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Article Dans Une Revue Journal of Neurology, Neurosurgery and Psychiatry Année : 2010

AUTONOMIC INNERVATION IN MULTIPLE SYSTEM ATROPHY AND PURE AUTONOMIC FAILURE

Résumé

Background. Pure autonomic failure (PAF) and multiple system atrophy (MSA) are both characterized by chronic dysautonomia although presenting different disability and prognosis. Skin autonomic function evaluation by indirect tests has disclosed conflicting results in these disorders. Here we report the first direct analysis of skin sympathetic fibers including structure and function in PAF and MSA to ascertain different underlying autonomic lesion sites which may help differentiate the two conditions. Methods. We studied 8 patients with probable MSA (mean age 60±5 years) and 9 patients fulfilling diagnostic criteria for PAF (64±8 years). They underwent head-up tilt test (HUTT), extensive microneurographic search for muscle and skin sympathetic nerve activities from peroneal nerve and punch skin biopsies from finger, thigh and leg to evaluate cholinergic and adrenergic autonomic dermal annexes innervation graded by a semiquantitative score presenting a high level of reliability. Results. MSA and PAF patients presented a comparable neurogenic orthostatic hypotension during HUTT and high failure rate of microneurographic trials to record sympathetic nerve activity, suggesting a similar extent of chronic dysautonomia. In contrast, they presented different skin autonomic innervation in the immunofluorescence analysis. MSA patients showed a generally preserved skin autonomic innervation with a significantly higher score than PAF patients showing a marked postganglionic sympathetic denervation. In MSA patients with long disease duration morphological abnormalities and/or a slightly decreased autonomic score could be found in the leg reflecting a mild postganglionic involvement. Conclusion. Autonomic innervation study of skin annexes is a reliable method which may help differentiate MSA from PAF.
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Dates et versions

hal-00559610 , version 1 (26-01-2011)

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Vincenzo Donadio, Pietro Cortelli, Mikael Elam, Vitantonio Di Stasi, Pasquale Montagna, et al.. AUTONOMIC INNERVATION IN MULTIPLE SYSTEM ATROPHY AND PURE AUTONOMIC FAILURE. Journal of Neurology, Neurosurgery and Psychiatry, 2010, 81 (12), pp.1327. ⟨10.1136/jnnp.2009.198135⟩. ⟨hal-00559610⟩

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