[Postoperative occurrence of reversible posterior encephalopathy in a patient with Crohn's disease]. / Postoperativ aufgetretene reversible posteriore Enzephalopathie bei einem Patientin mit Morbus Crohn.

Posterior encephalopathy (PE) is a rare condition with different origins and unknown pathogenesis. We report on a case of a 16-year-old boy with Crohn's disease who developed PE after peridural anesthesia with ropivacain. A possible connection between PE, ropivacain and Crohn's disease is discussed on the basis of the current literature.

[Percutaneous endovascular aneurysm repair without arteriotomy (Perclose system)]. / Perkutane endoprothetische Therapie des Aortenaneurysmas ohne Arteriotomie.

BACKGROUND AND OBJECTIVE: It is common clinical practice to perform an arteriotomy for the endovascular treatment of infrarenal and thoracic aortic aneurysms. Instead we used the percutaneous endovascular Perclose device to perform the aneurysm repair without arterial cut-down. PATIENTS AND METHODS: The Perclose device contains four needles with two suture loops for closing the femoral artery access site. The sutures were deployed after the arterial puncture, before introduction of sheaths (diameter 12 - 27 F = 4 - 9 mm). After the procedure the sutures were used to close the puncture site. We attempted to achieve hemostasis with the Perclose system in 104 femoral arteries in 60 patients (7 females, mean age 69 +/- 12 years). The mean vessel diameter was 10 +/- 2 mm. RESULTS: The percutaneous graft implantation was successfully achieved in 58 of 60 patients. The graft could not be forwarded into the aorta in two cases because of calcified iliac arteries. The Perclose suture technique was successfully used in 97 femoral arteries. In one case a false aneurysm developed and in another case a secondary hemorrhage occurred. Seven patients needed additional manual compression to achieve complete hemostasis. A surgical repair was not necessary. The time to hospital discharge ranged from 4.5 hours to 32 days (median: 18 hours). 20 patients stayed longer than 24 h in the hospital, only 2 of them for reasons related to the puncture site. CONCLUSION: Closing the access site with the Perclose system is technically feasible and effective, even with large sheaths up to 27 F = 9 mm. This technique reduces the invasiveness of the endovascular repair of aortic aneurysms and decreases the length of hospital stay, i. e. it allows treatment in outpatients.

[Megadolichobasilar artery as the etiology of sensorineural deafness in differential sudden deafness diagnosis]. / Die Megadolichobasilaris als Ursache einer sensorineuralen Schwerhörigkeit zur Differentialdiagnose des Hörsturzes.

The megadolichobasilar artery is a rare vascular anomaly that can cause a variety of clinical symptoms but is usually asymptomatic. Not much attention has been given as yet to a possible sensorineural hearing loss caused by a megadolichobasilar anomaly. Vascular compression of the vestibulocochlear nerve must be assumed to be the pathogenic factor. The megadolichobasilar anomaly represents a rare entity in the differential diagnosis of sudden idiopathic sensorineural hearing loss. More common causes are acoustic neuromas, monosymptomatic Menière's disease and rupture of the round window membrane. We present the case of a 71-year-old white male in whom magnetic resonance angiography combined with contrast-enhanced computed tomography in three-dimensional reconstruction eventually led to the diagnosis of megadolichobasilar anomaly. The combination of both imaging techniques may be required for the effective diagnosis of similar vascular anomalies.

Myotonic dystrophy. The role of large triplet repeat length in the development of mental retardation.

OBJECTIVE: To describe mental retardation and microcephaly as initial clinical signs in myotonic dystrophy (MD) with high trinucleotide repeats. PATIENTS AND METHODS: Two patients with maternally inherited MD were examined. Southern blot analysis was performed and trinucleotide repeat expansions were related to the findings of clinical and magnetic resonance imaging investigations. RESULTS: Both patients had the large CTG trinucleotide repeat expansions often seen in congenital MD, but they lacked the typical clinical signs. Mental retardation and microcephaly were the leading features present in infancy. Muscular weakness, in contrast, developed after age 35 years. Although there was no evidence for perinatal asphyxia or sleep apnea, magnetic resonance imaging disclosed reduced brain volume and subcortical demyelination. CONCLUSIONS: Mental retardation preceding the development of muscle weakness suggests that the cerebral involvement in MD is a direct consequence of the genetic disorder and not mediated by muscle disease. Careful clinical examination of the parents for signs of MD should be considered in patients with cognitive deficits even without apparent muscular involvement.

Meningeal involvement in Wegener's granulomatosis confirmed and monitored by positive circulating antineutrophil cytoplasm in cerebrospinal fluid.

