Fahr's syndrome is defined by the presence of striato-pallido-dentate calcifications. It is a rare entity with clinical polymorphism, and it occurs in patients with dysparathyroidism, especially those with hypoparathyroidism. It must be distinguished from Fahr's disease (FD), which is defined by the presence of intracerebral calcifications without phosphocalcic metabolism abnormality. In this paper, we report the particulars of five patients diagnosed with Fahr's syndrome revealed by neurological and cognitive disorders, seizures, and abnormal movements associated with tetany crisis. In all cases, brain imaging and biological examinations led to the diagnosis of Fahr's syndrome related to hypoparathyroidism. The evolution was favorable after treatment. Fahr's syndrome is a rare and serious condition for which treatment is simple and effective. Our observations shed light on the necessity of evaluating phosphocalcic metabolism and exploring cerebral calcifications in patients with neurological disorders.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10350282 | PMC |
http://dx.doi.org/10.7759/cureus.40502 | 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 PDFFront 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.
View Article and Find Full Text PDFRadiol Case Rep
January 2025
M Abdur Rahim Medical College Hospital, Dinajpur, Bangladesh.
Fahr's disease, also known as primary familial brain calcification, is a progressive neurological disorder that follows an autosomal dominant inheritance pattern, characterized by calcifications primarily located within the basal ganglia of the brain. This condition typically affects middle-aged individuals, who present with a combination of neurological and psychiatric symptoms; however, this case report discusses a 16-year-old male patient. The patient initially exhibited general signs of infection, including fever and jaundice, before developing specific neurological symptoms, which progressed to systemic encephalopathy characterized by altered consciousness, seizures, and hypoglycemia, necessitating his admission to the ICU.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!