Summary
Ultrastructural examination of anterior tibial muscle from four patients with late-onset autosomal dominant distal myopathy of Welander-type revealed intrasarcoplasmic filamentous inclusions in association with rimmed vacuoles. In one of the patients, identical intranuclear filamentous inclusions were also found. These filamentous inclusions are similar to those desxribed in inclusion body myositis (IBM). They have also been observed in hereditary neuromuscular disorders including autosomal recessive distal myopathy. Thus, the filamentous inclusions occur in different neuromuscular conditions with different etiologies. These findings further raise the question of the specificity of the filamentous inclusions in IBM.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Borg K, Borg J, Edström L, Grimby L (1988) Effects of excessive use of remaining muscle fibers in prior polio and LV lesion. Muscle Nerve 11:1219–1230
Borg K, Solders G, Borg J, Edström L, Kristensson K (1989) Neurogenic involvement in distal myopathy (Welander). Histochemical and morphological observations on muscle and nerve biopsies. J Neurol Sci 91:53–70
Bouchard J-P, Gagné F, Tomé FMS, Brunet D (1989) Nuclear inclusions in oculopharyngeal muscular dystrophy in Quebec. Can J Neurol Sci 16:446–450
Brooke MH, Kaiser MM (1970) Muscle fiber types — How many and what kind? Arch Neurol 23:369–370
Carpenter S, Karpati G, Heller I, Eisen A (1978) Inclusion body myositis: a distinct variety of idiopathic inflammatory myopathy. Neurology 28:8–17
Chou SM (1986) Inclusion body myositis: a chronic persistent mumps myositis. Hum Pathol 17:765–767
Cole AJ, Kuzniecky R, Karpati G, Carpenter S, Andermann E, Andermann F (1988) Familial myopathy with changes resembling inclusion body myositis and periventricular leukoencephalopathy. A new syndrome. Brain 111:1025–1037
Coquet M, Vallat JM, Vital C, Fournier M, Barat M, Orgogozo JM, Julien J, Loiseau P (1983) Nuclear inclusions in oculopharyngeal dystrophy. An ultrastructural study of six cases. J Neurol Sci 60:151–156
Dieler R, Schröder JM (1990) Lacunar dilatations of intrafusal and extrafusal terminal cisternae, annulate lamellae, confronting cisternae and tubulofilamentous inclusions within the spectrum of muscle and nerve fiber changes in myotonic dystrophy. Pathol Res Pract 186:371–382
Edström L (1975) Histochemical and histopathological changes in skeletal muscle in late-onset hereditary distal myopathy (Welander). J Neurol Sci 26:147–157
Engel AG, Angelini C, Gomez MR (1972) Fingerprint body myopathy: a newly recognized congenital muscle disease. Proc Mayo Clin 47:377–382
Engel WK, Cunningham GC (1963) Rapid examination of muscle tissue: an improved trichrome method for fresh-frozen biopsy sections. Neurology 13:919
Fardeau M, Tomé FMS, Chevallay M, Collin H, Lebon P, Fournier JG (1986) “Inclusion body myositis-like” filamentous inclusions in several cases of hereditary neuro-muscular disorders. Muscle Nerve 9 [Suppl]: 215
Fardeau M, Askanas V, Tomé FMS, Engel WK, Alvarez R, McFerrin J, Chevallay M (1990) Hereditary neuromuscular disorder with inclusion body myositis-like filamentous inclusions: clinical, pathological, and tissue culture studies. Neurology 40 [Suppl 1]:120
Figarella-Branger D, Pellissier JF, Bianco N, Devictor B, Toga M (1990) Inflammatory and non-inflammatory inclusion body myositis. Characterization of the mononuclear cells and expression of the immunoreactive class I major histocompatibility complex product. Acta Neuropathol 79:528–536
Fukuhara N, Kumamoto T, Tsubaki T (1980) Rimmed vacuoles. Acta Neuropathol (Berl) 51:229–235
Jakobsson F, Borg K, Edström L (1990) Fibre type composition, structure and cytoskeletal protein localisation of fibres in anterior tibial muscle: comparison between young adults and physically active aged humans. Acta Neuropathol 80: 459–468
