Click on Images for Enlarged View
Findings: Image #1 is an axial CT image which demonstrates marked calcification of the basal ganglia as well as calcification within the subcortical white matter and bilateral cerebellum.
Image #2 is an axial T2 weighted image, which demonstrates markedly decreased signal within the bilateral basal ganglia.
Image # 3 is an axial proton density MR image which demonstrates moderately decreased signal within the bilateral basal ganglia.
Diagnosis: Fahr disease.
Discussion: Fahr disease is a rare neurologic condition, which is actually not a single entity, but represents a diverse group of disorders. The condition is characterized by abnormal calcification within certain regions of the brain, most predominantly within the basal ganglia. Symptoms develop when the deposits accumulate, including progressive deterioration of mental function, loss of previous motor development, spastic paralysis, and athetosis. In addition, optic atrophy may occur. The condition is generally considered inherited, idiopathic cases have been identified.
References:
Submitted by: