Abstract
We report the electrophysiological findings and the management of 5 subjects with progressive systemic sclerosis (PSS) and clinical evidence of nerve entrapment. Three had carpal tunnel syndrome (CTS), 1 bilateral CTS and right tarsal tunnel syndrome (TTS) and 1 Guyon's canal syndrome. Only 1 patient (with CTS) showed significant clinical improvement after surgical decompression; the other 4 demonstrated a slight recovery of conduction without lasting clinical relief after conventional treatment. To explain these failures we hypothesized that these entrapment syndromes were the clinical expression of underlying diffuse damage to the peripheral nervous system (PNS). The conduction values of nerves unaffected by entrapment syndromes were within normal limits, but almost all distal velocities were below the mean of controls. Such subclinical distal peripheral neuropathy was also verified in a selected sample of 17 patients with PSS, without clinical symptoms or signs of PNS involvement. In these 17 cases the mean distal sensory and motor conduction findings of the median, ulnar, sural and tibial nerves were significantly lower than those of a control group, while no significant differences were found in the more proximal tracts of the same nerves. Furthermore, 3 of the 17 patients showed classical electrophysiological evidence of TCS and TTS without any clinical symptoms. We concluded that the subjects with PSS had subclinical polyneuropathy which may become plain polyneuropathy or nerve entrapment syndromes perhaps induced by other risk factors.
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References
Ashworth B, Tait GBW (1971) Trigeminal neuropathy in connective tissue disease. Neurology 21:609–614
Averbuch-Heller L, Steiner I, Abramsky O (1992) Neurologic manifestations of progressive systemic sclerosis. Arch Neurol 49:1292–1295
Barr WG, Blair SJ (1988) Carpal tunnel syndrome as the initial manifestation of scleroderma. J Hand Surg 13A:378–380
Beigelman PM, Goldmer F, Bayles TB (1953) Progressive systemic sclerosis. N Engl J Med 249:45–58
Beighton P, Gumpel JM, Comes NGM (1968) Prodromal trigeminal sensory neuropathy in progressive systemic sclerosis. Ann Rheum Dis 27:367–369
Currie DM, Bradshaw DC (1979) Polyneuropathy in mixed connective tissue disease presenting as progressive systemic sclerosis (scleroderma): case report and literature review. Arch Phys Med Rehabil 60:549
Dierckx RA, Aichner F, Gerstenbrand F, Fritsch P (1987) Progressive systemic sclerosis and nervous system involvement: a review of 14 cases. Eur Neurol 26:134–140
Di Trapani G, Tulli A, La Cara A, Laurienzo P, Mazza S, David P (1986) Peripheral neuropathy in course of progressive systemic sclerosis. Light and ultrastructural study. Acta Neuropathol (Berl) 72:103–110
Ebeling P, Gilliat RW, Thomas PK (1960) A clinical and electrical study of ulnar nerve lesions in the hand. J Neurol Neurosurg Psychiatry 23:1–9
Farrel DA, Medsger TA (1982) Trigeminal neuropathy in progressive systemic sclerosis. Am J Med 73:57–62
Giannini F, Passero S, Cioni R, Paradiso C, Battistini N, Giordano N, Vaccai D, Marcolongo R (1991) Electrophysiologic evaluation of local steroid injection in carpal tunnel syndrome. Arch Phys Med Rehabil 72:738–742
Gordon RM, Silverstein A (1970) A neurologic presentation in progressive systemic sclerosis. Arch Neurol 22:126–134
Hankey GJ, Gubbay SS (1988) Compressive mononeuropathy of the deep palmar branch of the ulnar nerve in cyclists. J Neurol Neurosurg Psychiatry 51:1588–1590
