Adenosarcoma of the uterine body initially presenting as an interstitial small tumor of the uterus: a case report.

Adenosarcoma of the uterine body is a rare mixed tumor in which a benign epithelial component is mixed with a malignant stromal element. It has been considered that this tumor originates from the endometrium and its most common finding of imaging is a polypoid tumor occupying the uterine cavity. The authors herein present a case of 37-year-old female with a complaint of abnormal vaginal bleeding. At the first visit, transvaginal ultrasound and magnetic resonance imaging (MRI) showed a round mass with a diameter of one cm in the uterine wall. No malignant pathological finding was detected. The patient visited the authors again one year later, because of continuous bleeding. At that time, they found a polypoid tumor in the uterine cavity, which turned out to be adenosarcoma with sarcomatous overgrowth. The round mass in the uterus detected at first time seems to have been incipience of adenosarcoma. Prodromal sign of adenosarcoma has not been reported previously.

Altered alpha1-syntrophin expression in myofibers with Duchenne and Fukuyama muscular dystrophies.

Alpha1-syntrophin, a scaffolding adapter and modular protein, is a cytoplasmic component of the dystrophin glycoprotein complex. This study investigated immunohistochemically the expression of alpha1-syntrophin in Duchenne and Fukuyama muscular dystrophies (DMD and FCMD, respectively). Biopsied muscles of five DMD, five FCMD, five normal controls and five disease controls (three myotonic and two facioscapulohumeral dystrophies) were analyzed. Immunoblot analysis showed that anti-alpha1-syntrophin antibody had a decreased reaction in both DMD and FCMD muscle extracts. Biopsied muscle sections and their serial sections were immunostained with rabbit anti-alpha1-syntrophin and rabbit anti-muscle-specific beta-spectrin antibodies, respectively. Immunoreactive patterns of sarcolemma were classified into (i) a continuously positive immunostaining pattern, (ii) a partially positive immunostaining pattern, (iii) a negative immunostaining pattern and (iv) a faint but entire surface positive immunostaining pattern. The group mean percentages of alpha1-syntrophin and beta-spectrin immunonegative myofibers in the DMD group were 39.3% and 10.8%, respectively, while those in the FCMD group were 45.5% and 10.4%, respectively. These values were statistically significant compared with those of disease control and normal control muscles. Thus we found that dystrophin-deficient DMD muscles contained significant numbers of alpha1-syntrophin-positive fibers and significant numbers of alpha1-syntrophin-negative fibers were present in dystrophin-positive muscles of severe muscular dystrophy such as FCMD. Alpha-dystrobrevin immunoreactivity was tested in DMD muscles and appreciable amounts of alpha-dystrobrevin that binds to syntrophin were found in DMD muscle membranes.

Altered aquaporin 4 expression in muscles of Fukuyama-type congenital muscular dystrophy.

This study was undertaken to investigate the expression of aquaporin 4 (AQP4) in the muscle plasma membrane of children with Fukuyama-type congenital muscular dystrophy (FCMD) at protein and mRNA levels. The biopsied six muscles with FCMD, six histochemically normal muscles and eight disease control muscles were analyzed by means of immunoblots, immunohistochemistry and reverse-transcription polymerase chain reaction (RT-PCR). Immunoblots showed that the band of FCMD muscle extracts stained with anti-AQP4 antibody was faint in comparison with that of normal muscle extracts. The immunohistochemistry revealed that most of the FCMD myofibers showed negative immunostaining with anti-AQP4 antibody, although the partially positive immunostaining of sporadic FCMD myofibers was noted. The immunoreactivity was positive with anti-dystrophin and anti-beta-spectrin antibodies in almost all FCMD myofibers. The quantitative RT-PCR demonstrated that the AQP4 mRNA level of the FCMD muscles was markedly reduced. On the basis of these findings, we conclude that the expression of AQP4 in FCMD myofibers is reduced and the reduced content of AQP4 mRNA in FCMD muscles may be related to the decreased expression of AQP4 at the muscle plasma membrane of FCMD myofibers.

