Neuromuscular Diseases: A Practical Approach to Diagnosis by Michael Swash MD, FRCP, MRCPath, Martin Schwartz MD (auth.)

By Michael Swash MD, FRCP, MRCPath, Martin Schwartz MD (auth.)

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Extra resources for Neuromuscular Diseases: A Practical Approach to Diagnosis and Management

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Bar 10 IDS. duration of these positive sharp waves is about 4 ms (Buchthal and Rosenfalck 1966), but many are of longer duration. Their firing rate is similar to that of fibrillation potentials. The origin of positive sharp waves is uncertain but it has been suggested that, like fibrillation potentials, they originate from single muscle fibres; the initial positive deflection is due to current flow passing outward through an unexcited and damaged membrane, while the absence of a negative component implies failure of excitability of the membrane.

Unless reinnervation occurs, fibrillations will persist. The precise point of origin of fibrillation potentials in denervated muscle has been studied by stimulation experiments in which it was shown that denervated muscle fibres, probably corresponding to the small angulated fibres typically found in biopsies of patients with neurogenic disorders, contained discrete, low-threshold sites at which the fibres could be activated electrically (Trontelj and Stalberg 1983a). At these sites ephaptic activation from other fibres occurred, giving rise to paired discharges-a common finding in spontaneous fibrillations in chronic partial denervation .

1983; Hibi et al. 1984). In normal. It is also usually normal in peripheral myotonic dystrophy the CAIII level is also raised, neuropathies and other neurogenic disorders. How- probably reflecting Type 1 fibre involvement in this ever, in some severe chronic neurogenic disorders, disease (Mokuno et al. 1986). such as Kugelberg-Welander syndrome, the CK Mokuno et al. (1984) have reported that levels level may be moderately raised (see Chap. 6). of muscle-specific enolase, particularly those forms The elevation of CK level that may follow exer- containing the p subunit found in skeletal and carcise, even in normal subjects, has been utilised in the diac muscle, are elevated in muscular dystrophies diagnosis of McArdle's syndrome.

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