Summary
Localized hypertrophic neuropathy (hypertrophic mononeuropathy) is a rare benign condition that generally occurs in people under 40 years of age. Our immunocytochemical (S-100 protein) study of four new cases confirms previous observations that the cells forming the hypertrophic onion bulb are composed of perineurial cells. These observations and previously published illustrations, reveal a curious hyalinization of the outer perineurium of affected fascicles which suggests the absence of a perineurial barrier. Compartmentation (compartmentalization) of the endoneurium in hypertrophic mononeuropathy closely mimics the transient compartmentation which occurs in the distal nerve stumps of axotomized nerves, particularly in nerves in which re-innervation is prevented. Compartmentalization also can be produced by resection of the perineurial sheath. These findings suggest that hypertrophic mononeuropathy may be a reactive condition due to focal damage to the perineurial barrier.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Asbury AK, Johnson PC (1978) Pathology of peripheral nerve. Saunders, New York, pp 190–193
Bilbao JM, Khoury NJS, Hudson AR, Briggs J (1984) Perineurioma (localized hypertrophic neuropathy). Arch Pathol Lab Med 108:557–560
Boker DK, Schoenber F, Gullotta F (1984) Localized hypertrophic neuropathy — A rare, clinically almost unknown syndrome. Clin Neuropathol 3:228–230
Cajal SRY (1959) Degeneration and regeneration of the nervous system, vol 1. Hafner Publishing, New York, pp 198–204
de los Reyes RA, Chason JL, Rogers JS, Ausman JI (1982) Hypertrophic neurofibrosis with onion bulb formation in an isolated element of the brachial plexus. Neurosurgery 8:397–399
Grossiord A, Lapresle J, Lacert P, Appeloig E (1968) A propos d'une forme localisée de nevrite hypertrophique. Rev Neurol (Paris) 119:248–252
Hawkes CH, Jefferson JM, Jones EL, Smith WT (1974) Hypertrophic mononeuropathy. J Neurol Neurosurg Pschiatry 37:76–81
Hirose T, Sano T, Hizawa K (1986) Ultrastructural localization of S-100 protein in neurofibroma. Acta Neuropathol (Berl) 69:103–110
Imaginario JDG, Coelho B, Tome F, Luis MLS (1964) Nevrite interstitielle hypertrophique monosymptomatique. J Neurol Sci 1:340–347
Lallemand RC, Weller RO (1973) Intraneural neurofibromas involving the posterior interosseous nerve. J Neurol Neurosurg Psychiatry 36:991–996
Mitsumoto H, Wilbourn AJ, Goren H (1980) Perineuroma as the cause of localized hypertrophic neuropathy. Muscle Nerve 3:403–412
Morris JH, Hudson AR, Weddell G (1972) A study of degeneration and regeneration in the divided rat sciatic nerve based on electron microscopy. IV. Changes in fascicular microtopography, perineurium and endoneurial fibroblasts. Z Zellforsch 124:165–203
Nesbitt JA, Acland RD (1980) Histopathological changes following removal of the perineurium. J Neurosurg 53:233–238
Pearson JMG, Weddell AGM (1975) Perineurial changes in leprosy. Lepr Rev 46:51–67
Peckham NH, O'Boynick PL, Meneses A, Kepes J (1982) Hypertrophic mononeuropathy. Arch Pathol Lab Med 106:534–537
Roytta M, Salonen V, Pettonen J (1987) Reversible endoneurial changes after nerve injury. Acta Neuropathol (Berl) 73:323–329
Seddon HJ (1943) Three types of nerve injury. Brain 66:237–288
Simpson DA, Fowler M (1966) Two cases of localized hypertrophic neurofibrosis. J Neurol Neurosurg Psychiatry 29:80–84
Snyder M, Cancilla PA, Batzdorf U (1977) Hypertrophic neuropathy simulating a neoplasm of the brachial plexus. Surg Neurol 7:131–134
Spencer PS, Weinber HJ, Raine CS, Prineas W (1975) The perineurial window — A new model of focal demyelination and remyelination. Brain Res 96:323–329
Thomas PK, Jones DG (1967) The cellular response to nerve injury. 2. Regeneration of the perineurium after nerve section. J Anat 101:45–55
Weinberg HJ, Spencer PS (1976) The fate of Schwann cells isolated from axonal contact. J Neurocytol 7:555–569
Author information
Authors and Affiliations
Additional information
Reported in part at the American Association of Neuropathologists meeting in Minneapolis, MN, 1986
Rights and permissions
About this article
Cite this article
Johnson, P.C., Kline, D.G. Localized hypertrophic neuropathy: possible focal perineurial barrier defect. Acta Neuropathol 77, 514–518 (1989). https://doi.org/10.1007/BF00687253
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00687253