BibTex RIS Cite

Fahr Sendromu: Acil Serviste İdiyopatik Hipoparatiroidili Bir Hasta

Year 2013, Volume: 4 Issue: 2, 1 - 3, 01.04.2013

Abstract

Acil servise nonspesifik nörolojik semptomlarla başvuran hastalarda
oldukça geniş bir nörolojik ve metabolik bir ön tanı yelpazesi
mevcuttur. Burada servisimize epizodik periferik parestezik şikayetlerle
acil servise başvuran ve bilateral striyatopallidodental kalsifikasyonla
karakterize nadir bir klinik durum olan Fahr Sendromu
tanısıyla sonlanan bir hastayı sunmayı amaçladık. Yetmiş sekiz yaşında
bayan hasta acil servise sol kol ve bacakta uyuşma ve bir gün
öncesinde 4 kez ataklar şeklinde olan yüzde uyuşma şikayetiyle
başvurdu. Nörolojik muayenesinde lateral motor defisiti olmayan
hastanın Chvostek testi (+) bulundu. Diğer sistem muayenelerinde
ek herhangi bir patoloji saptanmadı. Serum biyokimyasında Ca++
düşüklüğü (5.8 mg/dL) tespit edilen hastanın nörolojik şikayetlerinin
ayırıcı tanısı için çekilen beyin BT’sinde bazal ganglion ve serebellumda
bilateral kalsifikasyonlar saptandı. Nöroloji ve Endokrinoloji
bölümleri ile konsulte edilen hastada mevcut bulgularla Fahr
Sendromu düşünüldü. Nonspesifik akut nöropsikiyatrik şikayetlerle
acil servise başvuran hastalarda, özellikle Ca++ metabolizması ile
ilgili patoloji varlığında Fahr sendromu da akla getirilmeli ve ileri
tetkikler bu yönde planlanmalıdır.

References

  • Açıkgöz NP, Kamışlı Ö, Altınayar S, Özcan C, Fahr Sendromu Olguları. Par- kinson Hast. Hareket Boz. Der 2007; 10: 41-5.
  • Harrington MG, Macpherson P, McIntosh WB, Alam BF, Bone I. The signifi- cance of the incidental finding of basal ganglia calcification on computed tomography. J Neurol Neurosurg Psychiatry 1981; 44: 1168-70. [Crossref]
  • Fenelon G, Gray F, Paillard F, Thibierge M, Mahieux F, Guillani A. A pro- spective study of patients with CT detected pallidal calcifications. J Neurol Neurosur Psychiatry 1993; 56: 622-5. [Crossref]
  • Kökeş Ü, Hız F, Özden DL, Çınar M. Psödohipoparatiroidinin yol açtığı bir Fahr sendromu olgusu. Parkinson Hast Hareket Boz Der 2009; 12: 18-24.
  • Windeck R, Menken U, Benker G, Reinwein D. Basal ganglia calcification in pseudohypoparathyroidism type II. Clin Endocrinol 1981; 15: 57-63. [Crossref]
  • Beall SS, Patent BM. Abnormal systemic metabolism of iron, porphyrin and calcium in Fahr”s syndrome. Ann Neurol 1989; 26: 569-75. [Crossref]
  • Manyam BV. What is and what is not ‘Fahr’s disease’. Parkinsonism Relat Disord 2005; 11: 73-80. [Crossref]
  • Hanağası H, Hanağası F, Bilgiç B, Gürvit H, Yazıcı J, Emre M. Bilateral strio- pallidodentat kalsinosis’in klinik spektrumu. Türk Nöroloji Dergisi 2004; 5: 430-7.
  • Ramonet D, de Yebra L. Similar calcification process in acute and chron- ic human brain pathologies. J Neurosci Res 2006; 83: 147-56. [Crossref]
  • Morgante L, Vita G. Di Perri R. Fahr’s syndrome: local inflammatory factors in the pathogenesis of calcification. J Neurol 1986; 233: 19-22. [Crossref]
  • Karimi M, Habibzadeh F. Hypoparathyroidism with extensive intracere- bral calcification in patients with beta-thalassemia major. J Pediatr En- docrinol Metab 2003; 16: 883-6. [Crossref]
  • Paprocka J, Jamroz E. Neurological picture and 1H MRS in 4 children with hypoparathyroidism. Przegl Lek 2005; 62: 680-4.

Fahr’s Syndrome: A Patient with Idiopathic Hypoparathyroidy in the Emergency Department

Year 2013, Volume: 4 Issue: 2, 1 - 3, 01.04.2013

Abstract

Patients attending emergency departments (EDs) with nonspecific neurological symptoms have a wide range of differential diagnoses. In this case report, we present a female patient with episodic peripheral paresthaesia, who ended up with a diagnosis of Fahr’s syndrome, a rare pathology characterised by bilateral striatopallydodental calcifications. A 78 year old female presented to our ED with numbness in the left arm and leg, and a history of four episodes of facial numbness which occurred during the previous day. Physical examination of the patient revealed no lateralising neurological deficits, but Chvostek’s sign was (+).The only notable pathology of the patient arising from routine lab work was the Ca level of Ca++ (5.8 mg/dL). A computed tomography scan of the brain showed bilateral calcifications in the basal ganglia and cerebellum. The patient was examined by a neurology and endocrinology consultant in the ED and hospitalised for hypocalcaemia replacement and further diagnostic endocrinology investigations related to Fahr’s syndrome. In patients with acute nonspecific neuropsychiatric symptoms, especially in the case of Ca++ metabolism disorders, Fahr’s syndrome should be a part of differential diagnosis, and diagnostic investigations performed in the ED should include this syndrome

