POEMS syndrome with Guillan-Barre syndrome-like acute onset: a case report and review of neurological progression in 30 cases.

POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome is a rare cause of demyelinating neuropathy with monoclonal plasma cell proliferation, and POEMS neuropathy is usually chronically progressive. Herein, the authors report a 34-year-old woman with POEMS syndrome presenting as acute polyneuropathy. Within 2 weeks of disease onset, she became unable to walk with electrodiagnostic features of demyelination and was initially diagnosed as having Guillan-Barré syndrome. Other systemic features (oedema and skin changes) developed later, and an elevated serum level of vascular endothelial growth factor led to the diagnosis of POEMS syndrome. She received high-dose chemotherapy with autologous peripheral blood stem cell transplantation, resulting in good recovery. The authors also reviewed patterns and speed of progression of neuropathy in the 30 patients with POEMS syndrome; 22 (73%) of them were unable to walk independently with the median period of 9.5 months from POEMS onset (range 0.5-51 months). Whereas the speed of neuropathy progression varies considerably among patients, some POEMS patients can show acute or subacute polyneuropathy. The early diagnosis and treatment could result in rapid improvement as shown in the present patient.

A case of adenoid cystic carcinoma (ACC) of the breast and review of the utility of preoperative imaging diagnose.

A case of adenoid cystic carcinoma (ACC) of the breast in a 66-year-old woman is reported herein. ACC accounts for about 0.1% of all breast cancers. Our patient presented with a small, elastic and hard mass, measuring 2.0x2.0 cm, between both outer quadrants of the right breast. Although physical examination, ultrasonography and magnetic resonance (MR) mammography suggested a benign tumor, aspiration biopsy cytology (ABC) was performed twice, and the second ABC specimen was evaluated as suspicious for breast carcinoma. Breast conserving surgery with a level II lymph node dissection was subsequently performed. There was no lymph node metastases and estrogen receptor (ER) status was negative. Light microscopy revealed various growth patterns, with the cells showing biphasic cellularity. According to immunohistochemical analyses, CEA, actin and vimentin were positive, S-100 protein was negative, and the cytokeratin reaction was partially positive. Therefore, ACC of the breast was diagnosed. Although ACC of the breast is a rare neoplasm, it should be considered in the differential diagnosis even if various diagnostic imaging studies suggest a benign tumor of the breast. Awareness of this tumor will help prevent misdiagnosis.

Herniation through both Winslow's foramen and a lesser omental defect: report of a case.

The herniation of small bowel through Winslow's foramen is a rare type of internal hernia which can cause ileus; however, a hernia traversing the lesser sac is even more unusual. To the best of our knowledge, only 25 cases of herniation through Winslow's foramen and 10 cases of lesser sac hernia have been reported in the Japanese literature. We describe herein the case of a 33-year-old man who presented to our hospital complaining of abdominal pain in whom a plain abdominal radiograph revealed small bowel gas with air-fluid levels, suggesting ileus. Following admission, an ileus tube was inserted, but the intestinal shadow did not improve and surgery was performed based on suspicion of an internal hernia. Approximately 100 cm of ileum was found to have herniated through a defect in the lesser omentum after passing through Winslow's foramen. Since the herniated bowel was viable, manual reduction without resection was performed. The patient had a satisfactory postoperative course, and was discharged on postoperative day 11. There are many unknown aspects surrounding the etiology of Winslow's foramen hernia and lesser sac hernia, and although internal hernia is a rare cause of ileus, its possibility should be kept in mind.

Hypothermia with acute renal failure in a patient suffering from diabetic nephropathy and malnutrition.

