[Ambiguous unilateral visual impairment with hyperopia].
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Biomedical subjects
Publications and source records attributed to H Wilhelm.
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Multiple sclerosis (MS) is the most frequent chronic inflammatory disease of the central nervous system. Mostly young adults present with a variety of different symptoms due to the multiple localisations of the inflammatory lesions. Up to one-third of MS patients experience symptoms of optic neuritis as the initial symptom. That is the reason why the ophthalmologist often is the first physician contacted by patients later on diagnosed with MS. Today, it is known that there is already a significant irreversible axonal loss in MS patients progressing from the beginning of the disease. Therefore early, diagnosis and application of available therapeutic options are necessary for the patient's benefit. The therapeutic aim in early immunomodulatory treatment is to decrease the number of relapses and to slow down the development of clinical disability. This interdisciplinary overview presents guidelines for the clinical routine: how to assess the individual risk of each patient and how to treat the patient in accordance with current pathogenic, diagnostic and therapeutic knowledge.
We report on results of specific heat measurements on single crystals of the frustrated quasi-2D spin-1/2 antiferromagnet Cs2CuCl4 (T(N)=0.595 K) in external magnetic fields B<12 T and for temperatures T>30 mK. Decreasing B from high fields leads to the closure of the field-induced gap in the magnon spectrum at a critical field Bc approximately = 8.51 T and a magnetic phase transition is clearly seen below Bc. In the vicinity of Bc, the phase transition boundary is well described by the power law Tc(B) proportional, variant (Bc-B)(1/phi), with the measured critical exponent phi approximately =1.5. These findings are interpreted as a Bose-Einstein condensation of magnons.
PURPOSE: Since completion of the AREDS study, evidence has accumulated to suggest that some form of dietary supplementation may be of value in special individuals who are at risk of progressive age-related macular degeneration (ARMD). We wanted to find out which of the many supplements available are recommended in practice and which instructions, promises, and purchasing arguments are used by German pharmacies. METHODS: A blinded consultation following a standardized protocol was carried out in 60 pharmacies. RESULTS: In 36 pharmacies specific products for dietary supplementation were recommended, but the dosage never complied with the AREDS study, and in 24 pharmacies, the necessity for a medical examination or consultation with an ophthalmologist was not mentioned. Possible interactions with other drugs or side effects were generally excluded. CONCLUSIONS: Potential risks of supplement intake were underestimated. Medical knowledge of the most frequent cause of blindness in the Western world was insufficient. A clear policy of recommendations according to evidence-based criteria is needed.
BACKGROUND: Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a chronic inflammatory disease of unknown origin that is morphologically characterized by a local fibrotic thickening of the dura mater with a sterile lymphocytic infiltrate. Patients usually present with chronic headache, ataxia and cranial nerve palsies. Because of the great diversity of symptoms and the fact that IHCP can be associated with many other disorders, there is often a long time between the occurrence of clinical features and making the diagnosis. During this time symptoms can further increase. In addition, this is the third case report describing bone involvement in this disorder. CASE REPORT: In May 2000, a 51-year-old woman presented with reduced visual acuity on both eyes, complete palsies of the fourth and sixth cranial nerve, a partial palsy of the third cranial nerve and numbness and pain concerning the area of the fifth cranial nerve on the left side, painful eye movement on the left side, headache, and numbness and palsies on the left side of her body. Magnetic resonance imaging of the brain showed a focal gadolinium-enhanced small thickening of the dura mater close to the left hemisphere and a homogeneous bone thickening in the same area close to the thickened dura mater. Cerebrospinal fluid analysis revealed an elevated cell count (lymphocytic pleocytosis), routine blood counts and serum chemistry showed an elevated white blood count, an elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Therapy with corticosteroids resulted in a complete remission in November 2000 except for a persistent numbness of the left side of her face and body and reduced visual acuity on both sides. The following three years were characterized by repeated clinical deteriorations followed by a temporary increase of the daily steroid dose. Altogether, daily oral steroid therapy could slow down the progression of disease and improve the visual acuity when compared with the first examination. CONCLUSIONS: Chronic headache, ataxia and cranial nerve palsies in combination with inflammation and dural thickening should call to mind idiopathic hypertrophic cranial pachymeningitis. In principle, every ophthalmological or neurological symptom can be associated with IHCP.
