ReviewNeurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2019
Basal ganglia calcifications (Fahr's syndrome): related conditions and clinical features.
Review in Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 90 papers, 1 of them a synthesis that pooled it.
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Who cites it
90 citing papers in PubMed, 1 synthesis or guideline pooled it, 163 citations in OpenAlex.
- Safety and Efficacy of PTH 1-34 and 1-84 Therapy in Chronic Hypoparathyroidism: A Meta-Analysis of Prospective Trials.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2022Pooled it
- The role of calcium serum level in clinical manifestation and progress of psoriasis vulgaris in a patient with Fahr's syndrome.Dermatology reports · 2026Article
- Multimodal PET Findings in MYORG-Related Primary familial Brain Calcification Caused by a Novel Homozygous Frameshift Mutation.Cerebellum (London, England) · 2026Article
- Letter to the Editor: Potential pitfalls in deep learning-based imaging for spontaneous intracerebral hemorrhage.World journal of radiology · 2026Article
- Bilateral Basal Ganglia Calcification (Fahr's Syndrome) Secondary to Hypoparathyroidism: A Case Series.Clinical case reports · 2026Article
- White Matter Matters: A Magnetic Resonance Imaging Study with Clinical Correlates in Primary Brain Calcification.Movement disorders : official journal of the Movement Disorder Society · 2026Article
- A Novel Frameshift Mutation inBiomedicines · 2026Article
- Early-onset Fahr's disease with acute psychosis in a 20-year-old male with SLC20A2 mutation: A case report and literature review.Radiology case reports · 2026Article
- Generalized Dystonia in Fahr's Syndrome Secondary to Idiopathic Hypoparathyroidism: Improvement After Metabolic Correction.Journal of clinical neurology (Seoul, Korea) · 2026Article
- [A case report of severe hypocalcemia secondary to Fahr's syndrome].Anales del sistema sanitario de Navarra · 2026Article
- First case of Fahr's disease with homozygous mutations of SLC20A2, among the Libyan children.BMC pediatrics · 2026Article
- Endocrine Disorders of Calcium Signaling in Children: Neuroendocrine Crosstalk and Clinical Implications.Cells · 2026Review
- Superior sagittal sinus occlusion combined with dural arteriovenous fistula presenting as Fahr-like pattern: a case report.Frontiers in surgery · 2026Article
- Late-onset atypical pseudohypoparathyroidism type 1A with intracranial calcification and epilepsy associated with a novelFrontiers in endocrinology · 2026Article
- Thalamic quantitative susceptibility mapping and susceptibility source separation: a critical narrative review of evidence and interpretation.Frontiers in neurology · 2026Review
- Fahr's Syndrome Secondary to Hypoparathyroidism Presenting With Neurological and Cardiac Manifestations.Cureus · 2025Article
- Review
- A rare case of Fahr's disease with posterior circulation (basilar tip) aneurysm- pathophysiology, management, and complications.Journal of cerebrovascular and endovascular neurosurgery · 2025Article
- Delirious Hyperactivity and Agitation in a Young Male Unveiling an Intriguing Underlying Diagnosis: Case Report.Clinical practice and cases in emergency medicine · 2025Article
- Rare Movement Disorders-An Approach for Clinicians.International journal of molecular sciences · 2025Review
30 more citing papers are in PubMed but not listed here.
Corrections and comments
- Erratum issued
Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Basal ganglia calcifications could be incidental findings up to 20% of asymptomatic patients undergoing CT or MRI scan. The presence of neuropsychiatric symptoms associated with bilateral basal ganglia calcifications (which could occur in other peculiar brain structures, such as dentate nuclei) identifies a clinical picture defined as Fahr's Disease. This denomination mainly refers to idiopathic forms in which no metabolic or other underlying causes are identified. Recently, mutations in four different genes (SLC20A2, PDGFRB, PDGFB, and XPR1) were identified, together with novel mutations in the Myogenic Regulating Glycosylase gene, causing the occurrence of movement disorders, cognitive decline, and psychiatric symptoms. On the other hand, secondary forms, also identified as Fahr's syndrome, have been associated with different conditions: endocrine abnormalities of PTH, such as hypoparathyroidism, other genetically determined conditions, brain infections, or toxic exposure. The underlying pathophysiology seems to be related to an abnormal calcium/phosphorus homeostasis and transportation and alteration of the blood-brain barrier.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.