Characteristics of neurological Wilson's disease without Kayser-Fleischer ring

Jinyoung Youn, Ji Sun Kim, Hee Tae Kim, Jee Young Lee, Phil Hyu Lee, Chang Seok Ki, Jin Whan Cho

Research output: Contribution to journalArticle

9 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)183-186
Number of pages4
JournalJournal of the Neurological Sciences
Volume323
Issue number1-2
DOIs
Publication statusPublished - 2012 Dec 15

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Hepatolenticular Degeneration
Nervous System Diseases
Copper
Ceruloplasmin
Brain
Neurologic Manifestations
Liver Cirrhosis
Medical Records
Magnetic Resonance Imaging
Demography

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology

Cite this

Youn, Jinyoung ; Kim, Ji Sun ; Kim, Hee Tae ; Lee, Jee Young ; Lee, Phil Hyu ; Ki, Chang Seok ; Cho, Jin Whan. / Characteristics of neurological Wilson's disease without Kayser-Fleischer ring. In: Journal of the Neurological Sciences. 2012 ; Vol. 323, No. 1-2. pp. 183-186.
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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.",
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Characteristics of neurological Wilson's disease without Kayser-Fleischer ring. / Youn, Jinyoung; Kim, Ji Sun; Kim, Hee Tae; Lee, Jee Young; Lee, Phil Hyu; Ki, Chang Seok; Cho, Jin Whan.

In: Journal of the Neurological Sciences, Vol. 323, No. 1-2, 15.12.2012, p. 183-186.

Research output: Contribution to journalArticle

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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

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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.

AB - 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.

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