Aquaporin 4: lack of mRNA expression in the rat regenerating muscle fiber under denervation.

The recently identified water channel aquaporin 4 is a major component of the orthogonal arrays observed with freeze-fracture electron microscopy. We examined the expression of aquaporin 4 mRNA and protein in rat regenerating muscle under innervated and denervated conditions. We found decreased sarcolemmal immunostaining of aquaporin 4 in denervated regenerating muscle as opposed to innervated muscle. Quantitative reverse transcription-polymerase chain reaction revealed that aquaporin 4 mRNA was expressed in the innervated regenerating muscle; whereas it was not expressed in denervated muscle. Thus, lack of aquaporin 4 protein may be due to lack of aquaporin 4 mRNA in the denervated regenerating muscle. We conclude that the nerve supply influences expression of aquaporin 4 at the mRNA level in regenerating muscle.

[Juvenile onset Creutzfeldt-Jakob disease with the history of neurosurgical operation].

Juvenile onset Creutzfeldt-Jakob disease with the history of neurosurgical operation: a case report A 15-year-old boy gradually developed gait disturbance and dementia. After three months, his condition was deteriorated and he became a state of akinetic mutism. Then, he was transferred to our hospital. He had a history of neurosurgical operation for arterio-venous malformation in his right occipital lobe at the age of five. He showed myoclonus, periodic synchronous discharges in electroencephalogram, and a high cerebrospinal fluid level of neuron-specific enolase. He also revealed progressive brain atrophy by CT examination. He was diagnosed as having Creutzfeldt-Jakob disease (CJD). We could not detect gene mutation of prion protein. As far as we know, this is the youngest case of CJD in Japan. He is too young for the sporadic form of CJD. So the possibility of new variant form of CJD or iatrogenic CJD was considered, the former having been reported in England and the neighboring countries in Europe. However, new variant form of CJD was less likely because of the presence of definite PSD in EEG and the absence of the history of his stay in England. The relationship between the CJD onset and the neurosurgical operation was suggested, but no evidence such as frozen dura matter graft was proved.

[Respiratory function impairment in patients with Parkinson's disease--a consideration on the possible pathogenetic relation to autonomic dysfunction].

To investigate the characteristics and clinical significance of respiratory function in patients with Parkinson's disease (PD), we studied 38 patients (male, 19; female, 19: mean age, 65.5 years: mean duration of disease, 6.7 years) who had no history of respiratory disease and smoking. Fifty three non-respiratory disease subjects (male, 26; female, 27: mean age, 67.6 years) were served as age-matched control. We measured spirometry and maximal expiratory flow-volume curve in all patients, and analyzed the relations between respiratory function variables and clinical profiles. The clinical disability of PD was indicated by Hoehn-Yahr (H-Y) scale. The number of PD patients was 15 in H-Y 2, 18 in H-Y 3 and 5 in H-Y 4, respectively. The values of % VC, %FEV 1, FEV 1/FVC, %PEFR, % V50 in H-Y 4 group were significantly smaller than those in H-Y 2 and 3 groups. Small airway dysfunction (SAD) was represented by abnormality of % V25, % V50/V25. The prevalence of impairment in % V25 and % V50/V25 was detected in 13 patients (34.2%) and 15 patients (39.5%), respectively, this was significantly higher than age-matched controls. However, the mean value and prevalence of impairment in % V25, % V50/V25 were not affected by H-Y scale and duration of disease or ideal body weight (%predicted value). Twenty seven patients showed normal ventilatory function based on % VC over 80% and FEV 1/FVC over 70%. The prevalence of impairment in % V25, % V50/V25 was detected in 8 patients (29.6%), 9 patients (33.3%), respectively, among 27 patients with normal ventilatory function. These results suggest that ventilatory dysfunction is concerned with clinical disability but SAD which is independent of clinical disability seen prevalently in patients with PD. It is widely accepted that patients with PD frequently have cardiac or bowel dysfunction based on the visceral autonomic dysfunction. We hypothesize that SAD may also be caused by possible autonomic dysfunction in patients with PD.

