Summary
We examined post-mortem material of the peripheral nervous system of 26 cases of motor neuron disease (MND) for the presence of lymphocyte subsets and macrophages. Findings were quantified and compared with those in control nerves. Lymphocytes in chronic and acute axonal degeneration were studied in sural nerve biopsy and animal material. Signs of demyelination were studied in MND and controls with infiltrates of T cells. A few T lymphocytes were scattered diffusely within the fascicles. The numbers did not differ between MND and controls. About half of the T cells was positive for CD45RA, the other half being positive for CD45RO. T cells were negative for CD25, CD54 and major histocompatibility complex (MHC)-class II. There were hardly and B lymphocytes. The numbers of lymphocytes in nerves with and without axonal degeneration did not differ. Increased MHC class II expression was present on denervated Schwann cells and macrophages in MND and in sural nerves with axonal degeneration. Macrophages were increased in number and in size, both in MND and in control material with axonal degeneration. No signs of demyelination were present either in MND or in controls. It is concluded that a T cell-mediated process in peripheral nerves in MND is very unlikely.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Appel SH, Stockton-Appel V, Stewart SS, Kerman RH (1986) Amyotrophic lateral sclerosis: associated clinical disorders and immunologic evaluations. Arch Neurol 43:234–238
Appel SH, Engelhardt JI, Garcia J, Stefani E (1991) Autoimmunity and ALS: a comparison of animal models of immunemediated motor neuron destruction and human ALS. Adv Neurol 56:405–412
Burnstone MS (1961) Histochemical demonstration of phosphatases in frozen sections with naphthol AS-phosphatases. J Histochem Cytochem 9:146–153
Dayan AD, Graveson GS, Illis LS, Robinson PK (1969) Schwann cell damage in motoneuron disease. Neurology 19:242–246
Dyck PJ, Stevens JC, Mulder DW, Espinosa RE (1975) Frequency of nerve fiber degeneration of peripheral motor and sensory neurons in amyotrophic lateral sclerosis: morphometry of deep and superficial peroneal nerves. Neurology 25:781–787
Graham RC, Lundholm U, Karnovsky JJ (1965) Cytochemical demonstration of peroxidase activity with 3-amino-9-ethylcarbazole. J Histochem Cytochem 13:150–152
Gurney ME (1984) Suppression of sprouting at the neuromuscular junction by immune sera. Nature 307:546–548
Gurney ME, Belton AC, Cashman N, Antel JP (1984) Inhibition of terminal axonal sprouting by serum from patients with amyotrophic lateral sclerosis. N Engl J Med 311:933–939
Hanyu N, Oguchi K, Yanagisawa N, Tsukagoshi H (1982) Degeneration and regeneration of ventral root motor fibres in amyotrophic lateral sclerosis. Morphometric studies of cervical ventral roots. J Neurol Sci 55:99–115
Hilderink PH, Veldman H, Jennekens FGI (1989) A simple method for immunostaining of axons in plastic embedded material. Proceedings of the 30th Dutch Federation Meeting. Dutch Foundation Federation of Medical Scientific Societies, Maastricht, Abstract no. 169
Kawamura Y, Dyck PJ, Masatake S, Okazaki H, Tateishi J, Doi H (1981) Morphometric comparison of the vulnerability of peripheral motor and sensory neurons in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 40:667–675
Kerkhoff H, Jennekens FGI, Troost D, Veldman H (1991) Nerve growth factor receptor immunostaining in the spinal cord and peripheral nerves in amyotrophic lateral sclerosis. Acta Neuropathol 81:649–656
Lampson LA, Kushner PD, Sobel RA (1988) Strong expression of class II major histocompatibility antigens in the absence of detectable T cell infiltration in amyotrophic lateral sclerosis (ALS) spinal cord. J Neuropathol Exp Neurol 47:353
Lampson LA, Kushner PD, Sobel RA (1990) Major histocompatibility complex antigen expression in the affected tissues in amyotrophic lateral sclerosis. Ann Neurol 28:365–372
