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  • 1
    ISSN: 1590-3478
    Keywords: Carpal tunnel syndrome ; Natural history ; Neurophysiological classification
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario Sebbene siano stati effettuati molti studi sulla sindrome del tunnel carpale (STC), in letteratura sono riportati pochi dati sulla storia naturale di tale patologia. La conoscenza dell'evoluzione naturale ha molte implicazioni cliniche e terapeutiche. Abbiamo eseguito prospetticamente il follow-up di 80 mani affette da STC non trattate. La valutazione è stata basata su un questionario auto -somministrato e sull'esame neurofisiologico. Le mani sono state divise secondo la classificazione neurofisiologica in STC negativa, minima, lieve, medio, grave, estrema. Il peggioramento è apparso inversamente correlato al grado di sofferenza del nervo, al contrario il miglioramento è apparso proporzionale alla compromissione della funzione nervosa (ad eccezione dei casi estremi the non sono mai migliorati), circa un terzo dei casi lievi e medi sono migliorati. La maggior parte dei casi minimi sono rimasti neurofisiologicamente invariati. Le nostre osservazioni suggeriscono che in una buona percentuale dei casi affetti da STC avviene un'autolimitazione della sindrome. I nostri dati suggeriscono ulteriori studi sulla naturale evoluzione di questa comune patologia.
    Notes: Abstract Although many studies on carpal tunnel syndrome (CTS) have been reported, few data on the natural history of CTS are available. Knowledge of the natural course of the disease has significant clinical and therapeutic value. We prospectively followed up 80 cases of untreated CTS. The evaluation was based on self-administered questionnaires and on neurophysiological investigation. According to the neurophysiological classification, cases of CTS were divided into six groups on the basis of impairment severity: negative, minimal, mild, moderate, severe, and extreme. Disease worsening was inversely related to severity of nerve entrapment. On the contrary, improvement was proportionally related to nerve function impairment (except for extreme cases that never improved), and about one-third of mild and moderate cases improved. Most minimally cases remained neurophysiologically unchanged. We hypothesize that in a good percentage of CTS cases, nerve impairment is self-limited. Our data suggest the utility of further studies on the natural course of this common disease.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 63 (1984), S. 96-107 
    ISSN: 1432-0533
    Keywords: Hereditary coproporphyria ; Hereditary hepatic porphyrias ; Sural nerve biopsy ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In spite of several cases reported in the literature, the exact pathogenetic mechanism of neuropathic changes in porphyric neuropathy remains uncertain. Various authors have ascribed the neuropathologic findings to either a dying-back axonal degeneration or segmental demyelination. In recent years, the hypothesis of an axonal and myelinic disorder has received support by the demonstration of a combined and simultaneous involvement of both these structures. Such different opinions are also a consequence of the reduced number of detailed bioptic observations in the different forms of acute porphyria not only during acute phases but also between attacks. In this paper we report the results of light- and electronmicroscopic examination of two sural nerve biopsies from subjects with hereditary coproporphyria. The first was performed 6 months after an acute attack, the second specimen was obtained from a patient without acute attacks, who had clinical and electrophysiologic signs of a chronic progressive neuropathy. In both cases a dying-back axonal degeneration is considered the primary change. The pathogenetic mechanism of peripheral nerve lesions in porphyric neuropathy will be discussed finally.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Recuding body myopathy ; Desmin storage peripheral nerves [3, 16, 20] ; We describe a new condition of desmin storage in a patient with typical reducing body myopathy (RBM)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe clinical, morphological and biochemical findings of a patient with reducing body myopathy (RBM). This 15-year-old patient was affected by severe limb-girdle progressive myopathy with asymmetric distribution. Muscle biopsy showed many fibers with cytoplasmic polymorphic masses, which stained dark purple with modified Gomori's trichrome, associated with proliferation of cytoplasmic bodies. Cytoplasmic polymorphic masses showed marked reducing activity with menadione-nitro blue tetrazolium reaction. Ultrastructurally, there was great amount of highly electron-dense tubular-filamentous structures of 16–17 nm in diameter. Immunohistochemistry showed that many fibers were positive for desmin. Sodium dodecyl sulfate-electrophoresis disclosed an increase in two bands of approximately 53 and 70 kDa, and Western blot demonstrated that the 53-kDa band was desmin. It was not possible to characterize the 70-kDa protein further.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 83 (1992), S. 543-546 
    ISSN: 1432-0533
    Keywords: Giant axonal neuropathy ; Intermediate filaments
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report on a 5 1/2 year-old boy with chronic progressive polynèuropathy, ataxia, and pyramidal signs. His hair was not curled. Sural nerve biopsy disclosed many axons enlarged by accumulation of 10-nm neurofilaments and a marked variability in the number of myelinated fibers as well as in the amount of axonal enlargements among different fascicles. These findings and the electrophysiological data were consistent with a giant axonal polyneuropathy with a multifocal fiber loss.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Key words: Reducing body myopathy – Desmin storage
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. We describe clinical, morphological and biochemical findings of a patient with reducing body myopathy (RBM). This 15-year-old patient was affected by severe limb-girdle progressive myopathy with asymmetric distribution. Muscle biopsy showed many fibers with cytoplasmic polymorphic masses, which stained dark purple with modified Gomori's trichrome, associated with proliferation of cytoplasmic bodies. Cytoplasmic polymorphic masses showed marked reducing activity with menadione-nitro blue tetrazolium reaction. Ultrastructurally, there was great amount of highly electron-dense tubular-filamentous structures of 16 – 17 nm in diameter. Immunohistochemistry showed that many fibers were positive for desmin. Sodium dodecyl sulfate-electrophoresis disclosed an increase in two bands of approximately 53 and 70 kDa, and Western blot demonstrated that the 53-kDa band was desmin. It was not possible to characterize the 70-kDa protein further.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1459
