Serious side effects with D-penicillamine therapy for Wilson's disease]

Immunocomplex nephritis as one of the serious side-effects is dealt with on the basis of 41 patients with Wilson's Disease who have been treated for many years and were stabilised on D-penicillinamine. In one quarter of the patients, proteinuria was found 1 to 5 years after the beginning of the therapy. Until now, an immunocomplex nephritis with diffuse granular, mainly epimembranous IgG and C3 deposits on the glomerular basement membran was found in four patients. No circulating antibodies against cell nuclei were found. The finding of immunocomplex nephritis calls for the discontinuation of the therapy for an at present unknown period of time.     

Results of nuclear medical, electroencephalographic, and angiographic examinations after brain tumor operations]

The methods of nuclear medicine and electro-encephalography allow, in combination, in almost every case the answer to the question of the cerebral tumour relapse. Our evaluation confirms the discovery of the greater sensitivity of the isotopic methods with relapses of glioblastomas, while relapses of meningiomas, astrocytomas, oligodendrogliomas and spongioblastomas can be recognized more frequently only by electro-encephalographic methods. The value of angiography is limited in cases of cerebral tumour relapses without pathological vascularisation, since the swelling can spread in the resection cavity without retroaction on the surrounding areas. For this reason vascular displacement (an only be observed very late on. Clinical judgement is furthermore complicated by the fact that after the removal of the primary tumour, often no reposition of the arteries ensues. After our exainations, a negative angiographic condition can be rule out a diagnosis of a relapse which has been established by electro-encephaolographic and isotopic methods.     

Some problems of Wilson's disease

The Leipzig Center for Wilson's Disease in the GDR is charged with the registration and diagnosis of all homozygous Wilson gene carriers, the clarification of all suspected cases, including the heterozygote test, and the co-ordination of long-term treatment. At present, there are 78 recorded Wilson gene carriers living. On the basis of our own comprehensive observations and investigations over prolonged periods of time, questions concerning pathogenesis, genetics, diagnosis and therapeutic measures, including their side-effects, are dealt with.     

Diphenylhydantoin stimulation test in Wilson's disease

Coeruloplasmin and diphenylhydantoin levels were studied both in patients who were stabilised for the first time on diphenylhyantoin (300 mg daily) up to reaching a stable serum concentration (N = 6) and during steady-state concentration (N = 25). No significant DPH-dependent influence on the coeruloplasmin level in the serum was found. In our opinion, DPH is no suitable for the stimulation of coeruloplasmin and thus for the diagnosis of Wilson's disease, particularly in the preclinical stage, because absence of coeruloplasmin increase in the serum being found also in healthy persons.     

Relations between pneumoencephalographic and electroencephalographic findings in childhood]

During simultaneous testing of electro-encephalograms and pneumoencephalograms in 200 children of various ages a proven correlation of statistics between both test procedures emerged as a well-defined parameter for evaluation. When making a statement about the correlation in findings between E.E.G. and P.E.G. the age of development in a child must be taken into consideration. Thus it emerged that in infants pronounced enlargement of the ventricular system correlates with active tension E.E.G. at increased inter-cranial pressure, while the coincidence of focal changes in E.E.G. and P.E.G. with advancing years becomes at first more pronounced after the tenth year of life (or puberty) but afterwards lessens.     

Wilson's disease in East Germany: in retrospect and perspectives -- an evaluation

Wilson's disease is an autosomal recessive inherited metabolic disorder due to a disturbance of copper metabolism. Although the primary genetic defect is not known a longlife treatment is necessary for establishing a negative copper balance by removing the metal of the abnormal body stores. Experiences in handling with this disease in our country over a period of 20 years are reported. Especially epidemiologic findings, the diagnostic procedures and the strategies in therapeutic regimes are discussed. Future advances in genomic diagnostics are mentioned.     

Episodic psychoses in epileptic juveniles with reference to electroencephalographic findings]

The present paper describes three juveniles affected with epilepsy, who suffered from episodic psychoses. Such disorders are a very rare occurrence in young persons. The patients had grand mal epilepsy with "secondary temporalization". Clinical and electroencepholographic aspects are discussed by reference to these particular cases. Also, the authors deal with questions of classification and etiopathogenesis. Finally, possible causes producing such psychoses and giving rise to the psychopathological character thereof are pointed out within the framework of a multiconditional approach.     

