An atypical case of mononeuritis multiplex in primary Sjogren's syndrome

We present an atypical case of peripheral nervous system (PNS) involvement in Sjogren's syndrome in a 63 year-old woman. Symptoms of an entrapment neuropathy were the first manifestation of the systemic disease and they were subsequently coupled to those of a mononeuritis multiplex. Clinical and laboratory signs for the diagnosis of Sjogren's syndrome became subsequently overt. The mononeuritis multiplex remained clinically limited to the upper limbs and characterized by unusually severe motor symptoms which progressed up to the development of a final complete deplegia. By contrast, sensory symptoms at the upper limbs remained mild over the entire course of the disease and the lower limbs revealed a subclinical sensory-motor damage only during the late stage.     

Surgical correction of convergent strabismus: its relationship to prism compensation

Esotropic patients whose angle of strabismus has been corrected by prisms frequently increase their angle deviation to compensate for the prismatic correction. This sensorio-motorial reaction to prism correction has been given the name of anomalous movements (a.m.). Quantification of a.m. has been made according to the amount of prisms that an esotropic patient is capable of compensating for (progressive prism compensation test - p.p. test). Some esodeviation does not compensate for any prisms at all since a.m. have not yet developed. Other cases compensate for as much as 40 or 60 prism diopters and more of over-correction of the angle deviation and they therefore have powerful a.m. The interference of these innervational forces acting on the medial recti to corrective surgery has been studied in 126 operated esotropic patients. A significant decrease from the expected surgical result (p < 0.001) has been found in patients having powerful a.m., as can be judged by the p.p. test. It is believed that a.m. are an important drawback contributing to vitiate any formula on the amount of muscle surgery to be performed in patients having no possibilities of restoring normal binocular vision.Practical advice on how to eliminate this drawback and theoretical reasoning on the significance of a.m. are offered.     

Visual and psychomotor performance of elite,intermediate and novice table tennis competitors

The purpose of this cross-sectional study was to determine whether measures on a selected groups of visual and psychomotor variables distinguished between groups of elite, intermediate and novice table tennis players. The variables included commonly of Melbourne measured clinical functions such as static visual acuity, extent of visual field and oculomotor balance and more experimental visual tasks such as recognition of, peripheral targets, saccadic latency and dynamic visual acuity. Psychomotor performance was assessed by measures of simple reaction time, choice response time and hand movement time. Although elite level competitors had significantly better dynamic visual acuity, a wider visual field and superior recognition of peripheral targets compared to less skilled competitors, the magnitude, or practical significance of these differences was not great and individually accounted for less than 5 per cent of population variance. Elite competitors had significantly faster psychomotor responses than novice players with die skill variables individually accounting for between 21 per cent and 62 per cent of population variance in psychomotor performance. These results indicate that the psychomotor parameters could be a useful part of a test battery for talent identification amongst table tennis players.     

Neuropeptides: Animal behaviour and human psychopathology

Summary Animal studies have demonstrated that neuropeptides modulate nervous system functions. It has been postulated that disturbances in neuropeptide systems may be aetiological factors in psychiatric and neurological disorders. Neuropeptides related to ACTH/MSH, including ORG 2766, increase motivation and attention and facilitate recovery processes after nerve damage. These peptides may be effective during the early stage of dementia. Vasopressin and related peptides improve memory processes in animals and humans. In addition, these peptides influence social behaviour, mood and addictive behaviour. The non-opioid -type endorphins have neurolepticlike activities in animals and antipsychotic effects in a category of schizophrenic patients. Peptides related to CCK have also been found to be effective in these patients. Some neuropeptides, e.g. TRH and PLG, have been reported to exert antidepressant effects. Further research may eventually produce neuropeptides with therapeutic action in psychiatric and neurological diseases.Parts of this article were presented on the occasion of the inauguration ceremony of the Department of Psychiatry of the University of Mainz on April 2 and 3, 1987     

Sigma-movement and optokinetic nystagmus elicited by stroboscopically illuminated stereopatterns