    Keywords: Key words Ocular palsies ; Ptosis ; Diplopia ; Ocular myasthenia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We studied prospectively 105 unselected patients complaining of ptosis and/or diplopia due to extrinsic ophthalmic muscle palsies without other neurological signs. All patients underwent the same diagnostic protocol. The presenting symptoms were: ptosis, 35 patients (33%); diplopia, 27 patients (26%); ptosis and diplopia, 43 patients (41%). The oculomotor nerve was most frequently involved, followed by the abducens nerve. The final diagnoses were: ocular myasthenia, intracranial and/or orbital pathology, thyroid ophthalmopathy, diabetic ophthalmoplegia, mitochondrial myopathy, oculopharyngeal muscular dystrophy. In 26 patients (25%) the cause remained undetermined. Our study confirms the difficulty of establishing an aetiological diagnosis in patients with isolated ocular palsies.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1106
    Keywords: Key words Magnetic stimulation ; Motor cortex ; Corpus callosum ; Descending volleys ; Interhemispheric inhibition
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Electromyographic (EMG) responses evoked in hand muscles by a magnetic test stimulus over the motor cortex can be suppressed if a conditioning stimulus is applied to the opposite hemisphere 6–30 ms earlier. In order to define the mechanism and the site of action of this inhibitory phenomenon, we recorded descending volleys produced by the test stimulus through high cervical, epidural electrodes implanted for pain relief in three conscious subjects. These could be compared with simultaneously recorded EMG responses in hand muscles. When the test stimulus was given on its own it evoked three waves of activity (I-waves) in the spinal cord, and a small EMG response in the hand. A prior conditioning stimulus to the other hemisphere suppressed the size of both the descending spinal cord volleys and the EMG responses evoked by the test stimulus when the interstimulus interval was greater than 6 ms. In the spinal recordings, the effect was most marked for the last I-wave (I3), whereas the second I2-wave was only slightly inhibited, and the first I-wave (I1) was not inhibited at all. We conclude that transcranial stimulation over the lateral part of the motor cortex of one hemisphere can suppress the excitability of the contralateral motor cortex.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1106
    Keywords: Key words Brain stimulation ; Electrical stimulation ; Motor cortex ; Descending volleys
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The spinal volleys evoked by electric anodal and cathodal stimulation over the cerebral motor cortex hand area were recorded from a bipolar electrode inserted into the cervical epidural space of two conscious human subjects. We measured the size of volleys elicited by electric stimulation at active motor threshold and at 3% of maximum stimulator output above this value with subjects at rest and during maximum voluntary contraction of the contralateral first dorsal interosseous muscle. Surface EMG activity was recorded at the same time. Electrical anodal stimulation evoked a single negative wave that we termed D-wave in analogy with data in experimental animals. Cathodal stimulation evoked a single negative wave with a latency of 0.2 ms longer than the D-wave recruited by anodal stimulation. At both intensities tested, voluntary contraction did not modify the amplitude of the descending waves. We conclude that changes in cortical excitability induced by voluntary activity do not modify the corticospinal volley evoked by electric stimulation and that the D-waves evoked by both anodal and cathodal electric stimulation are probably initiated several nodes distant to the cell body.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1106
    Keywords: Key words Intracortical excitatory circuits ; Brain stimulation ; Motor cortex ; Descending volleys ; Magnetic stimulation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Under certain conditions, EMG responses evoked by pairs of transcranial magnetic stimuli over the motor cortex are larger than the sum of the responses to each stimulus given alone. This occurs with interstimulus intervals of around 1.3, 2.5 and 4.3 ms and could be due to interaction between the responses to each stimulus at either the cortex or spinal cord. We recorded the descending volleys set up by such pairs of stimuli from the cervical epidural space of five patients implanted with chronic stimulators for pain control. Interstimulus intervals of 1, 1.2, 1.4 and 2 ms were used to investigate the first peak of facilitation. Enhanced EMG responses occurred after pairs of stimuli at 1, 1.2 and 1.4 ms, and these were accompanied by larger and more numerous descending volleys than expected from the sum of each stimulus alone. We conclude that facilitatory interaction between the stimuli can occur within the cerebral cortex. This may involve elements that produce repetitive I-wave activity in response to a single stimulus.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1106
    Keywords: Key words Intracortical inhibitory circuits ; Brain stimulation ; Motor cortex ; descending volleys ; Human
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  A magnetic transcranial conditioning stimulus given over the motor cortex at intensities below threshold for obtaining electromyographical (EMG) responses in active hand muscles can suppress responses evoked in the same muscles at rest by a suprathreshold magnetic test stimulus given 1–5 ms later. In order to define the mechanism of this inhibitory effect, we recorded descending volleys produced by single and paired magnetic transcranial stimulation of motor cortex through high cervical, epidural electrodes implanted for pain relief in two conscious subjects with no abnormality of the central nervous system. The conditioning stimulus evoked no recognisable descending activity in the spinal cord, whilst the test stimulus evoked 3–4 waves of activity (I-waves). Conditioning stimulation suppressed the size of both the descending spinal cord volleys and the EMG responses evoked by the test stimulus. Inhibition of the descending spinal volleys was most pronounced at ISI 1 ms and had disappeared by ISI 5 ms. It was evident for all components following the I1-wave, while the I1-wave itself was not inhibited at all. We conclude that a small conditioning magnetic stimulus can suppress the excitability of human motor cortex, probably by activating local cortico-cortical inhibitory circuits.
    Type of Medium: Electronic Resource
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