Literature DB >> 7788483

Case report: concordant traumatic brainstem contusion delayed diagnosis in a young man with Wilson's disease.

A Marcus1, C Ammermann, M Klein, M H Schmidt.   

Abstract

Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism. The corresponding gene locus has been localized on the long arm of chromosome 13. Three different clinical variants of the disease can be distinguished: hepato-cerebral, abdominal/hepatic, and central nervous type. The heterogeneity of symptoms can cause problems in differential diagnosis, especially when another concordant disorder can also explain the pathogenesis of symptoms. The case report of a young man who suffered from brainstem contusion demonstrates the possibilities of misinterpretation because presenting symptoms could be attributed either to traumatic brain injury followed by adjustment disorder or Wilson's disease. Clinical signs included leftsided hemiparesis, bilateral gaze direction nystagmus, marked dysarthria with consecutive pervasive mutism, choreo-athetoid movements, spasmodic torticollis and diplopia dependent on gaze direction. Slit lamp examination showed Kayser-Fleischer's corneal ring. EEG- and computer assisted tomography investigations revealed non-specific findings. The patient was treated with D-Penicillamine. Alternative treatment with oral zinc preparations is discussed.

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Year:  1995        PMID: 7788483     DOI: 10.1007/bf01987966

Source DB:  PubMed          Journal:  Eur Child Adolesc Psychiatry        ISSN: 1018-8827            Impact factor:   4.785


  29 in total

1.  Atypical MR presentation of Wilson disease: a possible consequence of paramagnetic effect of copper?

Authors:  P Brugieres; C Combes; F Ricolfi; J D Degos; J Poirier; A Gaston
Journal:  Neuroradiology       Date:  1992       Impact factor: 2.804

2.  EEG changes in Wilson's disease.

Authors:  P Hansotia; R Harris; J Kennedy
Journal:  Electroencephalogr Clin Neurophysiol       Date:  1969-11

3.  Psychologic investigation of Wilson's disease.

Authors:  L J Davis; N P Goldstein
Journal:  Mayo Clin Proc       Date:  1974-06       Impact factor: 7.616

Review 4.  Wilson disease.

Authors:  G J Brewer; V Yuzbasiyan-Gurkan
Journal:  Medicine (Baltimore)       Date:  1992-05       Impact factor: 1.889

5.  Wilson disease: clinical presentation, treatment, and survival.

Authors:  W Stremmel; K W Meyerrose; C Niederau; H Hefter; G Kreuzpaintner; G Strohmeyer
Journal:  Ann Intern Med       Date:  1991-11-01       Impact factor: 25.391

6.  Presenting symptoms and natural history of Wilson disease.

Authors:  T Saito
Journal:  Eur J Pediatr       Date:  1987-05       Impact factor: 3.183

7.  Treatment of Wilson's disease with zinc: XI. Interaction with other anticopper agents.

Authors:  G J Brewer; V Yuzbasiyan-Gurkan; V Johnson; R D Dick; Y Wang
Journal:  J Am Coll Nutr       Date:  1993-02       Impact factor: 3.169

8.  Improvement of cerebral CT abnormalities following liver transplantation in a patient with Wilson disease.

Authors:  W E Rothfus; W L Hirsch; J J Malatack; I Bergman
Journal:  J Comput Assist Tomogr       Date:  1988 Jan-Feb       Impact factor: 1.826

9.  Correlation of evoked potential and MRI findings in Wilson's disease.

Authors:  L M Selwa; C W Vanderzant; J A Brunberg; G J Brewer; I Drury; A Beydoun
Journal:  Neurology       Date:  1993-10       Impact factor: 9.910

10.  Evidence for linkage between Wilson disease and esterase D in three kindreds: detection of linkage for an autosomal recessive disorder by the family study method.

Authors:  B Bonné-Tamir; L A Farrer; M Frydman; H Kanaaneh
Journal:  Genet Epidemiol       Date:  1986       Impact factor: 2.135

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