Summary
Ultrastructural studies on muscle biopsies from three patients with Becker's muscular dystrophy showed that the i.m. nerves presented loss or disarrangement of the neurofilaments and an increased number of glycogen granules and/or myelin figures not infrequently in the myelinated and unmyelinated nerve fibers. The neuromuscular junctions showed markedly widened sole-plate areas, and several terminal axons frequently abutted and formed neuromuscular junctions on the same fiber. The secondary synaptic clefts were markedly decreased in number and short in length in type I fibers but not in type II fibers. Most terminal axons showed no degenerative changes. Therefore, the participation of a neural factor might be suggested as the cause of Becker's muscular dystrophy, although it does not mean denervation in the conventional sense of an axonal degeneration.
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References
Appenzeller O (1975) Pathogenesis of muscular dystrophies. Sympathetic neurovascular components. Arch Neurol 32: 2–4
Becker PE (1962) Two new families of benign sex-linked recessive muscular dystrophy. Rev Can Biol 21:551–566
Becker PE, Kiener F (1955) Eine neue X-chromosomale Muskeldystrophie. Arch Psychiatr Nervenkr 193:427–448
Bradley WG, Jones MZ, Mussini J-M, Fawcett PRW (1978) Becker-type muscular dystrophy. Muscle Nerve 1:111–132
Brooke MH, Engel WK (1969) The histographic analysis of human muscle biopsies with regard to fiber types. 2. Diseases of the upper and lower motor neuron. Neurology (Minneap) 19:378–393
De Harven E, Coërs C (1959) Electron microscope study of the human neuromuscular junction. J Biophys Biochem Cytol 7–10
Dubowitz W, Brooke MH (1973) Muscle biospy: A modern approach. Saunders, London Philadelphia Toronto
Emery EAH, Smith CAB, Sanger R (1969) The linkage relations of the loci for benign (Becker type) x-borne muscular dystrophy, colour blindness and Xg blood. Ann Hum Genet 32:261–269
Engel AG, Santa T (1971) Histometric analysis of the ultrastructure of the neuromuscular junction in myasthenia gravis and in the myasthenic syndrome. Ann NY Acad Sci 183:46–63
Fukuhara N (1976) Ultrastural changes of the neuromuscular junction in myasthenic syndrome. Adv Neurol Sci 20:635–643
Fukuhara N, Takamori M, Gutmann L, Chou SM (1972) Eaton-Lambert syndrome. Ultrastructural study of the motor endplates. Arch Neurol 27:67–78
Fukuhara N, Yuasa T, Tsubaki T, Kushiro S, Takasawa N (1978) Nemaline myopathy: histological, histochemical, and ultrastructural studies. Acta Neuropathol (Berl) 42:33–41
Furukawa T, Peter JB (1977) X-linked muscular dystrophy. Ann Neurol 2:414–416
Goebel HH, Prange H, Gullota F, Kiefer H, Jones MZ (1979) Becker's X-linked muscular dystrophy. Histological, enzyme-histochemical, and ultrastructural studies of two cases, originally reported by Becker. Acta Neuropathol (Berl) 46:69–77
Harriman DGF (1976) A comparison of the fine structure of motor end-plates in Duchenne dystrophy and in human neurogenic diseases. J Neurol Sci 28:233–247
Harris JB (1980) Denervation supersensitivity — some pharmacological curiosities. Trends Neurol Sci 3:224–225
Hausmanowa-Petrusewicz I, Borkowska J (1978) Intrafamilia variability of x-linked progressive muscular dystrophy. Mild and acute form of x-linked muscular dystrophy in the same family. J Neurol 218:43–50
Jacobs PA, Hunt PA, Mayer M, Bart RD (1981) Duchennne muscular dystrophy (PMD) in a female with an x/autosome translocation. Further evidence that the DMD locus is at Xp21. Am J Hum Genet 33:513–518
Jędrzejowska H, Johnson AG, Woolf AL (1965) The intramuscular nerve endings in muscular dystrophy. A biopsy study. Acta Neuropathol (Berl) 5:225–242
Jerusalem F, Engel AG, Gomez MR (1974) Duchenne dystrophy. II. Morphometric study of motor end-plate fine structure. Brain 97:123–130
Kingstom HM, Thomas NST, Pearson PL, Safarazi M, Harper PS (1983) Genetic linkage between Becker muscular dystrophy and a polymorphic DNA sequence on the short arm of the x chromosome. J Med Genet 20:255–258
Korènyi-Both A, Szobor A, Laps K, Szathmáry I (1973) Fine structural studies in myasthenia gravis. II. Lesions of the neuromuscular junction. Eur J Neurol 10:311–323
Kuhn E, Fihn W, Schröder JM, Assmus H, Wagner A (1979) Early myocardial disease and cramping myalgia in Becker dystrophy: A kindered. Neurology (Minneap) 29:1144–1149
Mechler F, Mastaglia FL, Haggith J, Gardner-Medwin D (1980) Adrenergic receptor responses of vascular smooth muscle in Becker dystrophy. A muscle blood flow study using the133Xe clearance method. J Neurol Sci 46:291–302
Okinaka S, Kajiyama M, Ohshima K (1936) Drei Sektionsfälle von Dystrophia musculorum progressiva mit besonderer Berücksichtigung des autonomen Systems. Tokyo J Med Sci 50:1569–1583
Sakakibara H, Engel AG, Lambert EH (1977) Duchenne dystrophy: Ultrastructural localization of the acetylcholine receptor and intracellular microelectrode studies of neuromuscular transmission. Neurology (Minneap) 27:741–745
Saltis LM, Mendell JR (1974) The fine structural differences in human muscle fiber types based on peroxidatic activity. J Neuropathol Exp Neurol 33:632–640
Santa T, Engel AG (1973) Histometric analysis of neuromuscular junction. Ultrastructure in rat red, white, and intermediate muscle fibers. In: Desmedt JE (ed) New development in electromyography and clinical neurophysiology, vol 1. Karger, Basel, pp 41–54
Sjöström M, Ängquist K-A, Bylund A-C, Frieden J, Gustavsson L, Scherstén T (1982a) Morphometric analysis of human muscle fiber types. Muscle Nerve 5:538–553
Sjöström M, Kidman S, Larsén KH, Ängquist K-A (1982b) Z-and M-band appearance in different histochemically defined types of human skeletal muscle fibers. J Histochem Cytochem 30:1–11
Takamori M (1975) Contractility and supersensitivity to adrenaline in dystrophic muscle. J Neurol Neurosurg Psychiatry 38:483–492
ten Heuten R, De Visser M (1984) Histopathologic findings in Becker-type muscular dystrophy. Arch Neurol 41:729–733
Zacks SI, Blumberg JM (1961) Observations on the fine structure of mouse and human neuromuscular junctions. J Biophys Biochem Cytol 10:517–528
Zatz M, Itskan SB, Sanger R, Frota-Pessoa O, Saldanha PH (1974) New linkage data for the x-linked types of muscular dystrophy and G6 P-D variants colour blindness and Xg blood group. J Med Genet 11:321–327
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Fukuhara, N., Suzuki, M., Tsubaki, T. et al. Ultrastructural studies on the neuromuscular junctions of Becker's muscular dystrophy. Acta Neuropathol 66, 283–291 (1985). https://doi.org/10.1007/BF00690960
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DOI: https://doi.org/10.1007/BF00690960