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Fahr’s disease with an initial presentation of crescendo TIA
  1. Paul Smith1,
  2. Kalun Ng1 and
  3. Kailash Krishnan2
  1. 1Nottingham University Hospitals NHS Trust, Nottingham, UK
  2. 2Stroke Medicine, Nottingham University Hospitals NHS Trust, Nottingham, UK
  1. Correspondence to Dr Paul Smith; paul-smith{at}doctors.org.uk

Abstract

A 51-year-old man presented with vertigo, slurred speech and left facial droop. He had been previously diagnosed with transient ischaemic attack (TIA) and had a prior lacunar infarct. Imaging showed heavy symmetrical calcification in the globus pallidus, frontal white matter and cerebellar dentate nuclei/deep white matter. The imaging was pathognomonic for Fahr’s disease and diagnosis was confirmed when other secondary causes of hypercalcemia were excluded. Fahr’s disease is a rare, autosomal dominant, neurological condition characterised by primary brain calcification. Patients present with progressive neurological and psychiatric symptoms; commonly, Parkinsonian movement disorders, seizures, headaches, dysarthria, cognitive decline, psychosis and personality changes. There is an association with intracerebral ischaemic events. This case supports a growing body of anecdotal evidence of this association and is the first in which crescendo TIA may be the initial presentation of Fahr’s disease. Referral for genetic counselling and symptomatic relief for neurological symptoms are the main management strategies.

  • neurology
  • movement disorders (other than Parkinsons)
  • neuroimaging
  • stroke
  • radiology

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Footnotes

  • Contributors PS: lead author, submitting author, responsible for reviewing patient information, obtaining consent, writing of the report, obtaining images, review of the literature, revision of initial drafts and overall content guarantor. KN: identification of suitable case for the report, guidance of content and review. KK: review of imaging, supervision, feedback and review.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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