Neurogenic bladder in Lambert-Eaton myasthenic syndrome and its response to 3,4-diaminopyridine.

Autonomic dysfunction, as well as neuromuscular involvement, is a common manifestation of Lambert-Eaton myasthenic syndrome (LEMS). Dry mouth and impotence have been described as typical features of autonomic dysfunction, but neurogenic bladder is infrequent or subclinical in LEMS. We report a patient with neurogenic bladder secondary to LEMS whose condition responded to 3,4-diaminopyridine (3,4-DAP). In this patient's serum, results of repeated measurement with P/Q-type VGCC antibodies proved positive, but not with N-type VGCC and synaptotagmin antibodies. A review of the literature turned up a few patients with voiding dysfunction related to LEMS, but no urodynamic studies were done on these patients. Ours is the first case in which 3,4-DAP was efficacious in treating LEMS and neurogenic bladder. Responses of 3,4-DAP in urodynamic studies suggest that in this LEMS patient neurogenic bladder was caused by defective neurotransmission both in the autonomic detrusor and skeletal abdominal muscles.

Paraganglioma of the urinary bladder: A case report and review of the Japanese literature.

We describe a patient with primary paraganglioma of the urinary bladder associated with typical clinical symptoms. Systolic blood pressure frequently increased to 300 mm Hg immediately after micturition. Levels of urinary vanillylmandelic acid excretion and serum norepinephrine were significantly elevated. The tumor was removed by partial cystectomy. Histological examination of the tumor revealed paraganglioma of the urinary bladder. Compared with a review of 53 similar cases reported in Japan, the distribution of age, sex and tumor location in the bladder differed from those described worldwide.

[A case of ovarian cyst presenting as urinary retention].

A 54-year-old woman presented with repeated episodes of urinary retention during an approximately 7-month period of dysuria. Computerized tomography scan revealed a right ovarian cyst and a small uterine myoma. At operation, the ovarian cyst was deeply incarcerated into the pouch of Douglas displacing the bladder neck and the uterine cervix in the anterocephalad direction. Histological diagnosis of the resected cyst was benign mucinous cystadenoma. This is the 5th reported case of urinary retention caused by ovarian cyst in the Japanese literature.

[Efficacy and safety of intramuscular imipenem/cilastatin (IPM/CS) for complicated urinary tract infections].

An intramuscular preparation of imipenem/cilastatin (IPM/CS, 500 mg/500 mg) was administered to 59 patients with complicated urinary tract infections (UTI; cystitis and pyelonephritis) to evaluate its efficacy and safety. The obtained results are summarized as follows: In patients with cystitis, evaluations based on daily frequencies of administration were also performed. 1) According to the treating doctors, the drug showed an overall efficacy rate of 80% (45/56 patients). The efficacy rate was 89% in patients with cystitis treated by a u.i.d. regimen. Among patients treated by a b.i.d. regimen, the efficacy rate was 67% for cystitis cases and 84% for pyelonephritis cases. 2) When clinical efficacy was assessed according to the criteria for UTI drug efficacy evaluation, the drug was 'markedly effective' in 14 patients, 'effective' in 23, and ineffective in 11 patients, for an efficacy rate of 77% (37/48 patients). 3) The microbiological eradication rate was 88% (59/67 strains). The rate was 95% (20/21 strains) for Gram-positive bacteria and 85% (39/46 strains) for Gram-negative bacteria. The efficacy for Enterobacter faecalis and Pseudomonas aeruginosa was 100% and 73%, respectively. 4) As side effects, pain at the injection site was reported by one patient and abnormal laboratory test values were observed in 2 patients. All of these reactions were mild and resolved shortly after the completion of treatment. Based on these findings, it is concluded that this intramuscular preparation of IPM/CS is effective for treating complicated urinary tract infections.

Patent vitelline duct in an adult deceptively appeared to be acquired umbilical urachal sinus: a case report.

Here is presented a surprisingly rare case in a 40-year-old male who had patent vitelline duct by nature. However, his congenital disease appeared deceptively to be an acquired umbilical urachal sinus on the diagnostic evaluations including fistulography before surgery. The diagnosis was definitely confirmed after the successful surgical procedure. The principal reason why these diseases were indistinguishable was reviewed. The incidence of each disease and incidence of association with umbilical fistula in each disease were discussed. With regard to these incidences, we compared urachal anomalies with vitelline duct anomalies through reference of several literatures. This is the most unique event we have ever clinically experienced.

[Percutaneous Bacillus Calmette-Guerin perfusion of the upper urinary tract for carcinoma in situ].