[A case of non-herpetic acute encephalitis presenting high intensity lesion at unilateral temparal cortex on MR FLAIR image].

We reported a case of non-herpetic acute encephalitis with unilateral temporal cortex lesion revealed by MR imaging and SPECT study. The patient was an eighteen years old woman who developed tonic-clonic seizure after common cold symptom. She was healthy before this episode. Neurological abnormality was only a single convulsion at onset and there was no other abnormal physical and neurological signs except for low grade fever. Electroencephalogram showed spike and slow wave complex of 2 Hz focused on a right posteriotemporal point (T 6) and an MR FLAIR (fluid-attenuated inversion recovery) image revealed a high signal intensity area at right temporal cortex. There was a decrease of cerebral blood flow in the same portion on SPECT study. This lesion was obscure on T1 and T2 MR images. Cerebrospinal fluid showed pleocytosis with normal glucose level and protein concentration. Bacterial and fungal cultures of CSF were negative and a detection of tubercule bacillus by PCR hybridization method was also negative. Although CSF findings suggested viral infection of CNS, virological study could not demonstrate infections of herpes simplex virus type 1, type 2, varicella-zoster virus, cytomegalovirus, measles virus, mumps virus, Japanese encephalitis virus, and influenza virus type A and B. After infusion of acyclovir and antibiotics, the patient was discharged from our hospital without sequelae of encephalitis. EEG was normal at this point and a high intensity area of MR FLAIR image disappeared two months later. SPECT findings were normalized six months later. The encephalitis presenting unilateral temporal cortex lesion without the infection of herpes simplex virus is thought to be very rare. Our case was distinguished from non-herpetic acute limbic encephalitis by an extent of the lesion and clinical manifestations. MR FLAIR image was useful for the detection of the lesion in this case.

Expression of selectin families and their ligand sialyl Lewis X in the muscles of inflammatory myopathies: an immunohistochemical study.

OBJECT: Adhesion molecules are suggested to play important roles in the pathogenesis of inflammatory diseases. We examined the expression of adhesion molecules in the muscles of human inflammatory myopathies. METHODS: We immunohistochemically studied the expression and distribution of two molecules in the selectin family (E- and P-selectin) and their common ligand sialyl Lewis X in 18 inflammatory myopathies, 13 disease controls, and 16 normal controls. RESULTS: In inflammatory myopathies, E- and P-selectin were upregulated on the surface of blood vessels, especially on the endothelial cells of the venules. Sialyl Lewis X was upregulated in the blood vessels, infiltrating leukocytes, and the surface of some atrophic myofibers. Some control muscles also showed weakly positive staining with these molecules, however, expression of these molecules was most striking in the muscles of inflammatory myopathies. CONCLUSION: The results suggested that these molecules are upregulated in inflammatory myopathies and might play a role in the pathogenesis of inflammatory myopathies.

[Reversible posterior leukoencephalopathy in a patient receiving cyclosporin therapy].

We reported a case of reversible posterior leukoencephalopathy syndrome (RPLS) that occurred during cyclosporin A (CyA) therapy for fulminant hepatitis. A 22-year-old man was given an intravenous drip of interferon-beta, metylprednisolone sodium succinate and CyA, and also received plasma exchange and hemodiafiltration. On the 7th day of the intravenous CyA therapy, in which its dose had been increased from 60 mg/day to 84 mg/day, he became somnolent and had headache, double vision, hallucination and then a generalized tonic-clonic seizure. The blood CyA concentration increased to a level as high as 455 ng/ml. Brain computed tomography (CT) scan without contrast medium revealed symmetric low-density areas in the bilateral occipital white matter and partly in the cortex. T2-weighted magnetic resonance imaging (MRI) showed an increased signal intensity, and single-photon emission CT using 99 mTc showed a hypoperfusion of cerebral blood flow in those areas. After CyA administration was changed to 100 mg/day orally to decrease its uptake in the blood, his consciousness and vision recovered within 4 weeks. Then abnormalities in MRI findings completely disappeared. On the basis of the clinical course and time-sequential change of serum CyA level in this patient, he was diagnosed as having RPLS caused by CyA therapy. Recently, the number of cases of RPLS has increased in the Western countries. However, there are few reports of RPLS after CyA therapy in Japan. From this case, we emphasize that careful following up the patient's neurological findings during CyA therapy is very important and that a cranial MRI is an essential tool for the diagnosis of RPLS.

