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Autonomic innervation in multiple system atrophy and pure autonomic failure
V Donadio1, P Cortelli, M Elam
1Department of Neurological Sciences, University of Bologna, Bologna, Italy. vincenzo.donadio@unibo.it
Journal of Neurology, Neurosurgery, and Psychiatry
|July 28, 2010
Summary
Skin autonomic innervation analysis reliably differentiates pure autonomic failure (PAF) from multiple system atrophy (MSA). This direct assessment reveals distinct sympathetic denervation patterns, aiding in diagnosing these chronic dysautonomia conditions.
Area of Science:
- Neurology
- Autonomic Neuroscience
- Dermatology
Background:
- Pure autonomic failure (PAF) and multiple system atrophy (MSA) are distinct neurological disorders characterized by chronic dysautonomia.
- Previous indirect tests of skin autonomic function in PAF and MSA yielded conflicting results.
- Direct analysis of skin sympathetic nerve fibers in PAF and MSA is needed to identify differing lesion sites and improve differential diagnosis.
Purpose of the Study:
- To directly analyze the structure and function of skin sympathetic nerve fibers in patients with PAF and MSA.
- To identify distinct autonomic lesion sites that could help differentiate between PAF and MSA.
- To evaluate the reliability of skin autonomic innervation studies for differential diagnosis.
Main Methods:
- Eight patients with probable MSA and nine patients with PAF underwent head-up tilt test (HUTT).
- Microneurography assessed sympathetic nerve activity from the peroneal nerve.
- Punch skin biopsies from the finger, thigh, and leg were analyzed for cholinergic and adrenergic innervation using immunofluorescence and a semiquantitative score.
Main Results:
- Both MSA and PAF patients exhibited comparable neurogenic orthostatic hypotension and high rates of failed microneurographic trials.
- Immunofluorescence analysis revealed significantly different skin autonomic innervation patterns between the two groups.
- MSA patients generally had preserved skin autonomic innervation, while PAF patients showed marked postganglionic sympathetic denervation.
Conclusions:
- Skin autonomic innervation studies provide a reliable method for differentiating between MSA and PAF.
- Distinct patterns of postganglionic sympathetic denervation in skin appendages are characteristic of PAF.
- While generally preserved, mild postganglionic involvement may be observed in the leg of MSA patients with long disease duration.
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