Lee Han Do, Chang Min Cheol
Department of Physical Medicine and Rehabilitation, College of Medicine, Yeungnam University, Namku, Daegu, Republic of Korea.
Medicine (Baltimore). 2017 Dec;96(50):e9195. doi: 10.1097/MD.0000000000009195.
In this case report, we describe a Parkinson's disease (PD) patient with limb-kinetic apraxia (LKA) in whom degeneration of the corticofugal tract (CFT) from the supplementary motor area (SMA) was observed in diffusion tensor tractography (DTT).
A 63-year-old woman presented with a loss of dexterity in both upper extremities, which indicated LKA, and typical PD-related symptoms, including a gait disturbance with a short step, resting tremor in both upper extremities, and rigidity, and these symptoms had been present for 2 years. The F-florinated-N-3-fluoropropyl-2-β-carboxymethoxy-3-β-(4-lodophenyl) nortropane positron emission tomography scanning findings were consistent with PD. Based on the clinical symptoms and imaging findings, we diagnosed the patient with PD. In a coin-rotation test that was used to evaluate the severity of the LKA, the patient's results significantly decreased compared to the results of the normal controls.
The DTT showed that the CFTs from the SMAs in both hemispheres were partially torn and thinned. The fractional anisotropy values and CFT volumes in both SMAs were >2 standard deviations lower than those of the normal controls.
The patient was treated with an initial dose of 150/37.5 mg/day of levodopa/benserazide, and the dose was gradually increased to 400/100 mg/day.
After treatment, although the bradykinesia, rigidity, and resting tremor of the patient significantly decreased, the dexterity of the patient's hands did not improve.
These observations indicated degeneration of the CFTs from the SMAs in both hemispheres in the patient. This degeneration might have, at least in part, contributed to the patient's LKA. The results of this study suggest that CFT degeneration could be one of the pathological mechanisms underlying LKA in patients with PD.
在本病例报告中,我们描述了一名患有肢体运动性失用症(LKA)的帕金森病(PD)患者,在弥散张量纤维束成像(DTT)中观察到其来自辅助运动区(SMA)的皮质传出束(CFT)发生退变。
一名63岁女性出现双上肢灵活性丧失,提示LKA,以及典型的帕金森病相关症状,包括短步幅步态障碍、双上肢静止性震颤和强直,这些症状已持续2年。氟代-N-3-氟丙基-2-β-羧甲氧基-3-β-(4-碘苯基)去甲托烷正电子发射断层扫描结果与帕金森病相符。基于临床症状和影像学检查结果,我们诊断该患者为帕金森病。在用于评估LKA严重程度的硬币旋转试验中,该患者的结果与正常对照组相比显著降低。
DTT显示双侧半球SMA的CFT部分撕裂且变细。双侧SMA的各向异性分数值和CFT体积比正常对照组低2个标准差以上。
患者初始接受左旋多巴/苄丝肼治疗,剂量为150/37.5mg/天,随后逐渐增加至400/100mg/天。
治疗后,患者的运动迟缓、强直和静止性震颤虽显著减轻,但手部灵活性未改善。
这些观察结果表明该患者双侧半球SMA的CFT发生退变。这种退变可能至少部分导致了患者的LKA。本研究结果提示CFT退变可能是帕金森病患者LKA的病理机制之一。