Predominantly cerebral manifestation in Urbach-Wiethe's syndrome (lipoid proteinosis cutis et mucosae): a clinical and pathomorphological study

The neurological and psychiatric manifestations in a case of Urbach-Wiethe's disease are reported. The present case is characterized by the predominance of cerebral symptoms: ataxia, psychomotoric seizures, paranoic hallucinatory psychosis and attacks of rage. At autopsy, deposits of amyloid-like material in the walls of the brain vessels could be observed. This finding, together with the mucocutaneous alterations, confirmed the diagnosis of the rare disease know as "lipoid proteinosis cutis et mucosae".     

The effect of propranolol on exercise induced tachycardia is determined by plasma concentration and the density of adrenergic receptors on leukocytes

Summary The chronotropic response to a single oral dose of propranolol in 23 healthy subjects has been related to the plasma propranolol concentration and the density of -adrenoceptors on peripheral polymorphonuclear leucocytes. The percentage reduction in exercise-induced tachycardia was significantly correlated with the log plasma propranolol concentration within subjects but not between subjects. Taking the concentration of the active metabolite 4-hydroxypropranolol into account did not improve the interindividual correlation. The reduction in exercise-induced tachycardia was significantly correlated with the maximum binding density of (¹²⁵I)-hydroxybenzylpindolol on polymorphonuclear leucocyte membrane fragments measured before medication. A response index (% reduction in exercise-induced tachycardia/plasma propranolol concentration) was correlated with the maximum binding density of (¹²⁵I)-hydroxybenzylpindolol (predrug) at 2 h (r_s=0.72), 4 h (r_s=0.84) and 6 h (r_s=0.73) after dosing. The data suggest that interindividual variation in the response to propranolol after a single oral dose is determined by interindividual differences both in plasma propranolol and adrenoceptor density.     

Deciphering the molecular effects of non-ablative Er:YAG laser treatment in an in vitro model of the non-keratinized mucous membrane

Lasers in Medical Science -     

A GPS logger and software for analysis of homing in pigeons and small mammals

A detailed analysis of homing in pigeons and small mammals has remained difficult because the paths of the animals could not be reconstructed precisely. Here, we describe a lightweight global position system (GPS) data logger (35 g including battery and casing; 40 x 68 x 18 mm) that records the flight of pigeons and the path of dogs with an accuracy of +/-12 m. With one battery, the logger runs in continuous mode (1 fix/s) for 3.5 h and in power-saving mode (1 fix/5 s) for about 16 h, and stores a maximum of 100,000 data points that are downloaded to a PC. A module of our public domain software WINTRACK permits a detailed numerical and graphical analysis of path geometry, phases of resting and moving, and path similarity. The device can be adapted to different species provided that satellite signals can be received reliably and that the loggers can be recovered. We expect it to be useful for testing hypotheses about pigeon homing, assessing natural spatial behavior and orientation of many species, and anticipate further miniaturization.     

Cardiac myxomas

Cardiac myxoma is the most common primary tumor of the heart. Between 1970 and 1998, 33 myxomas from patients operated at the Cardiosurgical Department were submitted for pathological examination. A review of age, sex and clinical symptoms of the patients as well as of gross and histological features of the tumors is presented. Immunohistochemical examination was performed on 10 selected myxomas-reactivity to vimentin, desmin, S-100 protein, cytokeratin and FVIIIR-Ag. The necessity of histological examination of the embolectomy material is stressed.     

Carcinoid heart disease

Demonstrated are eight cases of carcinoid heart disease (7 females, 1 male; average age 63.4 y.). The primary tumor was localized in the ileum in 5, and in the cecal area in 3 patients, with liver metastases in each case. The characteristic heart lesion appears as an endocardial plaque composed of loose intercellular matrix with scattered smooth muscle cells, covered by an endothelial layer. The plaques are apposed on an otherwise normal valvular, or mural endocardium of the right heart. The resulting combined valvular lesions show a predominance o tricuspid incompetence, or of pulmonary stenosis. In one case with a widely patent foramen ovale there was involvement of left-heart valves in addition. Local effect of serotonin appears to play the main role in pathogenesis of the endocardial plaques.     

Postpartum psychiatric disorders

The literature on postpartum psychiatric disorders (PPD) is reviewed with particular reference to recent advances in the field. Despite the prevailing tendency to consider PPD as a manifestation of other pre-existing psychiatric disorders (i.e. affective, schizophreniform, organic, neurotic, etc.) a growing number of studies point to the uniqueness and specificity of postpartum symptomatology. Theories of etiology, psychosocial as well as biological, are still wide-ranging and unconvicing. Attempts to predict PPD and/or to identify mothers-to-be at risk are based largely on retrospective studies or else are hindered by the use of nonspecific instruments. Society's implicit belief in the "joys of motherhood" may have further contributed to the obscurity surrounding these frequently occurring disorders. Interventions follow conventional methods of psycho- and pharmacotherapy but the multidisciplinary treatment approach especially for women with acute PPD who require hospital admission is gaining wider acceptance. The inpatient psychiatric mother-baby intensive care unit model is part of this approach.