Abstract
A case of primary autonomic failure (AF) with uncomplicated Parkinson's disease is presented with clinical and neurophysiological data. Special emphasis is placed on new methods of examining impairment ofunmyelinated sympathetic and afferent C-fibres. Sympathetic vasoconstrictor responses in the skin induced by deep inspiration were examined quantitatively with laser Doppler flowmetry. The vasoconstriction was markedly depressed in primary AF compared with healthy controls and similar to secondary forms of AF. Peripheral nociceptive C-fibre function was quantitatively assessed by measurement of axon reflex vasodilatation induced by histamine iontophoresis. The axon reflex vasodilatation was completely intact in primary AF in contrast to patients with secondary peripheral small fibre neuropathy. The results indicate that sympathetic C-fibres are considerably affected by the degenerative disease, whereas the afferent C-fibres seem to be totally preserved. Modern neurophysiological methods of testing sympathetic and afferent small fibre function in combination with other neurophysiological tests, e.g. brain-stem auditory evoked potentials, might help to diagnose and differentiate primary AF in early stages and make it easier to distinguish between secondary autonomic neuropathies of unknown origin that often also involve unmyelinated afferent fibres.
Similar content being viewed by others
References
Ando Y, Araki S, Shimoda O; Kano T (1992) Role of autonomic nerve functions in patients with familial amyloidotic polyneuropathy as analyzed by laser Doppler flowmetry, capsule hydrograph, and cardiographic R-R interval. Muscle Nerve 15:507–512
Bannister Sir R (1988) Autonomic failure, 2nd edn. Oxford University Press, Oxford
Bannister R, Oppenheimer DR (1972) Degenerative diseases of the nervous system associated with autonomic failure. Brain 95:457–474
Bannister R, Sever P, Gross M (1977) Cardiovascular reflexes and biochemical responses in progressive autonomic failure. Brain 100:327–344
Bannister R, Davies B, Holly T, Rosenthal T, Sever P (1979) Defective cardiovascular reflexes and supersensitivity to sympathomimetic drugs in autonomic failure. Brain 102:163–176
Baser SM, Brown RT, Curras MT, Baucom CE, Hooper DR, Polinsky RJ (1991) Beta-receptor sensitivity in autonomic failure. Neurology 41:1107–1112
Bradbury S, Eggleston C (1925) Postural hypotension: an autopsy upon a case. Am Heart J 1:73–86
Chokroverty S, Baryon KD, Katz FH, Del Greco F, Sharp JT (1969) The syndrome of primary orthostatic hypotension. Brain 92:743–768
Dotson R, Ochoa J, Marchettini P, Cline M (1990) Sympathetic neural outflow directly recorded in patients with primary autonomic failure: clinical observations, microneurography, and histopathology. Neurology 40:1079–1085
Fox RH, Goldsmith R, Kidd DJ (1962) Cutaneous vasomotor control in the human head, neck and upper chest. J Physiol (Lond) 161:298–312
Hales JRS, Iriki M, Tsuchiya K, Kozama E (1978) Thermally induced cutaneous sympathetic activity related to blood flow through capillaries and arteriovenous anastomoses. Pflügers Arch 375:17–24
Heyer G, Hornstein OP, Handwerker HO (1989) Skin reactions and itch sensation induced by epicutaneous histamine application in atopic dermatitis and controls. J Invest Dermatol 93:492–496
Kafka MS, Polinsky RJ, Williams A, Kopin IJ, Lake CR, Ebert MH, Tokola NS (1984) Alpha-adrenergic receptors in orthostatic hypotension syndromes. Neurology 34:1121–1125
Low PA, Neumann C, Dyck PJ, Fealey RD (1992) Evaluation of skin vasomotor reflexes by using laser Doppler velocimetry. Mayo Clin Proc 58:583–592
Parkhouse N, LeQuesne PM (1988) Impaired neurogenic vascular response in patients with diabetes and neuropathic foot lesions. N Engl J Med 318:1306–1309
Polinsky RJ, Kopin IJ, Ebert MH, Weise W (1981) Pharmacologic distinction of different orthostatic hypotension syndromes. Neurology 31:1–7
Prasher DK, Bannister R (1986) Brainstem auditory evoked potentials in patients with multiple system atrophy with progressive autonomic failure (Shy-Drager syndrome). J Neurol Neurosurg Psychiatry 49:278–289
Roessmann U, Van den Noon S (1971) Idiopathic orthostatic hypotension. Ann Neurol 24:503–510
Shy GM, Drager GA, (1960) A neurological syndrome associated with orthostatic hypotension. A clinical-pathologic study. Arch Neurol 2:511–527
Singh N, Fahn S (1980) Electrophysiological studies in Shy-Drager syndrome. Neurology 30:394
Spokes EGS, Bannister R, Oppenheimer DR (1979) Multiple system atrophy with autonomic failure. J Neurol Sci 43:59–82
Stewart JD, Low PA, Fealey RD (1992) Distal small fiber neuropathy: result of tests of sweating and autonomic cardiovascular reflexes. Muscle Nerve 15:661–665
Swanson Beck J, Abbot NC, Samson PD, Butlin CR, Grange JM, Cree IA, Forster A, Khan F (1991) Impairment of vasomotor reflexes in the fingertips of leprosy patients. J Neurol Neurosurg Psychiatry 54:965–971
Vanderhaeghen J-J, Périer O, Sternon JE (1970) Pathological findings in idiopathic orthostatic hypotension. Ann Neurol 22:207–214
Yazawa M, Ikeda S-I, Ushiyama M, Yanagisawa N (1991) Noradrenergic nerve fibers of the rectal mucosa in autonomic disorders: comparison of histochemical study with clinical severity and changes in plasma noradrenaline induced by standing. J Neurol Sci 104:222–229
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Baron, R., Feldmann, R. & Lindner, V. Small fibre function in primary autonomic failure. J Neurol 241, 87–91 (1993). https://doi.org/10.1007/BF00869769
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00869769