Article type: Case Report
Article
title: Fahr's disease presenting with pure dementia: a case report and literature review
Journal: Apollo
Medicine
Year: 2019
Authors: Jamir Pitton Rissardo, Ana Letícia Fornari
Caprara, Juliana Oliveira Freitas Silveira
E-mail: jamirrissardo@gmail.com
ABSTRACT
Fahr's disease (FD) is a
rare inherited or sporadic disorder characterized by symmetrical calcium
deposits in the basal ganglia and dentate nuclei with the absence of
biochemical abnormalities. Clinical manifestations can start at different ages
with a variety of presentations. We report a case of a 68‑year‑old
male who presented to our service with clinical signs and symptoms of pure and
progressive dementia. Afterward, he developed mood changes, and finally,
movement disorders, probably due to a sporadic form of FD. Noncontrast cranial
computed tomography scan demonstrated scattered brain parenchyma, bilateral
calcifications in the basal ganglia, and dentate nuclei. Standard blood tests
were within normal limits. In this way, FD can present with pure dementia
at onset, reflecting a wide range of neurological manifestations. The diagnosis
is challenging, and we proposed an acronym to facilitate the learning of the
diagnostic criteria.
Keywords: Dementia, fahr's disease, idiopathic basal ganglia calcification, tomography
Full text
available at:
DOI
10.4103/am.am_54_19
Citation
Rissardo JP, Caprara AL, Silveira JO. Fahr's disease presenting with pure dementia: a case report and literature review. Apollo Med 2019;16:236-9