MRI and CSF investigations revealed meningeal involvement in a 29-year-old patient with biopsy-confirmed Wegener's granulomatosis. The intracranial manifestation of Wegener's granulomatosis was supported by the detection of pathologic circulating antineutrophil cytoplasm (c-ANCA) in the CSF. We monitored disease activity by c-ANCA measurement in the CSF. After repeated cycles of intrathecal administration of methotrexate and corticoids, progression of meningeal infiltration stopped, and CSF c-ANCA titers became negative.

[Results of an analysis of age and cause of death in 1,000 silicosis autopsies in the Thüringen region]. / Ergebnisse einer Analyse des Lebensalters und der Todesursachen von 1,000 obduzierten Silikosepatienten aus dem Thüringer Raum.

The authors have studied the clinical and autopsy records, including lung roentgenograms, of 1,000 silicotics deceased in the Thuringian districts of Gera, Erfurt and Suhl over the past 30 years. The main points of the analysis are clinical-pathological-anatomical comparisons in pneumoconiosis diagnoses and the identification of the cause of death. The roentgenological findings were found to be largely in correspondence with the pathological-anatomical ones. Surprisingly, the mean life span of male pneumoconiosis patients (71.3 years) is more than 2 years above the average life expectancy in the GDR, whereas the mean life span of the female patients differs little from the average GDR figure. 60.7 percent of the patients died of the sequelae of pneumoconiosis.

[The frequency of bronchial carcinoma in 1,000 autopsies of cases of silicosis]. / Uber die Häufigkeit des Bronchialkarzinoms unter 1,000 autoptisch untersuchten Silikosen.

In the present examination were analysed the anamneses, necropsies and x-ray photograms of 1,000 patients (743 men and 257 women), who died from silicosis in the Thuringian area. The analysis includes a period of 30 years. The material of examination comprises autopsies from 1954 to 1983. There were 45 lung cancers, found in 42 men and 3 women. A relation to smoking habit could not be examined. The lung cancer was mainly connected with a stadium of silicosis II. Only in 6 lung cancers there was a relation to the silicosis like a cicatrice cancer assumed.

The variable region of human immunoglobulins. I. Serologic and structural correlations of antigenic markers common to V lambda I and V lambda IV proteins (isotypic cross-reactivities).

Sixty-eight human lambda-chain sources including representatives of the five different subgroups were studied for the distribution of the serologic markers ST, 111, and VOR The variable region character of these antigenic determinants has been demonstrated for those proteins whose primary structures had been partially or completely determined previously. The lambda-chain sub-groups I and IV are readily distinguished antigenically but the specificity designated VOR is shared between them and is absent in the II, III, and V subtypes. The results of these studies are discussed with respect to the possible relationships of the structural genes controlling the synthesis of the lambda-chain subgroups, and some potential phylogenetic and functional meaning of the heterogeneities is revealed.

[Clinical significance of cationic proteins of leukocyte lysosomes: cationic leukocyte antigen (CLA) as a marker of blast crisis in chronic myelocytic leukaemia (author's transl)]. / Klinische Wertigkeit von kationischen lysosomalen Leukozytenproteinen: Bedeutung der immunologischen Bestimmung von Kationischem Leukozytenantigen (CLA) für die Diagnose der Blastenkrise bei chronischer Myelose.

Cationic leukocyte antigen (CLA) was estimated in urine of acute and chronic leukaemics using quantitative immuno-precipitation techniques. The studies demonstrated that CLA is a marker of the (myelo-) blast crisis in patients with chronic myelocytic leukaemia (CML). By these studies the value of the determination of CLA for the differential diagnosis of acute myeloblastic leukaemia versus initial (myelo-) blast crisis in CML and the lymphatic leukaemias, respectively, was evident. The terminal blastic transformation in CML was indicated early by an elevation of the urinary CLA-level. The blast-crisis was accompanied by a heavy CLA-uria. The results are documented by demonstration of special studies of characteristic cases and discussed in the light of recent results of immunological and cytochemical findings on the nature of blast crisis in CML.

Immunoglobulin diversity: correlation of non-allelic antigenic markers with the basic sequences of the variable region of human lambda chains.

Three forms of the amino terminal half (variable region) of human pathological lambda light chains of immunolglobulins were identified antigenically. By study of all completely sequenced Bence Jones proteins hitherto analyzed and a greater number of proteins subjected to automated sequence determination as well as normal light chains three distinct isotypic basic sequences were identified. The basic sequences are shown to be associated with characteristic antigenic markers representing three V region genes encoding the variable half of lambda chains of immunoglobulins.