Small fiber dysfunction in patients with Wilson's disease

Objective: Patients with Wilson’s disease (WD) may develop a wide variety of neuropsychiatric symptoms, but there are few reports of autonomic dysfunction. Here, we described evidence of small fiber and/or autonomic dysfunction in 4 patients with WD and levodopa-responsive parkinsonism. Method: We reviewed the charts of 4 patients with WD who underwent evaluation for the presence of neuromuscular dysfunction and water-induced skin wrinkling test (SWT). Results: Two men and 2 women (33±3.5 years) with WD were evaluated. They all had parkinsonism at some point during their disease course. Parkinsonism on patient 4 almost completely subsided with treatment of WD. Two patients had significant sensory and 2 significant autonomic complaints, including syncopal spells. NCS/EMG was normal in all but SWT was abnormal in half of them (mean 4-digit wrinkling of 0.25 and 1). Discussion: A subset of patients with WD exhibit evidence of abnormal skin wrinkling test (small fiber neuropathy).

Bibliographic Details

Main Authors:	Gondim,Francisco de Assis A., Araújo,Davi F., Oliveira,Ítalo S., Vale,Otoni Cardoso do
Format:	Digital revista
Language:	English
Published:	Academia Brasileira de Neurologia - ABNEURO 2014
Online Access:	http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2014000800592
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