Abstract
We evaluated the clinicopathophysiological features of three patients with acute autonomic and sensory neuropathy (AASN) who were followed for over 3 years. Signs of an autonomic disturbance including vomiting, anhidrosis, urinary disturbances, orthostatic hypotension and reduced coefficient of variation of the R-R interval on electrocardiography gradually improved about 1 year after onset. However, all three exhibited severe generalized sensory impairment for all modalities with the development of persistent sensory ataxia. No sensory nerve action potentials could be elicited and no somatosensory evoked potentials could be obtained. Sural nerve biopsy revealed severe axonopathy. In two patients, a high-intensity area was observed in the posterior column of the spinal cord on T2*-weighted axial magnetic resonance images. The level of neuron-specific enolase in cerebrospinal fluid was markedly elevated in two patients, indicating spinal nerve root or sensory neuron damage. Motor nerve function was well preserved in all patients. Our findings suggests that the major lesion in patients with AASN, particularly those with a sensory deficit, is present in the dorsal root ganglion neurons, that is there is a ganglioneuronopathy.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Appenzeller O, Kornfeld M (1973) Acute pandysautonomia. Arch Neurol 29:510–519
Colan RV, Snead OC, Oh SJ, Kashlan MB (1980) Acute autonomic and sensory neuropathy. Ann Neurol 8: 441–444
Fagius J, Westerberg CE, Olsson Y (1983) Acute pandysautonomia and sensory deficit with poor recovery. A clinical, neurophysiological and pathological case study. J Neurol Neurosurg Psychiatry 46:725–733
Fujii N, Tabira T, Shibazaki H, Kuroiwa Y, Ohnishi A, Nagaki J (1982) Acute autonomic and sensory neuropathy associated with elevated Epstein-Barr virus antibody. J Neurol Neurosurg Psychiatry 45:656–658
Hodson AK, Hurwitz BJ, Albrecht R (1984) Dysautonomia in Guillain-Barre syndrome with dorsal root ganglioneuropathy, Wallerian degeneration, and fatal myocarditis. Ann Neurol 15: 88–95
Hopkins A, Neville B, Bannister R (1974) Autonomic neuropathy of acute onset. Lancet I: 769–771
Inoue Y, Motegi T, Yuasa T, Atsumi T, Miyatake T (1989) Acute autonomic and sensory neuropathy associated with galactorrhea-amenorrhea syndrome and intractable anorexia. Clin Neurol (Tokyo) 29:1265–1271
Kachi T, Sobue G, Yamamoto M, Igata A (1994) Sensory conduction study in chronic sensory ataxic neuropathy. J Neurol Neurosurg Psychiatry 57: 941–944
Kimura S, Kato K, Semba R, Isobe T (1984) Regional distribution of S100ao, S-100a and S-100b in the bovine central nervous tissue determined with a sensitive enzyme immunoassay system. Neurochem Int 6: 513–518
Lichtenfeld P (1971) Autonomic dysfunction in the Guillain-Barré syndrome. Am J Med 50:772–780
Matsui M, Akiguchi I, Kameyama M (1983) A case of acute polyradiculoneuropathy with various autonomic symptoms and amenorrhea-galactorrhea syndrome. Clin Neurol (Tokyo) 23:526–530
Mokuno K, Kato K, Kawai K, Matsuoka Y, Yanagi T, Sobue I (1983) Neuron-specific enolase and S-100 protein levels in cerebrospinal fluid of patients with various neurological diseases. J Neurol Sci 60:443–451
Mokuno K, Kiyosawa K, Sugimura K, Yasuda T, Riku S, Murayama T, Yanagi T, Takahashi A, Kato K (1993) Prognostic value of cerebrospinal fluid neuron-specific enolase and S-100b protein in Guillain-Barré syndrome. Acta Neurol Scand 89:27–30
Nakagawa T, Kumamoto T, Watanabe S, Uchino M, Araki S (1988) A case of acute autonomic, sensory and motor neuropathy. Clin Neurol (Tokyo) 28: 260–267
Okada F, Yamashita I, Suwa N (1975) Two cases of acute pandysautonomia. Arch Neurol 32:146–151
Royds JA, Davies-Jones GAB, Lewtas NA, Timperley WR, Taylor CB (1983) Enolase isoenzymes in the cerebrospinal fluid of patients with diseases of the nervous system. J Neurol Neurosurg Psychiatry 46:1031–1036
Senda Y, Sugimura K, Natsume K, Koike Y, Takahashi A (1986) Acute autonomic and sensory neuropathy: a case report. Anton Nerv Syst 23: 276–286
Sindic CJM, Chalon MP, Cambiaso CL, Laterre EC, Masson PL (1982) Assessment of damage to the central nervous system by determination of 5100 protein in the cerebrospinal fluid. J Neurol Neurosurg Psychiatry 45: 1130–1135
Sobue G, Senda Y, Matsuoka Y, Sobue I (1983) Sensory ataxia: a residual disability of Guillain-Barré syndrome. Arch Neurol 40:86–89
Sobue G, Nakao N, Murakami K, Yasuda T, Sahashi K, Mitsuma T, Sasaki H, Sakai Y, Takahashi A (1990) Type I familial amyloid polyneuropathy. Brain 113:903–919
Sobue G, Yasuda T, Kachi T, Sakakibara T, Mitsuma T (1992) Chronic progressive sensory ataxic neuropathy: clinicopathological features of idiopathic and Sjögren's syndrome-associated cases. J Neurol 240: 1–7
Sterman AB, Schaumburg HH, Asbury AK (1980) The acute sensory neuronopathy syndrome: a distinct clinical entity. Ann Neurol 7:354–358
Sugenoya J, Ogawa T, Asayama M, Miyagawa T (1985) Characteristics of central sudomotor mechanism estimated by frequency of sweat expulsion. Jpn J Physiol 35:784–794
Sugisaki K, Okajima T, Ueno H (1987) Polyradiculoneuropathy with peculiar sensory and autonomic disturbances. Shinkeinaika 26:51–56
Thomashevsky AJ, Horwitz SJ, Feingold MH (1972) Acute autonomic neuropathy. Neurology 22:251–255
Tohgi H, Sano M, Sasaki K, Suzuki H, Sato T, Iwasaki T, Satodate R (1989) Acute autonomic and sensory neuropathy: report of an autopsy case. Acta Neuropathol (Berl) 77:659–663
Yasuda T, Sobue G, Hirose Y, Mimura M, Yanagi T (1994) MR of acute autonomic and sensory neuropathy. Am J Neuroradiol 15:114–115
Young RR, Asbury AK, Corbett JL (1969) Pure pandysautonomia with recovery. Trans Am Neurol Assoc 94: 355–357
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Yasuda, T., Sobue, G., Mokuno, K. et al. Clinico-pathophysiological features of acute autonomic and sensory neuropathy: A long-term follow-up study. J Neurol 242, 623–628 (1995). https://doi.org/10.1007/BF00866911
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00866911