Risk assessment of multi-factorial complications after transrectal ultrasound-guided prostate biopsy: a single institutional retrospective cohort study.

BACKGROUND: Transrectal ultrasound-guided prostate biopsy (TRUSPB) is widely used to diagnose prostate cancer (PCa). The aim of this study was to evaluate the risk of multi-factorial complications (febrile genitourinary tract infection (GUTI), rectal bleeding, and urinary retention) after TRUSPB. METHODS: N = 2053 patients were Japanese patients undergoing transrectal or transperineal TRUSPB for suspicious of PCa. To assess risk of febrile GUTI adequately, the patients were divided into four groups: low-risk patients before starting a rectal culture, low-risk patients after starting a rectal culture, high-risk patients, and patients undergoing transperineal TRUSPB. Furthermore, to identify risk of rectal bleeding and urinary retention, patients were divided into transrectal and transperineal group. RESULTS: Febrile GUTI significantly decreased owing to risk classification. The frequency of rectal bleeding was 1.43% (transrectal: 25/1742), while it did not happen in transperineal group. The patients with rectal bleeding had a significantly lower body mass index (BMI) (P < 0.01). The frequency of urinary retention was 5.57% (transrectal: 97/1742), while it did not happen in transperineal group. The patients with urinary retention had a significantly higher prostate-specific antigen (PSA) (P = 0.01) in transrectal group. CONCLUSIONS: Risk classification, rectal swab culture, and selected antimicrobial prophylaxis for transrectal TRUSPB were extremely effective to reduce the risk of febrile GUTI. Furthermore, lower BMI and higher PSA were novel clinical predictors for rectal bleeding and urinary retention, respectively. When urologists perform transrectal TRUSPB to their patients, they can correctly understand and explain each complication risk to their patients based on these novel risk factors.

Triple-disease etiology is common for LUTS in octogenarians: a neuro-urological approach.

OBJECTIVE: Older individuals often have multiple etiologies for their lower urinary tract symptoms (LUTS); i.e., both urologic (U) and neurologic (N) etiologies. Few studies have investigated 'triple disease' (typically one U and two N components) in the LUTS of older adults. Herein, we had specialists from both urology and neurology address triple- and quadruple-etiology disease. PATIENTS AND METHODS: This was a retrospective study with a 12-month recruiting period. We ascertained LUTS by standard questionnaires and bladder diaries. Urodynamics, sphincter EMG, prostate echography, and a neurologic examination were conducted for each patient as well as neuroimaging and neurophysiology examinations when appropriate. The diagnoses of the etiologies were based on published criteria. RESULTS: We analyzed the cases of 141 older (age > 65 years) adults with LUTS referred from both urology (27%) and neurology departments (73%). The final etiologies were U (n = 69, 49%), N (n = 136, 96%), and a combination (U and N) (n = 77, 55%, overlap counted). The majority of U diagnoses were benign prostatic hyperplasia. The majority of N diagnoses were dementia with Lewy bodies, white matter disease (brain); lumbar spondylosis, and diabetes (peripheral disease). We noted triple-disease etiology in 25% (n = 35), increasing with each decade of age (18.2% of sexagenarians, 23.5% of septuagenarians, 39.1% of octogenarians). However, the differences were not significant. CONCLUSION: Our results demonstrate that triple disease for LUTS is the most common in octogenarians, and clinicians thus need to untangle LUTS etiologies to provide appropriate care and management of older adults.

Impact of Lymphovascular Invasion on Prognosis in the Patients with Bladder Cancer-Comparison of Transurethral Resection and Radical Cystectomy.

(1) Background: This study aimed to evaluate the associations of lymphovascular invasion (LVI) at first transurethral resection of bladder (TURBT) and radical cystectomy (RC) with survival outcomes, and to evaluate the concordance between LVI at first TURBT and RC. (2) Methods: We analyzed 216 patients who underwent first TURBT and 64 patients who underwent RC at Toho University Sakura Medical Center. (3) Results: LVI was identified in 22.7% of patients who underwent first TURBT, and in 32.8% of patients who underwent RC. Univariate analysis identified ≥cT3, metastasis and LVI at first TURBT as factors significantly associated with overall survival (OS) and cancer-specific survival (CSS). Multivariate analysis identified metastasis (hazard ratio (HR) 6.560, p = 0.009) and LVI at first TURBT (HR 9.205, p = 0.003) as significant predictors of CSS. On the other hand, in patients who underwent RC, ≥pT3, presence of G3 and LVI was significantly associated with OS and CSS in univariate analysis. Multivariate analysis identified inclusion of G3 as a significant predictor of OS and CSS. The concordance rate between LVI at first TURBT and RC was 48.0%. Patients with positive results for LVI at first TURBT and RC displayed poorer prognosis than other patients (p < 0.05). (4) Conclusions: We found that the combination of LVI at first TURBT and RC was likely to provide a more significant prognostic factor.

