시냅스전 기능장애를 보인 반신파킨슨증-반신위축
Hemiparkisonism-Hemiatrophy with Presynaptic Dysfunction
Article information
J Korean Neurol Assoc. 2016;34(4):415-416
Department of Neurology, Veterans Health Service Medical Center, Seoul, Korea
aDepartment of Nuclear Medicine, Veterans Health Service Medical Center, Seoul, Korea
Address for correspondence: Sang Won Ha, MD Department of Neurology, Veterans Health Service Medical Center, 53 Jinhwangdo-ro, Gangdong-gu, Seoul 05368, Korea Tel: +82-2-2225-1324 Fax: +82-2-2225-1327 E-mail:
hippocam@naver.com
received : May 25, 2016 , rev-recd : June 17, 2016 , accepted : June 17, 2016 .
66세 남성이 30년 전에 발생한 좌측 안정시떨림으로 내원하였다. 좌측 상하지에 운동완만, 경미한 근긴장이상과 보행장애가 있었다. 좌측 팔꿈치와 종아리 둘레가 오른쪽보다 작았고, 뇌MRI에서 좌측 측뇌실확장과 대뇌반구 위축이 보여 반신파킨슨증-반신위축(hemiparkinsonism-hemiatrophy, HPHA)으로 진단하였다.
F-18 fluoropropylcarbomethoxyiodophenylnortropane (FP-CIT)-PET에서는 우측 기저핵에 CIT 섭취 감소가 뚜렷하였으나 F-18-fluoropropylcarbomethoxyiodophenylnortropane (FDG)-PET에서 양측 기저핵의 당 대사는 정상으로, 이는 반신파킨슨증-반신위축에서 드문 결과이다. 레보도파 투여 후 파킨슨 증상은 호전되었고, 외래에서 3년 동안 안정적으로 추적･관찰 중이다().
The patient had atrophy on his left upper and lower extremities. The arm circumference measured at 10 cm below the elbow joint is 25.5 cm in right side, 23 cm in left side. (A, B) The circumference of leg was 40.5 cm in right, 36 cm in left at 10 cm below knee joint. (C, D) Brain MRI showed mild atrophy on the left hemisphere and on the left lateral ventricular enlargement. (E, F) F-18-FP-CIT PET CT displayed asymmetrically decreased FP-CIT uptake in right caudate nucleus and right putamen. (G, H) F-18-fluorodeoxyglucose PET showed normal regional glucose metabolism in bilateral basal ganglia. MRI; magnetic resonance imaging, FP-CIT; fluoropropylcarbomethoxyiodophenylnortropane, PET CT; positron-emitting tomography computed tomography.
기존 PET연구에서 반신파킨슨증-반신위축 환자의 일부는 도파민수용체영상에 섭취 감소가 나타났고 대부분 환자가 FDG-PET에서 반대측 기저핵에 당대사가 저하되었다. 레보도파에 대한 반응도 다양하여 HPHA는 흑질줄무늬체계(nigrostiatal system)의 시냅스 전과 후의 장애가 다양하게 섞여있는 이질적인 질환군으로 알려져 있다[1,2]. 본 증례는 시냅스전 장애(presynaptic dysfunction)를 보인 드문 반신파킨슨증-반신위축이다.
References
1. Przedborski S, Giladi N, Takikawa S, Ishikawa T, Dhawan V, Spetsieris P, et al. Metabolic topography of the hemiparkinsonism-hemiatrophy syndrome. Neurology 1994;44:1622–1628.
2. Przedborski S, Goldman S, Levivier M, Giladi N, Bidaut LM, Hildebrand J, et al. Brain glucose metabolism and dopamine D2 receptor analysis in a patient with hemiparkinsonism-hemiatrophy syndrome. Mov Disord 1993;8:391–395.
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Copyright © 2016 Korean Neurological Association
Figure.
The patient had atrophy on his left upper and lower extremities. The arm circumference measured at 10 cm below the elbow joint is 25.5 cm in right side, 23 cm in left side. (A, B) The circumference of leg was 40.5 cm in right, 36 cm in left at 10 cm below knee joint. (C, D) Brain MRI showed mild atrophy on the left hemisphere and on the left lateral ventricular enlargement. (E, F) F-18-FP-CIT PET CT displayed asymmetrically decreased FP-CIT uptake in right caudate nucleus and right putamen. (G, H) F-18-fluorodeoxyglucose PET showed normal regional glucose metabolism in bilateral basal ganglia. MRI; magnetic resonance imaging, FP-CIT; fluoropropylcarbomethoxyiodophenylnortropane, PET CT; positron-emitting tomography computed tomography.
