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We experienced four patients who suffered from nephrotic syndrome after a successful allogeneic hematopoietic stem cell transplantation (HSCT). These cases were seen in the nineteen-year period from September, 1986 to June, 2005. Our data showed that the incidence of nephrotic syndrome was 0.51% (3 out of 585 HSCT patients) in our hospital. Pathological findings of their renal biopsy specimens revealed that 3 patients had membranous nephropathy and that one patient had minimal change disease. Three patients were positive for anti nuclear antibody. Administration of prednisolone or cyclosporine improved the nephrotic syndrome, leading all patients to a complete or almost complete remission. The nephrotic syndrome occurred at 17 to 25 months after HSCT and accompanied the relapse of chronic graft-versus-host disease (GVHD), possibly due to the termination or a decrease of immunosuppressant administration in all patients. This suggests that immunological abnormality associated with chronic GVHD may be partly involved.

Angiography by means of iodinated contrast material, before endovascular or surgical treatment, may result in serious complications in patients with renal insufficiency or previous anaphylactoid reaction to iodine. Alternative techniques, such as magnetic resonance angiography or carbon dioxide angiography, have their own limitations. We report effectiveness and safety of cerebral angiography using gadolinium for a patient with right vertebral artery and basilar artery occlusion and renal insufficiency. A 66-year-old woman with diabetes under medical treatment suffered loss of consciousness and left hemiparesis following dehydration for 24 hours. She was transferred to our hospital. Emergent MRI suggested brain stem and cerebellar infarction due to vertebral artery occlusion with diffusion-perfusion mismatch. Because of renal insufficiency, the digital subtraction cerebral angiography using gadolinium was performed. Appropriate diagnosis was achieved by angiography using gadolinium of 0.4 mmol/kg. There was no any complication including deterioration of renal function. Gadolinium appears to be a safe and useful intraarterial contrast agent in patients with renal insufficiency.

A 21-year-old man with neurofibromatosis type 1 (NF 1) had many widespread cutaneous neurofibroma on his right face. Magnetic resonance imaging (MRI) revealed basal meningocele due to dysplasia of the skull base. Carotid and vertebral angiograms revealed occlusion of the right internal carotid artery, persistent primitive trigeminal artery. We have reviewed the clinical and radiographic features of this case of neurofibromatosis, meningocele and cerebral arterial abnormalities. NF associated with both intracranial vascular malformation and meningocele is very rare, and in our case both were thought to arise congenitally as a manifestation of mesodermal dysplasia. Careful follow up using MRI and MR angiography should be performed for such patients.

The efficacy of 1/2 FAM, which consists of 5-fluorouracil (5-FU), adriamycin (ADM) and mitomycin C (MMC), was compared with that of palliative treatment in patients with unresectable pancreatic and biliary tract carcinomas in a multicenter randomized trial. The patients assigned to 1/2 FAM group were treated with 5-FU 200 mg/m2/day IV, ADM 15 mg/m2/day IV and MMC 5 mg/m2/day IV. These 3 drugs were given concurrently as the initial dose within a week after palliative operation, and this regimen was repeated for at least 2 whole courses, at 4-week intervals before the next course of therapy. Those randomized to the control group were subjected to palliative treatment alone. Completely eligible for analysis were 42 cases of the 1/2 FAM group and 41 of the control group. There was no significant difference between the groups with respect to the overall and differentiated survival times according to the tumor sites and the clinical efficacy. As for the duration of 50% inhibition of tumor progression, a significantly better outcome was obtained in 1/2 FAM group. Tumor progression was most significantly inhibited in patients with gallbladder carcinoma. In 1/2 FAM group, tumor reduction was achieved in 1 CR and 2 PR patients. The most frequent adverse reaction was gastrointestinal manifestations, along with diarrhea and alopecia. 1/2 FAM did not contribute to the life prolongation, but inhibited the tumor progression for a significantly longer duration and, to a lesser extent, reduced the tumor size in unresectable pancreatic and biliary tract carcinomas. This regimen is suggested to be useful particularly in the treatment of the latter carcinoma.

Total gastrectomy was performed in a patient with an advanced gastric cancer (stage III). One year and eleven months postoperatively, Krukenberg's tumor was recognized and the prognosis was considered to be poor. A recurrence of ascites was identified. After resection of tumor, CDDP was administered intraperitoneally, and 5-FU tablets were administered orally as a maintenance therapy for a long term. The patient has continued to be in a good condition without any sign of recurrence for three years and six months (ie, five years and five months after previous gastrectomy).

