MR imaging features of a scalp plexiform schwannoma

Radiologic findings of a case with scalp plexiform schwannoma-an unusual variant of the benign, solitary schwannoma in the skin-are reported. T2-weighted MR imaging exhibited the most specific features: a multinodular pattern and hypointense capsule that separated the tumor from surrounding soft tissue. A surgical specimen was histologically confirmed as schwannoma. The MR imaging findings reported herein may aid in the preoperative diagnosis of this relatively rare scalp tumor.     

MR imaging findings of spinal posterior column involvement in a case of Miller Fisher syndrome

Summary: The site of lesions causing ataxia in Miller Fisher syndrome (MFS) remains in dispute. A 43-year-old man manifested rapidly progressive left-sided ptosis, bilateral abducens palsy, areflexia, and severe ataxia. Initial MR imaging showed confined lesions of the cauda equina with gadolinium enhancement. A diagnosis of MFS was made, and the patient underwent immunotherapy. His ophthalmoplegia disappeared, but other symptoms remained. Five months after onset, MR imaging disclosed lesions confined to the spinal posterior column, which were considered to result from involvement of posterior nerve roots of the cauda equina and to be responsible for his remaining severe ataxia.     

Establishment of a binding assay for protein kinase C isozymes using synthetic C1 peptides and development of new medicinal leads with protein kinase C isozyme and C1 domain selectivity

Conventional and novel protein kinase C (PKC) isozymes contain two cysteine-rich C1 domains (C1A and C1B), both of which are candidate phorbol-12, 13-dibutyrate (PDBu)-binding sites. We synthesized C1 peptides of 50-70 residues corresponding to all PKC isozyme C1 domains using an Fmoc solid-phase strategy. These C1 peptides were successfully folded by zinc treatment, as monitored by electrospray ionization time-of-flight mass spectrometry. We measured the K(d)'s of [3H]PDBu for all PKC C1 peptides. Most of the C1 peptides, except for delta-C1A and theta-C1A, showed strong PDBu binding affinities with K(d)'s in the nanomolar range (0.45-7.4 nM) comparable with the respective whole PKC isozymes. The resultant C1 peptide library can be used to screen for new ligands with PKC isozyme and C1 domain selectivity. Non-tumor-promoting 1-oleoyl-2-acetyl-sn-glycerol and bryostatin 1 showed relatively strong binding to all CIA peptides of novel PKCs (delta, epsilon, and eta). In contrast, the tumor promoters (-)-indolactam-V, ingenol-3-benzoate, and PDBu bound selectively to all C1B peptides of novel PKCs. The preference of tumor promoters for the domain might be related to tumorigenesis since recent investigations proposed the involvement of novel PKCs in tumor promotion in vivo using transgenic or knockout mice. Moreover, we recently have found that a new lactone analogue of benzolactams (6) shows significant selectivity in PKCeta-C1B binding.     

High-risk age for rebleeding in patients with hemorrhagic moyamoya disease: long-term follow-up study

OBJECTIVE: The prevention of rebleeding is one of the most important issues in the successful treatment of moyamoya disease with hemorrhagic onset. However, the natural course of hemorrhagic moyamoya disease and the characteristics of rebleeding remain unclear. METHODS: To study the natural course of the disease in conservatively treated patients, we analyzed long-term follow-up reports on 36 patients (12 male and 24 female) treated without bypass surgery at our hospitals before 1994 (mean follow-up, 12.7 +/- 7.1 yr; range, 2.9-27.0 yr). RESULTS: Of our 36 patients, 22 (61.1%) experienced rebleeding; there were 29 rebleeding episodes. The outcomes in patients with rebleeding were worse than in patients who did not rebleed (P < 0.05); in most cases, unsatisfactory results were attributable to rebleeding. Kaplan-Meier and multivariate analysis of rebleeding-free survival showed that age at onset (>36 yr) was a statistically significant risk factor; sex, hypertension, type of intracranial bleeding, and outcome after the first bleeding episode were not. Although the intervals between the first and subsequent episodes varied, both the rebleeding rate and the number of rebleeding episodes were highest in patients 46 to 55 years old. CONCLUSION: Rebleeding is the most important factor in unsatisfactory outcomes of patients with hemorrhagic moyamoya disease. Rebleeding seems to be age-related; rebleeding occurs at an increased rate when patients reach the age range of 46 to 55 years. Patients with hemorrhagic moyamoya must be followed up throughout their lives, even if their neurological status is excellent.     

Tryptanthrin inhibits interferon-gamma production by Peyer's patch lymphocytes derived from mice that had been orally administered staphylococcal enterotoxin

Tryptanthrin, a biologically active compound found in the medicinal plant Polygonum tinctorium, reportedly has several biological activities. We investigated the effects of tryptanthrin on cytokine production by lymphocytes in response to staphylococcal enterotoxin B (SEB), which causes a variety of disorders in humans based on its induction of large amounts of immunostimulatory cytokines. Tryptanthrin dose-dependently inhibited interferon-gamma (IFN-gamma) and interleukin-2 production by mouse spleen cells and Peyer's patch (PP) lymphocytes in vitro. The efficacy of tryptanthrin was further studied in a mouse model in vivo. Tryptanthrin was administered orally 2 h after an oral challenge with SEB. Nineteen hours after SEB administration, PP lymphocytes were prepared, and IFN-gamma production by PP lymphocytes was examined. The production of IFN-gamma increased after SEB administration, and the elevated IFN-gamma production was significantly inhibited by tryptanthrin treatment. These results suggest that tryptanthrin may be effective in the treatment of disorders of the intestines, such as food poisoning, that are associated with activated lymphocytes.     