In a former study of a patient with cerebellar agenesis (HK) mild motor deficits, problems in delay eyeblink conditioning and mild to moderate deficits in IQ, planning behavior, visuospatial abilities, visual memory, and attention were found. The present study reports additional findings in the same patient. In the motor domain, impairments in fine motor manipulations, trace eyeblink conditioning and motor imagination in a functional magnetic resonance (fMRI) study were found. Based on fMRI findings; however, cortical areas involved in a tapping task did not significantly differ from a healthy control group. In the cognitive domain, deficits in speech comprehension as well as verbal learning and declarative memory were present. No significant affective symptoms were observed. Although problems in executive, visuospatial and language tasks are in agreement with the so-called cerebellar cognitive affective syndrome-other possibilities remain. Non-motor impairments in HK might also be a consequence of lacking motor abilities in development and motor deficits may interfere with the performance of parts of the cognitive tasks. In addition, lack of promotion and learning opportunities in childhood may contribute and mental retardation based on extracerebellar dysfunction cannot be excluded.
Pupil campimetry is an objective test of the visual field. In pupil campimetry, the pupil light response elicited by focal light stimuli at different locations within the visual field is measured. Pupil campimetry was performed in patients with visual field loss because of different pathologies. The match between reduced pupil response and visual field loss was assessed. The pupil field of 23 patients was recorded by infrared video-pupillography with light stimuli generated on a computer screen. A visual field was obtained on the same day with the Tuebingen Automated Perimeter. Both fields were compared by subjective judgement. Six of nine patients with large, two of five patients with incomplete, and three of six patients with minor field loss showed matching defects in their pupil and their visual fields. In functional loss of visual field sensitivity, no pupil field defect was seen (three patients). In most patients, the loss of visual field sensitivity could be revealed objectively by pupil campimetry.
AIM: To evaluate the properties of devices for measuring stray light and glare: the Nyktotest, Mesotest, "conventional" stray light meter and a new, computer implemented version of the stray light meter. METHODS: 112 subjects, divided in three groups: (1) young subjects without any eye disease; (2) elderly subjects without any eye disease, and (3) subjects with (early) cataract in at least one eye. All subjects underwent a battery of glare and stray light tests, measurement of visual acuity, contrast sensitivity, refraction, and LOCS III cataract classification. Subjects answered a questionnaire on perceived disability during driving. RESULTS: Repeatability values were similar for all glare/stray light tests. Validity (correlation with LOCS III and questionnaire scores), discriminative ability (ability to discriminate between the three groups), and added value (to measurement of visual acuity and contrast sensitivity) were all superior for both stray light meters. Results of successive measurements are interrelated for the conventional but not the new stray light meter. This indicates a better resistance to fraud for the latter device. CONCLUSIONS: The new computer implemented stray light meter is the most promising device for future stray light measurements.
