Sakakibara Ryuji, Uchiyama Tomoyuki, Yamanishi Tomonori, Hattori Takamichi
Department of Neurology, Chiba University, Chiba, Japan.
Neurourol Urodyn. 2004;23(2):154-8. doi: 10.1002/nau.20013.
Corticobasal degeneration (CBD) is a rare neurodegenerative disorder affecting cerebral cortex and basal ganglia, both of which are crucial for regulating the lower urinary tract function. However, urinary function of this disorder has not been fully delineated. We investigated urinary function in patients with CBD.
A questionnaire for storage and voiding urinary symptoms was performed in all 10 patients with CBD (four men, six women; mean age, 67.3 years; mean duration of disease, 3.9 years) and 11 age-matched control subjects (four men and seven women; mean age, 73.0 years). Urodynamic studies were performed in six of the patients and all control subjects, including electromyography (EMG)-cystometry and analysis of motor unit potentials of the external sphincter.
As compared to the control subjects (27%), patients with CBD had more common urinary symptoms (80%, P < 0.05). The urinary symptoms appeared 1-3 years after the onset of the disease, and were more common in patients with longer disease duration (>5 years) and in patients with forced grasp reflex. Nocturnal frequency was the most common and tended to be the initial urinary symptom (seven), followed by urinary incontinence (six), urinary urgency (six), diurnal frequency (five), and difficulty in voiding (five). None was in a state of urinary retention. While, one asymptomatic patient showed normal urodynamic finding, all five symptomatic patients showed various abnormalities, including decreased bladder capacity (four), detrusor overactivity (DO) (three), which was noted only in one of the control subjects, detrusor hypocontractility on voiding (three), and low compliance detrusor (one). None of the patients had post-void residuals, detrusor-sphincter dyssynergia, or neurogenic motor unit potentials of the external sphincter muscles.
Although the number of our patients was small, the present study suggests that urinary dysfunction is a common feature in patients with CBD. Since decreased bladder capacity and DO were common in the symptomatic patients, lesions in the supranuclear parasympathetic system are mainly responsible for their dysfunction.
皮质基底节变性(CBD)是一种罕见的神经退行性疾病,会影响大脑皮层和基底神经节,而这两者对于调节下尿路功能都至关重要。然而,这种疾病的泌尿功能尚未完全阐明。我们对CBD患者的泌尿功能进行了研究。
对所有10例CBD患者(4例男性,6例女性;平均年龄67.3岁;平均病程3.9年)和11例年龄匹配的对照者(4例男性和7例女性;平均年龄73.0岁)进行了关于储尿和排尿症状的问卷调查。对6例患者和所有对照者进行了尿动力学研究,包括肌电图(EMG)膀胱测压和外括约肌运动单位电位分析。
与对照者(27%)相比,CBD患者有更常见的泌尿症状(80%,P<0.05)。泌尿症状在疾病发作后1 - 3年出现,在病程较长(>5年)的患者和有强握反射的患者中更常见。夜尿是最常见的,且往往是最初的泌尿症状(7例),其次是尿失禁(6例)、尿急(6例)、日间尿频(5例)和排尿困难(5例)。无尿潴留状态。虽然,1例无症状患者尿动力学检查结果正常,但所有5例有症状患者均表现出各种异常,包括膀胱容量减少(4例)、逼尿肌过度活动(DO)(3例),仅在1例对照者中发现,排尿时逼尿肌收缩力减弱(3例),以及低顺应性逼尿肌(1例)。患者均无残余尿量、逼尿肌 - 括约肌协同失调或外括约肌神经源性运动单位电位。
尽管我们的患者数量较少,但本研究表明泌尿功能障碍是CBD患者的常见特征。由于膀胱容量减少和DO在有症状患者中常见,核上副交感神经系统的病变主要导致其功能障碍。