Fahr's Disease

Case:
# 8170534692
Section:
Author(s):

Dr. Ramon E.  Figueroa.

Dr. Joaquin Nunez G. 

Augusta University. Augusta, GA 30912 USA

Patient:
Female, 95 year(s)

CLINICAL HISTORY

95 years old Female "fall from toilet, unclear medications or medical history, actually with confusion, right flank/rib pain, abdominal distention.


IMAGING FINDINGS

Extensive dense symmetric calcifications throughout the bilateral globus pallidus, dentate nuclei, red nuclei, deep cerebral periventricular and supraventricular white matter, deep cerebellar white matter, and posterior limb internal capsules.

IMAGES

  • Fahr disease_01.jpg
  • Fahr disease_02.jpg

DISCUSSION

Fahr’s disease (or bilateral striopallidodentate calcinosis) is a rare neurodegenerative disorder of unknown cause characterised by symmetrical calcium deposition in the brain; it can be sporadic or autosomal dominant1
The German pathologist Karl Theodor Fahr first described it in 1930.2

Patients most commonly present with movement disorders. In addition, cognitive impairment, seizures, cerebellar signs and dysarthria can be seen 3
is characterized by the bilaterally symmetric deposition of calcium (and other
minerals) in the basal ganglia, thalamus, dentate  nuclei, and centrum semiovale in the absence of
hypoparathyroidism. The Globos pallidus is the most common site of Ca.
At neuroimaging, the condition is characterized by bilaterally symmetric dense calcifications in the basal ganglia, dentate nuclei, thalamus, and subcortical white matter of the cerebrum

Findings are favored to represent underlying Fahr disease

hypoparathyroidism
Pseudohypoparathyroidism

References

1.-Manyam BV. What is and what is not ‘Fahr’s disease’. Parkinsonism Relat Disord 2005;11:73–80.
2.- Fahr T. Idiopathische Verkalkung der Hirngefa¨ sse. Zentralblatt fu¨ r allgemeine Pathologie und pathologische Anatomie 1930–1931;50:129–33.
3.- Manyam BV, Walters AS, Narla KR. Bilateral Striopallidodentate Calcinosis: Clinical Characteristics of Patients Seen in a Registry. Mov Disord 2001;16:258–64.

citation

Author(s):

Dr. Ramon E.  Figueroa.

Dr. Joaquin Nunez G. 

Augusta University. Augusta, GA 30912 USA

Case Title:
Fahr's Disease
URL:
http://neuroradiologysilan.com/case/8170534692

Published:
29.09.2017
Section:
Brain |
Views:
223
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