Clinical, electroencephalographic and computer tomographic studies of children with West and Lennox-Gastaut syndrome

The results of clinical, electroencephalographic and repeated computed tomographic examination of 14 children suffering from epileptic seizures in connection with the West and Lennox-Gastaud syndrome are analyzed and compared. Computed tomography yields little information regarding the etiology of such forms of epilepsy. Distinct clinical and electroencephalographic findings were accompanied by normal computed tomographic results in a large proportion of the cases. To avoid misinterpretation of computed tomographic findings and consequent prognostic errors in these epileptic cases it is necessary to take possible side effects of ACTH and corticosteroid treatment into account. At the climax of hormone treatment computed tomography revealed imposing changes in the form of generalized atrophy in 10 of the 14 children examined. These receded completely upon termination of hormone treatment.     

Results of neurophysiologic studies in ophthalmic migraine

The influence in 8 of our patients with ophthalmic migraine of photic driving with sine-like flashing lights on EEG background activity was checked and the visually evoked potentials following the application of diffused simple light stimuli were analyzed using the recovery cycle technique. The results indicate a slightly increased tonic activity of the structures of the mesencephalic formatio reticularis, such as may be produced by slight functional disturbances. Some vegetative secondary symptoms of migraine attacks and some of the changes in EEG observed in patients with ophthalmic migraine are explicable by such an abnormality.     

A biopsychosocial approach to liver transplant evaluation in two patients with Wilson's disease

Wilson's disease (WD) is characterized by hepatic, neurological, and/or psychiatric disturbances. In some cases, liver transplantation is indicated. Because psychologists and other health care workers play an increasing role in the evaluation of individuals presenting for transplant, an understanding of the heterogeneous phenotype of WD is important for mental health professionals working in medical settings. This article reviews two cases of patients with WD (one probable, one confirmed) presenting for liver transplantation and a biopsychosocial assessment approach is demonstrated. Patients are presented in terms of medical, psychiatric, and psychosocial history, neuropsychological examination results, and the subsequent indications for liver transplantation. Both patients exhibited neurocognitive and psychiatric symptoms. One patient was determined to be a marginally suitable candidate for transplantation, whereas the other was considered at high risk for negative outcome post-transplant. This article demonstrates the importance of considering phenotypic presentation, neurocognitive function, psychiatric status, and psychosocial circumstances in assessing transplant readiness in patients with WD. A comprehensive and integrative biopsychosocial assessment approach is appropriate for evaluating patients with WD presenting for liver transplantation.     

Pattern of familial psychoses--systematic and unsystematic schizophrenia and cycloid psychoses]

604 cases were examined, 309 of which were systematic schizophrenics, 191 were unsystematic schizophrenics and 104 cycloid psychotics. With the systematic schizophrenics the average period spent in hospital amounted to 16.9 years, with the unsystematic schizophrenics 13.8 years, with the cycloid psychotics 8.2 years. The systematic schizophrenics were discharged from hospital on average 1.5 times, the unsystematic 2.3 times, the cycloid psychotics 4.1 times. Of the systematic schizophrenics, 37.5% were never discharged after their first admittance to hospital, of the unsystematic 25.7%, of the cycloid psychotics 9.6%. Bipolar fluctuations were detected in 1.6% of the systematic schizophrenics, 49.7% of the unsystematic and 78.8% of the cycloid psychotics. 15.9% of the systematic schizophrenics had occurrences of psychoses in their family compared to 50.8% of the unsystematic and 22.1% of the cycloid psychotics. The systematic schizophrenics had 17.5% of their family ill, the unsystematic 69.6%, the cycloid psychotics 25.0%. Of the parents of the systematic schizophrenics 2.3% were ill, of the parents of the unsystematic schizophrenics 11.6%, of the parents of the cycloid psychotics 5.0%. Of the brothers and sisters of the systematic schizophrenics 2.4% were ill, of the unsystematic schizophrenics 10.9%, of the cycloid psychotics 3.0%. These variations in the pattern and occurrence of psychoses in the family seem to prove that the three groups of endogenous psychoses are genetically separable. There are also differentiating features inside the groups. Periodic catatonia in particular is characterized by the great number of psychotics in the family.     