Female detrusor underactivity: Two uterine leiomyoma cases.

INTRODUCTION: Female urinary retention is rare. CASE PRESENTATION: Case 1, a 35-year-old nulliparous woman, and case 2, a 47-year-old nulliparous woman, had transient urinary retention. A urodynamics revealed increased bladder sensation in case 1 and detrusor underactivity with a large post-void residual in cases 1 and 2. Both women had a uterine leiomyoma of >10 cm in diameter. Soon after extraction of the tumor, retention episodes disappeared completely in case 1. CONCLUSION: Although rare, uterine leiomyoma should be listed as a cause of female detrusor underactivity.

[SIGNIFICANCE OF IgG4 IN IDIOPATHIC RETROPERITONEAL FIBROSIS].

(Objective)Retroperitoneal fibrosis is largely divided into the idiopathic and secondary types. Some idiopathic cases include IgG4-related diseases, which are often similar to malignant diseases, such as lymphoma and sarcoma. The diagnostic criteria for IgG4-related disease are used and pathologic examination is necessary for a definitive diagnosis of IgG4-related retroperitoneal fibrosis. The first choice of treatment for IgG4-related retroperitoneal fibrosis is steroid administration, but no consensus has been established regarding its dose and tapering schedule. We investigated the significance of IgG4 in diagnosis and treatment of idiopathic retroperitoneal fibrosis. (Patients and methods)We examined 14 cases diagnosed as idiopathic retroperitoneal fibrosis between April 2013 and March 2019. Serum IgG4 was measured at the time of diagnosis in 13 cases, and changes over time in serum IgG4 before and after the induction of steroid therapy were measured in 6 cases. Computed tomography-guided biopsy was performed on 4 cases. (Results)Of all cases, 1 patient was diagnosed as IgG4-related retroperitoneal fibrosis and 5 patients were classified as possible group. Ten patients were administered steroid therapy. Percutaneous nephrostomy tube was placed in 3 patients and was removed in 2 of these patients after steroid therapy. The serum high levels of IgG4 were confirmed in all 4 patients who were classified into the possible group and who were treated with steroids. (Conclusion)Although histologic examination is necessary for the diagnosis of retroperitoneal fibrosis, tissue collection by open or laparoscopic surgery is highly invasive. CT-guided biopsy may be useful in high-risk cases, such as elderly patients on anticoagulation. After excluding other diseases in high-risk cases, response to empiric steroid therapy may be diagnostic. In the possible group, changes in serum IgG4 levels may reflect the disease condition and might be useful in determining the maintenance dose of steroids.

Preoperative Clinical Predictors of Lymphovascular Invasion of Bladder Tumors at Transurethral Resection Pathology.

BACKGROUND: The assessment of lymphovascular invasion (LVI) on the specimens of a transurethral resection of bladder tumors (TURBT) is very important for risk stratification and decision-making on further treatment for bladder cancer. OBJECTIVES: The present study aimed to identify clinical predictors associated with the risk of bladder cancer with LVI before a first TURBT. METHODS: A total of 291 patients underwent a first TURBT for bladder cancer at Toho University Sakura Medical Center between January 2012 and December 2016. We analyzed predictors of LVI based on data from 217 patients and predictors of high grade and ≥ pT1 tumors based on data from the medical records of 237 patients for comparison with LVI risk factors. RESULTS: Univariate analysis significantly associated LVI with episodes of gross hematuria, positive urinary cytology, and larger, non-papillary and sessile tumors. Multivariate analysis selected larger tumors [odds ratio (OR) 1.39; 95 % confidence interval (CI) 1.08-1.78; p = 0.01], and non-papillary (OR 10.05; 95% CI 3.75-26.91; p < 0.01) and sessile (OR 2.65; 95% CI 1.18-5.93; p = 0.02) tumors as significant predictors of LVI. Some predictors such as tumor size and non-papillary tumors overlapped between high-grade and ≥ pT1 bladder cancer. CONCLUSIONS: These predictors can help clinicians to identify patients with, or who are at high-risk for LVI before undergoing a first TURBT and to determine priorities for preoperative evaluation and scheduling consecutive treatments.

