Joo Eun Yeon, Hong Seung Bong, Tae Woo Suk, Kim Jee Hyun, Han Sun Jung, Seo Dae Won, Lee Kyung-Han, Kim Myoung-Hee, Kim Seunghwan, Lee Mann Hyung, Kim Byung Tae
Department of Neurology, Samsung Medical Center & Center for Clinical Medicine, SBRI, Sungkyunkwan University School of Medicine, Seoul, Korea.
Eur J Nucl Med Mol Imaging. 2005 Oct;32(10):1205-9. doi: 10.1007/s00259-005-1814-z. Epub 2005 Jun 10.
Previous cerebral blood flow and glucose metabolism studies suggest that the basal ganglia or thalamus is involved in the pathogenesis of paroxysmal kinesigenic choreoathetosis (PKC). However, the underlying cerebral abnormalities in idiopathic PKC have not been elucidated. To localise cerebral perfusion abnormalities in PKC, we performed interictal brain perfusion 99mTc-ethylcysteinate dimer (ECD) single-photon emission computed tomography (SPECT) in PKC patients and in normal controls.
Sixteen patients with idiopathic PKC and 18 age- and sex-matched normal controls were included. The patients were de novo diagnosed as having PKC, or had not taken medication for at least 3 months; none of them had structural abnormalities on MRI. Patients had a history of PKC attacks of a duration not exceeding 5 min and starting either on one side or on both sides of the body. These attacks were always induced by a sudden initiation of voluntary movement. PKC attacks were recorded in a hospital after being induced by neurology staff in 13 of the 16 patients. Interictal brain perfusion 99mTc-ECD SPECT was performed in all 16 patients and 18 normal controls. Differences between the cerebral perfusion in the PKC group and the normal control group were tested by statistical parametric mapping. Student's t test was used for inter-group comparisons.
Compared with normal controls, patients with idiopathic PKC showed interictal hypoperfusion in the posterior regions of the bilateral caudate nuclei (false discovery rate-corrected P<0.001 with a small volume correction).
This study showed that cerebral perfusion abnormality of bilateral caudate nuclei is present in idiopathic PKC.
以往的脑血流量和葡萄糖代谢研究表明，基底神经节或丘脑参与阵发性运动诱发性舞蹈手足徐动症（PKC）的发病机制。然而，特发性PKC潜在的脑异常尚未阐明。为了定位PKC患者的脑灌注异常，我们对PKC患者和正常对照者进行了发作间期脑灌注99mTc-乙半胱氨酸二聚体（ECD）单光子发射计算机断层扫描（SPECT）。
纳入16例特发性PKC患者和18例年龄及性别匹配的正常对照者。患者被初诊为PKC，或至少3个月未服药；他们均无MRI结构异常。患者有PKC发作史，发作持续时间不超过5分钟，始于身体一侧或双侧。这些发作总是由突然开始的自主运动诱发。16例患者中有13例在神经内科工作人员诱发后在医院记录了PKC发作。对所有16例患者和18例正常对照者进行了发作间期脑灌注99mTc-ECD SPECT检查。采用统计参数映射法检测PKC组和正常对照组脑灌注的差异。组间比较采用Student t检验。
与正常对照相比，特发性PKC患者双侧尾状核后部在发作间期出现灌注减低（经小体积校正的错误发现率校正P<0.001）。
本研究表明，特发性PKC患者存在双侧尾状核脑灌注异常。
