Abstract

Idiopathic basal ganglia calcification, also known as “Fahr disease”, is a rare, genetically dominant, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement.

Case Presentation: We report a case of 58 years old male patient presented with neuropsychiatric, behavioural and cognitive impairements with movement disorder. Non contrast CT scan of brain was suggestive of “Fahr’s Disease or Syndrome”.

Conclusion: Fahr’s disease is a rare neurodegenerative disorder with characteristic bilateral symmetrical calcifications in brain especially in basal ganglia, The disease should be considered in the differential diagnosis in patients with psychiatric symptoms. Non contrast CT scan may help in early diagnosis

Keywords: Fahr’s disease, CT scan, Cognitive impairment, Tremors.

1.      Manyam BV, Walters AS, Narla KR. Bilateral Striopallidodentate Calcinosis: Clinical Characteristics of Patients Seen in A Registry. Movement Disorders: Official Journal of the Movement Disorder Society.2001;16(2):258–264.

2.      Fahr T. Idiopathische Verkalkung Der Hirngefässe. Zentrabl Allg Pathol.  1930; 50:129–133.

3.      Ahad MA, Bala C, Karim S. Fahr’s syndrome. Bangladesh Medical Journal Khulna. 2013;45(1–2):33–35.

4.      Manyam BV, Bhatt MH, Moore WD, et al. Bilateral Striopalliododentate Calcinosis: Cerebrospinal Fluid, Imaging and Elctrop-hysiological Studies. Ann Neural. 1992; 31:379–384.

5.      Billard C, Dulac O, Boulouche J, et al. Encephalopathy with Calcifications of Basal Ganglia in Children: A Reappraisal of Fahr’sDisease with Respect to 14 New Cases. Neuropediatrics. 1989;20:12–19.

6.      Chiu HF, Lam LC, Shum PP, Li KW (January 1993). "Idiopathic calcification of the Basal Ganglia". Postgrad Med J. 69 (807): 68–70. 

7.      Shakibai SV, Johnson JP, Bourgeois JA. Paranoid Delusions and Cognitive Impai-rment Suggesting Fahr's Disease.  Psychosomatics. 2005; 46 : 569–572.

8.      Shoyama M, Kitabata Y, Kaku T, Shinosaki K. Evaluation of Regional Cerebral Blood Flow in Fahr Disease with Schizophrenia-Like Psychosis: A Case Report. AJNR Am J euroradiol.  2005; 26:2527–2529.

9.      Shibayama H, Kobayashi H, Nakagawa M, Yamada K, Iwata H, Iwai K, et al. Non-Alzheimer non-Pick dementia with Fahr's Syndrome. ClinNeuropathol. 1992;11:237–250.

10.  Lam JS, Fong SY, Yiu GC, Wing YK. Fahr's Disease: A Differential Diagnosis of Frontal Lobe Syndrome. Hong Kong Med J. 2007;13:75–77.

11.  Geschwind DH, Loginov M, Stern JM (September 1999). "Identification of a Locus on Chromosome 14q For Idiopathic Basal Ganglia Calcification (Fahr Disea-se)".  Am. J. Hum. Genet. 65 (3): 764–72.

12.  Dai X, Gao Y, Xu Z, et al. (October 2010). "Identification of a Novel Genetic Locus on Chromosome 8p21.1-Q11.23 For Idiopathic Basal Ganglia Calcification".  Am. J. Med. Genet. B Neuropsychiatr. Genet. 153B (7): 1305–10.

13.  Volpato CB, De Grandi A, Buffone E, et al. (November 2009). "2q37 as a Susceptibility Locus for Idiopathic Basal Ganglia Calcification (IBGC) in a Large South Tyrolean family". J. Mol. Neurosci. 39 (3): 346–53.

14.  Oliveira JR, Spiteri E, Sobrido MJ, et al. (December 2004). "Genetic Heterogeneity in Familial Idiopathic Basal Ganglia Calcification (Fahr disease)".  Neurology. 63 (11): 2165–7.

15.  Wang C, Li Y, Shi L, et al. (March 2012). "Mutations in SLC20A2 Link Familial Idiopathic Basal Ganglia Calcification with Phosphate Homeostasis". Nat. Genet. 44 (3): 254–6. 

16.  Bonazza S, La Morgia C, Martinelli P, Capellari S (Aug 2011). "Strio-pallido-dentate calcinosis: a Diagnostic Approach in Adult Patients". Neurol. Sci. 32 (4): 537–45. 

17.  Morita M, Tsuge I, Matsuoka H, et al. (May 1998). "Calcification in the Basal Ganglia with Chronic Active Epstein-Barr Virus Infection". Neurology. 50(5):1485-8.

18.  Lippmann S. Emergency brain imaging: CT or MRI? Current Psychiatry.  2013; 12(3):47.

19.  Munir KM (February 1986). "The Treatment of Psychotic Symptoms in Fahr's Disease with Lithium Carbonate". J Clin Psychopharmacol. 6 (1): 36–8.

20.  Loeb JA (March 1998). "Functional Improvement in A Patient with Cerebral Calcinosis Using a Bisphosphonate". Mov. Disord. 13 (2): 345–9. 

21.  Hossain M. Neurological and Psychiatric Manifestations in Idiopathic Hypoparath-yroidism: Response to Treatment. J Neurol Neurosurg Psychiatry. 1970;33:153–157.

22.  Oliviera JR, Spiteri E, Sobrido MJ, et al. Genetic Heterogeneity in Familial Idiopathic Basal Ganglia Calcification (Fahr’s Disease) Neurology. 2004;63:2165–2167.

23.  Baba Y, Broderick DF, Uitti RJ, et al. Heredofamilial Brain Calcinosis Syndro-me. Mayo Clinic Proc.2005;80: 251–256.