대한치매학회지 (Dementia and Neurocognitive Disorders)

대한치매학회 (Korean Dementia Association)
권호 표지

경도인지장애와 운동과다증을 동반한 Fahr 환자 1예

A Case of Fahr Disease with Mild Cognitive Impairment and Hyperkinesia

  • 윤영신 (이화여자대학교 의과대학 부속목동병원 신경과학교실) ;
  • 강희진 (이화여자대학교 의과대학 부속목동병원 신경과학교실) ;
  • 김정은 (이화여자대학교 의과대학 부속목동병원 신경과학교실) ;
  • 김범산 (이화여자대학교 의과대학 핵의학과) ;
  • 박기덕 (이화여자대학교 의과대학 부속목동병원 신경과학교실) ;
  • 최경규 (이화여자대학교 의과대학 부속목동병원 신경과학교실) ;
  • 정지향 (이화여자대학교 의과대학 부속목동병원 신경과학교실)
  • Yoon, Young-shin (Department of Neurology, Cognitive and Neurodegenerative Disorder Clinic, Ewha Womans University Medical Center, Ewha Womans University School of Medicine) ;
  • Kang, Hee-Jin (Department of Neurology, Cognitive and Neurodegenerative Disorder Clinic, Ewha Womans University Medical Center, Ewha Womans University School of Medicine) ;
  • Kim, Jung-Eun (Department of Neurology, Cognitive and Neurodegenerative Disorder Clinic, Ewha Womans University Medical Center, Ewha Womans University School of Medicine) ;
  • Kim, Bum-Sahn (Department of Nuclear Medicine, Cognitive and Neurodegenerative Disorder Clinic, Ewha Womans University Medical Center, Ewha Womans University School of Medicine) ;
  • Park, Kee-Duk (Department of Neurology, Cognitive and Neurodegenerative Disorder Clinic, Ewha Womans University Medical Center, Ewha Womans University School of Medicine) ;
  • Choi, Kyoung-Gyu (Department of Neurology, Cognitive and Neurodegenerative Disorder Clinic, Ewha Womans University Medical Center, Ewha Womans University School of Medicine) ;
  • Jeong, Jee-H. (Department of Neurology, Cognitive and Neurodegenerative Disorder Clinic, Ewha Womans University Medical Center, Ewha Womans University School of Medicine)
  • 발행 : 2010.06.30
⟨ 이전 논문 다음 논문 ⟩

초록

A 66-yr-old woman presented with progressive cognitive decline and mild hyperkinetic motor symptoms. Neuropsychological evaluation revealed impairment of memory and frontal executive functions with normal activities of daily living, indicating a mild cognitive impairment. Brain imaging showed extensive calcification involving bilateral basal ganglia, thalamus, occipital cortex and cerebellum consistent with Fahr disease. FDG-PET showed diffuse glucose hypometabolism in bilateral fronto-temporo-parietal cortices, suggesting the disruption of frontal subcortical circuit to be probably associated with the cognitive impairment of the patient.

키워드

참고문헌

  1. Malik R, Pandya VK, Nalk D. Fahr disease? A rare neurodegenerative disorder. Ind J Radiol Imag 2004; 4: 383-4.
  2. Manyam BV. What is and what is not "Fahr's disease". Parkinsonism Relat Disord 2005; 11: 73-80.
  3. Moderego PJ, Mojonero J, Serrano M, Fayed N. Fahr's syndrome presenting with pure and progressive presenile dementia. Neurol Sci 2005; 26: 367-9. https://doi.org/10.1007/s10072-005-0493-7
  4. Heo JH, Choi YJ, Lee KM. A case of Fahr's disease presenting with frontal lobe dysfunction. J Korean Neurol Assoc 2007; 4: 549-52.
  5. Shin DA, Gong TS, Shin DG, Kim HI. Familial Idiopathic Basal Ganglia Calcification. J Korean Neurosurg Soc 2006; 40: 196-8.
  6. Cho CS, Park JH, Song KY, Hahn YK, Kim CH, Ha YI. Fahr's disease (=Idiopathic Strido-Pallido-Dentate Calcinosis): a case report. J Korean Neurosurg Soc 1995; 24: 835-40.
  7. Brodaty H, Mitchell P. Familial idiopathic basal ganglia calcification (Fahr's disease) without neurological, cognitive and psychiatric symptoms is not linked to the IBGC1 locus on chromosome 14q. Hum Genet 2002; 110: 8-14. https://doi.org/10.1007/s00439-001-0650-x
  8. Benke T, Kamer E. Subacute dementia and imaging correlates in a case of Fahr's disease. J Neurol Neurosurg Psychiatry 2004; 75: 1163-5. https://doi.org/10.1136/jnnp.2003.019547
  9. Kang Y, Na DL, Hahn S. A validity study on the Korean mini-mental state examination (K-MMSE) in dementia patients. J Korean Neurol Assoc 1997; 5: 300-8.
  10. Choi SH, Na DL, Lee BH, Mahm DS, Jeong JH, Yoon ST, et al. Estimating the validity of the Korean version of Expanded Clinical Dementia Rating (CDR) scale. J Korean Neurol Assoc 2002; 20: 8-14.
  11. Jung IK, Kwak DI, Shin KD, Lee MS, Lee HS, Kim JY. A reliability and validity of the Korean depression scale. J Korean Neuro Psychiatr Assoc 1997; 36: 103-12.
  12. Choi SH. Introduction of dementia questionnaire, activity of daily living (ADL), neuropsychiatric inventory. J Korean Neurol Assoc 1999; 2: 14-20.
  13. Choi SH, Na DL, Kwon HM, Yoon SJ, Jeong JH, Ha CK. The Korean version of the neuropsychiatri inventory: a scoring tool for neuropsychiatric disturbance in dementia patients. J Korean Med Sci 2000; 15: 309-15. https://doi.org/10.3346/jkms.2000.15.3.309
  14. Gulay A, Uygur, Yu Liu, Robert S. Hellman. Evaluation of regional cerebral blood flow in massive intracerebral clacifications. J Nucl Med 1995; 36: 610-2.
  15. Andreia V, Faria, Ines C. Pareira. Computerized tomography findings in Fahr's syndrome. Arq Neuropsiquiatr 2004; 62: 789-92. https://doi.org/10.1590/S0004-282X2004000500009
  16. Jung HW, Kim IO, Kim WS, Hwang WS, Yeon KM. Intracranial Tuberculosis in Children: CT Findings before and after Treatment. J Korean Radiol Soc 1996; 35: 273-8. https://doi.org/10.3348/jkrs.1996.35.2.273
  17. Hempel A, Henze M, Berghoff C, Garcia N, Ody R, Schroder J. PET findings and neuropsychological deficits in a case of Fahr's disease. Psychiatry Res 2001; 108: 133-40. https://doi.org/10.1016/S0165-1781(01)00308-0
  18. Benke T, Delazer M, Bartha L, Auer A. Basal ganglia lesions and the theory of fronto-subcortical loops: neuropsychological findings in two patients with left caudate lesions. Neurocase 2003; 1: 70-85.
  19. FA Middletone, PC Strick. The temporal lobe is a target of output from the basal ganglia. Proc Natl Acad Sci U S A 1996; 93: 8683-7. https://doi.org/10.1073/pnas.93.16.8683