Neurological features and management of Wilson disease in children: an evaluation of 12 cases.
OBJECTIVE
Wilson's disease is an autosomal recessive disorder of copper metabolism which leads to copper overload in different tissues of the body. The aim of this study was to present the neurologic features of Wilson's disease and to assess the clinical course of neurological findings in children receiving anti-copper treatment.


METHODS
Twelve children with a diagnosis of Wilson's disease and findings of central nervous system involvement who were followed up in the Department of Pediatric Neurology and Pediatric Gastroenterology of the School of Medicine at Erciyes University were enrolled in the study.


RESULTS
The study cases consisted of five boys (42%) and seven girls (58%). The mean age at the time of diagnosis was 9.9±3.4 years (5-15 years). The mean duration of follow-up was 49.0±36.4 months (15-128 months). Neurological findings at presentation included headache in seven cases (58%), tremor in seven cases (58%), dystonia in three cases (25%), ataxia in two cases (17%), dizziness in two cases (17%), numbness in the hands and acute weakness in one case (8%) and syncope in one case (8%). Headache, dizziness, syncope, numbness in hands and acute weakness symptoms resolved completely within six months after receiving treatment. Movement disorders either decreased or remained stable in seven of the eight cases. However, one patient developed progressively worsening dystonia despite to all treatments.


CONCLUSIONS
Wilson's disease can be manifested with signs and symptoms of central nervous system in the childhood. Wilson's disease should be considered in all children presenting with movement disorders. A complete neurological assessment should be carried out in all cases with Wilson's disease.
Department of Pediatrics, Division of Pediatric Neurology, Erciyes University School of Medidine, Kayseri, Turkey.
Ayşe Kaçar
AK
Bayram
Department of Pediatrics, Division of Pediatric Neurology, Erciyes University School of Medidine, Kayseri, Turkey.
Hakan
H
Gümüş
Department of Pediatrics, Division of Pediatric Gastroenterology, Erciyes University School of Medicine, Kayseri, Turkey.
Duran
D
Arslan
Department of Pediatrics, Division of Pediatric Neurology, Erciyes University School of Medidine, Kayseri, Turkey.
Güldemet Kaya
GK
Özçora
Department of Pediatrics, Division of Pediatric Neurology, Erciyes University School of Medidine, Kayseri, Turkey.
Sefer
S
Kumandaş
Department of Pediatrics, Division of Pediatric Gastroenterology, Erciyes University School of Medicine, Kayseri, Turkey.
Neslihan
N
Karacabey
Department of Pediatrics, Division of Pediatric Neurology, Erciyes University School of Medidine, Kayseri, Turkey.
Mehmet
M
Canpolat
Department of Pediatrics, Division of Pediatric Neurology, Erciyes University School of Medidine, Kayseri, Turkey.
Hüseyin
H
Per
Children; Wilson’s disease; movement disorders; neurotoxicity