Immunocytochemical studies of aquaporin 4 in the skeletal muscle of mdx mouse.

Immunostainability of anti aquaporin 4 antiserum was investigated in the muscles of dystrophin deficient mdx mice. Western blot analysis showed that the rabbit antiserum against aquaporin 4 reacted with a 28 kDa protein in extracts of normal mouse quadriceps femoris muscles but did not react with the protein in extracts of quadriceps femoris muscles of mdx mice. Immunoperoxidase staining of the muscles from normal and mdx mice revealed the positive immunoreaction at the myofiber surface of normal mice and the negative, or the faint and discontinuous immunostaining at the surface of mdx myofibers. Immunogold electron microscopy disclosed the localization of aquaporin 4 molecules at the myofiber plasma membranes of normal mice and the localization was consistent with that of orthogonal array particles in the protoplasmic face of normal muscle plasma membrane seen in freeze fracture replicas. This study demonstrated that the density of aquaporin 4 molecules was decreased in the muscle plasma membranes of mdx mice, resulting in the faulty function of mdx myofibers.

Ultrastructural localization of alpha-, beta- and gamma-sarcoglycan and their mutual relation, and their relation to dystrophin, beta-dystroglycan and beta-spectrin in normal skeletal myofiber.

Ultrastructural localization of alpha-, beta- and gamma-sarcoglycan and their mutual relation, and their relation to dystrophin, beta-dystroglycan and beta-spectrin were investigated in normal skeletal myofibers. Single-immunogold labeling electron microscopy showed that the signals of rabbit and sheep polyclonal antibodies against the synthetic peptide of the cytoplasmic domain of alpha-, beta or gamma-sarcoglycan were present along the inside surface of muscle plasma membrane and at the sarcoplasmic side of plasma membrane invaginations and vesicular structures in subsarcolemmal areas. These localizations were similar to that of dystrophin, beta-dystroglycan and beta-spectrin. Double-immunogold labeling disclosed the close association of alpha-, beta- and gamma-sarcoglycan each other and alpha-, beta-, gamma-sarcoglycan with dystrophin or beta-dystroglycan, and this was confirmed by statistical analysis. Monoclonal antibody against the extracellular domain of alpha-sarcoglycan was used with above-mentioned polyclonal anti-beta- and -gamma-sarcoglycan antibodies for triple-immunogold labeling, in which signals of alpha-sarcoglycan localized at the outer surface of muscle plasmalemma and those of beta- and gamma-sarcoglycans were present at the inside surface of plasma membrane. The triple immunolabeling showed an occasional closely associated presence of the three signals for alpha-, beta-and gamma-sarcoglycans, and a more frequent association for two signals out of alpha-, beta- and gamma-sarcoglycans. This study demonstrated that alpha-, beta- and gamma-sarcoglycan are closely located to one another and to dystrophin and beta-dystroglycan at the muscle plasma membrane.

The expression of cytokine mRNA in the cauda equina of Lewis rats with experimental allergic neuritis.

Autoreactive CD4+ T cells can transfer experimental allergic neuritis (EAN) to naive recipients. In order to further analyze the role of these T cells and their corresponding cytokines in EAN, we studied the expression of mRNA for IFN-gamma, IL-4, and IL-10 in the cauda equina of rats with EAN using a quantitative competitive reverse transcriptase PCR method. Nerves were studied on days 0 (pre-immunization), 10 (disease onset), 13 (clinical progression), 16 (disease peak), as well as 20, 24, and 34 post immunization (recovery). IFN-gamma messages increased at disease onset and peaked at day 13 p.i. IL-10 message remained at a very low level at disease onset and surged at day 16. Both messages were low in recovery stage. IL-4 message was undetectable at any time point. These data suggest a pro-inflammatory role of IFN-gamma and anti-inflammatory role of IL-10 in EAN lesions. It is also possible that a clonal switch from Th1 to Th2 occurs in EAN lesions during the disease course.

