Case of Heerfordt's syndrome with prolonged peripheral nerve involvement]

A 28-year-old man complaining of myiodesopsia was given a diagnosis of uveitis. Subsequently he complained facial nerve palsy and enlargement of parotid gland. Heerfordt's syndrome was diagnosed based on the results of several examinations. Facial nerve palsy, enlargement of the parotid gland and uveitis were improved by systemic corticosteroid therapy. At present he is receiving systemic corticosteroid therapy, but numbness in the mouth, thought to be the involvement of the trigeminal nerve, remains. Systemic corticosteroid therapy is usually effective for most cases with Heerford's syndrome. On the other hand, there are some cases with the prolonged peripheral nerve involvement despite systemic corticosteroid therapy, as seen in this case. If peripheral nerve involvement is prolonged, it is necessary to consider small-fibre neuropathy as one possible cause.     

MULTICENTER STUDY DESIGN OF THE EX VIVO EVALUATION OF ENDOCYTOSCOPY IN ESOPHAGEAL SQUAMOUS CELL CARCINOMA

Optical technological innovations enable us to visualize cellular nuclei endoscopically. Herein is described a protocol design for a multicenter study for the ex vivo evaluation of endocytoscopy. The present study was performed by the Endoscopy Forum Japan study group.     

STANDARD IMAGING TECHNIQUES OF ENDOSCOPIC ULTRASOUND‐GUIDED FINE‐NEEDLE ASPIRATION USING A CURVED LINEAR ARRAY ECHOENDOSCOPE

Standard imaging techniques using a curved linear array echoendocope are summarized to facilitate the attainment of expertise in endoscopic ultrasonography and endoscopic ultrasound‐guided fine needle aspiration, and to promote the widespread use of this diagnostic and therapeutic tool. Typical images of the mediastinal organs, the bilio‐pancreatic systems and neighboring organs by scanning from the esophagus, stomach, duodenal bulb, and descending portion of the duodenum, are shown in a sequential manner. The basic techniques of endoscopic ultrasound‐guided fine needle aspiration are also presented.     

Chromosomal Mapping of Genetic Locus Associated with Thymus-size Enlargement in BUF/Mna Rats

The thymoma-prone rat of the BUF/Mna strain is a useful model for human thymoma. In this strain thymoma development is regulated by a single autosomal susceptible gene, Tsr-1. At pre-thymoma age, BUF/Mna rats have extremely large thyrauses, when compared to those of other strains of rats. Genetic studies in crosses between BUF/Mna rats with large thymuses and WKY/NCrj rats with small thymuses suggested the presence of a major autosomal gene, Ten-1 , which contributes to thymus enlargement in a backcross population. Linkage studies between Ten-1 and microsatellite markers in backcross rats of (WKY/NCrj×BUF/Mna)Fl×BUF/Mna have led to the localization of Ten-1 in chromosome 1. This result may provide an approach to clone Tsr-1 , which could be allelic to Ten-1.     

Blood-brain barrier dysfunction in Binswanger’s disease; an immunohistochemical study

Binswanger’s disease is pathologically characterized by a combination of diffuse cerebrovascular white matter lesions and lacunar infarcts in the basal ganglia and white matter. Although a blood-brain barrier (BBB) dysfunction has been implicated in the pathogenesis of these white matter (WM) lesions, few authors have addressed this problem. In the present study, we describe BBB dysfunction and its regional differences in the brains of Binswanger’s disease patients. Twelve brains from Binswanger’s disease patients (group III) were examined and compared with those from five patients with non-neurological disease (group I) and five cortical infarct patients without significant WM lesions (group II). Immunohistochemistry was performed for glial fibrillary acidic protein and vimentin as astroglial cell markers, and for immunoglobulins, complements and fibrinogen as extravasated serum protein markers. The grading scores for IgG extravasation were significantly higher in group III as compared to group I, in both the periventricular WM and the subcortical WM (P < 0.01). In group III, the scores in the periventricular WM and subcortical WM were significantly higher than in the subcortical U fibers and cerebral cortex (P < 0.01 for the periventricular WM; P < 0.001 for the subcortical WM), respectively. Clasmatodendritic astroglia, which had swollen cell bodies and large cytoplasmic vacuoles with disintegrated processes, incorporated the serum components IgG, IgM, C3d, C1q and fibrinogen, both in the periventricular WM and subcortical WM in 5 out of 12 (42%) Binswanger’s disease brains. These results indicate that WM lesions in Binswanger’s disease are accompanied by BBB dysfunction, although it remains uncertain whether BBB dysfunction is secondary to either chronic cerebral ischemia or arterial hypertension. Received: 25 April 1997 / Revised, accepted: 21 July 1997     

Epidermal injury stimulates prenylation in the epidermis of hairless mice

Isoprenylation is the covalent attachment of isoprenyl groups, intermediates of the cholesterol biosynthesis pathway, to carboxy terminal cysteine residues of proteins. Numerous proteins are isoprenylated including small GTP binding proteins, trimeric G proteins, and nuclear lamins, and these prenylated proteins regulate a variety of cell functions, including cell growth, cytokinesis, and differentiation. Here, we quantitated protein prenylation and determined which proteins are prenylated in the epidermis of hairless mice by radiolabeling with ³ H-mevalonolactone following acute or chronic epidermal injury. In normal epidermis, four major radiolabeled bands, with molecular weights of 17–26, 48, 54, and 68 kDa, were observed. The levels of each of these bands increased by 24–63% 16 h following acute epidermal injury induced by topical acetone treatment or tape stripping, returning to normal by 24 h. On 2D gel electrophoresis, there were no major differences between the patterns of labeling following barrier disruption. Subacute epidermal injury induced by either acetone or tape stripping twice a day for 7 days and chronic injury induced by feeding an essential fatty acid-deficient (EFAD) diet, also resulted in a significant increase in protein prenylation. As with acute injury, SDS-PAGE and 2D gel electrophoresis did not reveal marked differences in the pattern of protein prenylation. These results demonstrate that the prenylation of proteins in the epidermis is stimulated by injury, suggesting that one or more of these prenylated species may be important in epidermal proliferation or differentiation. Received: 29 May 1996     