We report a rare case of hypothermia with acute renal failure in a patient suffering from diabetic nephropathy. A 71-year-old male who had been receiving insulin therapy for the treatment of diabetes mellitus complicated with advanced diabetic nephropathy since 1998 was malnourished with an extremely decreased muscle mass. Without any prolonged exposure to excessively low external temperatures or hypothyroidism, pituitary insufficiency, adrenal insufficiency, sepsis, hypoglycemia, and diabetic ketoacidosis, acute hypothermia appeared together with an aggravation of diabetic nephropathy. His skin temperature fell to below measurable levels and his rectal temperature fell to 30.0 degrees C. His consciousness was drowsy and the hypothermia was not accompanied by shivering. Skeletal muscle is known to play an important role as a center of heat production and shivering thermogenesis in skeletal muscle mainly operates on acute cold stress. Therefore, in this case, hypothermia may have occurred because the shivering thermogenesis could not fully act on the acute cold stress due to the dramatically reduced muscle mass. We should always keep in mind that older, malnourished diabetic patients can easily suffer from impairments of the thermoregulatory system.

Altered N-glycosylation of glucose transporter-1 associated with radiation-induced tumorigenesis of human cell hybrids.

Studies on human cell hybrids between a cervical carcinoma cell line, HeLa, and normal fibroblasts have indicated that their tumorigenicity is under the control of a putative tumor suppressor on chromosome 11. We have previously demonstrated that a tumorigenic cell hybrid CGL4 expresses a larger glucose transporter, GLUT1, due to altered glycosylation when compared to the nontumorigenic counterpart CGL1. In this study, we demonstrated this glycosylation change in GLUT1 in gamma-ray-induced tumorigenic mutants (GIMs) isolated from CGL1 cells as expressing a tumor-associated surface antigen, intestinal alkaline phosphatase. In contrast, GLUT1 in the gamma-irradiated nontumorigenic control cells (CONs) did not show this alteration. In accordance with this glycosylation change, affinity to 2-deoxyglucose in these GIM clones was increased by about twofold when compared to the nontumorigenic CONs. These results suggest a close correlation between the glycosylation change in GLUT1 with increased affinity to D-glucose and tumorigenicity of these human cell hybrids.

Spinal cord compression by heterotopic ossification associated with pseudohypoparathyroidism.

This report describes a 37-year-old man presenting with a gait disturbance due to spastic paraparesis. Physical findings showed typical features of Albright's hereditary osteodystrophy, including short stature, obesity, brachydactyly and dental hypoplasia. He was diagnosed as having pseudohypoparathyroidism type Ia, on the basis of his hypocalcaemia, hyperphosphataemia, increased plasma level of parathyroid hormone (PTH), and the unresponsiveness to exogenous PTH loading of his urinary excretion of both nephrogenous cyclic adenosine monophosphate and phosphate. Magnetic resonance imaging and myelographic computed tomographic scans clearly demonstrated severe compression of the spinal cord at T 9/10 by tumour-like ossifications of the paravertebral ligaments. Neurosurgical decompression therapy was, therefore, performed to alleviate his spastic paraparesis. This was a rare case of pseudohypoparathyroidism complicated with spinal cord compression caused by ectopic ossification of the ligaments.

The first case of insulin-dependent diabetes mellitus with prominent spurious hyperglucagonemia due to interference of immunoglobulin G in glucagon radioimmunoassay (OAL-123) system.

A 51-year-old male was admitted to our hospital because of diabetic ketoacidosis. His symptoms were promptly improved with intensive insulin therapy, but his plasma glucagon immunoreactivity measured by the OAL-123 radioimmunoassay (RIA) system showed persistently high values (3,090-3,210 pg/ml). A computed tomographic scan, abdominal angiography and endoscopic examination of his gastrointestinal tract showed no evidence of glucagonoma. After removing the immunoglobulin fraction from the plasma, his immunoreactive plasma glucagon level returned to normal. Moreover, the immunoglobulin G fraction purified from the patient's plasma inhibited the binding of [125I]glucagon to rabbit antiglucagon antiserum, OAL-123. Dot-blot analysis demonstrated that the immunoglobulin G of this patient cross-reacted against OAL-123. Therefore, it was considered that the prominent hyperglucagonemia in this patient was due to the presence of IgG, which interfered with the measurement of plasma-immunoreactive glucagon.

