Summary
The clinico-pathologic findings in two patients with chronic relapsing polyneuritis are reported.
The patients had several attacks of diffuse polyneuritis followed by partial recovery. The tendency to persisting disability increased following relapses and in the late stages there was a progressive motor deterioration suggesting a continuing activity of nerve damage.
The biopsies of peripheral nerves, obtained in the advanced stages of the disease, showed severe loss of myelinated fibers and aspectes of repeated demyelination and remyelination, with formation of onion bulb complexes. Completely demyelinated axons together with remyelinating fibers as well as remyelinated fibers invested by macrophages were seen at the same time.
The clinical and pathological evidences suggest that in chronic relapsing polyneuritis, at least in the late stages of the disease, besides clinical relapses, there is a continuing activity of nerve damage. Thus, due to this ongoing activity of demyelination and remyelination, the elimination of the supernumerary Schwann cells does not take place satisfactorily and hypertrophic changes of the nerve fascicles are produced.
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References
Arnason BGW (1975) Inflammatory polyradiculoneuropathies. In: Dyck PJ, Thomas PK, Lambert EH (eds) Peripheral neuropathy, vol 2. Saunders, Philadelphia, pp 1110–1168
Asbury AK, Arnason BG, Adams RD (1969) The inflammatory lesion in idiopathic polyneuritis. Medicine 48:173–216
Austin JH (1956) Observation on the syndrome of hypertrophic neuritis (the hypertrophic interstitial radiculo-neuropathies). Medicine 35:187–237
Austin JH (1958) Recurrent polyneuropathies and their corticosteroid treatment — with five-year observations of a placebo-controlled case treated with corticotrophin, cortisone and prednisone. Brain 81:157–194
Ballin RHM, Thomas PK (1969a) Electron microscope observations on demyelination and remyelination in experimental allergic neuritis. Part I. Demyelination. J Neurol Sci 8:1–18
Ballin RHM, Thomas PK (1969b) Electron microscope observations on demyelination and remyelination in experimental allergic neuritis. Part II. Remyelination. J Neurol Sci 8:225–237
Cazzato G (1965) Rapporti fra “poliradicolonevriti recidivanti” e “neurite interstiziale ipertrofica progressiva sporadica dell'adulto”. Riv Pat Nerv Ment 86:325–354
Dalakas MC, Engel WK (1980) Immunoglobulin and complement deposits in nerves of patients with chronic relapsing polyneuropathy. Arch Neurol 37:637–640
Dalakas MC, Houff SA, Engel WK, Madden DL, Sever JL (1980) CSF “monoclanal” bands in chronic relapsing polyneuropathy. Neurology 30:864–867
Dalakas MC, Engel WK (1981) Chronic relapsing (dysimmune) polyneuropathy: Pathogenesis and treatment. Ann Neurol [Suppl] 9:134–145
Dolman CL, Allan BM (1973) Relapsing hypertrophic neuritis. Arch Neurol 28:351–353
Harris W, Newcomb WD (1929) A case of relapsing interstitial hypertrophic polyneuritis. Brain 52:108–116
King RHM, Pollard JD, Thomas PK (1975) Aberrant remyelination in chronic relapsing experimental allergic neuritis. Neuropathol Appl Neurobiol 1:367–378
Krücke W (1955) Progressive hypertrophische Neuritis. In: Lubarsch O, Henke F, Rössle R (Hrsg) Handbuch der speziellen pathologischen Anatomie und Histologie, Bd 13 (Nervensystem), Teil 5 (Erkrankungen des peripheren und vegetativen Nervensystems), Springer, Berlin, S 84
Lampert PW (1969) Mechanism of demyelination in experimental allergic neuritis: Electron-microscopic studies. Lab Invest 20:127–138
Nattrass JF (1921) Recurrent hypertrophic neuritis. J Neurol Psychopathol 2:159–165
Pleasure DE, Towfighi J (1972) Onion bulb neuropathies. Arch Neurol 26:289–301
Pollard JD, King RHM, Thomas PK (1975) Recurrent experimental allergic neuritis. J Neurol Sci 24:365–383
Prineas JW (1971) Demyelination and remyelination in recurrent idiopathic polyneuropathy: An electron microscope study. Acta Neuropathol (Berl) 18:34–57
Prineas JW, McLeod JM (1976) Chronic relapsing polyneuritis. J Neurol Sci 27:427–458
Raine CS (1976) Experimental allergic encephalomyelitis and related conditions. In: Zimmerman M (ed) Progress in neuropathology, vol 3. Grune & Stratton, New York, pp 225–251
Raine CS (1977) Schwann cell responses during recurrent demyelination and their relevance to onion-bulb formation. Neuropathol Appl Neurobiol 3:453–470
Raine CS (1978) Pathology of demyelination. In: Waxman SG (ed) Physiology and pathology of axons. Raven Press, New York, pp 283–311
Raine CS, Wisniewski H, Prineas JW (1969) An ultrastructural study of experimental demyelination and remyelination. II. Chronic experimental allergic encephalomyelitis in the peripheral nervous system. Lab Invest 21:316–327
Raine CS, Wisniewski H, Dowling PC, Cook SD (1971) An ultrastructural study of experimental demyelination and remyelination. IV. Recurrent episodes and peripheral nervous system plaque formation in experimental allergic encephalomyelitis. Lab Invest 25:28–34
Thomas PK, Lascelles RG, Hallpike JF, Hewer RL (1969) Recurrent and chronic relapsing Guilian-Barré polyneuritis. Brain 92:589–606
Waksman BH (1963) Experimental immunological disease of the peirpheral nervous system. In: Rose A, Pearson CM (eds) Mechanism of demyelination. McGraw-Hill, New York
Waksman BH, Adams RD (1955) An experimental disease of rabbits induced by the injection of peripheral nerve tissue and adjuvants. J Exp Med 102:213–236
Webster HDeF, Schröder JM, Asbury AK, Adams RD (1967) The role of Schwann cells in the formation of “onion bulbs” found in chronic neuropathies. J Neuropathol Exp Neurol 26:276–299
Wisniewski H, Prineas JW, Raine CS (1969) An ultrastructural study of experimental demyelination and remyelination. I. Acute experimental allergic encephalomyelitis in the peripheral nervous system. Lab Invest 21:105–118
Wisniewski H, Brostoff SW, Carter H, Eylar EH (1974) Recurrent experimental allergic polyganglioradiculoneuritis. Arch Neurol 30:347–359
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Rizzuto, N., Moretto, G., Monaco, S. et al. Chronic relapsing polyneuritis. Acta Neuropathol 56, 179–186 (1982). https://doi.org/10.1007/BF00690633
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DOI: https://doi.org/10.1007/BF00690633