H3K9 Demethylases JMJD1A and JMJD1B Control Prospermatogonia to Spermatogonia Transition in Mouse Germline.

Histone H3 lysine 9 (H3K9) methylation is dynamically regulated by methyltransferases and demethylases. In spermatogenesis, prospermatogonia differentiate into differentiating or undifferentiated spermatogonia after birth. However, the epigenetic regulation of prospermatogonia to spermatogonia transition is largely unknown. We found that perinatal prospermatogonia have extremely low levels of di-methylated H3K9 (H3K9me2) and that H3K9 demethylases, JMJD1A and JMJD1B, catalyze H3K9me2 demethylation in perinatal prospermatogonia. Depletion of JMJD1A and JMJD1B in the embryonic germline resulted in complete loss of male germ cells after puberty, indicating that H3K9me2 demethylation is essential for male germline maintenance. JMJD1A/JMJD1B-depleted germ cells were unable to differentiate into functional spermatogonia. JMJD1 isozymes contributed to activation of several spermatogonial stem cell maintenance genes through H3K9 demethylation during the prospermatogonia to spermatogonia transition, which we propose is key for spermatogonia development. In summary, JMJD1A/JMJD1B-mediated H3K9me2 demethylation promotes prospermatogonia to differentiate into functional spermatogonia by establishing proper gene expression profiles.

Possible "Premotor" Multiple System Atrophy-Cerebellar Form.

We report the case of a 52-year-old Japanese man who, while he had no cerebellar ataxia or parkinsonism, was revealed to have silent cerebellar hypoperfusion/mild cerebellar atrophy and sacral autonomic disorder. His sacral autonomic disorder was urinary retention without marked prostate hyperplasia. Urodynamics-sphincter electromyography revealed detrusor hyperactivity with impaired contraction and neurogenic changes of the sphincter motor unit potentials. Although he did not have a motor disorder, these features suggested possible multiple system atrophy-cerebellar (MSA-C) form. The present case report suggests that neuroimaging helps in diagnosing "premotor" MSA-C form in situ.

Urodynamic and gait analyses in multiple system atrophy.

BACKGROUND: Limited attention has been paid to the relationship between bladder dysfunction and motor disorder in multiple system atrophy (MSA). OBJECTIVE: We aimed to correlate pressure-flow urodynamic parameters with video-gait analysis parameters in MSA. METHODS: We recruited 34 patients with MSA: 20 men, 14 women; age 64.0 + - 7.9 years; disease duration 2 years (1-4 years). Nineteen cases had the cerebellar form, and 15 had the parkinsonian form; the patients were taking levodopa 300 mg/day (100-400 mg). All patients underwent pressure-flow urodynamics (parameters: detrusor overactivity [noted in 72%] and Watts factor) and video-gait analysis (parameters: time and the number of strides taken to walk 5 m (simple task) and time for timed up and go (complex task). Statistical analysis was done using Student's t-test to analyze the relation between detrusor overactivity and gait, and Spearman's rank correlation coefficient test to analyze the relation between the remaining parameters and gait. RESULTS: We found no relation between filling-phase urodynamics (detrusor overactivity) and video-gait analysis parameters. Also, we found no relation between voiding-phase urodynamics (Watts factor, reflecting detrusor power) and all three video-gait analysis parameters in our MSA patients. CONCLUSION: The fact that neither detrusor overactivity nor the Watts factor was related with motor disorders in the present study suggests that bladder dysfunction occurs independently from motor disorder in MSA.

Development and external validation of a nomogram to predict high-grade papillary bladder cancer before first-time transurethral resection of the bladder tumor.

