Pathologic and immunologic profiles of a limited form of neuromyelitis optica with myelitis.

BACKGROUND: Neuromyelitis optica (NMO) is a demyelinating syndrome characterized by myelitis and optic neuritis. Detection of anti-NMO immunoglobulin G antibody that binds to aquaporin-4 (AQP4) water channels allows the diagnosis of a limited form of NMO in the early stage with myelitis, but not optic neuritis. However, the detailed clinicopathologic features and long-term course of this limited form remain elusive. METHODS: We investigated 8 patients with the limited form of NMO with myelitis in comparison with 9 patients with the definite form. RESULT: All patients with limited and definite form showed uniform relapsing-remitting courses, with no secondary progressive courses. Pathologic findings of biopsy specimens from the limited form were identical to those of autopsy from the definite form, demonstrating extremely active demyelination of plaques, extensive loss of AQP4 immunoreactivity in plaques, and diffuse infiltration by macrophages containing myelin basic proteins with thickened hyalinized blood vessels. Moreover, the definite form at the nadir of relapses displayed significantly higher amounts of the inflammatory cytokines interleukin (IL)-1beta and IL-6 in CSF than the limited form and multiple sclerosis. CONCLUSION: This consistency of pathologic findings and uniformity of courses indicates that aquaporin 4-specific autoantibodies as the initiator of the neuromyelitis optica (NMO) lesion consistently play an important common role in the pathogenicity through the entire course, consisting of both limited and definite forms, and NMO continuously displays homogeneity of pathogenic effector immune mechanisms through terminal stages, whereas multiple sclerosis should be recognized as the heterogeneous 2-stage disease that could switch from inflammatory to degenerative phase. This report is a significant description comparing the pathologic and immunologic data of limited NMO with those of definite NMO.

Sharing of polyglutamine localization by the neuronal nucleus and cytoplasm in CAG-repeat diseases.

The expansion of a trinucleotide cytosine adenine and guanine (CAG) repeat that codes for polyglutamine is a common gene mutation in the family of hereditary neurodegenerative diseases that includes Machado-Joseph disease (MJD) and dentatorubral-pallidoluysian atrophy (DRPLA). The presence of ubiquitinated neuronal intranuclear inclusions (NIIs) has been recognized as a neuropathological hallmark of these diseases, although the significance of NIIs in the pathogenesis remains a matter of controversy. In a previous study of DRPLA, we proposed that intranuclear diffuse accumulation of mutant proteins is another pathological characteristic of neurones, and that the variable prevalence of this characteristic may be relevant to the variation of clinical symptoms in patients with different repeat sizes. Recently, we also disclosed that polyglutamine tracts are localized in a subset of lysosomes in affected neurones. The present immunohistochemical study of autopsied MJD and DRPLA brains shows that the nucleus and cytoplasm of affected neurones share the subcellular distribution of expanded polyglutamine tracts, the pattern of distribution being specific to each diseased brain. The results suggest that in CAG-repeat diseases, mutant proteins are involved in both the ubiquitin/proteasome and endosomal/lysosomal pathways for protein degradation in different intraneuronal compartments, where their accumulation may exert distinct influences on neuronal physiology.

Magnetic resonance imaging of hyaline cartilage regeneration in neocartilage graft implantation.

BACKGROUND: The purpose of this study was to investigate the regenerative potential of hyaline cartilage in a neocartilage graft implant with the aid of MR cartilage imaging using a rabbit model. METHODS: Surgical osteochondral defects were created in the femoral condyles of 30 mature New Zealand rabbits. The findings of neocartilage in autologous cartilage grafts packed into osteochondral defects were compared with control group of no implant to the osteochondral defect. The outcome of the implantations was correlated with histologic and MR cartilage imaging findings over a 3-month interval. RESULTS: Neocartilage grafts packed into osteochondral defects showed regeneration of hyaline cartilage at the outer layer of the implant using MR cartilage imaging. Fibrosis of fibrocartilage developed at the outer layer of the autologous cartilage graft together with an inflammatory reaction within the osteochondral defect. CONCLUSION: This animal study provides evidence of the regenerative ability of hyaline cartilage in neocartilage transplants to repair articular cartilage.

Histopathological and CT features of pulmonary sclerosing haemangiomas.

AIM: To demonstrate the computed tomography (CT) features of pulmonary sclerosing haemangiomas. MATERIALS AND METHODS: Six pathologically proven sclerosing haemangiomas were included in this retrospective review. Patients consisted of five women and one man aged 20-54 years (mean, 34.5 years). Their CT features were recorded according to enhancement patterns and the presence of a tail sign, prominent pulmonary artery sign, air-trapping sign, presence of calcification or cystic spaces, consolidation, and interstitial infiltration. The predominant composition of an individual sclerosing haemangioma was documented by means of microscopy. RESULTS: Inhomogeneous enhancement was frequently present and depended on the various compositions of the tumours, especially those in sclerotic and predominantly papillary predominant types. The presence of a tail sign, intra-tumoural cystic areas, and a prominent artery sign were the frequent features in our cases. CONCLUSION: Sclerosing haemangioma should be considered in young or middle-aged female patients whose CT images show them having an inhomogeneous enhancing soft tissue mass with a smooth outline and with above the aforementioned features.