PURPOSE OF THE STUDY: To examine bicycling habits of visually handicapped people in order to learn about the visual requirements. MATERIAL AND METHODS: Survey of patients (age range: 14-60 years) suffering from macular or tapetoretinal degeneration with no additional relevant handicap using a questionnaire. RESULTS: A total of 232 questionnaires were returned (62% of the questionnaires received by the participants). The results showed that if visual acuity dropped below 0.1, only 15% of the patients were able to ride a bicycle without accidents or near-accidents. This percentage increased for higher acuities between 33% (<0.2 > or = 0.1) and 67% (> or = 0.2 < or = 0.4). For visual field diameters <60 degrees and > or = 20 degrees approximately 18% were able to ride a bicycle safely and only 8% if the visual field diameter was below 20 degrees. For visual fields larger than 60 degrees diameter approximately 40% were able to ride a bicycle without problems. With a central scotoma of more than 10 degrees diameter only 21% of the patients could cycle safely, whereas with a central scotoma of 10 degrees and less this percentage increased to 47%. CONCLUSIONS: A visual acuity of less than 0.1 is not sufficient to ride a bicycle, and a visual acuity of less than 0.2 leads to a higher accident rate. A visual field diameter of less than 60 degrees or a central scotoma of more than 10 degrees diameter for the vast majority of patients does not allow safe cycling
About 50 patients contact an ophthalmologist every month because of headache. Only 20 % can be helped by ophthalmological therapeutic efforts. The vast majority of those patients suffer from migraine. This contribution gives an overview of the most common causes of headache and their therapy. The ophthalmologist should not only exclude an ophthalmological cause of the headache but also contribute to making the correct diagnosis and initiating further treatment.
Direct or indirect trauma may damage the optic nerve and result in permanent visual loss. The diagnosis of traumatic optic neuropathy is not always straightforward and is complicated by unfavourable circumstances for the examination. The diagnosis should only by established if it can be based on a clear objective finding, a relative afferent pupillary defect or a pathological flash-evoked visual response. Concerning therapy, surgical decompression (even without direct injury to the nerve) or megadose steroids or both in combination have been recommended. But even spontaneous improvement may occur. A large multicentre study could not demonstrate a significant advantage for any of the measures recommended, not even against the spontaneous course. However, the study was not randomised. In animal experiments steroids have shown an unfavourable effect. Currently, therapeutic decisions have to be made on an individual basis, in the absence of any evidence-based recommendations.
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Fulminant processes of the orbit can cause considerable diagnostic difficulties with regards to the clinical, radiological and microbiological appearance. Rhino-orbito-cerebral mucormycosis is a rare but, when untreated, fatal orbital disease capable of destructive infiltration of soft tissue and bone. It occurs preferably in the ill adjusted diabetic or in the course of an immunodeficiency. The following case report describes a leukaemia patient developing a severe rhino-orbito-cerebral mucormycosis which was initially not recognised. Since untreated mucormycosis is fatal, an aggressive surgical approach with excision of the whole necrotic area is necessary even if the diagnosis cannot be confirmed with certainty.
The point at absolute zero where matter becomes unstable to new forms of order is called a quantum critical point (QCP). The quantum fluctuations between order and disorder that develop at this point induce profound transformations in the finite temperature electronic properties of the material. Magnetic fields are ideal for tuning a material as close as possible to a QCP, where the most intense effects of criticality can be studied. A previous study on the heavy-electron material YbRh2Si2 found that near a field-induced QCP electrons move ever more slowly and scatter off one another with ever increasing probability, as indicated by a divergence to infinity of the electron effective mass and scattering cross-section. But these studies could not shed light on whether these properties were an artefact of the applied field, or a more general feature of field-free QCPs. Here we report that, when germanium-doped YbRh2Si2 is tuned away from a chemically induced QCP by magnetic fields, there is a universal behaviour in the temperature dependence of the specific heat and resistivity: the characteristic kinetic energy of electrons is directly proportional to the strength of the applied field. We infer that all ballistic motion of electrons vanishes at a QCP, forming a new class of conductor in which individual electrons decay into collective current-carrying motions of the electron fluid.
