Fahr’s disease: a rare diagnosis requiring admission to the emergency department

Abstract

Fahr’s disease is a rare degenerative neurological disorder characterized by the presence of abnormal calcium deposition and associated cell loss in the areas of the brain that control movement, including the basal ganglia and cerebral cortex. Clinical findings associated with Fahr's disease include parkinsonism, dystonia, chorea, ataxia and psychiatric symptoms. Fahr's disease may result from metabolic disorders, especially parathyroid disorders. We report our experience with 4 patients admitted to our emergency department with complaints such as convulsions, hand spasms, loss of consciousness, and weakness. Computed tomography of all patients showed calcification in the bilateral basal ganglia. The purpose of this paper is to draw attention to a rare disorder involved in the etiology of elderly patients admitted to the emergency department for seizure and/or unconsciousness.

Keywords

• Fahr’s disease
• hypocalcemia
• intracranial calcification
• seizure
• unconsciousness

References

1. Paprocka J, Jamroz E, Wackerman-Ramos A, Sokol M, Marszal E. [Norological Picture and 1H MRS in 4 children with hypoparathyroidisim]. PrezeglLek 2005;62:680-4. [Article in Polish]
2. Manyam BV. What is and what is not ‘Fahr’s disease’. Parkinsonism Relat Disord 2005;11:73-80.
3. Kokes U, Hiz F, Ozden DL, Cinar M. Psodohipoparatroidinin yol actigi bir Fahr sendromu olgusu. Parkinson Hastaligi Hareket Bozukluklari Dergisi 2009;12;18-24.
4. Sambrook MA, Hill LF, Cerebrospinal fluid absorbtion in primary hypoparathroidisim. J Neurol Neurosurg Physchiatry 1997;40:1015-7.
5. Kazis AD. Contribution of CT scan to the diagnosis of Fahr’s syndrome. Acta Neurol Scand 1985;71:206-11.
6. Windeck R, Menken U. Benker G, Reinwein D.Basal ganglia calcification in pseudo hypoparathyroidism type II. Clin Endocrinol (Oxf.) 1981;15:57-63.
7. Morgante L, Vita G, Meduri M, Di Rosa AE, Galatioto S, Coraci MA, et al. Fahr’s syndrome: local inflammatory factors in the pathogenesis of calcification. J Neurol 1986;233:19-22.
8. Anderson JR. Intracerebral calcification in a case of systemic lupus erythematosus with neurological manifestations. Neuropathol App Neurobiol 1981;7:161-6.

9. Illum F. Calcification of basal ganglia following carbon monoxide poisoning. Neurodiology 1980;19:213-4.
10. Harati Y, Jackson JA, Benjamin E. Adult onset idiopathic familial brain calcifications. Arch Intern Med 1984;144:2425-7.
11. Manyam BV, Walters AS, Narla KR. Bilateral striopallido dentate calcinosis: clinical characteristics of patients seen in a registry. Mov Disord 2001;16:258-64.
12. Ramonet D, de Yebra L, Fredriksson K, Bernal F, Ribalta T, Mahy N. Similar calcification process in acute and chronic human brain pathologies. J Neurosci Res 2006;83:147-56.
13. Karimi M, Habibzadeh F, De Sanctis V. Hypoparathyroidism with extensive intracerebral calcification in patients with beta-thalassemia major. J Pediatr Endocrinol Metab 2003;16:883-6.
14. Harrington MG, Macpherson P, Mcintosh WB, Allam BF, Bone I. The significance of the incidental finding of basal ganglia calcification on computed tomography. J Neurol Neurosurg Psychiatry 1981;44:1168-70.
15. Taxer F, Haller R, Konig P. Clinical early symptoms and CT findings in Fahr syndrome. Nervenarzt 1986;57:583-8.
16. Ogi S, Fukumitsu N, Tsuchida D, Uchiyama M, Mori Y, Matsui K. Imaging of bilateral striopallidodentate calcinosis. Clin Nucl Med 2002;27:721-4.