BACKGROUND: The aim of this study was to identify the clinical predictors related to the risk of high-grade papillary bladder cancer before first-time transurethral resection of a bladder tumor (TUR-Bt), and to develop and validate a nomogram predicting the risk of high-grade papillary bladder cancer. METHODS: A retrospective clinical study of consecutive patients who underwent first-time TUR-Bt for papillary bladder cancer was performed. Medical records were reviewed uniformly, and the following data were collected: age, sex, episodes of urinary symptoms, tumor size, number of tumors, location of the largest tumor (lateral walls, base, posterior wall, dome, and anterior wall), tumor appearance (papillary or non-papillary, pedunculated or sessile), and urinary cytology. Data from 254 patients (Group A) were used for the development of a nomogram, while data from 170 patients (Group B) were used for its external validation. RESULTS: High-grade papillary bladder cancer was pathologically diagnosed in 51.6 and 74.6% of Group A and Group B patients, respectively. Based on univariable analyses in Group A, macrohematuria, tumor size, multiple tumors, appearance, and positive urinary cytology were selected as variables to incorporate into a nomogram. The AUC value was 0.81 for the internal validation (Group A), and 0.78 for the external validation (Group B). This novel nomogram can predict high-grade papillary bladder cancer accurately. CONCLUSIONS: The present nomogram can help clinicians calculate the probability in patients with bladder cancer before TUR-Bt and decide on earlier intervention and priorities for the treatment of patients diagnosed with bladder cancer.

Prion protein protects mice from lethal infection with influenza A viruses.

The cellular prion protein, designated PrPC, is a membrane glycoprotein expressed abundantly in brains and to a lesser extent in other tissues. Conformational conversion of PrPC into the amyloidogenic isoform is a key pathogenic event in prion diseases. However, the physiological functions of PrPC remain largely unknown, particularly in non-neuronal tissues. Here, we show that PrPC is expressed in lung epithelial cells, including alveolar type 1 and 2 cells and bronchiolar Clara cells. Compared with wild-type (WT) mice, PrPC-null mice (Prnp0/0) were highly susceptible to influenza A viruses (IAVs), with higher mortality. Infected Prnp0/0 lungs were severely injured, with higher inflammation and higher apoptosis of epithelial cells, and contained higher reactive oxygen species (ROS) than control WT lungs. Treatment with a ROS scavenger or an inhibitor of xanthine oxidase (XO), a major ROS-generating enzyme in IAV-infected lungs, rescued Prnp0/0 mice from the lethal infection with IAV. Moreover, Prnp0/0 mice transgenic for PrP with a deletion of the Cu-binding octapeptide repeat (OR) region, Tg(PrPΔOR)/Prnp0/0 mice, were also highly susceptible to IAV infection. These results indicate that PrPC has a protective role against lethal infection with IAVs through the Cu-binding OR region by reducing ROS in infected lungs. Cu content and the activity of anti-oxidant enzyme Cu/Zn-dependent superoxide dismutase, SOD1, were lower in Prnp0/0 and Tg(PrPΔOR)/Prnp0/0 lungs than in WT lungs. It is thus conceivable that PrPC functions to maintain Cu content and regulate SOD1 through the OR region in lungs, thereby reducing ROS in IAV-infected lungs and eventually protecting them from lethal infection with IAVs. Our current results highlight the role of PrPC in protection against IAV infection, and suggest that PrPC might be a novel target molecule for anti-influenza therapeutics.

External validation of two web-based postoperative nomograms predicting the probability of early biochemical recurrence after radical prostatectomy: a retrospective cohort study.

The present study aimed to validate and compare the predictive accuracies of the Memorial Sloan Kettering Cancer Center (MSKCC) and Johns Hopkins University (JHU) web-based postoperative nomograms for predicting early biochemical recurrence (BCR) after radical prostatectomy (RP) and to analyze clinicopathological factors to predict early BCR after RP using our dataset. The c-index was 0.72 (95% confidence (CI): 0.61-0.83) for the MSKCC nomogram and 0.71 (95% CI: 0.61-0.81) for the and JHU nomogram, demonstrating fair performance in the Japanese population. Furthermore, we statistically analyzed our 174 patients to elucidate prognostic factors for early BCR within 2 years. Lymphovascular invasion (LVI) including lymphatic vessel invasion (ly) was a significant predictor of early BCR in addition to common variables (pT stage, extraprostatic extension, positive surgical margin and seminal vesicle invasion). LVI, particularly ly, may provide a good predictor of early BCR after RP and improve the accuracy of the nomograms.

Rescuing the aberrant sex development of H3K9 demethylase Jmjd1a-deficient mice by modulating H3K9 methylation balance.

