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The current status of “Fahr’s disease” nosology
  1. J R M de Oliveira1,
  2. M F Oliveira2,
  3. R R Lemos3,
  4. M L Oliveira4
  1. 1
    Professor, Department of Neuropsychiatry and Keizo Asami Laboratory (LIKA)-Federal University of Pernambuco, Recife, Brazil
  2. 2
    Research Assistant, Department of Neuropsychiatry and Federal University of Pernambuco, Recife-PE, Brazil
  3. 3
    PhD student, Keizo Asami Laboratory (LIKA)-Federal University of Pernambuco, Recife, Brazil
  4. 4
    PhD student, Department of Neuropsychiatry and Federal University of Pernambuco, Recife-PE, Brazil
  1. Correspondence to J R M de Oliveira, Department of Neuropsychiatry and Federal University of Pernambuco, Recife-PE, Brazil; joao.ricardo{at}ufpe.br

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Shenoy et al described an interesting case with idiopathic brain calcification.1 But in the title and throughout the text, they used the expression “Fahr’s disease”. This term is a misnomer. The nosology of the heterogeneous phenotype of patients with brain calcification is unclear and needs standard diagnostic criteria. A new classification has been proposed recently and suggests this eponym should be abolished, particularly because brain calcification is often found by neuroimaging in a wide variety of medical conditions, usually with a variable combination of motor and cognitive symptoms, and indeed in normal people—some with extensive deposits (there are more deposits in symptomatic compared with asymptomatic subjects).2 Furthermore, the German pathologist Karl Theodor Fahr was not the first to described the “disease” in 1930, and nor the one who contributed most to understanding it. For example, authors such as Delacour and Bamberger had published similar pathological findings in the 19th Century.2 Furthermore, Geyelin and Penfield, in 1929, described familial mineralisation of brain structures in a father and four children. They found that mineralisation affected mainly vessels of the deep cortical layers and adjacent white matter, attributing the calcification to endarteritis with secondary calcification of the necrotic lesion.3

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  • Competing interests None.

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