neurological disorder with a prevalence of <1/1,000,000. It was first described by German neurologist Karl Theodor Fahr in 1930. It is characterized by abnormal deposition of calcium in areas of the brain that control movements including basal ganglia, thalamus, dentate nucleus, cerebral cortex, cerebellum, subcortical white matter, and hippocampus. Most cases present with extra pyramidal symptoms initially. Additionally, they may present with cerebellar dysfunction, speech difficulty, dementia and neuropsychiatric symptoms.
3. Symptoms:
  • Motor function deterioration.
  • Dementia.
  • Mental retardation.
  • Spastic paralysis.
  • Dysarthria (poorly articulated speech).
  • Spasticity (stiffness of the limbs).
  • Ocular (eye) problems.
  • Athetosis (involuntary, writhing movements).
4. Cultural Interrelation: We could mention episode 8 Perils of Paranoia from the TV series (2004-2012) House M. D. (season 8). Dr. Adams thought the paranoia might indicate Fahr's syndrome.

S: 1. OED – http://bit.ly/2tisbCc(external link) (last access: 11 July 2017); WNMDT – http://bit.ly/2tisIUI(external link) (last access: 11 July 2017). 2. NCBI – http://bit.ly/2Fe0mRd(external link) (last access: 11 July 2017). 3. MN - http://bit.ly/2oJR8lv(external link) (last access: 11 July 2017). 4. https://www.springfieldspringfield.co.uk/view_episode_scripts.php?tv-show=house-md&episode=s08e08(external link) (last access: 5 March 2018).

SYN: Fahr’s disease, Fahr’s syndrome, idiopathic basal ganglia calcification, striopallidodentate calcification and calcinosis nucleorum. (context)

S: NCBI – https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3853434/(external link) (last access: 11 July 2017).

CR: Parkinson's disease


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