A rare case of lumbo-sacral lipoma in an adult case is reported. A 55-year-old male was admitted to the Department of Neurosurgery, Mazda Hospital, after a history of one year of urinary incontinence. Neurologically, no motor or sensory disturbance of the lower extremities was found in this patient. MRI showed a mass with high signal intensity on T2-weighted image, located between L3 to S2 vertebral segments. Metrizamide-CT scan demonstrated the outline of this hypodense mass at the same location as shown on MRI image. A L3 through L5 laminectomy was performed and the tumor was subtotally removed. Microscopic examination revealed that the tumor mass was made up of mature lipoma cells. Postoperative course of the patient was uneventful. The urinary incontinence was improved slightly. No motor or sensory deficit was found. We thought that MRI was useful for the correct diagnosis of lumbosacral lipoma. And it is best managed by operative removal of the tumor as early as possible after it is diagnosed.

Clinicopathological evaluation of pineocytoma was performed in 4 patients. The subjects, 2 males and 2 females, ranged in age from 17 to 40. All the patients were clinically found to have the symptom of increased intracranial pressure on a monthly basis, but none of them were found to have dorsal midbrain dysfunction symptoms such as Parinaud's sign or Argyll Robertson pupil. Diagnostic imaging produced heterogeneous pictures indicating calcifications and cyst in 2 patients and homogeneous pictures of the tumor parenchyma in the other 2 patients. Histologically, the former cases were found to have many pineal-sand-like calcifications. Median sagittal MR images demonstrated expansive growth of pineocytoma. Quadrigeminal plates which kept their shapes were observed in 2 patients. Craniotomy was performed in all cases, removing the tumor totally in 2 patients. Radiation therapy was given to 3 patients, resulting in complete remission, but radiosensitivity varied according to cases. During the follow-up period of 12 to 42 months, one patient died of peritonitis caused by shunt infection. No recurrence of the tumor was seen in any of the patients. The incidence of pineocytoma was very low. Further evaluation of the tumor involving many cases is advisable.

A case of aspergillotic meningoencephalitis associated with trigeminal neuralgia was reported. The patient, a 41-year-old female, was admitted to our hospital on Nov. 20, 1977 with the chief complaint of right trigeminal neuralgia. On admission right facial paresthesia and right abducens palsy were found. The skull x-ray and tomogram showed enlargement of the right superior orbital fissure. Ct scan revealed an irregular high density around the right superior orbital fissure. The examination of spinal fluid showed 75 mg/dl protein, 72 mg/dl sugar and 11 cells. A biopsy of the mass and trigeminal rhizotomy were performed on Dec. 21, 1977. Microscopically, the specimen was composed of nonspecific granulomatous inflammatory tissue. Thereafter, loss of visual acuity, total ophthalmoplegia and facial paresis gradually appeared on the right side with high fever elevation. Immunologically, peripheral blood lymphocytes responded normally to PHA and PWM, but numbers of lymphocytes showed a tendancy of decrease with deterioration. On the other hand, serum IgG and IgM levels were rather increased. CT scan showed that an irregular high density mass extended to the right orbital apex and the pterygoid fossa. Spinal fluid revealed 260 mmH2O pressure with 76 mg/dl protein, 55 mg/dl sugar and 293 cells, but no organisms were demonstrated. Four months after the operation, swelling of the right subtemporal region became remarkable, in which region a puncture revealed much pus retention, and Aspergillus fumigatus was cultured from the aspirated pus. The patient became comatose and died on May 20, 1978. Autopsy showed thick, yellowish green pseudomembranes covering from the right temporal tip to the basal cistern. The both of cerebral hemispheres were swollen and revealed multiple small softenings all over the brain. Small hemorrhage occupying the right temporal subcortex and microabscess located in the left thalamus were also seen. Microscopical examination disclosed that the pseudomembrane was composed of the necrotizing suppurative inflammation with branched septate hypha of Aspergillus. There have been a few reports of aspergillotic meningoencephalitis associated with trigeminal neuralgia and enlargement of the superior orbital fissure like this case. Some discussion was made on the importance for the diagnosis of aspergillosis to perform fungal culture and histological examination of materials obtained from the inflammatory site, and immunological data of this case was also presented.

