Detection of novel rearrangement of the JC virus gene in a case of progressive multifocal leukoencephalopathy with adult T-cell leukemia]

A 53-year-old man with adult T-cell leukemia (ATL) developed progressive left hemiparesis and left homonymous hemianopsia. Magnetic resonance imaging (MRI) one month later showed multiple high-intensity lesions in the white matter of both occipital lobes, with predominance in the right side. Detection of JCV genome with polymerase chain reaction in his cerebrospinal fluid subsequently confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML). He was admitted to our hospital. The serum level of soluble interleukin-2 receptor in the patient increased, and both edema and new Gd-enhanced lesions were observed in the cortex of the occipital lobe. He was treated with systemic administrations of Pirarubicin. Cyclophosphamide, and Prednisolone. as well as intrathecal injection of Methotrexate and Cytarabine. Although these treatments temporarily alleviated the symptoms of PML. the ATL spread to the liver and kidney. He died of multiple organ failure. Analysis of his JCV genes revealed that there were three types of rearrangements in the regulatory domains of the JCV genes. All three types lacked the domain B. and two had duplicate domain A. This is the first report of the simultaneous detection of three different types of rearrangements in JCV genes in a single patient. It has been reported that white-matter lesions caused by typical PML are not enhanced in Gd-MRI. However. the lesions seen in this patient were enhanced in Gd-MRI. Such enhancement might be attributable to the modification of the lesions through the direct invasion of ATL cells to the central nervous system.     

A case of coronary sinus rupture following retrograde coronary perfusion for myocardial protection

A 48-year-old woman was admitted to our hospital suffering from chest and back pain. The aortogram and CT scanning revealed aortic dissection (DeBakey II type). Six days after onset, the emergent operation was carried out under the cardio-pulmonary bypass. Myocardial protection was made by retrograde coronary sinus perfusion (RCSP) with cold GIK. The ascending aorta was replaced with an artificial graft including the entry. Massive bleeding in the pericardial space and the rupture of coronary sinus was recognized immediately after declamping of the aorta. Repair was made successfully under induced electric ventricular fibrillation. Care for RCSP was discussed.     

Diagnostic/prognostic significance of photo-evoked eyelid microvibration in neonates with intracranial hemorrhage

Photo-evoked eyelid microvibration (PEMV) was recorded in neonates with intracranial hemorrhage in order to determine whether it serves as a useful indicator of clinical course and also prognosis. Although the latency of PEMV was prolonged or absent in the acute stage of ICH, it returned to a normal range in these subjects with the improvement of symptoms. Three infants whose PEMV were still prolonged in the convalescent stage developed mental retardation or cerebral palsy. PEMV may be a useful predictor of the prognosis of neonatal ICH and its prolongation may signal later neurological sequelae.     

Difference in central projection of primary afferents innervating facial and intraoral structures in the rat

Transganglionic transport of horseradish peroxidase-wheat germ agglutinin conjugate was used to study the central projection of primary afferent neurons innervating facial and intraoral structures. The examined primary neurons innervating the facial structures were those comprising the frontal and zygomaticofacial nerves and those innervating the cornea, while the primary neurons innervating the intraoral structures included those innervating the mandibular incisor and molar tooth pulps and those comprising the palatine nerve. The primary afferents innervating the facial structures project to the lateral or ventral parts of the trigeminal principal, oral and interpolar subnuclei, and to the rostral cervical spinal dorsal horn across laminae I through V, with a greater proportion being directed to the spinal dorsal horn. The primary afferents innervating the intraoral structures terminate in the dorsomedial subdivisions of the trigeminal principal, oral and interpolar subnuclei, and in laminae I, II, and V of the medial medullary dorsal horn, with a much denser projection being distributed to the rostral subnuclei. In addition to the above brain stem trigeminal sensory nuclear complex, they project to the supratrigeminal nucleus, caudal solitary tract nucleus, and paratrigeminal nucleus. These observations agree with previously reported data that the central projection of trigeminal nerve is organized in different manners for the facial and intraoral structures. Furthermore, the present findings in conjunction with our previous studies clarify that the central projection of primary afferents from the facial skin is organized in a clear somatotopic fashion and that the terminal fields of primary afferents from the intraoral structures extensively overlap in the brain stem trigeminal nuclear complex particularly in its rostral subdivisions. The central mechanism of trigeminal nociception is discussed with particular respect to its difference between the facial and intraoral structures.     

Pseudo-adrenal incidentaloma: magnetic resonance imaging in a patient with para-adrenal Castleman's disease.