References

  • Açıkgöz NP, Kamışlı Ö, Altınayar S, Özcan C, Fahr Sendromu Olguları. Par- kinson Hast. Hareket Boz. Der 2007; 10: 41-5.
  • Harrington MG, Macpherson P, McIntosh WB, Alam BF, Bone I. The signifi- cance of the incidental finding of basal ganglia calcification on computed tomography. J Neurol Neurosurg Psychiatry 1981; 44: 1168-70. [Crossref]
  • Fenelon G, Gray F, Paillard F, Thibierge M, Mahieux F, Guillani A. A pro- spective study of patients with CT detected pallidal calcifications. J Neurol Neurosur Psychiatry 1993; 56: 622-5. [Crossref]
  • Kökeş Ü, Hız F, Özden DL, Çınar M. Psödohipoparatiroidinin yol açtığı bir Fahr sendromu olgusu. Parkinson Hast Hareket Boz Der 2009; 12: 18-24.
  • Windeck R, Menken U, Benker G, Reinwein D. Basal ganglia calcification in pseudohypoparathyroidism type II. Clin Endocrinol 1981; 15: 57-63. [Crossref]
  • Beall SS, Patent BM. Abnormal systemic metabolism of iron, porphyrin and calcium in Fahr”s syndrome. Ann Neurol 1989; 26: 569-75. [Crossref]
  • Manyam BV. What is and what is not ‘Fahr’s disease’. Parkinsonism Relat Disord 2005; 11: 73-80. [Crossref]
  • Hanağası H, Hanağası F, Bilgiç B, Gürvit H, Yazıcı J, Emre M. Bilateral strio- pallidodentat kalsinosis’in klinik spektrumu. Türk Nöroloji Dergisi 2004; 5: 430-7.
  • Ramonet D, de Yebra L. Similar calcification process in acute and chron- ic human brain pathologies. J Neurosci Res 2006; 83: 147-56. [Crossref]
  • Morgante L, Vita G. Di Perri R. Fahr’s syndrome: local inflammatory factors in the pathogenesis of calcification. J Neurol 1986; 233: 19-22. [Crossref]
  • Karimi M, Habibzadeh F. Hypoparathyroidism with extensive intracere- bral calcification in patients with beta-thalassemia major. J Pediatr En- docrinol Metab 2003; 16: 883-6. [Crossref]
  • Paprocka J, Jamroz E. Neurological picture and 1H MRS in 4 children with hypoparathyroidism. Przegl Lek 2005; 62: 680-4.
There are 12 citations in total.

Details

Other ID JA43BR69VC
Journal Section Case Report
Authors

Cem Ertan This is me

Esma Karaman This is me

Hakan Oğuztürk This is me

Dilek Ertan This is me

Publication Date April 1, 2013
Submission Date April 1, 2013
Published in Issue Year 2013 Volume: 4 Issue: 2

Cite

APA Ertan, C., Karaman, E., Oğuztürk, H., Ertan, D. (2013). Fahr’s Syndrome: A Patient with Idiopathic Hypoparathyroidy in the Emergency Department. Journal of Emergency Medicine Case Reports, 4(2), 1-3.
AMA Ertan C, Karaman E, Oğuztürk H, Ertan D. Fahr’s Syndrome: A Patient with Idiopathic Hypoparathyroidy in the Emergency Department. Journal of Emergency Medicine Case Reports. April 2013;4(2):1-3.
Chicago Ertan, Cem, Esma Karaman, Hakan Oğuztürk, and Dilek Ertan. “Fahr’s Syndrome: A Patient With Idiopathic Hypoparathyroidy in the Emergency Department”. Journal of Emergency Medicine Case Reports 4, no. 2 (April 2013): 1-3.
EndNote Ertan C, Karaman E, Oğuztürk H, Ertan D (April 1, 2013) Fahr’s Syndrome: A Patient with Idiopathic Hypoparathyroidy in the Emergency Department. Journal of Emergency Medicine Case Reports 4 2 1–3.
IEEE C. Ertan, E. Karaman, H. Oğuztürk, and D. Ertan, “Fahr’s Syndrome: A Patient with Idiopathic Hypoparathyroidy in the Emergency Department”, Journal of Emergency Medicine Case Reports, vol. 4, no. 2, pp. 1–3, 2013.
ISNAD Ertan, Cem et al. “Fahr’s Syndrome: A Patient With Idiopathic Hypoparathyroidy in the Emergency Department”. Journal of Emergency Medicine Case Reports 4/2 (April 2013), 1-3.
JAMA Ertan C, Karaman E, Oğuztürk H, Ertan D. Fahr’s Syndrome: A Patient with Idiopathic Hypoparathyroidy in the Emergency Department. Journal of Emergency Medicine Case Reports. 2013;4:1–3.
MLA Ertan, Cem et al. “Fahr’s Syndrome: A Patient With Idiopathic Hypoparathyroidy in the Emergency Department”. Journal of Emergency Medicine Case Reports, vol. 4, no. 2, 2013, pp. 1-3.
Vancouver Ertan C, Karaman E, Oğuztürk H, Ertan D. Fahr’s Syndrome: A Patient with Idiopathic Hypoparathyroidy in the Emergency Department. Journal of Emergency Medicine Case Reports. 2013;4(2):1-3.