Guillain-Barré syndrome associated with central nervous system lesions.

We report the clinical course, and neurophysiological and neuroimaging findings of a patient with Guillain-Barré syndrome associated with central nervous system lesions. During a course of intravenous immunoglobulin therapy, she had headache with meningism. Cerebral magnetic resonance imaging showed lesions in both frontal and right occipital lobes. Cerebrospinal fluid showed a raised protein concentration accompanied by mild pleocytosis. Her symptoms resolved within two months. Subsequent magnetic resonance imaging revealed cavity formation in the deep white matter and atrophic changes in the right occipital lobes.

[Intermittent androgen deprivation treatment of prostate cancer restarted at low level of serum prostate specific antigen: a pilot study].

We report a pilot study on a novel protocol of intermittent androgen deprivation (IAD) treatment of prostate cancer (PC), in which androgen deprivation is restarted when serum prostatic specific antigen (PSA) level reached more than 2 ng/ml and is stopped when PSA level decreased below 0.3 ng/ml. Thirty-two patients (aged 60 to 86 years, median 74 years) with prostate cancer (Stage A in 4 patients, B in 20, C in 1, D in 5, and relapse after radical prostatectomy in 2) were treated with IAD. Median serum PSA prior to the start of endocrine therapy was 15.65 (range 2.67 to 306.3) ng/ml. Eleven patients were treated with lutenizing-hormone-releasing hormone (LHRH) agonist alone and 21 were treated with LHRH agonist plus an antiandrogen. Median duration of first endocrine therapy was 572 (range 100 to 1,543) days. Median serum PSA at the start of first off-phase was 0.038 (range 0.003 to 0.489) ng/ml. After a median of 207 days (range 140 to 843) of follow-up, 19 patients were in the first cycle, 9 in the second cycle, 3 in the third cycle, 1 in the fourth cycle. Two patients developed androgen-independent PC. The median duration of first off-phase of IAD was 287 days. There was a significant inverse relation between the duration of the first on-phase and testosterone level measured 4 months after the cessation of first on-phase therapy (R = -0.518). These results suggest that our protocol provides a reasonable length of off-phase duration and that the long term-androgen deprivation phase might delay the recovery of the testicular endocrine function which should be maintained during the off-phase of IAD.

[Systemic lupus erythematosus presenting as a brainstem infarction and hemorrhage during treating retroperitoneal abscess: a case report].

We report a case of a brainstem infarction and hemorrhage seen in a 21-year-old female with systemic lupus erythematosus (SLE) during treating retroperitoneal abscess. SLE has been treated with prednisolone and mizoribine for 4 years. The patient with right back and lower abdominal pain visited our hospital. Computed tomography (CT) revealed retroperitoneal abscess over surrounding right kidney, horizontal part of duodenum and in front of psoas muscle. Drainage was done with indwelled right single J catheter and penrose drain. Retroperitoneal abscess was much smaller, but, post drainage day 17, high fever, unconsciousness, down-blood pressure and down-beat nystagmus appeared suddenly. Brain CT scan revealed a brainstem wide infarction and hemorrhage spot. Pulse therapy with steroid was done, however unfortunately the patient was dead after 6 days.

[Experience of direct hemoperfusion using polymyxin B-immobilized fiber on patients with endotoxin shock from urosepsis].

We report the effects of direct hemoperfusion using polymyxin B-immobilized fiber (PMX treatment) on two patients with endotoxin shock from urosepsis. In both cases, urosepsis caused by pyelonephritis from a ureteral stone progressed to endotoxin shock and disseminating intravascular coagulation (DIC). Single J catheter was indwelt in the ureter for the purpose of drainage before PMX treatment. Drainage of the origin of infection, using effective antibiotics and PMX treatment improved shock state and DIC earlier than we expected.

Molecular cloning and expression of endo-beta-N-acetylglucosaminidase D, which acts on the core structure of complex type asparagine-linked oligosaccharides.

