A case of Fahr disease with transient slurred speech and abnormal movement of the left arm

Fahr disease is a rare neurological condition characterized by abnormal basal ganglia calcification. Some patients with Fahr disease have been reported to present to the emergency department (ED) with stroke-like symptoms. Here, we present a case of Fahr disease with transient focal neurological signs. A 63-year-old man came to the ED with complaints of slurred speech and abnormal movement of his left arm. His symptoms lasted only 5 minutes before he recovered. He had no focal neurological deficits when he arrived at the ED, had no previous medical history and had never taken any medication. A physical examination revealed that the patient was healthy, and the initial results of laboratory tests were all within normal range. A brain computed tomography (CT) without contrast enhancement revealed symmetric calcification of the basal ganglia, thalamus and dentate nucleus of the cerebellum. Magnetic resonance imaging of the brain and neck CT angiography revealed no evidence of acute cerebral infarction, intracranial large-vessel occlusion, or stenosis. Based on his clinical presentation and examination results, the patient was diagnosed with Fahr disease in the ED. He was recommended to be admitted for differential diagnosis of seizures and additional tests, such as electroencephalography, but opted for discharge against medical advice. Emergency physicians should consider the possibility of Fahr disease if patients have focal neurological signs with characteristic symmetric calcification on brain CT. A detailed history and examination should be performed to detect other secondary causes associated with Fahr syndrome.

Fahr disease; Basal ganglia calcification; Computed tomography

[1] Alam ST, Aswani Y, Anandpara KM, Hira P. CT findings in Fahr’s disease. Case Reports. 2015; 2015: bcr2014208812.

[2] Donzuso G, Mostile G, Nicoletti A, Zappia M. Basal ganglia calci-fications (Fahr’s syndrome): related conditions and clinical features. Neurological Sciences. 2019; 40: 2251–2263.

[3] Saleem S, Aslam HM, Anwar M, Anwar S, Saleem M, Saleem A, et al. Fahr’s syndrome: literature review of current evidence. Orphanet Journal of Rare Diseases. 2013; 8: 156.

[4] Modrego P, Mojonero J, Serrano M, Fayed N. Fahr’s syndrome presenting with pure and progressive presenile dementia. Neurological Sciences. 2005; 26: 367–369.

[5] G J, Bansal R, Ajmal M, Gupta P, Pathania M. Young male with seizure disorder and intracranial calcification: a case of Fahr’s syndrome. Cureus. 2022; 14: e22189.

[6] Yang C, Lo C, Wu M. Ischemic stroke in a young patient with Fahr’s disease: a case report. BMC Neurology. 2016; 16: 33.

[7] Harrington MG, Macpherson P, McIntosh WB, Allam BF, Bone I. The significance of the incidental finding of basal ganglia calcification on computed tomography. Journal of Neurology, Neurosurgery & Psychiatry. 1981; 44: 1168–1170.

[8] Kim T, Park I, Kim S, Lee K, Kim Y, Kim J. Striopallidodentate calcification and progressive supranuclear palsy-like phenotype in a patient with idiopathic hypoparathyroidism. Journal of Clinical Neurology. 2007; 3: 57.

[9] Otu AA, Anikwe JC, Cocker D. Fahr’s disease: a rare neurological presentation in a tropical setting. Clinical Case Reports. 2015; 3: 806–808.

[10] Shouyama M, Kitabata Y, Kaku T, Shinosaki K. Evaluation of regional cerebral blood flow in Fahr disease with schizophrenia-like psychosis: a case report. American Journal of Neuroradiology. 2005; 26: 2527–2529.

[11] Sava A, Dumitrescu G, Haba D, Hodorog D, Mihailov C, Şapte E. The Fahr syndrome and the chronic lymphocytic thyroiditis. Romanian Journal of Morphology and Embryology. 2013; 54: 195–200.

[12] Uygur G, Liu Y, Hellman R, Tikofsky R, Collier B. Evaluation of regional cerebral blood flow in massive intracerebral calcifications. Journal of Nuclear Medicine. 1995; 36: 610–612.

[13] Yang C, Lo C, Wu M. Ischemic stroke in a young patient with Fahr’s disease: a case report. BMC Neurology. 2016; 16: 33.

[14] Jaworski K, Styczyńska M, Mandecka M, Walecki J, Kosior DA. Fahr syndrome—an important piece of a puzzle in the differential diagnosis of many diseases. Polish Journal of Radiology. 2017; 82: 490–493.

[15] Amisha F, Munakomi S. Fahr syndrome. StatPearls: Treasure Island (FL). 2022.

[16] Asensio Moreno C, Arias Jiménez JL, Aramburu Bodas O, Ortega Calvo M, Pérez Cano R. Transient ischemic attack associated with a calcinosis cerebri syndrome. Anales de Medicina Interna. 2008; 25: 33–35. (In Spanish)

[17] Govindarajan A. Imaging in Fahr’s disease: how CT and MRI differ?Case Reports. 2013; 2013: bcr2013201523.

[18] Sahin N, Solak A, Genc B, Kulu U. Fahr disease: use of susceptibility-weighted imaging for diagnostic dilemma with magnetic resonance imaging. Quantitative Imaging in Medicine and Surgery. 2015; 5: 628–632.

[19] Rastogi R, Singh A, Rastogi U, Mohan C, Rastogi V. Fahr’s syndrome: a rare clinico-radiologic entity. Medical Journal Armed Forces India. 2011; 67: 159–161.