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Characteristics of neurological Wilson's disease without Kayser-Fleischer ring.
J Neurol Sci 2012; 323(1-2):183-6JN

Abstract

BACKGROUND

Although Kayser-Fleischer ring has been regarded as a key diagnostic feature of neurologic Wilson's disease, there have been previous reports of neurologic Wilson's disease patients without Kayser-Fleischer ring. We assessed the characteristics of neurologic Wilson's disease patients without Kayser-Fleischer ring.

METHODS

We enrolled neurologic Wilson's disease patients from 4 university hospitals by review of medical records in this study. Patients with neurologic Wilson's disease were diagnosed based on the neurologic symptoms and international scoring system for the diagnosis of Wilson's disease. All subjects were divided into two groups according to the presence of a Kayser-Fleischer ring. We compared demographic data, laboratory findings and imaging findings of the liver and brain between the two groups.

RESULTS

There were 12 (26.7%) patients without Kayser-Fleischer ring out of a total of 45 neurologic Wilson's disease patients. The Wilson's disease patients without Kayser-Fleischer ring demonstrated a higher ceruloplasmin concentration and serum copper level than those with Kayser-Fleischer ring. In addition, liver cirrhosis and typical signal changes in brain magnetic resonance imaging were less common in neurologic Wilson's disease patients without Kayser-Fleischer ring.

CONCLUSION

Based on our results, the absence of Kayser-Fleischer ring can be regarded as a form of neurologic Wilson's disease with less copper involvement. In addition, it is important to understand these features and to perform further investigations if patients without Kayser-Fleischer ring are suspected of having neurologic Wilson's disease.

Authors+Show Affiliations

Departments of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Multicenter Study

Language

eng

PubMed ID

23043908

Citation

Youn, Jinyoung, et al. "Characteristics of Neurological Wilson's Disease Without Kayser-Fleischer Ring." Journal of the Neurological Sciences, vol. 323, no. 1-2, 2012, pp. 183-6.
Youn J, Kim JS, Kim HT, et al. Characteristics of neurological Wilson's disease without Kayser-Fleischer ring. J Neurol Sci. 2012;323(1-2):183-6.
Youn, J., Kim, J. S., Kim, H. T., Lee, J. Y., Lee, P. H., Ki, C. S., & Cho, J. W. (2012). Characteristics of neurological Wilson's disease without Kayser-Fleischer ring. Journal of the Neurological Sciences, 323(1-2), pp. 183-6. doi:10.1016/j.jns.2012.09.013.
Youn J, et al. Characteristics of Neurological Wilson's Disease Without Kayser-Fleischer Ring. J Neurol Sci. 2012 Dec 15;323(1-2):183-6. PubMed PMID: 23043908.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Characteristics of neurological Wilson's disease without Kayser-Fleischer ring. AU - Youn,Jinyoung, AU - Kim,Ji Sun, AU - Kim,Hee-Tae, AU - Lee,Jee-Young, AU - Lee,Phil Hyu, AU - Ki,Chang-Seok, AU - Cho,Jin Whan, Y1 - 2012/10/05/ PY - 2012/06/25/received PY - 2012/09/03/revised PY - 2012/09/10/accepted PY - 2012/10/10/entrez PY - 2012/10/10/pubmed PY - 2013/4/20/medline SP - 183 EP - 6 JF - Journal of the neurological sciences JO - J. Neurol. Sci. VL - 323 IS - 1-2 N2 - BACKGROUND: Although Kayser-Fleischer ring has been regarded as a key diagnostic feature of neurologic Wilson's disease, there have been previous reports of neurologic Wilson's disease patients without Kayser-Fleischer ring. We assessed the characteristics of neurologic Wilson's disease patients without Kayser-Fleischer ring. METHODS: We enrolled neurologic Wilson's disease patients from 4 university hospitals by review of medical records in this study. Patients with neurologic Wilson's disease were diagnosed based on the neurologic symptoms and international scoring system for the diagnosis of Wilson's disease. All subjects were divided into two groups according to the presence of a Kayser-Fleischer ring. We compared demographic data, laboratory findings and imaging findings of the liver and brain between the two groups. RESULTS: There were 12 (26.7%) patients without Kayser-Fleischer ring out of a total of 45 neurologic Wilson's disease patients. The Wilson's disease patients without Kayser-Fleischer ring demonstrated a higher ceruloplasmin concentration and serum copper level than those with Kayser-Fleischer ring. In addition, liver cirrhosis and typical signal changes in brain magnetic resonance imaging were less common in neurologic Wilson's disease patients without Kayser-Fleischer ring. CONCLUSION: Based on our results, the absence of Kayser-Fleischer ring can be regarded as a form of neurologic Wilson's disease with less copper involvement. In addition, it is important to understand these features and to perform further investigations if patients without Kayser-Fleischer ring are suspected of having neurologic Wilson's disease. SN - 1878-5883 UR - https://www.unboundmedicine.com/medline/citation/23043908/Characteristics_of_neurological_Wilson's_disease_without_Kayser_Fleischer_ring_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0022-510X(12)00505-9 DB - PRIME DP - Unbound Medicine ER -