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Restless legs syndrome in Wilson's disease: frequency, characteristics, and mimics
Author(s) -
Trindade M. C.,
Bittencourt T.,
LorenziFilho G.,
Alves R. C.,
Andrade D. C.,
Fonoff E. T.,
BorSengShu E.,
Machado A. A.,
Teixeira M. J.,
Barbosa E. R.,
Tribl G. G.
Publication year - 2017
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/ane.12585
Subject(s) - restless legs syndrome , evening , polysomnography , neurological examination , medicine , cohort , pediatrics , chorea , dystonia , neurology , physical therapy , neurological disorder , psychology , disease , central nervous system disease , surgery , psychiatry , physics , apnea , astronomy
Objective To determine characteristics, clinical significance, frequency, and mimics of restless legs syndrome ( RLS ) in a cohort of Wilson's disease ( WD , n = 42/f = 18), compared to healthy, matched controls. Materials and methods Structured clinical interviews (patients and caregiving family members), repeated neurological examinations (afternoon and presleep), comprehensive laboratory tests, WD ‐, RLS ‐, and sleep‐specific rating scales, and video‐polysomnography. Results Thirteen patients with WD (13/42 = 31.0%) clearly fulfilled the five diagnostic criteria of RLS ; in eight patients (19.1%), the burden of RLS was clinically significant. The RLS was of moderate severity, equally distributed among sexes, manifested mainly in the evening and before falling asleep, and had developed mostly after clinical manifestation of WD (time elapsed 10.2 ± 14.5 years), still at a young mean age (27.5 ± 11.5 years). The known RLS ‐associated features were absent (normal iron and kidney parameters) or rare (positive family history, polyneuropathy). Compared to WD patients without RLS , patients with RLS were significantly elder and had suffered longer from WD . WD ‐specific RLS mimics as well as RLS confounding motor comorbidities (dystonia, tremor, chorea) were frequent and a diagnostic challenge; in difficult cases, the differentiation was reached by clinical observation of the motor behavior in the evening or at nighttime. Conclusion RLS was frequent in this cohort of WD and might be causally related to WD . RLS should be included in the diagnostic work‐up of WD . In complex motor disorders, differential diagnosis of RLS might require evening/nighttime examination and video‐polysomnography. In WD patients with a clinically significant RLS , treatment with dopaminergic substances may be considered.