Lehmann J, Ziegan J, Oertel G, Lössner J, Kühn H-J (1986) Myopathy with mitochondrial abnormalities and rimmed vacuoles. Acta Neuropathol (Berl) 70:86–90
Lindberg C, Borg K, Edström L, Hedström A, Oldfors A (1991) Inclusion body myositis and Welander distal myopathy — A clinical, neurophysiological, structural and immunohistological comparison. J Neurol Sci (in press)
Lindholm T (1968) The influence of uraemia and electrolyte disturbances on muscle action potentials and motor nerve conduction in man. Acta Med Scand [Suppl]:491
Lotz BP, Engel AG, Nishino H, Stevens JC, Litchy WJ (1989) Inclusion body myositis. Observations in 40 patients. Brain 112:727–747
Matsubara S, Tanabe H (1982) Hereditary distal myopathy with filamentous inclusions. Acta Neurol Scand 65:363–368
Mhiri C, Gherardi R (1990) Inclusion body myositis in French patients. A clinical pathological evaluation. Neuropathol Appl Neurobiol 16:333–344
Mikol J (1986) Inclusion body myositis. In: Engel AG, Banker BQ (eds) Myology: basic and clinical. McGraw-Hill, New York, pp 1423–1438
Nonaka I, Sunohara N, Ishiura S, Satoyosi E (1981) Familial distal myopathy with rimmed vacuole and lamellar (myeloid) body formation. J Neurol Sci 51:141–155
Nishino H, Engel AG, Rima BK (1989) Inclusion body myositis: the mumps virus hypothesis. Ann Neurol 25:260–264
Padykula HA, Herman E (1955) The specificity of the histochemical method of adenosine triphosphatase. J Histochem Cytochem 3:170–183
Radner S (1962) Knappnålsteknik för iterativ muskelbiopsi. Trans Swed Soc Med Sci 19:94
Rammohan KW, Wolinsky I, Omerza J, Gales T, Kiessel I, Mendell J (1988) Mumps virus in IBM: studies implicating cross-reactivity accounting for antigen localization. Neurology 38 [Suppl 1]:151
Scarpelli DG, Hess R, Rears AGE (1958) The cytochemical localization of oxidative enzymes. J Biophys Biochem Cytol 4:747–752
Sunohara N, Nonaka I, Kamei N, Satoyoshi E (1989) Distal myopathy with rimmed vacuole formation. A follow-up study. Brain 122:65–83
Thornell L-E, Edström L, Billeter R, Butler-Browne GS, Kjörell U, Whalen RG (1984) Muscle fibre type composition in distal myopathy (Welander). An analysis with enzyme- and immuno-histochemical and gel electrophoretic and ultrastructural techniques. J Neurol Sci 65:269–292
Tomé FMS, Fardeau M (1973) “Fingerprint inclusions” in muscle fibres in dystrophia myotonica. Acta Neuropathol (Berl) 24: 62–67
Tomé FMS, Fardeau M (1980) Nuclear inclusions in oculopharyngeal dystrophy. Acta Neuropathol (Berl) 49:85–87
Tomé FMS, Fardeau M, Lebon P, Chevallay M (1981) Inclusion body myositis. Acta Neuropathol (Berl) [Suppl] VII:287–291
Tomé FMS, Gounon P, Collin H, Ploton D, Shelanski ML, Fardeau M (1989) Intranuclear inclusions in oculopharyngeal muscular dystrophy: Further studies. Neurology 39 [Suppl 1]:335
Tomé FMS, Gounon P, Borg K, Collin H, Ohayon H, Fardeau M (1990) Immunocytochemistry of the muscle fiber nucleus. J Neurol Sci 98 [Suppl]:59
Welander L (1951) Myopathia distalis tard hereditaria. Acta Med Scand 141:1–124
Yunis EJ, Samaha F (1971) Inclusion body myositis. Lab Invest 25:240–248
Author information
Authors and Affiliations
Additional information
Supported by grants from the Swedish Medical Research Council (proj. 3875, visiting research grant K. Borg), the Swedish MS foundation, the Swedish Society of Medicine, the Swedish Society for Traffic and Polio Disabled, Erik and Edith Fernströms Foundation for Medical Research and from the Karolinska Institute
Rights and permissions
About this article
Cite this article
Borg, K., Tomé, F.M.S. & Edström, L. Intranuclear and cytoplasmic filamentous inclusions in distal myopathy (Welander). Acta Neuropathol 82, 102–106 (1991). https://doi.org/10.1007/BF00293951
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00293951