Summary Sigma-movement is an apparent movement seen when a stationary periodic visual pattern of the period P_s is illuminated Stroboscopically at the flash frequency f_s and smooth gaze pursuit eye movements are performed across the pattern at an angular velocity V_e = P_s · f_s deg · s^–1. Sigma-movement leads to an optokinetic nystagmus (Sigma OKN) which in turn sustains Sigma-movement perception. (1) Sigma-movement was also seen in an apparent three-dimensional periodic stripe pattern generated by two periodic monocular stimulus patterns with a certain degree of horizontal binocular disparity. (2) Sigma-movement perception and Sigma-OKN were also elicited by a Stroboscopically illuminated, stationary, random dot stereostripe pattern. The periodicity P_s of this pattern is generated on the cyclopean retina (Julesz 1971). The equation described above was also valid. When the time delay t between left eye and right eye flashes was varied, the apparent depth of the random dot stereostripe pattern decreased with increasing t, but the Sigmaeffects were not affected. (3) Sigma-movement illusion and Sigma-pursuit movements can also be induced when real three-dimensional objects composed of periodic components are Stroboscopically illuminated and adequate gaze or eye pursuit movements are induced. Sigma-movement is related to gaze movement and is therefore elicitable by eye, head or body movements. (4) Sigma-movement is presumably caused by the interaction of efference copy signals (generated in a cortical gaze pursuit system) and afferent visual signals. The present data indicate that neuronal mechanisms for this interaction are located — at least in part — at or beyond the level of binocular fusion and stereopsis.Supported by grants of the Deutsche Forschungsgemeinschaft (Gr161)     

Pathology of inverted Takotsubo cardiomyopathy

Myocardial dysfunction without coronary involvement may occur in acute cerebral diseases. The inverted Takotsubo pattern has been recently recognized as a novel heart neurologic stress-related syndrome. We report on the case of a 40-year-old woman presenting with massive subarachnoid hemorrhage and brain death. Echocardiography revealed an extensive left ventricular circumferential akinesis except at the apex. Histologic analysis of the heart confirmed the absence of myocardial infarction and revealed only sparse foci of myocyte necrosis with contraction bands in the akinetic areas.     

Other genomic disorders and congenital heart disease

Congenital heart disease (CHD) is the common birth defect worldwide. Despite its recognized burden on public health, the etiology in the vast majority of individuals remains unknown. Chromosomal abnormality plays an important role, frequently observed as large cytogenetically visible rearrangement or small submicroscopic structural variation in the genome. Several genomic disorders are now recognized that are increasingly responsible for CHD with variable penetrance. Single gene disorders, epigenetic alterations, and environmental etiologies are also significant contributors. Our understanding of the genetic basis of CHD has increased exponentially with the escalating use of next generation sequencing to identify ever so small submicroscopic genomic imbalances at the level of coding exons in CHD. This review focuses on genomic disorders other than 22q11.2 deletion, that are major players in the etiology of human cardiac malformations.     

Pre-screening via sleep logs in sleep-disordered patients—Adaptational effects, yes or no?

Summary Question of the study  Sleep logs are common tools in sleep research and clinical routine. Usually sleep logs have to be completed during a 2-week period, with the first week serving as an adaptation to the instrument itself. In the present study, we investigated whether there is indeed such an adaptation bias or not. Patients and methods  A total of 236 chronically sleep-disordered outpatients completed the standardized sleep log ‘Abend-Morgen-Protokoll’ during a 2-week pre-screening period prior to the first visit in our sleep ambulance. Two sets of items were established, the ‘instrumental’ and the ‘therapeutic’ set. The respective ratings of the first and second week (week A, B) were compared to evaluate clinically relevant changes. Results  The ratings of several ‘instrumental’ items significantly differed between week A and B. However, these changes—on average—were only marginal and therefore of little clinical importance. Regarding the ‘therapeutic’ set of items, no systematic variations could be ascertained over the assessment period. Conclusion  The present investigation could not confirm the presence of adaptation biases (instrumental, therapeutic) in a large sample of chronically sleep-disordered outpatients. Therefore, we consider a 1-week pre-screening period via sleep log as sufficient for the diagnostic process in these patients.