Two cases of sphenoethmoidal meningoencephalocele are reported. Patients are a 1 year 7 month old boy and a 3 year old boy. Although sphenoethmoidal meningoencephalocele is reasonably classified as a type of basal meningoencephalocele, the authors could not find out any reports on cases designated as such. The reason must be in difficulty to differentiate the sphenoethmoidal type from the transsphenoidal type. Authors could differentiate them using CT scan. Two other cases were found in cases reported as transsphenoidal type. Further, it is assumed that these four cases including our two cases were accompanied with agenesis of corpus callosum and median cleft lip and palate. Despite the fact that the respective anomalies are rare, they are completely the same, which signifies the possibility that 1) sphenoethmoidal meningoencephalocele, 2) agenesis of corpus callosum and 3) median cleft lip and palate are a single unit of congenital anomalies. It probably should be considered that the cause for these malformations were present even before the formation of the lips which takes place earliest, that is, sixth week of gestation.

Magnetic resonance (MR) findings of two patients with a hypothalamic hamartoma are discussed. The two girls showed clinical symptoms and endocrinological signs of precocious puberty. MR imaging was of diagnostic value superior to that of CT in the demonstration of the characteristic location of this tumor and relationships to the neighboring structures because of its multi-dimensional utility. Although it has been reported that CT showed this lesion as isodense to the grey matter with and without injection of contrast medium, MR imaging depicted the lesion as a high signal intensity area on T 2-weighted images in both patients. MR imaging is a useful method for the evaluation of the hypothalamic hamartoma.

A case of arteriovenous malformation (AVM) with angiographically visualized extravasation of contrast medium on carotid angiography has been reported. The patient, a 9-year-old girl, came to our clinic with chief complaints of unconsciousness and left hemiplegia. On right carotid angiogram, done 4 hours after stroke, an AVM with a large intracerebral hematoma at the region of the right basal ganglia was recognized. The AVM was feeded from several lenticulostriate arteries and a small branch from precentral artery, and drained into thalamostriate vein. The extravasation of contrast medium was seen in a sash like fashion through arterial and venous phase. Extravasation of contrast medium from AVM has been extremely rarely reported, and this is considered as the third reported case. From the study of these 3 cases, we have discussed about the rarity of the extravasation from AVM and the causative factors.

It has been known that significant narrowing of the subclavian or innominate artery may cause cerebrovascular ischemic symptoms, especially vertebrobasilar insufficiency. This condition has been named as the subclavian steal syndrome (SSS). Recently, percutaneous transluminal angioplasty (PTA) has been developed and used in treating occlusive vascular lesions. In this report, we presented a 61-year-old man who suffered from SSS and treated by PTA. He complained of dizziness and dysesthesia on his left upper extremity which got worse on exertion. The left radial pulse was diminished and the left brachial blood pressure was lower about 50 mmHg than the right. Bruits were recognized over the left suprasubclavicular area. Arch aortogram revealed an 80% stenosis of the proximal left subclavian artery and retrograde flow of the left vertebral artery on late arterial phase. PTA was successfully performed at the same time as angiography via the right femoral artery without any complications. His clinical symptoms improved immediately following PTA. A month after PTA, left retrograde vertebral angiogram demonstrated that the dilation of the treated segment of the left subclavian artery continuously remained. Because of several advantages such as a technical easiness and a more safety, PTA will be a useful therapeutic tool for not only SSS but also other obstructive diseases of brachiocephalic arteries prior to some surgical treatments. However, it is necessary to avoid some possible complications, especially embolism into the cerebral circulation in this technique, and the careful procedure should be done under the co-operation with vascular surgeons and radiologists.

We report a case of a 26-year-old woman who had developed decrease of visual acuity, and restriction of the temporal visual field of the left eye in the 30th week of gestation. A skull roentgenogram showed no abnormality, but a pituitary mass was visualized by plain computed tomographic scan. Magnetic resonance imaging (MRI) demonstrated a symmetric sellar mass which had homogeneous signal intensity in all pulse sequences. T1 relaxation time of the mass was elongated as compared with that of a normal pituitary gland. The height of the mass was 11mm, and optic chiasm appeared compressed by the mass. Her visual disturbance improved before delivery, and the size of the pituitary mass regressed spontaneously. Although no histological examination was carried out, the most likely explanation for this phenomenon is lymphocytic adenohypophysitis. Early surgical intervention is not required for lymphocytic adenohypophysitis, because this disorder may be self-limiting, and may resolve itself. Most of these cases have been reported in women, often coincident with pregnancy. So we consider that MRI is the most useful and safest method for diagnosis and follow-up of this disease.