Ultrastructural localization of alpha 1-syntrophin and neuronal nitric oxide synthase in normal skeletal myofiber, and their relation to each other and to dystrophin.

We investigated the ultrastructural localization of alpha 1-syntrophin and neuronal nitric oxide synthase (nNOS) in normal human skeletal myofibers and analyzed their relation to each other and to dystrophin using single and double immunogold-labeling electron microscopy. Single immunolabeling showed antibodies to alpha 1-syntrophin and nNOS on the inner surface of the muscle plasma membrane, the sarcoplasmic side of plasma membrane invaginations, and the sarcoplasm near mitochondria of subsarcolemmal areas. The epitopes of alpha 1-syntrophin and nNOS tended to be present in clusters. Double immunolabeling revealed that epitope combinations of alpha 1-syntrophin-dystrophin, alpha 1-syntrophin-nNOS, and nNOS-dystrophin occurred more frequently in doublet form than did other epitope combinations, such as alpha 1-syntrophin-beta- spectrin and nNOS-beta-spectrin. These increased frequencies were noted both at the muscle plasma membrane undercoat and near mitochondria of subsarcolemmal areas. A significantly higher percentage of doublets comprised antibodies against alpha 1-syntrophin and dystrophin (28.5 +/- 1.5%, group mean +/- SE) than those against alpha 1-syntrophin and beta-spectrin (9.2 +/- 0.8%, P < 0.01). Furthermore, nNOS formed doublets significantly more frequently with dystrophin (25.2 +/- 3.3%) and alpha 1-syntrophin (26.0 +/- 4.1%) than with beta-spectrin (13.9 +/- 2.3%; P < 0.05). These data support the association of dystrophin, alpha 1-syntrophin, and nNOS at the inner surface of the muscle plasma membrane and near mitochondria of subsarcolemmal areas of normal human skeletal myofibers.

[Multiple solitary lesions in deep white matter in the early stage of Creutzfeldt-Jakob disease. A case report].

A 56-year-old housewife was admitted to our hospital because of involuntary movement on her left arm. Her neurological examination on admission showed mild weakness of her left arm and cerebellar ataxia. She developed periodic synchronous discharge on electroencephalogram and her cerebrospinal fluid revealed elevated level of neuron-specific enolase. Thereafter she developed dementia, followed by apallic state and diagnosed as having Creutzfeldt-Jakob disease (CJD). Interestingly, her MRI on admission revealed multiple solitary lesions in deep cerebral white matter, which were detected as high signal intensity by T2 weighed image. A few months later, these lesions tended to extend, and finally fused around the lateral ventricle in parallel with remarkable cortical atrophy. We excluded other diseases such as cerebrovascular disorders. Finally, we concluded the white matter change seen from early stage in this case may be the lesion associated with CJD, and the CJD case with early white matter changes has been seldomely described.

[Allergic bronchopulmonary aspergillosis successfully treated with itraconazole].

A 67-year-old man was admitted to our hospital because of coughing, a low-grade fever, and abnormal shadows on a chest X-ray film. He had had asthma as a child, but had no asthmatic symptoms on admission. A CT scan showed collapse of the right middle lobe and mucoid impactions in the lingula. Bronchoscopy revealed thick mucus obstructing the right middle-lobe bronchus and the left upper-lobe bronchus. The eosinophil count and the IgE level were abnormally high. Aspergillus fumigatus was detected in his sputum. Tests for immediate skin reaction and precipitating antibody to aspergillus antigen were positive. After treatment with itraconazole he became asymptomatic. Radiographic abnormalities had resolved by 1 month after the start of treatment; a high resolution CT scan obtained after clinical improvement revealed central bronchiectasis. In this patient with allergic bronchopulmonary aspergillosis, a course of itraconazole alone was followed by satisfactory improvement.