We report a case of para-adrenal angiofollicular lymph node hyperplasia (Castleman's disease) of the hyaline-vascular type. The mass could not be differentiated from an adrenal tumor by ultrasonography and computed axial tomography (CT). However, magnetic resonance imaging (MRI) suggested the possibility of an extra-adrenal origin of the mass. The intensity of the mass by MRI was homogeneous and of a higher intensity in the T2-weighted image than in the T1-weighted image, a finding similar to lymphadenopathy, lymphatic tumorous mass or metastatic tumor of the lymph node. Ultrasonography, CT and MRI may not be useful in characterizing Castleman's disease, but MRI was useful to distinguish asymptomatic para-adrenal masses from those of adrenal origin.     

Surgical treatment of native valve endocarditis]

Twenty eight patients with native valve endocarditis (NVE) were subjected to this study. Thirteen patients underwent an operation at the chronic phase, and 15 patients at the active phase. One of the 13 patients at the chronic phase died of cardiac rupture due to myocardial infarction which had occurred preoperatively, and one of 10 patients at active phase without annular infection died of rupture of mycotic cerebral aneurysm early postoperatively. Among 5 patients at the active phase with annular infection, prosthetic valve endocarditis occurred in one patient 1.5 months after supraannular aortic valve replacement, and the second operation with a translocation technique was needed. This patient was lost from low output syndrome. Another patient in this group, who underwent a translocation technique because of mycotic annular abscess, died of intestinal infarction late postoperatively. The other 24 patients went a good postoperative course. Five patients with annular infection at the active phase had a shorter duration from the infectious onset to operation (20 days to 2 months, average 38 days), and the causative microorganisms were streptococcus faecalis, staphylococcus epidermidis and gram-negative coccus. One patient, who died of mycotic cerebral aneurysmal rupture, had candida albicans as a causative microorganism. For patients with NVE, an early aggressive operation is essential before infection extends to the annulus or to other vital organs, especially when these microorganisms are identified.     

Clinical and hemodynamic sequelae of mitral prostheses evaluated by Doppler echocardiography]

To determine the relative superiority of a prosthesis in the mitral position, in vivo hemodynamics were examined by Doppler echocardiography, and the results were compared with other types of mechanical mitral valves including 63 Bj?rk-Shiley convexo-concave (BS) values, 30 Duromedics (DM) valves, and 58 Medtronic Hall (MH) valves. For this comparison, the following indices were evaluated: peak velocity of mitral flow (PV), mitral valve orifice area (MVA), mitral valvular regurgitation, New York Heart Association (NYHA) classification, pulmonary capillary wedge pressure (PC), cardiac index (CI) and valve-related complications. On Doppler echocardiograms, PV ranged from 1.2 to 2.0 m/sec with a mean of 1.6 m/sec. There was no evident relationship between the PV and the valve size in each type of prosthesis, and no significant difference in the PVs among the valves. The mean MVA was 2.6 cm2 (25 mm DM, 25 mm MH), which was regarded satisfactory from a clinical standpoint. MVA increased with the increase in the valve size in all types of valves, and of all sizes, MVA was larger in the DM and MH groups than in the BS group. Similarly, the incidence of valvular regurgitation was relatively low in all groups, and the degree of regurgitation proved to be grade II or less in all cases. As for the clinical results, clinical symptoms (NYHA) and hemodynamic states (PC, CI) improved postoperatively, with the differences among the types of prosthetic valves being insignificant. The incidences of thromboembolism, valvular thrombosis, valve failure and prosthetic endocarditis were relatively low in all groups.(ABSTRACT TRUNCATED AT 250 WORDS)     

Genetic changes in JC virus possibly associated with progressive multifocal leukoencephalopathy]

Progressive multifocal leukoencephalopathy (PML) is a fetal demyelinating disease in the central nervous system. PML was once a rare disease, but it is now relatively common among AIDS (acquired immunodeficiency syndrome) patients. The immunological state of patients mainly contributes to the pathogenesis of PML. Genetic changes of the etiological agent, however, may also be involved in the development and progression of the disease. The major genetic changes possibly associated with PML include the regulatory region rearrangement and the VP1 loop mutation. Both changes have been identified as genetic changes usually occurring in JCV (JCvirus) DNAs from the brain and cerebrospinal fluid of PML patients. Although it remained to be clarified how these changes are involved in the pathogenesis of PML, accumulating evidence suggests that the VP1 loop mutation is associated with the progression of PML. Here we overview studies (mainly those performed by ourselves) on these genetic changes.