Lange DJ, Trojaborg W, Latov N, et al (1992) Multifocal motor neuropathy with conduction block: Is it a distinct clinical entity? Neurology 42:497–505
Mancardi GL, Cadoni A, Zicca A, et al (1988) HLA-DR Schwann cell reactivity in peripheral neuropathies of different origins. Neurology 38:848–851
Ohnishi Y, Makifuchi T, Ikuta F (1980) Morphometric investigation of the myelinated fibres of the anterior spinal roots in amyotrophic lateral slcerosis and Shy-Drager syndrome. Clin Neurol (Tokyo) 20:809–815
Pestronk A, Li F (1991) Motor neuropathies and motor neuron disorders: association with antiglycolipid antibodies. Adv Neurol 56:427–432
Pollard JD, McCombe PA, Baverstock J, Batenby PA, McLeod JG (1986) Class II antigen expression and T lymphocyte subsets in chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol 13:123–134
Pollard JD, Baverstock J, McLeod JG (1987) Class II expression and inflammatory cells in the Guillain-Barré syndrome. Ann Neurol 21:337–341
Roitt I (1991) The acquired immune response. Production of effects: In: Essential Immunology, 7th edn. Blackwell Scientific Publications, Oxford, pp 129–151
Sadiq SA, Latov N (1991) Monoclonal gammopathy and motor neuron disease. Adv Neurol 56:413–420
Sadiq SA, Thomas FP, Kilidireas K, et al (1990) The spectrum of neurologic disease associated with anti-GM1 antibodies. Neurology 40:1067–1072
Santoro M, Thomas FP, Fink ME, et al (1990) IgM deposits at nodes of Ranvier in a patient with motor neuropathy, anti-GM1 antibodies and multifocal motor conduction block. Ann Neurol 28:373–379
Schröder HD, Olsson T, Solders G, Kristensson K, Link H (1988) HLA-DR expressing cells and T lymphocytes in sural nerve biopsies. Muscle Nerve 11:864–870
Shy ME, Rowland LP, Smith T, et al (1986) Moto neuron disease and plasma cell dyscrasia. Neurology 36:1429–1436
Sobue G, Matsuoka Y, Mukai E, Takayanagi T, Sobue I (1981) Pathology of myelinated fibres in cervical and lumbar ventral spinal roots in amyotrophic lateral sclerosis. J Neurol Sci 50:413–421
Sobue G, Matsuoka Y, Mukai E, Takayanagi T, Sobue I, Hahizume Y (1981) Spinal and cranial motor nerve roots in amyotrophic lateral sclerosis and X-linked recessive bulbospinal muscular atrophy: morphometric and teased fiber study. Acta Neuropathol (Berl) 55:227–235
Steck AJ, Adams D (1991) Motor neuron syndromes and monoclonal IgM antibodies to gangliosides. Adv Neurol 56:421–425
Sumner AJ (1991) Separating motor neuron diseases from pure motor neuropathies. Multifocal motor neuropathy with persistent conduction block. Adv Neurol 56:399–403
Troost D, Van den Oord JJ, De Jong JMBV, Swaab DF (1989) Lymphocytic infiltration in the spinal cord of patients with amyotrophic lateral sclerosis. Clin Neuropathol 8:289–294
Troost D, Van den Oord JJ, De Jong JMBV (1990) immunohistochemical characterization of the inflammatory infiltrate in amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol 16:401–410
Williams DB, Windebank AJ (1991) Subject review: motor neuron disease (amyotrophic lateral sclerosis). Mayo Clin Proc 66:54–82
Younger DS, Rowland LP, Latov N, et al (1990) Motor neuron disease and amyotrophic lateral sclerosis: relation of high CSF protein content to paraproteinemia and clinical syndromes. Neurology 40:595–599
Younger DS, Rowland LP, Latov N, et al (1991) Lymphoma, motor neuron diseases and amyotrophic lateral sclerosis. Ann Neurol 29:78–86
Author information
Authors and Affiliations
Additional information
Supported by a grant from the Dutch Foundation for Research into ALS and Spinal Muscular Atrophy
Rights and permissions
About this article
Cite this article
Kerkhoff, H., Troost, D., Louwerse, E.S. et al. Inflammatory cells in the peripheral nervous system in motor neuron disease. Acta Neuropathol 85, 560–565 (1993). https://doi.org/10.1007/BF00230497
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00230497