2020, Number 1
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Rev Cubana Neurol Neurocir 2020; 10 (1)
Fahr syndrome due to secondary hypoparatiroidism
León CR, Real CRM, Domínguez GWH, Linares SEY, Durán TG, Gómez VN
Language: Spanish
References: 26
Page: 1-10
PDF size: 453.84 Kb.
ABSTRACT
Objective: To describe the etiological diagnosis process of Fahr syndrome in a patient with no family history of brain calcifications.
Clinical case report: We report a female patient who was admitted because of psychiatric disorders, seizures and involuntary movements. A computed tomography scan of the skull was performed, which showed thick and symmetrical calcifications in the basal ganglia, cerebellum, and white matter. Fahr syndrome was diagnosed from secondary hypoparathyroidism because of the injuries observed, the results of hormonal tests, and a history of thyroidectomy from several years. She was treated with calcium gluconate and phenytoin, and subsequently died of cardiogenic shock.
Conclusions: The analysis of the clinical characteristics, the neuroimaging findings, the laboratory tests, the pathological results and the surgical history, allowed the etiological diagnosis of Fahr syndrome in this patient.
REFERENCES
Calili DK, Mutlu NM, Mutlu Titiz AP, Akcaboy ZN, Aydin EM, Turan IO. Unexplained neuropsychiatric symptoms in intensive care: A Fahr Syndrome. J Pak Med Assoc. 2016;66(8):1029-31.
Saleem S, Aslam HM, Anwar M, Anwar S, Saleem M, Saleem A, et al. Fahr’s syndrome: literature review of current Evidence. Rom J Intern Med. 2013;8(1):156-65.
Dos Santos VM, Da Mata AM, Ribeiro KR, Calvo IC. Fahr’s Syndrome and Secondary Hypoparathyroidism. Rom J Intern Med. 2016;54(1):63-5.
Guzmán GE, Arévalo Espejo O, Jiménez Penagos K, Pérez O. Síndrome de calcificación cerebral asociado a hipoparatiroidismo. Acta Neurol Colomb. 2014;30(2):114-7.
Delacour A. Ossification des capillaires du cerveau. Ann Med-Psychol. 1859;458:61.
Fahr T. Idiopathipathische verkalkung der hirngefässe. Zentrabl Allg Pathol. 1930;50:129-33.
Savino E, Soavi C, Capatti E, Borrelli M, Vigna GB, Passaro A, et al. Bilateral strio-pallido-dentate calcinosis (Fahr’s disease): report of seven cases and revision of literature. BMC Neurol. 2016;16:165-76.
Manyam BV. What is and what is not ‘Fahr’s disease’. Parkinsonism Relat Disord. 2005;11(2):73-80.
Dade E, Saint-Joy V, Haynes NA, Berkowitz AL. Teaching NeuroImages: Fahr syndrome caused by Hypoparathyroidism. Neurology. 2017;88(23):e233.
Etcharry-Bouyx F, Ceccaldi M, Poncet M, Pellissier JF. Fahr’s disease and mitochondrial myopathy. Rev Neurol (Paris). 1995;151(12):731-3.
Sue CM, Crimmins DS, Soo YS, Pamphlett R, Presgrave CM, Kotsimbos N, et al. Neuroradiological features of six kindreds with MELAS tRNA(Leu) A2343G point mutation: implications for pathogenesis. J Neurol Neurosurg Psychiatry. 1998;65(2):233-40.
Mufaddel AA, Al-Hassani GA. Familial idiopathic basal ganglia calcification (Fahr’s disease). Neurosciences. 2014;19(3):171.
Forstl H, Krumm B, Eden S, Kohlmeyer K. Neurological disorders in 166 patients with basal ganglia calcification: a statistical evaluation. J Neurol. 1992;239(1):36-8.
Ostling S, Andreasson LA, Skoog I. Basal ganglia calcification and psychotic symptoms in the very old. Int J Geriatr Psychiatry. 2003;18(11):983-7.
Sarkar DN, Sarkar D, Zahin AKM, Ohab MA, Miah MS, Haque MM. Fahr's Syndrom e: A rare case- Presented as Acute Ischaemic Stroke. Medicine today. 2017;29(1):45-6.
Rizvi SA, Ahmed SS, Papillon F, Branly R, Salah AM, Ahmed J. FAHR SYNDROME. Consultant. 2016;56(9):848-9.
Sucre-Grimaldo FM, Casares-Cruz K, Sandoval-Paredes J. Calcinosis bilateral en los núcleos estriado, pálido y dentado e hipoparatiroidismo: a propósito de un caso. Anales de Radiología México. 2015;14:285-91.
König P. Psychopathological alterations in cases of symmetrical basal ganglia sclerosis. Biol Psychiatry. 1989;25(4):459-68.
Chepuri VR, Panta H. A case of Fahr's syndrome with rare atypical presentation as hemiplegia. NUJHS. 2015;5(4):77-9.
Khalil I, Aziz Siham El, Chadli A. Fahr syndrome revealing primary hypoparathyroidism: About two cases. En: 21st European Congress of Endocrinology. BioScientifica. 2019;63:507. Doi: 10.1530/endoabs.63.P507.
Halili G, Papajani M, Grabova S, Rroji A, Kruja J. Fahr syndrome: A case series analysis. J Neurol Sci. 2019;405:355.
Gurcan O, Gurcay AG, Kazanci A, Goker T, Eylen O, Turkoglu OF. Chronic subdural hematoma associated with fahr syndrome: A clinical association or just a simple coincidence? Asian J Neurosurg. 2018;13(1):90-2.
Chen J. SUN-510 Pseudohypoparathyroidism Type 1b Presenting with Fahr Syndrome in An Adolescent. Journal of the Endocrine Society. 2019;3(1):SUN-510.
Kumar S, Gaad AA, Irshad Abbasi M, Afzal M, Shah S, Kumar D. Association of fahr disease with Rhabdomyolysis and hypoparathyroidism. Pakistan Journal of Neurological Sciences (PJNS). 2018;13(4):27-30.
Dembélé K, Cissé L, Djimdé S, Coulibaly Y, Diarra S, Yalcouyé A, et al. Fahr's syndrome with hyperparathyroidism revealed by seizures and proximal weakness. eNeurologicalSci. 2019;15:100192.
Jeon I, Cho KH, Kim SW. Concomitant Fahr’s syndrome and thoracic ossification of the posterior longitudinal ligament caused by idiopathic hypoparathyroidism –case report. BMC Musculoskelet Disord. 2019;20(1):1-4.