Effect of myasthenic IgG on degradation of junctional acetylcholine receptor

We investigated the effect of the lgG from patients with myasthenia gravis (MG) on the degradation of normal rat junctional acetylcholine receptor (AChR) labeled with ¹²⁵l-α-bungarotoxin (BuTx) and calculated the degradation rate (DR). The DR for the lgG from these patients was significantly higher than that from healthy volunteers and patients with other autoimmune diseases. For MG, DR was significantly correlated with the severity of the disease but not with anti-AChR antibody titer. DR was accelerated by lgG from patients with generalized MG whose antibody titers were in the normal range and by lgG from patients with ocular MG. These results indicate that measurement of the DR of junctional AChR in normal rats is more closely correlated with the severity of the disease than is measurement of anti-AChR antibody and that the former is a sensitive and confirmatory method for evaluating MG. © 1993 John Wiley & Sons, Inc.     

Morphology of single mesencephalic trigeminal neurons innervating periodontal ligament of the cat

The morphology of single neurons in the trigeminal mesencephalic nucleus (Vmes) that innervate periodontal ligament was studied in cats by the method of intraaxonal injection of horseradish peroxidase (HRP). Two kinds of Vmes neurons were distinguished on the basis of differences in axon profile and its central projection. The first type of Vmes neurons was unipolar in shape and its axon was divided into united (U), peripheral (P), and central axons (C). The U axon traveled caudally within the Vmes from the soma to the dorsolateral aspect of trigeminal motor nucleus (Vmo), where it split into the P and C axons with a T-shaped appearance. The P axon joined the spinal trigeminal tract across the trigeminal principal nucleus and ran within the tract and sensory root to exit the brainstem. The C axon traveled caudally within Probst's tract. All 3 axons issued axon collaterals. Axon collaterals from the U, P and the proximal C axons sent their terminal branches into the supra (Vsup) and intertrigeminal regions (Vint). Most axon collaterals from the C axon sent their terminal branches into the juxtatrigeminal regions (Vjuxta). The second type of Vmes neurons was bipolar and issued P and C axons. The C axon ran a short distance in the Vmes to leave the Vmes, and then it traveled caudolaterally in the rostrodorsomedial aspect of the Vmo. Finally, it entered in the Vmo and traveled caudally in the dorsolateral subdivision of the nucleus to its rostrocaudal mid-level. The C axon gave off massive axon collaterals.(ABSTRACT TRUNCATED AT 250 WORDS)     

A Comparison of Two Simple Measures to Evaluate the Health Status of Asthmatics: The Asthma Bother Profile and the Airways Questionnaire 20

Simple and concise measures for health status are desirable in clinical practice. The Asthma Bother Profile (ABP), which consists of 23 items, has been developed to assess how much asthma bothers patients. The Airways Questionnaire 20 (AQ20) is a simple instrument which consists of 20 items. The purpose of this study was to investigate how the ABP and AQ20 evaluate the health status of patients with asthma. A total of 166 patients with chronic asthma (age: 48 ± 16 yr, 77 males) completed pulmonary function testing, measurement of airway hyperresponsiveness, dyspnea rating, assessments of their anxiety and depression (HADS; Hospital Anxiety and Depression Scale), and assessments of their health status. The health status was assessed using the ABP, AQ20, the short-form 36 health survey questionnaire (SF-36), the Living With Asthma Questionnaire (LWAQ) and the Asthma Quality of Life Questionnaire (AQLQ). The Japanese version of the ABP included only 15 'bother' items out of the original 23 items due to cultural differences. The scores on the ABP were widely distributed, whereas the scores on the AQ20 were skewed towards the milder end of the scale. The ABP had a strong correlation with the Avoidance and Distress constructs on the LWAQ, and Anxiety and Depression on the HADS (Rs = 0.56 ∼ 0.79), and its strongest correlation with the General Health (Rs = - 0.64) scale among the 8 subscales on the SF-36. The AQ20 had a less significant correlation with the LWAQ, AQLQ, and SF-36 than the ABP. The ABP and AQ20 were short and simple to complete, and both measures could easily be used in clinical practice. The ABP can evaluate patients more specifically with respect to distress and bother than the AQ20.     

Spontaneous and isolated dissection of the main trunk of the superior mesenteric artery.

A 42-year-old man was admitted to another hospital complaining of acute abdominal pain that was induced by eating. Abdominal computed tomography and selective angiography revealed an intimal flap separating true and false lumens that was located 3 cm from the origin of the superior mesenteric artery (SMA). Emergency surgery was performed because of the sudden recurrence of diffuse abdominal pain after eating and abdominal aorta-SMA bypass grafting was done using a radial artery graft. Postoperative angiography revealed that the graft showed good patency. The postoperative course was uneventful and abdominal pain no longer occurred after eating. This excellent result was achieved by early diagnosis using CT scanning and angiography plus an aggressive surgical repair with a radial artery bypass graft for isolated dissection of the superior mesenteric artery.