Endo-beta-N-acetylglucosaminidase D (Endo D) produced by Streptococcus pneumoniae cleaves the di-N-acetylchitobiose structure in asparagine-linked oligosaccharides. The enzyme generally acts on complex type oligosaccharides after removal of external sugars by neuraminidase, beta-galactosidase, and beta-N-acetylglucosaminidase. We cloned the gene encoding the enzyme and expressed it as a periplasm enzyme in Escherichia coli. The first 37 amino acids in the predicted sequence are removed in the mature enzyme, yielding a protein with a molecular mass of 178 kDa. The substrate specificity of the recombinant enzyme is indistinguishable from the enzyme produced by S. pneumoniae. Endo-beta-N-acetylglucosaminidase A (Endo A) from Arthrobacter protophormiae, the molecular mass of which is 72 kDa, had 32% sequence identity to Endo D, starting from the N-terminal sides of both enzymes, although Endo A hydrolyzes high-mannose-type oligosaccharides and does not hydrolyze complex type ones. Endo D is not related to endo-beta-N-acetylglucosaminidases H, F(1), F(2), or F(3), which share common structural motifs. Therefore, there are two distinct groups of endo-beta-N-acetylglucosaminidases acting on asparagine-linked oligosaccharides. The C-terminal region of Endo D shows homology to beta-galactosidase and beta-N-acetylglucosaminidase from S. pneumoniae and has an LPXTG motif typical of surface-associated proteins of Gram-positive bacteria. It is possible that Endo D is located on the surface of the bacterium and, together with other glycosidases, is involved in virulence.

[Extracorporeal shock wave lithotripsy for upper urinary tract stones using piezoelectric shock wave lithotripsy].

Between August 1999 and July 2000, 123 cases of renal stones and 52 cases of ureteral stones in 116 males and 59 females were treated with the New Piezolith 2500. The average number of sessions required for renal and ureteral stones was 2.50 and 1.48, respectively. At 3 months postoperatively, stone-free rates for renal and ureteral stones were 64.2% and 72.7%, respectively. Assessing residual stones less than 4 mm in diameter as an effective treatment outcome, the efficacy rates for renal and ureteral stones were 94.3% and 86.4%, respectively. Side effects were encountered in 5 cases (2.9%) of high fever and one case (0.57%) of renal subcapsular hematoma. New Piezolith 2500 is effective and safe for the treatment of upper urinary stones.

Congenital ureteral diverticulum coexistent with hydronephrosis caused by vascular compression involving the uterine artery and umbilical ligament: report of a case.

Coexistence of congenital ureteral diverticulum and hydronephrosis caused by vascular compression is a rare entity. The authors experienced a case of congenital ureteral diverticulum coexistent with hydronephrosis caused by aberrant vascular compression by uterine and umbilical arteries in a 14-year-old girl. The authors could not diagnose accurately this abnormality preoperatively by 3-dimensional computed tomography. After partial ureterectomy, including resection of the saccular lesion and ureteroneocystostomy, the frequency of urinary tract infection decreased.

[Liposarcoma of the spermatic cord in the left scrotum and inguinal region: a case report].

We report a rare case of liposarcoma of the spermatic cord in the left scrotum and inguinal region. The patient was a 75 year old male, who visited our hospital with the complaint of painless left scrotal swelling and inguinal mass which had been noticed for 3 to 4 years. Ultrasonography and computed tomography (CT) showed a heterogenous mass in the left inguinal region and intrascrotum. We suspected a left testicular tumor and removed it by high inguinal orchiectomy. Histological examination revealed a well-differentiated liposarcoma of sclerosing type. Tumor local recurrence was not recognized at 8 months after operation. This is the 43rd case of liposarcoma of the spermatic cord in the Japanese literature.

[Segmental infarction of testicle presenting as right acute scrotum: a case report].