Pathogenesis of diabetic nephropathy in non-insulin-dependent diabetes mellitus.

To elucidate the characteristics of diabetic nodular lesions and the process of progression of diabetic glomerulosclerosis, kidney specimens obtained from 185 patients with non-insulin-dependent diabetes mellitus (NIDDM) were observed using light, electron, and immunofluorescence microscopes. The results suggest the following. First, there are two distinct subtypes of nodular lesions: One is formed by the progression and expansion of diffuse lesions; the other, showing a concentrically layered structure, is probably formed in the process of reconstruction of mesangiolysis. Second, there are three phases in the process of progression of diabetic glomerulosclerosis: In the first phase, arteriolosclerosis and diffuse lesions appear; in the second phase, mesangiolysis and nodular lesions develop in association with moderately advanced arteriolosclerosis; and in the third phase, exudative lesions and hyalinized glomeruli appear in association with advanced arteriolosclerosis together with advanced interstitial lesions. In the progression of the phases and in the development of mesangiolysis and layered nodular lesions, disturbed blood flow into glomeruli in consequence of diabetic arteriolosclerosis could be essential.

Carcinosarcoma of the esophagus. An immunohistochemical and electron microscopic study.

Immunohistochemical and electron microscopic examinations were made of a carcinosarcoma of the esophagus in an 80-year-old man. An immunohistochemical examination showed that sarcomatous spindle cells were vimentin-positive, whereas squamous carcinoma cells were keratin-positive. No coexistence of vimentin and keratin in a single tumor cell was found. Electron microscopically, the sarcomatous spindle cells were characterized by well-developed rough endoplasmic reticulum, abundant intermediate filaments, and the occasional presence of peripheral aggregates of microfilaments. No definite desmosomes were identified among these cells. These results appear to indicate that most of the spindle-shaped tumor cells assume fibroblastic cellular features and synthesize the intermediate filament protein usually expressed in mesenchymal cells, even though such tumor cells could be epithelial in origin.

Impaired plasma cyclic AMP response to exogenous glucagon in acute liver injury.

The response of plasma cyclic AMP to glucagon was investigated in 18 patients with acute liver injury to determine its value as a marker for the severity of the hepatic damage. We also investigated the interrelationship between plasma cyclic AMP response and the hyperglucagonemia often seen in this disease. Plasma cyclic AMP response to glucagon was reduced significantly in patients with acute hepatitis, particularly in severe cases with bridging hepatic necrosis. There was a significant negative correlation between log (peak % cAMP) and prothrombin time (r = -0.715, p less than 0.01), and also total bilirubin (r = -0.819, p less than 0.01). In fatal cases, the early phase of plasma cyclic AMP response after glucagon stimulation was blunted. In patients with acute liver injury, basal IRG levels were significantly high and a significant negative correlation was found between log (peak % cAMP) and basal IRG levels (r = -0.816, p less than 0.01). Our results suggest that the response of plasma cyclic AMP to exogenous glucagon could be useful in evaluating the severity of acute liver injury. The lower cyclic AMP response may be attributed to reduced hepatic reserve and endogenous hyperglucagonemia.

Plasma insulin and C-peptide levels in cirrhotic and uremic patients.

Fifty grams of glucose were administered orally to twelve cirrhotics, twelve uremics and ten normal controls and the plasma insulin and C-peptide responses were measured and expressed as molar concentration. Glucose intolerance, hyperinsulinemia and elevated C-peptide levels were found in cirrhotics after glucose loading and in uremics during the later part of the glucose response. The molar ratio of C-peptide to insulin was lower in cirrhotics and higher in uremics than in controls. It is suggested that insulin is mainly degraded by the liver and C-peptide by the kidneys, and that C-peptide is not affected by the liver damage but is present in raised concentration in subjects with injured kidneys. Elevated C-peptide level in cirrhotics is consistent with hyperfunction of pancreatic B-cells.