Histone H3 lysine 9 (H3K9) methylation is a hallmark of heterochromatin. H3K9 demethylation is crucial in mouse sex determination; The H3K9 demethylase Jmjd1a deficiency leads to increased H3K9 methylation at the Sry locus in embryonic gonads, thereby compromising Sry expression and causing male-to-female sex reversal. We hypothesized that the H3K9 methylation level at the Sry locus is finely tuned by the balance in activities between the H3K9 demethylase Jmjd1a and an unidentified H3K9 methyltransferase to ensure correct Sry expression. Here we identified the GLP/G9a H3K9 methyltransferase complex as the enzyme catalyzing H3K9 methylation at the Sry locus. Based on this finding, we tried to rescue the sex-reversal phenotype of Jmjd1a-deficient mice by modulating GLP/G9a complex activity. A heterozygous GLP mutation rescued the sex-reversal phenotype of Jmjd1a-deficient mice by restoring Sry expression. The administration of a chemical inhibitor of GLP/G9a enzyme into Jmjd1a-deficient embryos also successfully rescued sex reversal. Our study not only reveals the molecular mechanism underlying the tuning of Sry expression but also provides proof on the principle of therapeutic strategies based on the pharmacological modulation of epigenetic balance.

Prions amplify through degradation of the VPS10P sorting receptor sortilin.

Prion diseases are a group of fatal neurodegenerative disorders caused by prions, which consist mainly of the abnormally folded isoform of prion protein, PrPSc. A pivotal pathogenic event in prion disease is progressive accumulation of prions, or PrPSc, in brains through constitutive conformational conversion of the cellular prion protein, PrPC, into PrPSc. However, the cellular mechanism by which PrPSc is progressively accumulated in prion-infected neurons remains unknown. Here, we show that PrPSc is progressively accumulated in prion-infected cells through degradation of the VPS10P sorting receptor sortilin. We first show that sortilin interacts with PrPC and PrPSc and sorts them to lysosomes for degradation. Consistently, sortilin-knockdown increased PrPSc accumulation in prion-infected cells. In contrast, overexpression of sortilin reduced PrPSc accumulation in prion-infected cells. These results indicate that sortilin negatively regulates PrPSc accumulation in prion-infected cells. The negative role of sortilin in PrPSc accumulation was further confirmed in sortilin-knockout mice infected with prions. The infected mice had accelerated prion disease with early accumulation of PrPSc in their brains. Interestingly, sortilin was reduced in prion-infected cells and mouse brains. Treatment of prion-infected cells with lysosomal inhibitors, but not proteasomal inhibitors, increased the levels of sortilin. Moreover, sortilin was reduced following PrPSc becoming detectable in cells after infection with prions. These results indicate that PrPSc accumulation stimulates sortilin degradation in lysosomes. Taken together, these results show that PrPSc accumulation of itself could impair the sortilin-mediated sorting of PrPC and PrPSc to lysosomes for degradation by stimulating lysosomal degradation of sortilin, eventually leading to progressive accumulation of PrPSc in prion-infected cells.

Development of novel nomograms to predict renal functional outcomes after laparoscopic adrenalectomy in patients with primary aldosteronism.

PURPOSE: Most patients with primary aldosteronism (PA) show a significant decrease in kidney function after surgery. Glomerular hyperfiltration peculiar to PA can mask mild renal failure before surgery. The aim of this retrospective study was to investigate postoperative renal functional outcomes in PA patients from different viewpoints and to develop novel nomograms that can predict renal functional outcomes in PA patients after surgery. METHODS: 130 Japanese PA patients treated by unilateral laparoscopic adrenalectomy were retrospectively surveyed. Pre- and postoperative changes of estimated glomerular filtration rates (eGFRs) and the distribution of eGFR classification were compared. Furthermore, predictors of the following renal functional outcomes were investigated: (I) the percentage decrease >25% in eGFR and (II) the presence of new-onset eGFR <45 ml/min/1.73 m2. Finally, two nomograms that predicted postoperative renal functional outcomes were developed and internally validated. RESULTS: At 6 months, the average decrease in eGFR was 16.7 mL/min/1.73 m2 (corresponding percent decrease: 19.7%). Upstaging of eGFR classification was observed in 54.6% of patients. Age, potassium, plasma aldosterone concentration, and initial eGFR were incorporated into a nomogram predicting a >25% postoperative decrease in eGFR. Duration of hypertension and initial eGFR were incorporated into a nomogram predicting new-onset eGFR <45 ml/min/1.73 m2. The value of the area under the receiver operating characteristics curve for each nomogram was 0.82 and 0.74, respectively. CONCLUSION: The first nomograms that can predict postoperative renal outcomes in PA patients were developed. They will help clinicians calculate the probability of renal dysfunction in PA patients after laparoscopic adrenalectomy.

