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Fahr Disease
Miral D. Jhaveri, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Fahr disease (FD)
              • Synonyms

                • Primary familial brain calcification (PFBC)
                • Familial idiopathic basal ganglia (BG) Ca⁺⁺
                • Bilateral striopallidodentate Ca⁺⁺
                • Idiopathic familial cerebrovascular ferrocalcinosis
              • Definitions

                • Rare degenerative neurologic disorder
                  • Extensive bilateral BG Ca⁺⁺
                  • Can lead to progressive dystonia, parkinsonism, neuropsychiatric manifestations

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Bauer M et al: MYORG mutations: a major cause of recessive primary familial brain calcification. Curr Neurol Neurosci Rep. 19(10):70, 2019
                                          2. Pourshahid S et al: Fahr's disease: a differential to be considered for various neuropsychiatric presentations. Cureus. 10(3):e2304, 2018
                                          3. Quintáns B et al: Primary familial brain calcifications. Handb Clin Neurol. 147:307-17, 2018
                                          4. Kostić VS et al: Brain calcification and movement disorders. Curr Neurol Neurosci Rep. 17(1):2, 2017
                                          5. Calabrò RS et al: Fahr's disease presenting with dementia at onset: a case report and literature review. Behav Neurol. 2014:750975, 2014
                                          6. Mufaddel AA et al: Familial idiopathic basal ganglia calcification (Fahr`s disease). Neurosciences (Riyadh). 19(3):171-7, 2014
                                          7. Westenberger A et al: The genetics of primary familial brain calcifications. Curr Neurol Neurosci Rep. 14(10):490, 2014
                                          8. Bekiesinska-Figatowska M et al: Basal ganglia lesions in children and adults. Eur J Radiol. 82(5):837-49, 2013
                                          9. Shirahama M et al: A young woman with visual hallucinations, delusions of persecution and a history of performing arson with possible three-generation Fahr disease. Acta Psychiatr Scand. 121(1):75-7, 2010
                                          10. Smith AB et al: Bilateral thalamic lesions. AJR Am J Roentgenol. 192(2):W53-62, 2009
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Fahr disease (FD)
                                                      • Synonyms

                                                        • Primary familial brain calcification (PFBC)
                                                        • Familial idiopathic basal ganglia (BG) Ca⁺⁺
                                                        • Bilateral striopallidodentate Ca⁺⁺
                                                        • Idiopathic familial cerebrovascular ferrocalcinosis
                                                      • Definitions

                                                        • Rare degenerative neurologic disorder
                                                          • Extensive bilateral BG Ca⁺⁺
                                                          • Can lead to progressive dystonia, parkinsonism, neuropsychiatric manifestations

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR Findings

                                                            • Nuclear Medicine Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Bauer M et al: MYORG mutations: a major cause of recessive primary familial brain calcification. Curr Neurol Neurosci Rep. 19(10):70, 2019
                                                                                  2. Pourshahid S et al: Fahr's disease: a differential to be considered for various neuropsychiatric presentations. Cureus. 10(3):e2304, 2018
                                                                                  3. Quintáns B et al: Primary familial brain calcifications. Handb Clin Neurol. 147:307-17, 2018
                                                                                  4. Kostić VS et al: Brain calcification and movement disorders. Curr Neurol Neurosci Rep. 17(1):2, 2017
                                                                                  5. Calabrò RS et al: Fahr's disease presenting with dementia at onset: a case report and literature review. Behav Neurol. 2014:750975, 2014
                                                                                  6. Mufaddel AA et al: Familial idiopathic basal ganglia calcification (Fahr`s disease). Neurosciences (Riyadh). 19(3):171-7, 2014
                                                                                  7. Westenberger A et al: The genetics of primary familial brain calcifications. Curr Neurol Neurosci Rep. 14(10):490, 2014
                                                                                  8. Bekiesinska-Figatowska M et al: Basal ganglia lesions in children and adults. Eur J Radiol. 82(5):837-49, 2013
                                                                                  9. Shirahama M et al: A young woman with visual hallucinations, delusions of persecution and a history of performing arson with possible three-generation Fahr disease. Acta Psychiatr Scand. 121(1):75-7, 2010
                                                                                  10. Smith AB et al: Bilateral thalamic lesions. AJR Am J Roentgenol. 192(2):W53-62, 2009