Hietaharju A, Rintti V, Korpela M, Frey H (1993) Nervous system involvement in systemic lupus erythematosus, Sjogren syndrome and scleroderma. Acta Neurol Scand 87:382–387
Hietaharju A, Jääskeläinen S, Kalimo H, Hietarinta M (1993) Peripheral neuromuscular manifestations in systemic sclerosis (scleroderma). Muscle Nerve 16:1204–1212
Ishida K, Kamata T, Tanizaki Y, Tsukagoshi (1993) Progressive systemic sclerosis with CNS vasculitis and cyclosporin A therapy. J Neurol Neurosurg Psychiatry 56:720
Kabati UM, Sinkoff MW (1977) Trigeminal neuralgia in progressive systemic sclerosis. Postgrad Med J 61:168–180
Kibler RF, Rose FC (1960) Peripheral neuropathy in the “collagen diseases”. A case of scleroderma neuropathy. BMJ 1:1781–1784
Lee P, Bruni J, Sukenin S (1984) Neurological manifestations in systemic sclerosis (scleroderma). J Rheumatol 11:480–483
Mondelli M, Rossi A, Passero S, Guazzi GC (1993) Involvement of peripheral sensory fibres in amyotrophic lateral sclerosis: electrophysiological study of 64 cases. Muscle Nerve 16:166–172
Ofstad E (1960) Scleroderma (progressive systemic sclerosis). A case involving polyneuritis and swelling of the lymph nodes. Acta Rheum Scand 6:65–75
Omdal R, Mellgren SI, Husby G, Salvesen R, Henriksen OA, Torbergsen T (1993) A controlled study of peripheral neuropathy in systemic lupus erythematosus. Acta Neurol Scand 88:41–46
Piper WN, Helwig EB (1955) Progressive systemic sclerosis — visceral manifestations in generalized scleroderma. Arch Dermatol 72:535–546
Potts F, Shamani BT, Young RR (1990) A study on the coincidence of carpal tunnel syndrome and generalized peripheral neuropathy. Muscle Nerve 3:440A
Quinones CA, Perry HO, Rushton JG (1966) Carpal tunnel syndrome in dermatomyositis and scleroderma. Arch Dermatol 94:20–25
Richter RB (1954) Peripheral neuropathy and connective tissue disease. J Neuropathol Exp Neurol 13:168–180
Ru Dusky BM (1964) Neurologic manifestations of collagen disease. Penn Med 67:30–34
Schady W, Sheard A, Hassell A, Holt L, Jayson MIV, Klimiuk P (1991) Peripheral nerve dysfunction in scleroderma. Q J Med 80:661–675
Sigal LH (1987) The neurologic presentation of vasculitic and rheumatologic syndromes: a review. Medicine (Baltimore) 66:157–179
Silverstein A (1963) Cerebrovascular accidents as the initial major manifestation of Lupus Erythematosus. NY State J Med 63:2942–2948
Stava Z, Stein J (1961) Electroencephalography in scleroderma. Dermatologica 123:375–390
Stewart JD (1987) The ulnar nerve. In: (eds) Focal peripheral neuropathy. Elsevier, New York, pp 163–193
Teasdall RD, Fraya RA, Shulman LE (1980) Cranial nerve involvement in systemic sclerosis (scleroderma): a case report of 10 cases. Medicine (Baltimore) 59:149–159
Tola MR, Granieri E, Caniatti L, Paolino E, Monetti C, Dovigo L, Scolozzi R, De Bastiani P, Carreras M (1992) Systemic lupus erythematosus presenting with neurological disorders. J Neurol 239:61–64
Tuffanelli D, Winkelmann RK (1961) Systemic scleroderma: a clinical study of 727 cases. Arch Dermatol 84:359–371
Wu J-S, Morris JD, Hogan GR (1985) Ulnar neuropathy at the wrist: case report and review of literature. Arch Phys Med Rehabil 66:785–788
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Mondelli, M., Romano, C., Della Porta, P. et al. Electrophysiological evidence of “nerve entrapment syndromes” and subclinical peripheral neuropathy in progressive systemic sclerosis (scleroderma). J Neurol 242, 185–194 (1995). https://doi.org/10.1007/BF00919590
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DOI: https://doi.org/10.1007/BF00919590