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.     

Oculomotor programming disturbances in the dementia syndrome

Three types of oculomotor programming disturbances are described in patients with the dementia syndrome. This report describes two of these abnormalities, poorly regulated gaze patterns and a programming type of gaze perseveration. All patients showed poorly regulated gaze patterns in contrast to the normal controls, whereas on the whole only the moderately affected patients showed the programming type of gaze perseveration. The third type of oculomotor programming disturbance is efferent perseveration of gaze (a hypokinetic, "staring" type of gaze pattern) and can be seen with severe dementia. The possible significance of these findings is discussed.     

Axiomatic and numerical conjoint measurement: An evaluation of diagnostic efficacy

Synthetic data are used to examine how well axiomatic and numerical conjoint measurement methods, individually and comparatively, recover simple polynomial generators in three dimensions. The study illustrates extensions of numerical conjoint measurement (NCM) to identify and model distributive and dual-distributive, in addition to the usual additive, data structures. It was found that while minimum STRESS was the criterion of fit, another statistic, predictive capability, provided a better diagnosis of the known generating model. That NCM methods were able to better identify generating models conflicts with Krantz and Tversky's assertion that, in general, the direct axiom tests provide a more powerful diagnostic test between alternative composition rules than does evaluation of numerical correspondence. For all methods, dual-distributive models are most difficult to recover, while consistent with past studies, the additive model is the most robust of the fitted models.Douglas Emery is now at the Krannert Graduate School of Management, Purdue University, West Lafayette, IN, on leave from the University of Calgary.     

Experimentally induced phonological paraphasias.

A motor aphasic patient had to repeat word lists that contained, among other things, words with the sequences r - r - l and l - l - r. These sequences were often repeated as l - r - l (anticipatory distant assimilation) and r - l - r (same type, but less frequently), respectively. These assimilations violate the cross-over constraint (or relevancy condition or adjacency principle) and thus represent another instance of a flagrant contradiction between pathological and normal (natural) phonological processes.     

A head-mounted scene holder for eye movement research

An inexpensive head-mounted scene holder is described. In contrast to the usual biteboard/fixed-head approach, the present apparatus allows head movements without introducing substantial artifact. This enables eye movement investigations of brain-damaged or psychiatric subjects who often cannot maintain prolonged head immobility; also, the unit makes possible subjects’ verbal reports coincident with visual scanning.     

Aortic isthmus stenosis from a neurological viewpoint

The etiology, pathogenesis, and clinical treatment of stenoses of the isthmus aortae are outlined. Following this, the particular case of a 41-year-old patient with typical stenosis of the isthmus of the aorta is reported. The course of disease required the need for a surgical operation, which had not initially been taken into consideration, to be reexamined. The patient, after performance on him of vascular restoration, is now on a part-time job. Using age as the sole circumstance indicating the inadvisability of performing a surgical operation in the case of stenosis of the isthmus aortae cannot any longer be considered justifiable.