Fahr Syndrome

Case contribution: Dr Radhiana Hassan


  • A 30-years old with underlying psoriasis and pseudohypoparathyroidism
  • Presented with hallucination and behavioural change
  • No fitting episode
  • No obvious neurological deficit

CT scan findings:

  • CT brain non contrast in axial and coronal plane, soft tissue window
  • Extensive dense coarse calcifications at bifrontoparietal deep white matter, basal ganglia, and cerebellum
  • Bilateral and almost symmetrical involvement.
  • No acute intracranial bleed. No focal hypodensity to suggest infarct.
  • Grey and white matter differentiation still preserved.
  • No midline shift. No hydrocephalus.

Diagnosis: Fahr syndrome


  • Fahr disease = idiopathic striopallidodentate calcinosis = cerebrovascular ferrocalcinosis
  • Fahr syndrome = same appearance as Fahr Disease but secondary to underlying disorder (e.g endocrine disease)
  • The condition that has been closely described with this appearance is primary hypopara-thyroidism.
  • Other causes include lupus, tuberous sclerosis, Alzheimer’s disease, myotonic muscular dystrophy and mitochondrial encephalopathies
  • Causing progressive dystonia, parkinsonism, neuropsychiatric manifestations
  • Bimodal pattern of clinical onset: early adulthood (psychosis) and 6th decade (extrapyramidal syndrome, subcortical dementia)
  • Imaging shows extensive calcifications involving the basal ganglia (globus pallidus), putamen, caudate, thalami and periventricular deep white matter
  • Although Fahr’s syndrome and Fahr’s disease are rare entities they should be suspected in patients with neuropsychiatric disturbances and seizure disorder.
  • Routine biochemical investigations should always be performed to rule out metabolic causes.
  • Conversely, all patients with incidentally detected striopallidodentate calcinosis should be subjected to thorough neuropsychiatric examination and if required, biochemical tests.
  • Knowledge of the associated conditions will not only help to rectify the treatable cause but will also prevent unnecessary treatment in others.


Author: radhianahassan