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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European archives of psychiatry and clinical neuroscience 237 (1988), S. 230-236 
    ISSN: 1433-8491
    Keywords: Emery-Dreifuss syndrome ; Autosomal dominant inheritance ; Single-fiber EMG ; Fiber type-I atrophy ; Spinal muscular atrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The first German family with autosomal dominant Emery-Dreifuss syndrome (EDS) is described, with electrophysiologic and myopathologic results providing evidence of a primary neurogenic disease. According to classification of the scapulo peroneal syndrome without cardiomyopathy, we conclude that there are two variants of EDS: one myopathic, the other neurogenic in origin. Therefore, the term Emery-Dreifuss muscular dystrophy should be avoided. Instead, each case of EDS should be classified as myopathic or neurogenic with X chromosome recessive or autosomal dominant inheritance.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1459
    Keywords: Botulism ; Ophthalmoplegia ; Single-fibre electromyography ; Jitter ; Blockings
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of complete bilateral internal ophthalmoplegia as the sole clinical sign of botulism is reported. Diagnosis was immediately confirmed by single-fibre electromyography (SFEMG), which revealed abnormally high blocking (14.3%), contrasting with moderately increased jitter (mean consecutive difference in the extensor digitorum communis muscle, 43.9 μs). After giving equine botulinum antitoxin and simultaneous forced emptying of the bowels, ocular symptoms completely disappeared within 2 days. Six days, 5 weeks and 6 months after the first SFEMG study, the jitter was still abnormal, even becoming more so with time. Blocking, however, was only rarely observed in the follow-up studies. It is concluded that SFEMG may serve as a useful and sensitive method for the rapid diagnosis of botulinum intoxication, even in cases where no clinical signs of general muscular weakness are apparent.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Research in experimental medicine 189 (1989), S. 129-140 
    ISSN: 1433-8580
    Keywords: Auscultation ; Abdomen ; Bowel sounds ; Intestinal motility ; Acustic analysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In an attempt to find a non-invasive method yielding a comprehensive insight into a patient's intestinal activity, continuous multichannel registration and analysis of acustic phenomena as produced by the intestines were performed. Previous work, described by others, was further developed with respect to topographical interpretation of the data obtained, and to the possible introduction into general clinical use by installing different methods of artifact detection and reduction. To record and process bowel sounds, a device developed for this purpose (Phonoenteroanalyzer PEA-06, G-W Elektronik, Munich, FRG) was used. Five specially designed microphones were placed on the abdominal wall. After amplification and filtering, further processing of the signal was achieved by means of rectifying, peak detection, and logarithmic transformation techniques, yielding a topographically discernible pattern of intestinal activity. Noise and movement artifacts, although a basic problem of this technique, could be adequately managed, e.g., by using a control microphone as well as simultaneous registration of breathing. Ten normal subjects as well as eight patients with manifest or suspected gastrointestinal problems, had their bowel sounds registered either continuously overnight or during adequate stimulatory tests (gastrocolonic response, neostigmine test, rectal distension). Bowel sounds were registrated as short “clicks” or longer lasting, sometimes rhythmically appearing, complex acustic phenomena. Overnight registrations revealed periodical changes in general or local intestinal activity, which in turn showed remarkable interindividual variabilities. Stimulatory tests provoked an enhancement of intestinal activity, which could well be documented with this method. In conclusion, analysis of acustic phenomena provoked by the bowels should henceforth allow an objective, continuous, long-term, non-invasive, indirect, and at any time repeatable topographical registration of intestinal activity.
    Type of Medium: Electronic Resource
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