Klinische Pr?sentation und Prognose von Hirnstamminfarkten Eine Auswertung der Schlaganfall-Datenbank der Stiftung Deutsche Schlaganfall-Hilfe Eine Auswertung der Schlaganfall-Datenbank der Stiftung Deutsche Schlaganfall-Hilfe

Angesichts der vielf?ltigen klinischen Klassifikationssysteme und Syndrome fehlt bislang ein repr?sentativer überblick über ?tiologie und Prognose von Hirnstamminfarkten. Aus der Deutschen Schlaganfall-Datenbank untersuchten wir daher 455 Patienten mit bildmorphologisch nachgewiesenem, akuten Hirnstamminfarkt im Vergleich zu Patienten mit anderer Infarktlokalisation. Durch Nachbefragung der Patienten nach 3 und 12 Monaten wurden au?erdem das funktionelle Ergebnis sowie die Reinsultrate erhoben.     

Estrone and estradiol in patients with cirrhosis of the liver: effects of ACTH and dexamethasone.

To study the influence of the adrenal gland on plasma estrogen levels in male patients with hepatic cirrhosis, estrone and estradiol were measured under a variety of experimental conditions. Compared to controls, estradiol levels were moderately elevated by 26% (P is less than 0.05) in patients with hepatic cirrhosis (28.5 +/- 5.4 vs. 36.0 +/- 4.7 pg/ml plasma; n: 12), whereas estrone levels exhibited a two- to threefold increase under basal conditions (32.5 +/- 5.6 vs. 67.8 +/- 20.8 pg/ml; P is less than 0.01). ACTH application resulted in a striking increase in plasma estrone levels in both patients with hepatic cirrhosis and in normal subjects (61.8 +/- 27.5 vs. 27.3 +/- 7.8 pg/ml). During stimulation with ACTH, estradiol levels showed no significant changes. After suppression of the adrenal gland by dexamethasone administered for 5 days, plasma concentrations of estrone and estradiol were found to be reduced. The absolute decrease of estrone was significantly greater in patients with hepatic cirrhosis than in healthy male subjects (35.5 +/- 12.6 vs. 21.3 +/- 6.0 pg/ml; P is less than 0.05; n: 8). Estrogen values, however, were still high in patients with hepatic cirrhosis after 5 days of dexamethasone administration (37.1 +/- 17.6 pg estrone/ml and 23.9 +/- 3.6 pg estradiol/ml plasma). It is suggested that elevated plasma values of estrogens in this disorder may be derived predominantly from adrenal production. Peripheral conversion of androgens to estrone rather than to estradiol appears to be more effective in sustaining plasma levels of estrogens in patients with hepatic cirrhosis.     

Filamin C-related myopathies: pathology and mechanisms

The term filaminopathy was introduced after a truncating mutation in the dimerization domain of filamin C (FLNc) was shown to be responsible for a devastating muscle disease. Subsequently, the same mutation was found in patients from diverse ethnical origins, indicating that this specific alteration is a mutational hot spot. Patients initially present with proximal muscle weakness, while distal and respiratory muscles become affected with disease progression. Muscle biopsies of these patients show typical signs of myofibrillar myopathy, including disintegration of myofibrils and aggregation of several proteins into distinct intracellular deposits. Highly similar phenotypes were observed in patients with other mutations in Ig-like domains of FLNc that result in expression of a noxious protein. Biochemical and biophysical studies showed that the mutated domains acquire an abnormal structure causing decreased stability and eventually becoming a seed for abnormal aggregation with other proteins. The disease usually presents only after the fourth decade of life possibly as a result of ageing-related impairments in the machinery that is responsible for disposal of damaged proteins. This is confirmed by mutations in components of this machinery that cause a highly similar phenotype. Transfection studies of cultured muscle cells reflect the events observed in patient muscles and, therefore, may provide a helpful model for testing future dedicated therapeutic strategies. More recently, FLNC mutations were also found in families with a distal myopathy phenotype, caused either by mutations in the actin-binding domain of FLNc that result in increased actin-binding and non-specific myopathic abnormalities without myofibrillar myopathy pathology, or a nonsense mutation in the rod domain that leads to RNA instability, haploinsufficiency with decreased expression levels of FLNc in the muscle fibers and myofibrillar abnormalities, but not to the formation of desmin-positive protein aggregates required for the diagnosis of myofibrillar myopathy.     

Relationship of plasma sex hormones to different parameters of obesity in male subjects

Relationships between plasma sex hormones and different parameters of obesity (weight, ideal body weight [IBW], overweight, fat mass, and body surface) were investigated in 70 healthy nonobese and obese males, 20–40 yr of age and with a body weight of 85%–245% of IBW. Plasma sex hormones remained unaffected by weight up to approximately 160% of the IBW. Only in the massively obese subjects was plasma testosterone decreased to 40% of controls (from 6.2 to 2.5 ng/ml), whereas free testosterone remained almost constant. On the other hand, plasma estrone and estradiol exhibited significant increases in obese subjects, ranging from 31.5 ± 5.3 to 52.3 ± 5.8 pg/ml for estrone, and 25.4 ± 5.4 increasing to 44.7 ± 5.0 pg/ml for estradiol. Similarly, free estradiol was shown to significantly increase with obesity in men from 505 ± 118 to 991 ± 123 fg/ml (p < 0.001). The ratios of testosterone/androstenedione, as well as of estradiol/estrone, were not affected by obesity, suggesting that reduction of the 17-oxo-group of the steroids is not influenced by the amount of fat tissue. A significant (p < 0.001) correlation was found between IBW and estrone (r = 0.80) and estradiol (r = 0.75), as well as the ratios of estrone/androstenedione (r = 0.62) and estradiol/testosterone (r = 0.86). This is consistent in its evidence indicating that fat tissue may be able to aromatize androgens. In the obese subjects, there were significant correlations between plasma sex hormones (testosterone, estrone, estradiol, and free estradiol) and the parameters of obesity used. Among these, correlations were best with IBW, overweight, and fat mass (r = 0.74–0.89; p < 0.001); body weight and body surface were less favorable.     

Diagnosis of middle ear tuberculosis

Middle ear tuberculosis is underdiagnosed. Clinical suspicion requires extensive diagnostic and therapeutic procedures. Due to a high degree of error in bacteriological and histological examination they must often be repeated. Indications for tentative diagnosis are given.     

Tympanoplasty in children

Tympanoplasty in childhood is not always the same as in adults. This applies to the indication for the operation, to the operation itself and also to the pre- and postoperative treatment. The rapidly growing and expanding juvenile cholesteatoma offers special problems, and probably has a different cause than the slow growing pearl tumour found in adults. Traumatic cholesteatomas also give the impression of growing faster in childhood. The prognosis for tympanoplasty in cases of mesotympanic chronic otitis media must not be too optimistic because the condition of the mucosa in juveniles is different to that in adults. Pre-operative treatment may take a long time and postoperative treatment may be difficult.