Cases of cerebellar agenesis are rare. The degree of motor impairment is a matter of discussion. It has been claimed that normal motor function can be observed. Detailed descriptions of neurological findings, however, are lacking. Neuropsychological testing in cerebellar agenesis is of additional interest based on recent findings of impaired non-motor functions in cerebellar disease. The case of an elderly woman with cerebellar agenesis is presented. 3D-MR imaging was used to confirm the diagnosis. Neurological and neuropsychological examination was performed including video documentation (see the authors' own website). To assess deficits of motor learning eyeblink conditioning was investigated. Neurological examination revealed mild to moderate signs of cerebellar dysarthria, upper and lower limb ataxia and ataxia of stance and gait. Motor learning was affected as shown by inability to acquire conditioned eyeblink responses. In addition, neuropsychological testing disclosed mild to moderate deficits in IQ, planning behavior, visuospatial abilities, memory and attention. Cerebellar ataxia, although clearly present, was less than one would expect in almost complete absence of the cerebellum. Neuropsychological deficits, on the other hand, appeared to be more marked than one would expect in cerebellar disease. No conclusion, however, could be drawn whether impaired cognitive development and neuropsychological test performance were directly related to lack of cerebellar function, or caused by impaired motor development and performance.
AIM: To determine the relation between perceived driving disability and vision screening tests. METHODS: 93 subjects, aged 50 years and over, with binocular visual acuity of at least 20/80. Perceived driving disability (PDD) was assessed by a questionnaire. Subtracting daytime from night-time driving question scores revealed PDD at night (PDDN), subtracting scores of questions for driving in familiar places from those in unfamiliar places revealed PDD at unfamiliar places (PDDU). RESULTS: PDD was strongly related to visual acuity, contrast sensitivity and useful field of view (UFOV). Specific relations existed between PDDN and Nyktotests and Mesotests and between PDDU and UFOV. These associations were enhanced in a subset of subjects with better visual acuities. CONCLUSIONS: Vision screening tests correlate well with perceived driving disabilities, especially when a subtraction method is used in the questionnaire to reveal condition dependent disabilities. Additional tests for visual acuity are useful, especially in subjects with better visual acuity.
BACKGROUND: Primary optic nerve sheath meningioma (ONSM) is a rare but almost invariably blinding tumour when its natural history is observed in a "wait and see" strategy. Surgery has hitherto only been advocated in case of progressive disease involving intracranial structures, as it leads to iatrogenic blindness in the overwhelming majority of cases. Therefore, treatment options bearing lesser risk of functional deterioration are highly desirable, both in cases of intracranial involvement as well as during earlier phases of the disease which are currently generally left untreated. The authors report the outcome of the largest series of patients to date treated by stereotactic fractionated irradiation as a new treatment approach in ONSM at all stages. METHODS: 15 patients (16 nerves) underwent stereotactic fractionated conformal irradiation with a total dose of 54 Gy, using standard fractionation. Main outcome parameters included visual acuity and visual field, as well as three dimensional remission as documented by imaging. RESULTS: Tumour control was confirmed in all 15 patients undergoing stereotactic fractionated conformal irradiation (mean follow up 37 (range 12-71) months). No patient developed functional deterioration during or after treatment. Moreover, visual acuity improved by more than two lines in one patient and the visual field improved in six cases. Visual outcome in the other patients remained unchanged. There were no significant side effects of radiation therapy. CONCLUSION: These data provide convincing evidence that stereotactic fractionated conformal irradiation is an effective treatment option for primary ONSM with minimal treatment related morbidity. It should therefore be considered as therapeutic option both in early stage ONSM where surgery cannot be justified as well as in later stages, where surgery is so far considered the first line approach.
During a scientific examination in July 1999 both crypts below the St. Martin's Church in Grünstadt, Germany, were opened and 9 coffins from the county family of Leiningen examined. This paper is concentrating on one of these persons: Georg Hermann (1679-1751), count of Leiningen-Westerburg-Altleiningen, who gave during the 18. century the city its barock character. He was also responsible for the rebuilding of the church. His skeleton revealed interesting pathological changes. Few years before his death the count had the accident to get run over by a heavy wagon which crushed the distal part of his legs. The fractures healed, but gave him an ancylotic and shortened left leg, which must have caused him a lot of suffering in his last years.