Chronic spinal muscular atrophy

Pearce, John; Harriman, D. G. F.

doi:10.1136/jnnp.29.6.509

Cited by 28 publications

(6 citation statements)

References 16 publications

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“…In some ways they resemble the cases described by Pearce and Harriman (1966) under the heading 'chronicspinal muscular atrophy', but differ in that the onset of the disease in their cases was considerably earlier, and the progression even slower. Further, two of their cases showed upper motor neurone signs.…”

Section: A Case With Long Duration and Associated Withmentioning

confidence: 63%

The central nervous system in motor neurone disease

Brownell

1970

Journal of Neurology, Neurosurgery & Psychiatry

456

193

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Section: A Case With Long Duration and Associated Withmentioning

confidence: 63%

The central nervous system in motor neurone disease

Brownell

1970

Journal of Neurology, Neurosurgery & Psychiatry

456

193

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“…Muscle Histology with conventional stains reveals atrophy of groups of fibres (group atrophy) supplied by the affected anterior horn cells (Pearce and Harriman, 1966;Gardner-Medwin et al, 1967;Hausmanowa-Petrusewicz et al, 1968;Meadows et al, 1969a and b;Munsat et al, 1969;Buchthal and Olsen, 1970;Engel, 1970). Group atrophy is pathognomic of spinal muscular atrophy (Fig.…”

Section: Diagnosismentioning

confidence: 98%

“…In neurogenic atrophy ( Fig. 4) there is branching of subterminal intramuscular nerve fibres (rarely seen in normal muscle) with collateral reinnervation, and degeneration of end-plates Pearce and Harriman, 1966;Hausmanowa-Petrusewicz et al, 1968). Branching of nerve fibres seems to be more profuse in the more benign juvenile form of spinal muscular atrophy than in the infantile form (Hausmanowa-Petrusewicz et al, 1968).…”

Section: Muscle Histochemistrymentioning

confidence: 99%

The nosology of the spinal muscular atrophies.

Emery¹

1971

Journal of Medical Genetics

146

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“…It is described in chronic neurogenic disorders (Pearce and Harriman, 1966;Drachman Murphy, Nigam, and Hills, 1967) though not in the more acute forms, perhaps as a result of a 'functional overload' in some of the remaining, innervated, hypertrophied fibres (Cazzato, 1970). In Duchenne type muscular dystrophy the incidence of splitting is lowest in the early and preclinical stages, increases to a maximum in the 1 Present address: Division of Neurology, Medical College of Virginia, Health Sciences Division, Virginia Commonwealth University, 1200 East Broad Street, Richmond, Virginia 23219, U.S.A. 2 Reprint requests to W.G.B.…”

mentioning

confidence: 99%

Longitudinal fibre splitting in muscular dystrophy: a serial cinematographic study

Isaacs

Bradley

Henderson

1973

Journal of Neurology, Neurosurgery & Psychiatry

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SUMMARY A technique of block surface-staining and serial cinematography was modified to review serial sections of normal and dystrophic muscle from the Bar Harbor 129 Re strain of mice as a preliminary study of fibre splitting in dystrophic muscle. Using this technique, muscle fibres were reconstructed for up to 1-5 mm of their length without difficulty. Split fibres were identified only when the actual separation of fibres was observed. Splitting was seen to be a significant cause of the variations in fibre diameter and was at times responsible for the formation of groups of small atrophic fibres which resembled those seen in denervation atrophy. Complex multiple splitting and recombination of daughter and parent fibres was also observed and reconstructed to scale. These results may have considerable significance for the interpretation of physiological data on both human and murine dystrophic muscle.The longitudinal splitting of muscle fibres is one of the degenerative changes which may be identified in transverse sections of muscle tissue. Daughter fibres share a common endomysial sheath but possess their own sarcolemmal membrane (Adams, Denny-Brown, and Pearson, 1962). Central migration of sarcolemmal nuclei appears to precede the longitudinal separation of the sarcoplasm (Banker, 1960). Usually, from two to five daughter fibres can be delineated in a split fibre.One of the underlying causes for splitting seems to be related to increased functional stress (Edgerton, 1970;Hall-Craggs, 1970). It is described in chronic neurogenic disorders (Pearce and Harriman, 1966;Drachman Murphy, Nigam, and Hills, 1967) though not in the more acute forms, perhaps as a result of a 'functional overload' in some of the remaining, innervated, hypertrophied fibres (Cazzato, 1970 813moderately affected stage, and is significantly lower in the more severely handicapped patient confined to a wheelchair (Bell and Conen, 1968). In murine muscular dystrophy, animals which are 'too sickly' for sale to research laboratories, and which therefore have a more severe disease show no splitting (West and Murphy, 1960).The full extent and complexities of fibre splitting have never been completely described. As a preliminary approach to this problem, a technique of serial cinematography (Hegre and Brashear, 1947;Hegre, 1951;Read, Hegre, and Russi, 1953;Hegre, 1967) was modified and adapted as a unique method to view and study relatively large numbers of serial sections of both normal and dystrophic muscle. In this preliminary study, muscle from mice with muscular dystrophy was studied. METHODSPortions of gracilis muscle were obtained from 8-12 weeks old normal and dystrophic mice of the Bar Harbor 129 Re strain. The specimens were placed in isotonic saline for 6-8 hours, allowing the muscle fibres to relax and separate. The tissue was then fixed in Susa and double embedded using a modified Peterfi technique. The muscle was embedded in

show abstract

Chronic spinal muscular atrophy

Cited by 28 publications

References 16 publications

The central nervous system in motor neurone disease

The central nervous system in motor neurone disease

The nosology of the spinal muscular atrophies.

Longitudinal fibre splitting in muscular dystrophy: a serial cinematographic study

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