Hepatic inflammatory mass secondary to toothpick perforation of the stomach: triphasic CT appearances.

Unintentional ingestion of foreign bodies is common in daily life. The unintentional ingestion of sharp foreign bodies such as toothpicks, fish bones and chicken bones carries a significant risk of perforation of the gastrointestinal tract. Herein, we report a case of hepatic inflammatory mass complicated by the perforation of an ingested toothpick that had a distinctive enhancement pattern on dynamic computed tomograms (CT). Understanding the phenomenon of enhancement in triphasic CT study is the basis in differentiating liver neoplasms.

Diagnostic sensitivity of ultrasound-guided needle biopsy in soft tissue masses about superficial bone lesions.

We evaluated the value of ultrasound-guided needle biopsy in 20 soft tissues masses about superficial bone lesions in 20 oncology patients. Sonographically guided needle biopsies were performed without an on-site pathologist. A diagnostic sensitivity of 95% and specificity of 100% in separating a benign or a malignant lesion was obtained. Fine needle aspiration cytology allowed the specific cell type of malignancy to be diagnosed in 80% of cases, while core needle biopsy allowed it in 91%. Real-time ultrasonographic guidance permits precise needle placement into the targets, avoidance of hypervascular areas, and flexibility of patient positioning so that needle biopsy can be performed quickly and safely on soft tissue masses about superficial bone lesions.

Dynamic CT features of mediastinal hemangioma: more information for evaluation.

Dynamic enhancing feature in mediastinal hemangioma was rarely reported. Slow, gradually increasing and prolonged contrast stains are the diagnostic hints in our case that avoid the hemorrhagic catastrophe from transthoracic core needle biopsy. A rare finding of an aberrant vessel entering into the tumor was valuable for operative planning. Dynamic computed tomography (CT) can provide more information for evaluation in such cases.

Biliary ascariasis: CT, MR cholangiopancreatography, and navigator endoscopic appearance--report of a case of acute biliary obstruction.

A case of acute common bile duct obstruction caused by a roundworm in a 6-year-old girl is reported. Computed tomography (CT) and magnetic resonance imaging (MRI) showed "bull's-eye" and "eye-glass" appearances of the ascaris in the common bile duct. On reformation of the transverse CT and coronal MR images, the tubular Ascaris was better depicted. MRI and navigator endoscopic demonstrations of the common bile duct ascaris have not been described previously in the literature. These CT and MRI findings may be helpful in the diagnosis of Ascaris.

Lymphangiectasia within angiomyolipoma in a tuberous sclerosis patient.

This is a presentation of a case of tuberous sclerosis with multifocal hamartomas in the retroperitoneum, both kidneys, and the liver. Lymphangiectasia and numerous ectatic lymphatic channels penetrating through the retroperitoneal angiomyolipoma were demonstrated by lymphography and computed tomography. The findings in our study may document the presence of degenerative change in the lymphatic system--not just in elastic vessels--an association which has not yet been reported in the radiological literature.

Total knee arthroplasty in a rheumatoid arthritic knee with large geode: a case report.

Geodes (subchondral cysts) are a well-known manifestation of rheumatoid arthritis. Solitary cysts or cysts larger than 2 cm are not generally found in the knee joint of patients with rheumatoid arthritis (RA). We report a case of RA involving both knees with a giant geode over the right proximal tibia. Surgical treatment was performed including synovectomy, cyst enucleation and packing of autogenous bone chips followed by primary total knee arthroplasty. The postsurgical result was excellent with the knee restored to good function and complete healing of the cystic lesion.

MRI of multiple biliary hamartomas.

Multiple biliary hamartomas (MBH) are uncommon benign malformations of the bile ducts. A case of MBH simulating liver metastases is reported, the diagnosis having been ascertained by US-guided biopsy. US and CT showed multiple hepatic nodules scattering in both lobes of the liver. Angiography showed small hypovascular lesions as well as hypervascular nodules. Only two cases with MRI studies have previously been reported. In this case, the finding was of hepatic cysts containing intermediate signal mural nodules. Demonstrating hepatic cysts with mural nodules on imaging will considerably increase the accuracy of diagnosis.

CT findings of bronchial transection.

A case of right bronchial rupture demonstrated by computed tomography (CT) is reported. Chest radiographs of a 55-year-old man who sustained blunt chest trauma showed bilateral pneumomediastinum, hydropneumothorax, and subcutaneous emphysema with fracture of the left 3rd and 4th ribs. CT showed wall defect over the right main bronchus with the air in airway contiguous to that in the mediastinum. Bronchoscopy and thoracostomy confirmed the CT findings and the patient was treated by bronchorrhaphy with end-to-end anastomosis. The airway should be meticulously evaluated in cases of mediastinal and subcutaneous emphysema, especially after trauma.