Summary: A 69-year-old male presents with progressive parkinsonian movement abnormalities and altered mental status. Serum calcium, phosphorus, and
parathyroid hormone levels are normal. Computed tomography (CT) and magnetic resonance (MR) of the brain were obtained.
Fahr disease (familial cerebral
ferrocalcinosis, bilateral striadopallidodentate calcinosis)
Noncontrast CT demonstrates diffuse calcification of basal ganglia, subcortical white matter,
and dentate nuclei of the cerebellum. MR images demonstrate subtle FLAIR
hyperintensity at the margins of calcification in the cerebellum and to a
lesser extent the basal ganglia.
Fahr disease is a rare neurodegenerative disorder characterized by progressive psychosis, cognitive
impairment, dementia, movement disorders, and parkinsonian symptoms. The
condition is thought to be heritable with variable expressivity and typically
presents ages 40 to 60. Prognosis is generally poor and there is no known
CT classically demonstrates diffuse mineralization of the globus pallidus, putamen, caudate, thalamus,
cerebellum, corona radiata, and subcortical white matter. Calcification begins
several decades prior to neurological deterioration. MR occasionally reveals a
similar distribution of T2/FLAIR hyperintensities, which have been postulated
to represent a progressive metabolic or inflammatory process preceding calcification.2
The differential for basal ganglia calcification is broad, but the widespread calcification, including
dentate nuclei calcification, narrows the differential to Fahr disease and
disorders of calcium metabolism, specifically hypoparathyroidism and pseudohypoparathyroidism.3
However, with the appropriate clinical history and normal serologic studies, this
pattern is pathognomonic for Fahr disease.
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- Avrahami E, Cohn DF, Feibel M, et al. MRI demonstration and CT correlation of the brain in
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- Hedge AN, Mohan S, Lath N, Lim CCT. Differential diagnosis for bilateral abnormalities of the basal ganglia
and thalamus. Radiographics. 2011;31:5-30.