Effects of prion protein devoid of the N-terminal residues 25-50 on prion pathogenesis in mice.

The N-terminal polybasic region of the normal prion protein, PrPC, which encompasses residues 23-31, is important for prion pathogenesis by affecting conversion of PrPC into the pathogenic isoform, PrPSc. We previously reported transgenic mice expressing PrP with residues 25-50 deleted in the PrP-null background, designated as Tg(PrP∆preOR)/Prnp 0/0 mice. Here, we produced two new lines of Tg(PrP∆preOR)/Prnp 0/0 mice, each expressing the mutant protein, PrP∆preOR, 1.1 and 1.6 times more than PrPC in wild-type mice, and subsequently intracerebrally inoculated RML and 22L prions into them. The lower expresser showed slightly reduced susceptibility to RML prions but not to 22L prions. The higher expresser exhibited enhanced susceptibility to both prions. No prion transmission barrier was created in Tg(PrP∆preOR)/Prnp 0/0 mice against full-length PrPSc. PrPSc∆preOR accumulated in the brains of infected Tg(PrP∆preOR)/Prnp 0/0 mice less than PrPSc in control wild-type mice, although lower in RML-infected Tg(PrP∆preOR)/Prnp 0/0 mice than in 22L-infected mice. Prion infectivity in infected Tg(PrP∆preOR)/Prnp 0/0 mice was also lower than that in wild-type mice. These results indicate that deletion of residues 25-50 only slightly affects prion susceptibility, the conversion of PrPC into PrPSc, and prion infectivity in a strain-specific way. PrP∆preOR retains residues 23-24 and lacks residues 25-31 in the polybasic region. It is thus conceivable that residues 23-24 rather than 25-31 are important for the polybasic region to support prion pathogenesis. However, other investigators have reported that residues 27-31 not 23-24 are important to support prion pathogenesis. Taken together, the polybasic region might support prion pathogenesis through multiple sites including residues 23-24 and 27-31.

Clinical predictors for high-grade bladder cancer before first-time transurethral resection of the bladder tumor: a retrospective cohort study.

The aim of this study was to identify the clinical predictors related to the risk of high-grade bladder cancer before first-time transurethral resection of the bladder tumor (TUR-Bt) and to externally validate the accuracy of Shapur's nomogram predicting the risk of high-grade bladder cancer in Japanese patients. As a result, episode of gross hematuria (odds ratio: 2.68, P = 0.02), larger tumor size (odds ratio: 1.89, P < 0.01) and positive urinary cytology (odds ratio: 8.34, P < 0.01) were found to be significant predictors for high-grade bladder cancer. Furthermore, the nomogram showed a high predictive accuracy in our Japanese population (area under the curve: 0.79). Clinicians will be able to predict high-grade bladder cancer using the common factors in Shapur's study and ours, such as tumor size and urinary cytology, and gross hematuria as the additional factor first identified here to decide priorities for the treatment of patients diagnosed with bladder cancer.

Urodynamic findings in patients with Creutzfeldt-Jakob disease: a case report.

AIM OF STUDY: Urinary dysfunction in Creutzfeldt-Jakob disease (CJD) patients is attributed to functional incontinence, since they often have immobility and loss of motivation. In contrast, previously no urodynamic findings are available in CJD patients. CASE REPORT: We had 2 CJD patients who had urinary frequency and urinary retention. We performed urodynamics with the spouse's informed consent in order to explore the mechanism of urinary dysfunction in those cases. Case 1 had typical acute cognitive deterioration with incontinence and urinary retention, while case 2 had subacute cognitive deterioration (that started after admission) and nocturia. The urodynamic findings were diverse. One feature was detrusor overactivity during bladder filling in case 1. Another feature of urodynamic finding includes neurogenic change of sphincter EMG in case 1 and decreased bladder sensation in case 2. CONCLUSION: Urodynamics in our two CJD patients revealed detrusor overactivity and neurogenic sphincter electromyogram, presumably reflecting pathological lesions in the prefrontal cortex/basal ganglia as well as the sacral spinal cord in CJD.