69 year-old with progressive dysarthria, extrapyramidal signs, and ataxia

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Findings:

Calcification in the putamen, caudate nucleus, dentate nucleus, thalamus, and cerebral white matter

DDX:

Normal symmetric basal ganglia calcifications in the elderly.
Pathologic basal ganglia calcifications from endocrine causes or Fahr’s syndrome
Post-inflammatory causes, such as TB, toxoplasmosis, cystercercosis, congenital HIV

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Diagnosis:

Fahr’s syndrome

Discussion:

Fahr’s Syndrome (Disease) or familial idiopathic basal ganglia calcification is characterized by bilateral basal ganglia calcification particularly the globus pallidus but also the caudate, dentate, and cerebral white matter. The calcium deposits occur in the extracellular and extravascular space often surrounding the capillaries. It is not clear whether the calcification in Fahr Disease is a metastatic deposition, secondary to local disruption of blood brain barrier, or is due to disorder of neuronal calcium metabolism. Typically the age at onset of clinical symptoms is 30 to 60 years. There is neither a cure for Fahr Disease, nor a standard course of treatment. The prognosis is variable and hard to predict.

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Submitted by Asako Miyakoshi, MD, UW Neuroradiology