We report a case of segmental infarction of a testicle seen in a 28-year-old man with right lower abdominal pain who visited our hospital. On physical examination, the right testicle was slightly elevated and Prehn's sign was positive. As torsion of the right spermatic cord was suspected, ultrasonography and color Doppler sonography were performed, revealing sufficient blood supply in the right spermatic cord and in the most part of the right testicle. However, a low echogenic area without blood flow was noted in the upper pole of testicle. Since CT and MRI findings couldn't rule out a testicular tumor, right high orchiectomy was performed. The specimen revealed a hemorrhagic area, histologically proved to be segmental hemorrhagic infarction of the testicle. Segmental infarction of a testicle is rare and our case is the 31st case in the world literature (the 7th case in Japan).

[Three cases of gastric cancer (Borrmann type IV) presenting with right back pain caused by right hydronephrosis as the first symptom].

We experienced three cases of right hydronephrosis, which were later diagnosed to have been caused by gastric cancer (Borrmann type IV). The patients were 25-, 38-, and 50-year-old women who complained of right back pain. Ultrasound sonography revealed right hydronephrosis in all three cases. We conducted drip infusion pyelography, computed tomographic scan and retrograde pyelography, but there were no signs of urinary stones or tumors, except for the presence of right ureteral stenosis. Since the patients had upper abdominal discomfort, they underwent gastrofiberscopy, which revealed scirrhous gastric cancer. We suspected that the right ureteral stenosis was caused by metastasis of gastric cancer. After a double J catheter was indwelt at the right ureter, combination chemotherapy of methetrexate + 5-fluorouracil was conducted. The right hydronephrosis diminished and all three patients became catheter-free.

[Bilateral synchronous multilocular epididymal cysts: a case report].

A rare case of bilateral synchronous multilocular epididymal cysts is reported. A fifty-six year old man visited to our hospital with a chief complaint of swelling of bilateral intrascrotal contents. Ultrasonographic findings demonstrated multilocular lesions of the bilateral intrascrotal contents. Preoperative diagnosis was bilateral multilocular hydrocele testes. Operative procedure revealed bilateral cysts originating from the head, body and tail of the epididymis without the cysts of the tunica vaginalis. Operative sight was bilateral synchronous multilocular epididymal cysts, and bilateral epididymal cystectomy were performed. The specimen size was right diameter 12 x 6 cm and left its 8 x 5.5 cm. The puncture of the cystic fluid revealed many spermatozoa in both sides. The acquired cysts of the epididymis generally are the result of tubular obstruction with dilation of tubules adjacent to the obstruction. The dilated tubules are filled variably with viable and degenerating spermatozoa. The cysts originate most commonly from the head of the epididymis, and are unilateral, unilocular or multilocular and are usually within 1 cm in diameter. In our case, bilateral synchronous epididymal cysts originating from not only the head, but also the body and the tail is a rare case.

[Tuberous sclerosis associated with renal angiomyolipoma, pulmonary lymphangioleiomyomatosis and subungual fibroma: report of a case].

We report a case of tuberous sclerosis associated with bilateral renal angiomyolipomas (AMLs), pulmonary lymphangioleiomyomatosis (LAM) and subungual fibroma of hands and feet. A 42-year-old woman who was diagnosed as tuberous sclerosis at the age of 18 complained of left flank pain and abdominal fullness. Bilateral renal AMLs were pointed out when complete examinations were performed for hypertension at the age of 32. She suffered from severe left flank pain and abdominal distension due to the left renal tumor. Left nephrectomy and excision of the renal hilar tumor were performed. The left renal tumor weighed 1120 g, the perirenal space was filled with the tumor. histopathological diagnosis of the left renal tumor and renal hilar tumor was AML. In our case, bilateral pneumothorax appeared, and chest CT scan revealed bilateral multiple pulmonary cysts. Histopathological diagnosis of pulmonary cysts was LAM. Other complications of our case are intracranial calcification and adenoma sebaceum.