Abstract
Background
Wilson's disease (WD) is an inherited disorder of copper metabolism. Although well
documented in many disorders, gender hasn't been directly addressed in WD; therefore,
our aim was to evaluate gender differences in WD.
Methods
We analyzed data on 627 consecutive WD patients entered into our registry (1958–2010).
Results
We observed a male predominance in our population of WD patients (327 males vs. 290
females; p<0.05). At disease diagnosis, 510/627 patients were symptomatic, most patients had
the neuropsychiatric WD form (345/510; p<0.01). The neuropsychiatric form occurred predominantly in men versus women (209/278
vs. 136/232; p<0.01), especially the rigidity-tremor (71/111 vs. 40/111; p<0.05), rigidity (23/33 vs. 10/33; p=0.07) and psychiatric forms (46/71 vs. 25/71; p=0.06). The hepatic form occurred more frequently in women (96/165 vs. 69/165; p<0.01) and women developed the neuropsychiatric form 2 years later than men (29.4 vs. 27.1; p<0.05).
Conclusions
According to our findings, the neuropsychiatric form of WD is predominant at diagnosis
in both genders. The hepatic form of WD occurs more frequently in women, and women
develop the neuropsychiatric form of disease almost 2 years later than men. We speculate these differences may be due to the protective
effect of estrogens and iron metabolism differences.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of the Neurological SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Wilson's disease.Semin Neurol. 2007; 27: 123-132
- Frameshift and nonsense mutations in the gene for ATPase7B are associated with severe impairment of copper metabolism and with an early clinical manifestation of Wilson's disease.Clin Genet. 2005; 68: 524-532
- Diagnosis and phenotypic classification of Wilson's disease.Liver Int. 2003; 23: 139-142
- Detection of the His1069Gln mutation in Wilson disease by rapid polymerase chain reaction.Ann Intern Med. 1997; 127: 21-26
- The impact of apolipoprotein E genotypes on age at onset of symptoms and phenotypic expression in Wilson's disease.Brain. 2000; 123: 585-590
- Clinical presentation, diagnosis and long-term outcome of Wilson's disease: a cohort study.Gut. 2007; 56: 115-120
- Copper metabolism in normal adults and in clinically normal relatives of patients with Wilson's disease.J Clin Pathol. 1958; 11: 441-447
- Gender differences in neurological disease: role of estrogens and cytokines.Endocrine. 2006; 29: 243-256
- Hormones, genetic factors, and gender differences in cardiovascular disease.Cardiovasc Res. 2002; 53: 550-557
- Incidence of Parkinson's disease: variation by age, gender, and race/ethnicity.Am J Epidemiol. 2003; 157: 1015-1022
- Gender differences in Parkinson's disease.Gend Med. 2007; 4: 8-18
- Gender specific role of mitochondria in the vulnerability of 6-hydroxydopamine-treated mesencephalic neurons.Biochim Biophys Acta. 2010; 1797: 1178-1188
- Gender and liver fibrosis in chronic hepatitis: the role of iron status.Aliment Pharmacol Ther. 2005; 21: 1445-1451
- Influence of gender, race and ethnicity on suspected fatty liver obese adolescents.Pediatrics. 2005; 115: 561-565
- Brain ferritin iron may influence age- and gender-related risks of neurodegeneration.Neurobiol Aging. 2007; 28: 414-423
- Age, gender, and hemispheric differences in iron deposition in the human brain: an in vivo MRI study.Neuroimage. 2008; 40: 35-42
- Wilson's disease.Q J Med. 1987; 65: 959-966
- Wilson's disease.Handb Clin Neurol. 1986; 49: 223-228
- Association between male gender and cortical Lewy body pathology in large autopsy series.J Neurol. 2010; 257: 1875-1881
- Gender differences in Parkinson's disease.J Neurol Neurosurg Psychiatry. 2007; 78: 819-824
- Gender differences in Parkinson's disease: clinical characteristic and cognition.Mov Disord. 2010; 25: 2695-2703
- Parkinson's disease in women: a call for improved clinical studies and for comparative effectiveness research.Maturitas. 2010; 65: 352-358
- Increased risk of parkinsonism in women who underwent oophorectomy before menopause.Neurology. 2008; 70: 200-209
- Risk of Parkinson's disease after tamoxifen treatment.BMC Neurol. 2010; 10: 23
- Occupational factors and risk of Parkinson's disease: a population-based case–control study.Am J Ind Med. 2010; 53: 217-223
- Occupational and environmental risk for Parkinson's disease.Parkinsonism Relat Disord. 2002; 8: 297-309
Article info
Publication history
Published online: September 14, 2011
Accepted:
August 22,
2011
Received in revised form:
August 18,
2011
Received:
April 17,
2011
Identification
Copyright
© 2011 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