GPi pallidotomy for Parkinson's disease with drug-induced psychosis

Levodopa-induced psychosis may seriously threaten the ability of patients with Parkinson's disease (PD) to continue leading an independent life. A retrospective assessment of the therapeutic effects of the globus pallidus internus (GPi) pallidotomy on the activities of daily living (ADL) of seven PD patients presenting with mild or moderate degrees of psychosis was carried out. Their scores according to the Unified Parkinson's Disease Rating Scale (UPDRS) Part I-2 (maximum=4) were 2 or 3 (mean +/- SD=2.4 +/- 0.5). Bilateral procedure was needed in 5 out of 7 patients to obtain sufficient improvement of motor symptoms. At 3 months after surgery, UPDRS part III motor scores in the 'off' state were significantly decreased and motor fluctuations were abolished. Nevertheless, their score of Schwab and England (S-E) ADL scale scores responded poorly to the surgery, while the scores in other 12 patients without psychosis was significantly improved after pallidotomy. The data indicate that GPi pallidotomy ameliorates the motor symptoms in patients with drug-induced psychosis (DIP), but has no significant impact on their consequent daily activities. A regression model for all 19 patients who underwent pallidotomy revealed that postoperative S-E scale was affected by the preoperative UPDRS Part I-2 rather than by Part III motor score. The present study suggested that DIP, even if its degree is not severe, may be a limiting factor of the therapeutic potential of pallidotomy in patients with PD.     

Preventive effect of Coriandrum sativum (Chinese parsley) on localized lead deposition in ICR mice

The preventive effect of Coriandrum sativum, Fam. UMBELLIFERAE (Chinese parsley) on lead deposition was investigated in male ICR mice given lead (1000 ppm) as lead acetate trihydrate in drinking water for 32 days. Administration of Chinese parsley to mice by gastric intubation was performed for 25 days from day 7 after the start of lead exposure up to the end of the experiment. The mice were then sacrificed for comparison of lead distribution. The lead reached its highest concentration in the femur but localized lead deposition in the femur was significantly decreased by meso-2,3-dimercaptosuccinic acid (DMSA), a chelating agent used as a positive control to validate this experimental model. Administration of Chinese parsley also significantly decreased lead deposition in the femur and severe lead-induced injury in the kidneys. In addition, urinary excretion of delta-aminolevulinic acid (ALA) which is known to increase with lead intake was significantly decreased after administration of Chinese parsley. The MeOH extract of Chinese parsley also reduced lead-induced inhibition of delta-aminolevulinic acid dehydratase (ALAD) activity in vitro. These results suggest that Chinese parsley has suppressive activity on lead deposition, probably resulting from the chelation of lead by some substances contained in Chinese parsley.     

Successful treatment with bypass and interventional surgery for a ruptured pseudo carotid artery aneurysm after transsphenoidal surgery: a case report

We report case of a patient who suffered a pseudo internal carotid artery (IC) aneurysm following transsphenoidal surgery. He was successfully treated with bypass surgery and IC occlusion involving the pseudoaneurysm using Guglielmi detachable coils (GDCs). This 50-year-old man with recurrent FSH-releasing pituitary adenoma suffered profuse arterial bleeding during transsphenoidal surgery. The hemorrhage was managed, using oxidized cellulose with bio-bond. His postoperative course was uneventful, but, he developed massive epistaxis 20 days after surgery. Cerebral angiograms showed a pseudoaneurysm arising from the C4 portion of the left IC. He could not tolerate the balloon occlusion test. Using GDCs, we immediately performed left IC occlusion involving the pseudoaneurysm followed by bypass surgery between the left EC and the left middle cerebral artery. Postoperative angiograms showed that the pseudoaneurysm was completely occluded and the bypass was fully patent. When massive arterial bleeding is encountered during transsphenoidal surgery, the patient should be carefully monitored to detect early the development of a pseudoaneurysm. When such an aneurysm is found or has ruptured, interventional surgery has proved effective in the management of this complication.     

Endovascular treatment for facial arteriovenous malformations using new particles: report of two cases

Facial arteriovenous malformations (FAVM) are difficult to treat because of their highly vascular networks. Intravascular treatment using liquid material to occlude the FAVM occasionally results in skin necrosis after embolization. The use of particulate materials to obliterate the nidus often fails to obtain a permanent cure due to arterial recanalization. We report two patients with FAVM who were successfully treated with endovascular embolization using a new type of particulate material. One patient was treated with embolization only, the other was treated with embolization followed by surgical resection. Both patients showed clinical and angiographic improvement. Intravascular treatment using particles with a smooth surface and optimal size is safe and effective in the treatment of patients with FAVM.     

Suprasellar hemangioblastoma in a patient with von Hippel-Lindau disease confirmed by germline mutation study: case report and review of the literature

BACKGROUND: Hemangioblastoma (HBL) in the suprasellar region is extremely rare.CASE DESCRIPTION: A suprasellar mass was found in a 33-year-old woman with retinal HBL and bilateral adrenal pheochromocytomas. The diagnosis of von Hippel-Lindau (VHL) disease was confirmed preoperatively not only by these clinical manifestations but also by germline mutation study. The existence of VHL disease indicated a diagnosis of HBL for the suprasellar mass. The results of our mutation study indicated that this patient had type II VHL disease, suggesting that careful follow-up is essential for the early detection of renal cell carcinoma, which is often associated with type II VHL disease. Here, we summarize the previously reported features of sellar and suprasellar HBLs.CONCLUSIONS: HBLs in this region may be one manifestation of VHL disease. Genetic testing of the VHL gene of our patient could provide useful information to determine appropriate medical care and management.