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Findings

Dense symmetric calcifications in the basal ganglia

Calcifications also seen within the thalamus and dentate nucleus, and to a lesser extent in the cerebral white matter

No post gadolinium enhancement

Differential diagnosis

Normal

Endocrinologic disorders

Hyperparathyroidism, hypoparathyroidism, post thyroidectomy, pseudohyperparathyroidism

Neuropsychiatric disorders

Fahr disease

Fahr disease

Rare degenerative neurological disorder characterized by extensive bilateral basal ganglia calcifications that can lead to progressive dystonia, parkinsonism, and neuropsychiatric manifestations.

Imaging findings

Globus pallidus is the most common location

Lateral pallidum more affected than medial

Additional areas of involvement

Putamen, caudate, thalami

Cerebellum (dentate nuclei)

Decreased perfusion to calcified lesions

Functional abnormalities may precede morphological changes

Pathology

Calcium-phosphorus metabolism usually normal

Calcification along capillary vessels, medial walls/adventitia of larger arteries, veins

Clinical manifestation

Neuropsychiatric disturbance

Cognitive impairment (subcortical dementia)

Extrapyramidal movement disorders

Schizophrenia like psychosis predominates with onset in early adulthood.

Onset in 6th decade with extrapyramidal syndrome and subcortical dementia.

Natural history

Progressive mental deterioration and loss of motor accomplishments

Degenerative rather than developmental disorder

Calcium deposition begins in third decade with neurological deterioration 2 decades later

Symmetrical spastic paralysis and sometimes athetosis appear, progressing to a decerebrate state

Treatment

No specific treatment to limit progression of calcification

Theoretical improvement using chelator