ArticleBMC neurology2016
Bilateral strio-pallido-dentate calcinosis (Fahr's disease): report of seven cases and revision of literature.
Article in BMC neurology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 55 citations in OpenAlex.
- Article
- Challenging Diagnosis of Fahr's Disease Mimicking Parkinson's Disease: A Case Report.Clinical case reports · 2025Article
- Fahr's disease in a patient with recurrent pneumonias, parkinsonism and dementia.BMJ case reports · 2024Article
- First-time seizure revealing late-onset Fahr's disease: a case report and brief literature review.Frontiers in human neuroscience · 2024Article
- Article
- Advanced Early-Onset Fahr's Disease: A Case Report.Cureus · 2023Article
- A rare case of Fahr disease revealed by an epileptic seizure.Radiology case reports · 2023Article
- Epidemiology and Financial Burden of Adult Chronic Hypoparathyroidism.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2022Review
- Intracranial calcification and psychotic symptoms after irradiation in a patient with Fanconi anemia: A case report.PCN reports : psychiatry and clinical neurosciences · 2022Article
- Neuropsychiatric Manifestations of Fahr's Disease, Diagnostic and Therapeutic Challenge: A Case Report and a Literature Review.Clinical neuropsychiatry · 2022Article
- Familial Idiopathic Basal Ganglia Calcification: A Father-Son Dyad Demonstrate Heterogeneity of Presentation and Disease Progression.Archives of clinical neuropsychology : the official journal of the National Academy of Neuropsychologists · 2022Article
- Article
- Hereditary and non-hereditary etiologies associated with extensive brain calcification: case series.Metabolic brain disease · 2021Article
- From cranial nerve palsy to seizures-All the signs that lead to secondary Fahr's syndrome.Clinical case reports · 2021Article
- Basal ganglia calcification in hypoparathyroidism and pseudohypoparathyroidism: local and systemic metabolic mechanisms.Journal of endocrinological investigation · 2021Review
- Basal ganglia calcifications (Fahr's syndrome): related conditions and clinical features.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2019Review
- Concomitant Fahr's syndrome and thoracic ossification of the posterior longitudinal ligament caused by idiopathic hypoparathyroidism - case report.BMC musculoskeletal disorders · 2019Article
- Intracranial calcifications on CT: an updated review.Journal of radiology case reports · 2019Review
- Article
- Unusual Cause of Hypotension in a Polytrauma Victim: A Case of Fahr's Syndrome.Indian journal of critical care medicine : peer-reviewed, official publication of Indian Society of Critical Care Medicine · 2018Article
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Authors and funding
7 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundFahr'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. CASES PRESENTATION: 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.
conclusionBased 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.
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