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Skin Biopsy May Help to Distinguish Multiple System Atrophy–Parkinsonism from Parkinson's Disease With Orthostatic Hypotension
Author(s) -
Donadio Vincenzo,
Incensi Alex,
Rizzo Giovanni,
De Micco Rosa,
Tessitore Alessandro,
Devigili Grazia,
Del Sorbo Francesca,
Bonvegna Salvatore,
Infante Rossella,
Magnani Martina,
Zenesini Corrado,
Vignatelli Luca,
Cilia Roberto,
Eleopra Roberto,
Tedeschi Gioacchino,
Liguori Rocco
Publication year - 2020
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.28126
Subject(s) - pure autonomic failure , atrophy , skin biopsy , parkinsonism , parkinson's disease , orthostatic vital signs , medicine , pathology , autonomic nervous system , biopsy , anatomy , disease , blood pressure , heart rate
Background The differential diagnosis between multiple system atrophy parkinsonism type (MSA‐P) and Parkinson's disease with orthostatic hypotension (PD+OH) is difficult because the 2 diseases have a similar clinical picture. The aim of this study is to distinguish MSA‐P from PD+OH by immunostaining for abnormal phosphorylated α‐synuclein at serine 129 (p‐syn) in cutaneous nerves. Method We recruited 50 patients with parkinsonism and chronic orthostatic hypotension: 25 patients fulfilled the diagnostic criteria for MSA‐P and 25 patients for PD+OH. The patients underwent a skin biopsy from the cervical area, thigh, and leg to analyze somatic and autonomic skin innervation and p‐syn in skin nerves. Results Intraneural p‐syn positivity was found in 72% of patients with MSA‐P, mainly in distal skin sites. More important, p‐syn deposits in MSA‐P differed from PD+OH because they were mainly found in somatic fibers of subepidermal plexi, whereas scant autonomic fiber involvement was found in only 3 patients. All patients with PD+OH displayed widely distributed p‐syn deposits in the autonomic skin fibers of proximal and distal skin sites, whereas somatic fibers were affected only slightly in 4 patients with PD+OH. Skin innervation mirrored p‐syn deposits because somatic innervation was mainly reduced in MSA‐P. Sympathetic innervation was damaged in PD+OH but fairly preserved in MSA‐P. Conclusions The p‐syn in cutaneous nerves allows the differentiation of MSA‐P from PD+OH; MSA‐P mainly shows somatic fiber involvement with relatively preserved autonomic innervation; and by contrast, PD+OH displays prevalent abnormal p‐syn deposits and denervation in autonomic postganglionic nerves. © 2020 International Parkinson and Movement Disorder Society