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





Lateral scout                                                                


    
CT noncontrast


 
CT noncontrast                                                                                      





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



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.



Submitted by Asako Miyakoshi, MD, UW Neuroradiology

Return to UW Neuroradiology Teaching Files