Literature DB >> 23966348

Acute onset anarthria without hepatic manifestation: a rare presentation of Wilson disease.

Rajesh Verma1, Aveg Bhandari, Navin Tiwari, Tejendra S Chaudhari.   

Abstract

Wilson disease (WD) is one of the few inherited but treatable disorder mainly affecting the liver and brain resulting in severe disability or death if left untreated. Hence, it is important to keep a high index of suspicion for diagnosing this clinical entity in appropriate clinical settings. The clinical presentation can be quite variable and they may present solely with neurological features sans hepatic symptoms. Such neurological manifestations usually follow subacute to chronic course. Acute onset anarthria as the heralding and predominant presenting feature has been rarely reported in the literature. We reported a case of a 12-year-old girl who presented with acute onset anarthria and dystonia of 1-month duration. On further evaluation, a diagnosis of WD was made. The patient showed partial improvement after she was started on copper chelating agents and anticholinergics.

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Mesh:

Year:  2013        PMID: 23966348      PMCID: PMC3761678          DOI: 10.1136/bcr-2013-010415

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  4 in total

Review 1.  Wilson's disease.

Authors:  Aftab Ala; Ann P Walker; Keyoumars Ashkan; James S Dooley; Michael L Schilsky
Journal:  Lancet       Date:  2007-02-03       Impact factor: 79.321

2.  Wilson disease in septuagenarian siblings: Raising the bar for diagnosis.

Authors:  Aftab Ala; Jimo Borjigin; Arnold Rochwarger; Michael Schilsky
Journal:  Hepatology       Date:  2005-03       Impact factor: 17.425

Review 3.  Wilson's disease: an update.

Authors:  Shyamal K Das; Kunal Ray
Journal:  Nat Clin Pract Neurol       Date:  2006-09

4.  Wilson's disease--early onset and lessons from a pediatric cohort in India.

Authors:  V Kalra; D Khurana; R Mittal
Journal:  Indian Pediatr       Date:  2000-06       Impact factor: 1.411

  4 in total
  1 in total

1.  Acute onset neurological symptoms in Wilson disease after traumatic, surgical or emotional events: A cross-sectional study.

Authors:  Liang-Yong Li; Xiao-Qun Zhu; Wei-Wei Tao; Wen-Ming Yang; Huai-Zhen Chen; Yu Wang
Journal:  Medicine (Baltimore)       Date:  2019-06       Impact factor: 1.817

  1 in total

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