Training of the neurologist for the 21st century.
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Biomedical subjects
Publications and source records attributed to L J Endtz.
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A case is presented in which sensory ataxic neuronopathy developed after serologically proven infection with Rickettsia conorii and continued to be progressive after appropriate antibiotic treatment. Electrophysiological studies showed decreasing sensory nerve conduction velocities ending with the absence of sensory nerve action potentials as well as peripheral and cortical somatosensory evoked potentials. Histological studies revealed a profound loss of myelinated fibres due to primarily axonal degeneration. The clinical course and the electrophysiological and histological findings suggest primary involvement of the dorsal root ganglion. Peripheral neuropathy due to infection with R. conorii is rare and usually of the mixed motor and sensory type. We believe this to be the first report of sensory ataxic neuronopathy associated with R. conorii infection.
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In recent years the name of Brown-Séquard has been assigned to syndromes caused by spinal cord damage differing widely in extension. On the basis of a review of more than 600 published cases, we propose that Brown-Séquard-plus syndromes be distinguished from the pure Brown-Séquard syndrome. This distinction is shown to be justified on clinical, anatomic, and historical grounds. Two case histories are reported herein to illustrate the essential differences between these syndromes.
A 69-year-old woman suffered transient global amnesia as a complication following coronary angiography. Three reports have been described earlier. The etiology of this condition is discussed. Possibly, this complication is overlooked in some cases.
Some of the patients with homonymous hemianopia are unaware of any visual defect, whereas others perceive this defect more of less consciously. Analysis of the CT scans of 41 cases showed that patients who are partially or fully aware of the hemianopia have purely occipital lesions. The patients who were not aware of the visual defect were found to have larger and more anteriorly situated lesions. Parietal lesions or lesions interrupting the associative pathways to the primary or secondary visual association cortex are considered to be responsible for the lack of awareness of the defect.
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This is the second report of a case in which a uterine myoma, one of the most common benign tumours of women, was the cause of a lumbosacral plexus neuropathy. The possibility of uterine myoma should be considered in the differential diagnosis of neuropathy of the lumbosacral plexus in women.
Visual suppression of caloric nystagmus was studied in five patients with hereditary ataxia before and after administration of physostigmine. All patients had an initial abnormal ocular fixation index that improved after physostigmine was given. The data indicate that there is a partly reversible disturbance of visual-vestibular interaction in patients with hereditary ataxia, caused by an impairment of a central cholinergic mechanism.
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