Psychophysiologic chronometry: detection of facilitating and inhibiting processes in information processing using electroencephalographic and electromyographic data]

The objective of the present research was to tally the potential usefulness of psychophysiological measures in the systematic analysis of human information processing. Different context conditions were established in two cued choice-reaction time experiments. The context could either facilitate or inhibit the processing of an imperative stimulus. Event-related brain potentials (EEG-ERP) and event-related electromyograms (EMG-ERP) were recorded and from these biosignals latencies and duration times for different aspects of stimulus evaluation and movement execution were derived. The EEG-ERP provided measures for the time of primary stimulus categorization (N2), the time necessary to test actual stimulus information against stimulus expectancies (P3b), and the time necessary to check the decision about the stimulus-response mapping (pSW). From the EMG-ERP, processing times were derived which are related to the initiation of a motor program and to distinct sections of the movement trajectory. Results show that these latency measures react differently to different context manipulations. EEG-ERP measures, for example, reflected differences during stimulus evaluation which were not detectable in response- or EMG-derived movement times. The parallel analysis of EEG-ERPs and EMG-ERPs allowed delineation of processing modules which are responsible for particular context-dependent facilitation and inhibition effects. Moreover, the data revealed substantial overlap of evaluation times and movement times. This finding seems to be more compatible with a parallel processing model than with a strictly sequential model.     

肝豆状核变性(Wilson病)治疗策略的变革及思考

评析肝豆状核变性(WD)当前的治疗方法,主要有低铜饮食,铜的螯合剂青霉胺及曲恩汀,锌制剂和四硫钼酸胺,肝移植.介绍WD治疗策略的变革:不再提倡驱铜疗法以去除堆积的铜,而是使血液中的游离铜离子浓度常态化以逆转铜中毒,并结合2007年《柳叶刀》WD治疗专家共识进行思考.最后,展望未来对WD可能的根治疗法,即基因治疗、肝细胞移植及现在可施行的肝移植.     

肝豆状核变性（Wilson病）治疗策略的变革及思考

评析肝豆状核变性(WD)当前的治疗方法,主要有低铜饮食,铜的螯合剂青霉胺及曲恩汀,锌制剂和四硫钼酸胺,肝移植。介绍WD治疗策略的变革:不再提倡驱铜疗法以去除堆积的铜,而是使血液中的游离铜离子浓度常态化以逆转铜中毒,并结合2007年《柳叶刀》WD治疗专家共识进行思考。最后,展望未来对WD可能的根治疗法,即基因治疗、肝细胞移植及现在可施行的肝移植。     

The epidemiology of Wilson's disease in the German Democratic Republic and current problems from the viewpoint of population genetics

Between 1949 and Nov. 30, 1977 a total of 123 cases of Wilson's disease have been registered in the GDR. Using the available statistics the incidence rate of the disease is assumed to be about 2.9/100,000, the gene frequency approx. 0.53 per cent and the heterozygote incidence 1.05 per cent, supposing that the Hardy-Weinberg law applies to a large extent. Conclusions are drawn from the available material with a view to detection of the disease infuture and to genetic family counselling.     

Results of catamnestic studies on the social prognosis of childhood epilepsies

Owing to the progress in pharmakotherapy the individual prognosis of epilepsy is characterized above all by brainorganic or psychoreactive caused disorders of the adaptability. The results of the examination of 153 children are presented, which were stationary treated for epilepsy. The special problems in the family, the school and in the choice of a job are referred.     

Relevant dimensions of cyberbullying — Results from two experimental studies

Cyberbullying is a prevalent problem of adolescents. However, several conceptual and measurement questions, regarding its defining characteristics and relevant dimensions in comparison to conventional bullying, remain unanswered. To this end we conducted two studies with experimental methods. Study I shows that power imbalance in terms of perceived popularity is relevant for the affective, cognitive, and behavioral experience of cyberbullying. Cyberbullying by a popular bully is more distressing than cyberbullying by an unpopular bully. Study II shows that factors unique to cyberbullying are also relevant for the experience of cyberbullying, namely the media and the type of cyberbullying. For example, different types of cyberbullying are related to different patterns of relevant coping strategies. Therefore, cyberbullying seems both a unique phenomenon and closely related to conventional bullying.     

Illuminating the nomological network of digital aggression: Results from two studies

We examined the nomological network surrounding digital aggression (DA), in regards to other forms of aggression/antisocial behavior and individual difference variables commonly associated with other forms of aggression/antisocial behavior. Two large samples of university students (N = 713 and 633, respectively) completed a series of questionnaires and in some cases, an additional experience sampling study. Results revealed that, in emerging adulthood, DA appears to have a unique demographic profile relative to the other forms of aggression and antisocial behavior. Results further suggested that, when examined simultaneously, only social aggression and non‐aggressive rule‐breaking evidenced independent associations with DA. These associations persisted even in the absence of social media use. Unique associations with most individual difference variables dissipated once we controlled for social aggression and rule‐breaking, although DA was independently associated with extraversion and alcohol use problems. Such findings begin to embed DA in its broader nomological network and suggest clear functional links with both social aggression and non‐aggressive rule‐breaking.