Fahr’s syndrome and idiopathic hypoparathyroidism – A case report

Dejan M Marinković; Tamara Dragović; Saša Kiković; Snežana Kuzmić Janković; Zorana Đuran; Zoran Hajduković

doi:10.2298/VSP150916109M

Dejan M Marinković Clinic for Endocrinology, Military Medical Academy, Belgrade, Serbia
Tamara Dragović Clinic for Endocrinology, Military Medical Academy, Belgrade, Serbia; Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia.
Saša Kiković Clinic for Endocrinology, Military Medical Academy, Belgrade, Serbia;
Snežana Kuzmić Janković Clinic for Endocrinology, Military Medical Academy, Belgrade, Serbia
Zorana Đuran Clinic for Endocrinology, Military Medical Academy, Belgrade, Serbia
Zoran Hajduković Clinic for Endocrinology, Military Medical Academy, Belgrade, Serbia; Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia

DOI: https://doi.org/10.2298/VSP150916109M

Keywords: hypoparathyroidism, comorbidity, diagnosis, differential, drug therapy, neurodegenerative diseases, calcinosis, basal ganglia,

Abstract

Introduction. Fahr´s syndrome is a rare, slowly progressive, neurodegenerative disorder, characterised by extensive, bilateral, and symmetrical basal ganglia calcification. It is associated with neuropsychiatric manifestations and gradually progressive cognitive impairment. Fahr's syndrome is the secondary form of brain calcification that is caused by various metabolic, infectious, or degenerative diseases. Case report. We presented a middle-aged male with Fahr's syndrome due to primary idiopathic hypoparathyroidism. Clinical diagnosis was based on signs and symptoms of hypocalcemia, progressive neuropsychiatric illnesses, laboratory evidence of hypoparathyroidism, and radiological signs of calcifications in the basal ganglia. The patient improved after only a few days of intravenous rehydration and calcium substitution, followed by oral supplemental calcitriol. Conclusion. Timely recognition of idiopathic and iatrogenic hypoparathyroidism allows appropriate treatment that can prevent the development and clinical manifestations of Fahr´s syndrome and potentially slow its progression.

References

Saleem S, Aslam HM, Anwar M, Anwar S, Saleem M, Saleem A, et al. Fahr's syndrome: Literature review of current evidence. Orph J Rare Dis 2013; 8(1): 156.

Asokan AG, D’Souza S, Jeganathan J, Pai S. Fahr's Syndrome- An Interesting Case Presentation. J Clin Diagn Res 2013; 7(3): 532−3.

Kavirayani DK, Jammulapati S. Fahr's syndrome with psychotic features: Review and Case report. NMJ 2014; 3(2): 71−4.

Kumar S, Sher K, Ahmed S, Naik S, Ayub S, Fatima BM, et al. Fahr's Disease: A Rare Neurological Disease Frequently Mis-diagnosed as Acute Psychosis, or Mood Disorder. J Neurol Disord 2013; 1: 130.

Shoback D. Clinical practice. Hypoparathyroidism. N Engl J Med 2008; 359(4): 391−403.

Goswami R, Sharma R, Sreenivas V, Gupta N, Ganapathy A, Das S. Prevalence and progression of basal ganglia calcification and its pathogenic mechanism in patients with idiopathic hypopar-athyroidism. Clin Endocrinol (Oxf) 2012; 77(2): 200−6.

Mitchell DM, Regan S, Cooley MR, Lauter KB, Vrla MC, Becker CB, et al. Long-term follow-up of patients with hypoparathy-roidism. J Clin Endocrinol Metab 2012; 97(12): 4507−14.

Caliskan T, Gurbuz S. Fahr's Disease. J Contemp Med 2013; 3(2): 133−5.

Manyam BV. What is and what is not 'Fahr's disease'. Parkin-sonism Relat Disord 2005; 11(2): 73−80.

Eaton LM, Camp JD, Lore JG. Symmetric cerebral calcification, particulary of basal ganglia, demonstrable roentgen nograph-ically; calcific ation of the finer cerebral blood ressels. Arch Neurol Psychiatres 1939; 41: 921−42.

Krishnamurthy K, Sonasree J. Fahr’s syndrome with psychotic fea-tures – Review and Case report. NMJ 2014; 3(2): 71−4.

Rastogi R, Singh AK, Rastogi UC, Mohan C, Rastogi V. Fahr's syndrome: A rare clinico-radiologic entity. Med J Armed Forc-es India 2011; 67: 159−61.

Soares FB, Amorima FF, Santana AR, de Moura EB, Margalho SB, Amorim AP, et al. Fahr's Syndrome due to Hypoparathyroid-ism Following Thyroidectomy. J Med Cases 2013; 4(6): 380−4.

Fénelon G, Gray F, Paillard F, Thibierge M, Mahieux F, Guillani A. A prospective study of patients with CT detected pallidal calci-fications. J Neurol Neurosurg Psychiatr 1993; 56(6): 622−5.

Moskowitz MA, Winickoff RN, Heinz ER. Familial calcification of the basal ganglions: A metabolic and genetic study. N Engl J Med 1971; 285(2): 72−7.

Mufaddel AA, Al-Hassani GA. Familial idiopathic basal ganglia calcification (Fahr`s disease). Neurosciences (Riyadh) 2014; 19(3): 171−7.

Ahad MA, Bala CS, Karim SR. Fahr's Syndrome. Bang Med J (Khulna) 2012; 45(1−2): 33−5.

Rizvi I, Ansari NA, Beg M, Shamim MD. Widespread intracranial calcification, seizures and extrapyramidal manifestations in a case of hypoparathyroidism. N Am J Med Sci 2012; 4(8): 369−72.

Chowdhury AW, Majumder SN, Amin MG, Islam KN, Saleh MA, Sabah KM, et al. Recurrent Seizure: An Uncommon Presenta-tion of Post Thyroidectomy Hypoparathyroidism. Bangladesh J Vet Med 2015; 16(1): 56−8.

Zisimopoulou V, Siatouni A, Tsoukalos G, Tavernarakis A, Gatzonis S. Extensive bilateral intracranial calcifications: A case of iat-rogenic hypoparathyroidism. Case Rep Med 2013; 2013: 932184.

Castro MC, Singer BH. Agricultural settlement and soil quality in the Brazilian Amazon. Popul Environ 2012; 34(1): 22−43.

Ghosh K, Sengupta N, Sau TJ, Chatterjee A. Rare presentation of a common disease: Idiopathic hypoparathyroidism presenting with extrapyramidal symptoms and status epilepticus. Indian J Endocr Metab 2012; 16(6): 1035−6.

Panduranga P, Sulaiman K. Is there an association between Fahr's disease and cardiac conduction system disease?: A case report. J Res Med Sci 2012; 17(1): 96−100.

Winer KK, Zhang B, Shrader JA, Peterson D, Smith M, Albert PS, et al. Synthetic human parathyroid hormone 1-34 replacement therapy: a randomized crossover trial comparing pump versus injections in the treatment of chronic hypoparathyroidism. J Clin Endocrinol Metab 2012; 97(2): 391−9.

Winer KK, Cutler GB. Treatment of Hypoparathyroidism with Parathyroid Hormone 1-34. In: Brandi ML, Brown EM, editors. Hypoparathyroidism. Milano: Springer-Verlag Italia; 2015. p. 319−31.