We performed percutaneous perfusion of the upper urinary tract with Bacillus Calmette-Guerin (BCG) in 3 patients. Two of them had undergone unilateral nephrectoureterectomy for ureter carcinoma in situ and one had undergone radical cystectomy with bilateral ureterocutaneostomy for invasive bladder carcinoma. However, they suffered recurrent upper urinary tract carcinoma in situ within 2 years after their operation. Under ultrasound control a percutaneous nephrostomy tube was placed in the patient. Before BCG perfusion unobstructed flow from the renal pelvis to the bladder was confirmed and pyelovenous or pyelolymphatic back flow was excluded under fluoroscopy. A dose of 240 mg BCG was dissolved in 150 ml 0.9% saline. The flask was placed 20 cm above the kidney of the resting patient. A continuous flow of approximately 1 ml per minute was maintained. The perfusion was stopped after 2 hours and the nephrostomy tube was closed. Therapy was repeated at weekly intervals for a total of 6 perfusions (1 treatment course). In each of them urine cytology results became negative after 1 treatment course. No severe side effects were observed. Further investigation is also needed to determine whether BCG perfusion of the upper urinary tract could become a conservative treatment for carcinoma in situ of the upper urinary tract.

Novel complications with HTLV-1-associated myelopathy/tropical spastic paraparesis: interstitial cystitis and persistent prostatitis.

Lower urinary symptoms associated with HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) are common, but have been regarded as 'neurogenic' due to spinal involvements. However, in some cases, these symptoms are persistent, progressive, and not directly correlated with the severity of other neurologic symptoms of the lower spinal cord. These findings prompted us to locate organic lesions in the lower urinary tract and to correlate them with HTLV-1 infection. Among 35 HAM patients with lower urinary symptoms, we found 4 cases with the symptoms persistent and progressive: 3 with contracted bladder and another with persistent prostatitis. Histological or cytological examinations indicated local lymphocytic infiltrations in the lower urinary tract in all cases: 3 by the infiltration in the bladder and the other by a high concentration of lymphocytes in expressed prostatic secretions. Of 3 cases whose urinary samples were available, 2 showed significant increase in the concentration of urinary anti-HTLV-1 antibody of IgA class. The urinary IgA antibody of the third case was not elevated, but the sample had been obtained after resection of the affected bladder. None of the control cases showed significant anti-HTLV-1 IgA antibody in urine except for a case of gross hematuria due to chemotherapy directed against adult T-cell leukemia. We suggest inclusion of these processes into the spectrum of complications for HAM/TSP. The elevated excretion of anti-HTLV-1 of IgA class in urine may be an indicator of these complications.

[Postoperative adjuvant M-VAC chemotherapy for invasive bladder cancer].

Between 1986 and 1989, 14 patients undergoing cystectomy for bladder cancer, in pathological stage high risk pT2 group, pT3-4 and/or with N+ disease, received postoperative adjuvant methotrexate, vinblastine, doxorubicin and cisplatin (M-VAC) chemotherapy. Of the 14 patients 10 were alive with no evidence of disease for an average of 41 months. Tumor recurrence was seen in 4 patients (bone in 2, lungs in 1, brain in 1 patient). Of the 4 patients, 3 patients died of cancer progression at an average of 26 months and 1 patient was alive with tumor for 30 months. Their actual survival rate at 64 months was 70%, which was significantly higher than that of the historical control groups (1974-1981: 18%, 1982-1985: 46%). Although postoperative adjuvant M-VAC chemotherapy for invasive bladder cancer seemed effective in this study, a controlled randomized study will be necessary to conclude if it could be of real benefit for these patients.

Segmental stenosis of ureter: a late complication of intra-arterial chemotherapy for bladder cancer.

A 54-year-old Japanese male was treated with a single shot of cisplatin-phosphatidylcholine-lipiodol (CPL) suspension due to bladder tumour (stage T2N0M0). Seven months later, a right lower ureteral stenosis developed. The possible cause of ureteral stenosis due to intra-arterial chemotherapy is discussed.

[Bladder involvement in HTLV-I associated myelopathy].

The HTLV-I infection was endemic in south western Kyushu. This human T-lymphotropic virus type I may cause HTLV-I associated myelopathy (HAM), a neurological disease characterized by a spastic paraparesis. And one of the minor diagnostic features of HAM is the presence of cystorectal disturbance. We experienced 35 HAM patients with a neurogenic bladder. A gradually progressive contracted bladder was observed in 3 of them. The main pathological finding in these patients was submucosal infiltration of lymphocytes. These findings suggest that immunologic mechanisms account for the development of bladder lesions.

[Bladder replacement with sigmoid colon for bladder cancer].

From 1972 to 1989, 21 patients underwent bladder replacement with sigmoid colon after cystectomy for bladder cancer. A portion of sigmoid colon (about 15 to 20 cm) was isolated and anastomosed to the urethral stump. Then the ureters were implanted in the sigmoid colon via submucosal tunnels. There were 20 men and 1 woman, ranging in age from 24 to 71 years (average 51 years). Preoperative investigations showed that all the cases were free of metastasis. Five years survival rate was 60.7% and there was no operative mortality. There was urethral recurrence in one case and in that case postoperative histopathology revealed carcinoma in situ with tumor. All patients were able to void by themselves without any difficulty. Five patients complained mild incontinence at daytime and all patients had mild incontinence during deep sleep at night, but all of them could maintain their normal daily life like before. The major postoperative complications were: leak at the site of anastomosis (between sigmoid colon and urethra) in 6 cases, hydronephrosis in 2 cases, VUR in 2 cases and bladder stone in 1 case. The patients who underwent bladder replacement with sigmoid colon were well satisfied, because there were no external stoma and the patients could void by themselves. From now, we want to improve our operative method in order to avoid postoperative incontinence.

