Fahr disease (FD) is a well-defined rare neurodegenerative disease that is characterized by idiopathic bilateral symmetric extensive striopallidodentate calcifications. The patients may present with diverse manifestations, most commonly movement disorder, cognitive impairment, and ataxia. Computed tomography (CT) is considered to be critical for accurate diagnosis because it is difficult to reliably identify calcifications by routine magnetic resonance imaging (MRI). Susceptibility-weighted imaging (SWI) is a relatively new 3D gradient-echo (GE) MR sequence with special phase and magnitude processing. SWI phase images can recognize calcifications definitively with higher sensitivity compared to other MRI sequences. In this article, we present two cases of FD with different manifestations and neuroimaging in different age groups and genders, which were diagnosed by SWI and confirmed with CT, and we discuss the contribution of SWI in the diagnosis of FD. In conclusion, we suggest integrating SWI with MRI protocol to identify calcifications in suspicion of neurodegenerative disorders.
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http://dx.doi.org/10.3978/j.issn.2223-4292.2015.04.01 | DOI Listing |
Radiol Case Rep
February 2025
Department of Psychiatry, Datta Meghe Institute of Medical Sciences, Sawangi, Wardha, Maharashtra, India 442001.
Fahr's disease is a rare neurological disorder which is characterized by the presence of abnormal, symmetrical, and bilateral calcifications within the basal ganglia and other cerebral areas. Seizures are 1 of the symptoms that may aid in its diagnosis. Fahr's disease is diagnosed in adults mostly.
View Article and Find Full Text PDFClin Pract Cases Emerg Med
November 2024
University of Louisville Health, Louisville, Kentucky.
Introduction: Fahr disease and Fahr syndrome represent clinical entities that result in diffuse intracranial brain calcification, either by way of genetic mutation in the case of the former or by secondary endocrine dysfunction in the latter.
Case Report: We present a case of a middle-aged male with undiagnosed Fahr syndrome, identified during evaluation for symptoms of an acute posterior circulation cerebrovascular accident.
Conclusion: Fahr syndrome is a clinical constellation of symptoms and radiographic findings often seen in late-stage hypoparathyroidism.
Front Hum Neurosci
November 2024
Department of Internal Medicine, Jimma University, Jimma, Ethiopia.
Mov Disord Clin Pract
December 2024
Department of Neurology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Radiol Case Rep
January 2025
Radiology Department, Shifa International Hospital, Islamabad, Pakistan.
Fahr's disease is a rare neurodegenerative condition characterized by widespread cerebral calcium accumulation and cell death, mostly in the bilateral basal ganglia and dentate nuclei of the cerebellum. Concurrent findings of Fahr disease with multi territorial ischemic stroke and falx lipoma has been rarely reported till date. We discuss the case of a 75-year-old female Pakistani by birth, with Fahr's disease who presented with an ischemic stroke.
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