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Bilateral strio-pallido-dentate calcinosis (Fahr’s disease): report of seven cases and revision of literature

Overview of attention for article published in BMC Neurology, September 2016
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Title
Bilateral strio-pallido-dentate calcinosis (Fahr’s disease): report of seven cases and revision of literature
Published in
BMC Neurology, September 2016
DOI 10.1186/s12883-016-0693-1
Pubmed ID
Authors

Elisabetta Savino, Cecilia Soavi, Eleonora Capatti, Massimo Borrelli, Giovanni B. Vigna, Angelina Passaro, Giovanni Zuliani

Abstract

Fahr's disease is rare a neurodegenerative idiopathic condition characterized by symmetric and bilateral calcifications of basal ganglia, usually associated with progressive neuropsychiatric dysfunctions and movement disorders. The term "Fahr's syndrome" is used in presence of calcifications secondary to a specific cause, but the variability of etiology, pathogenesis, and clinical picture underlying this condition have raised the question of the real existence of a syndrome. Several classifications based on the etiology, the location of brain calcifications and the clinical presentation have been proposed. Here we describe seven clinical cases of basal ganglia calcifications, in order to search for pathognomonic features and correlations between clinical picture and imaging findings. The patients came to our attention for different reasons (most of them for memory/behavior disturbances); all underwent neuro-psychologic evaluation and neuro-imaging. All patients showed variable degrees of deterioration in cognitive function; anxiety and depression were frequent too, and resistant to treatment in all cases. Less frequent, but severe if present, were psychotic symptoms, with different grade of structure and emotional involvement, and always resistant to treatment. We observed only few cases of extrapyramidal disorders related to the disease itself; anyway, mild extrapyramidal syndrome occurred quite frequently after treatment with antipsychotics. Based on these findings we discourage the use of the term "Fahr's syndrome", and suggest to refer to Idiopathic or Secondary basal ganglia calcification. Unlike early onset forms (idiopathic or inherited), the clinical presentation of late onset form and Secondary basal ganglia calcification seems to be really heterogeneous. Case-control studies are necessary to determine the actual significance of basal ganglia calcification in the adult population and in the elderly, in cognitive, physical and emotional terms.

Twitter Demographics

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Mendeley readers

The data shown below were compiled from readership statistics for 74 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 74 100%

Demographic breakdown

Readers by professional status Count As %
Student > Master 13 18%
Student > Bachelor 12 16%
Student > Ph. D. Student 12 16%
Student > Doctoral Student 7 9%
Researcher 6 8%
Other 11 15%
Unknown 13 18%
Readers by discipline Count As %
Medicine and Dentistry 20 27%
Psychology 17 23%
Nursing and Health Professions 9 12%
Neuroscience 6 8%
Biochemistry, Genetics and Molecular Biology 2 3%
Other 2 3%
Unknown 18 24%

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 10 September 2016.
All research outputs
#7,222,563
of 8,355,069 outputs
Outputs from BMC Neurology
#1,149
of 1,254 outputs
Outputs of similar age
#210,409
of 252,739 outputs
Outputs of similar age from BMC Neurology
#53
of 56 outputs
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