[Induction therapy with CDDP and ADM for stage D prostatic cancer and its clinical response].

From September 1983 to September 1989, 20 patients with newly diagnosed stage D prostatic cancer were treated with cis-platinum (CDDP) and adriamycin (ADM) as the induction therapy. Analysis of histological and clinical effects on the induction therapy revealed partial response (PR) in 13 cases and no change (NC) in 7 cases according to Shimazaki's response criteria, and PR in 4 cases, NC in 15 cases and progression (PD) in 1 case according to NPCP criteria. Histological Ef 0-b effect was found in 2 cases, Ef 1 in 7 cases, Ef 2 in 3 cases, Ef 3 in 2 cases and Ef 4 in 2 cases. Analysis of long-term results revealed relapse in 9 cases and cancer death in 6 cases. The 1-year, 3-year and 5-year continued response rates for all cases were 85.7, 40.2 and 32.1%, respectively. The 1-year, 3-year and 5-year survival rates were 100, 64.3 and 53.6% respectively. Histologically, low responsive cases showed a tendency of relapse and cancer death more frequently than high responsive cases. These results suggest that CDDP and ADM therapy is more effective than hormone therapy in newly diagnosed stage D prostatic cancer as an induction therapy.

[A case of non-Cushingoid Cushing's syndrome].

A 42-year-old female was admitted to our hospital with a chief complaint of hypertension. Endocrinologically, the plasma cortisol level was increased, but its diurnal rhythm had disappeared and the plasma cortisol level was not suppressed by dexamethasone loading at 2 mg or 8 mg. The plasma ACTH level was low. Computerized tomographic scan, echography and adrenocortical scintigraphy showed an adrenocortical tumor on the right adrenal gland. Physical examination did not reveal typical Cushingoid symptoms such as moon face, central obesity and violaceous striae. Thus we diagnosed this case as non-Cushingoid Cushing's syndrome and performed right adrenalectomy. Histological examination showed adreno-cortical adenoma without malignancy.

[A clinicopathological study on patients with bladder cancer treated with cystectomy].

A clinical and histopathological investigation was made on 170 patients with bladder cancer who underwent total cystectomy at our institutions between 1982 and 1986. The overall 5-year survival rates of patients with pTis + pTa, pT1, pT2, pT3, pT3b and pT4 were 100, 71.8, 60.7, 39.2, 31.4 and 0% respectively, those of patients with G1, G2 and G3 were 100%, 67.6%, 35.7% respectively. As for histopathological growth and spread pattern (INF), intramural lymphatic invasion (ly) and venous invasion (v), INF beta, INF gamma, ly2, v (+) showed the worst prognosis. These histopathological factors were considered to be closely correlated to each other. Studies on these histopathological factors are very important in planning the subsequent therapy.

[Transitional cell carcinoma of the renal pelvis in a woman discovered at childbirth].

We report a case of transitional cell carcinoma of a renal pelvis in a woman discovered after childbirth. A 38-year-old woman, who delivered an immature male infant in cesarean section 29 days prior to hospitalization, was admitted complaining of asymptomatic gross hematuria. Excretory urography and retrograde pyelography showed a filling defect of the right renal pelvis, Spontaneous urine cytology indicated class 5. Renal computed tomographic scan demonstrated a mass lesion in the right kidney. Right total nephroureterectomy and partial cystectomy was performed for diagnosis and treatment. Pathological diagnosis was papillary transitional cell carcinoma (grade 2). This is the first case of transitional cell carcinoma of the renal pelvis occurring in a childbearing woman in Japan.

A case of deoxycorticosterone-producing benign adrenocortical tumor.

A case of deoxycorticosterone-producing benign adrenocortical tumor is presented. A review of the English literature revealed that this is the second case of deoxycorticosterone-producing adrenocortical adenoma.

A case of spontaneous rupture of ureter due to ureteral stone in G syndrome.

A case of spontaneous rupture of ureter with formation of urinoma due to ureteral stone, which was noticed in an infant with G syndrome, is described. Investigation revealed that the patient had bilateral vesicoureteric reflux. The urinary tract anomalies in G syndrome are discussed.

Carcinosarcoma of the bladder.

We report a case of carcinosarcoma of the bladder. Total cystectomy with ileal loop urinary diversion was performed. Histologically, the bulk of the tumor tissue was rhabdomyosarcoma and adenocarcinoma. At distant areas from the bulk tumor transitional cell carcinoma was noted. The literature is reviewed and the clinicopathologic condition of carcinosarcoma of the bladder is discussed.