Article

Autonomic innervation in multiple system atrophy and pure autonomic failure

Department of Neurological Sciences, University of Bologna, Bologna, Italy.
Journal of neurology, neurosurgery, and psychiatry (Impact Factor: 5.58). 12/2010; 81(12):1327-35. DOI: 10.1136/jnnp.2009.198135
Source: PubMed

ABSTRACT Pure autonomic failure (PAF) and multiple system atrophy (MSA) are both characterised by chronic dysautonomia although presenting different disability and prognosis. Skin autonomic function evaluation by indirect tests has revealed conflicting results in these disorders. Here, the authors report the first direct analysis of skin sympathetic fibres including structure and function in PAF and MSA to ascertain different underlying autonomic lesion sites which may help differentiate between the two conditions.
The authors studied eight patients with probable MSA (mean age 60±5 years) and nine 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.
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 a long disease duration, morphological abnormalities and/or a slightly decreased autonomic score could be found in the leg reflecting a mild postganglionic involvement.
Autonomic innervation study of skin annexes is a reliable method which may help differentiate MSA from PAF.

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    • "However, spontaneous muscle sympathetic nerve activity (MSNA) was preserved in these patients, which fits the clinical picture of there being little disturbance to blood pressure regulation in this condition . So, while pure autonomic failure affects both SSNA and MSNA (Donadio et al., 2010), microneurography has revealed that Ross syndrome affects SSNA but not MSNA. In another rare condition associated with anhidrosis – idiopathic pure sudomotor failure – microneurography revealed that SSNA is intact; evidently the failure occurs at the cholinergic synapse with the sweat glands in this condition (Nakazato et al., 2004). "
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    • "The combination of the tyramide-based amplification system with the sABC amplification system also enables amplification of two separate antigen signals when used in parallel. Without amplification, we and others (Donadio et al. 2008; Nolano et al. 2008; Donadio et al. 2010) have been unable to visualize co-localization of sympathetic cholinergic or sympathetic adrenergic fibers within sweat glands. Using the parallel amplification technique with sABC and TSA, we report the first co-localization of sympathetic cholinergic fibers and sympathetic adrenergic fibers within sweat glands, arrector pili muscles, and surrounding blood vessels (Figure 2). "
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