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NEUROLOGY 2009;73:e73
© 2009 American Academy of Neurology
Resident and Fellow Section

Teaching Video NeuroImages: Excessive grinning in Wilson disease

Formula

R. S. Cetlin, MD, G. R. Rodrigues, MD, M. A. Pena-Pereira, MD, D. S. Oliveira, MD, C. P. Souza, MD and V. Tumas, MD, PhD

From the Department of Neuroscience (R.S.C., M.A.P.-P., D.S.O., V.T.) and Movement Disorders Clinic (G.R.R., C.P.S.), School of Medicine of Ribeirao Preto, University of Sao Paulo, Brazil.

Address correspondence and reprint requests to Dr. Vitor Tumas, Department of Neurosciences and Behavior Sciences, Ribeirão Preto School of Medicine–University of São Paulo, Campus Universitário Monte Alegre, Ribeirão Preto–SP, Brazil 14048-900 tumasv{at}rnp.fmrp.usp.br

A 19-year-old man presented with a 3-month history of excessive grinning. Examination revealed unrestrained grinning and mild symmetric parkinsonism. Wilson disease was suspected and confirmed by the presence of Kayser-Fleischer ring (figure), suggestive brain MRI (figure), low ceruloplasmin, and high urinary copper levels.


Figure 118
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  		Figure (A, B) Excessive grinning, (C) Kayser-Fleischer ring, (D) "face of giant panda" sign and basal ganglia T2 hyperintensity

 

Wilson disease is a disorder of copper metabolism characterized by hepatic impairment and movement disorders. Typical facial manifestations, although not pathognomonic, include excessive grinning, in which the patient grins to trivial stimuli,1 as demonstrated in this report; sustained open-mouth smile, when a parkinsonian face is associated with a dystonic dropped jaw (sometimes referred to as "vacuous smile")2; and fixed forced smile, when facial dystonia produces a sustained spasm of risorius and zygomaticus muscles (also referred to as "risus sardonicus").

Supplemental data at www.neurology.org

Disclosure: The authors report no disclosures.


   REFERENCES
Top.
 REFERENCES
 

  1. Wilson SAK. Progressive lenticular degeneration (hepatolenticular degeneration, Wilson’s disease). In: Ninian-Bruce A, ed. Neurology. Baltimore: Williams & Wilkins;
  2. Das SK, Ray K. Wilson’s disease: an update. Nat Clin Pract Neurol 2006;2:482–493.[Medline]




This Article
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Right arrow Articles by Cetlin, R. S.
Right arrow Articles by Tumas, V.
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Right arrow Articles by Cetlin, R. S.
Right arrow Articles by Tumas, V.
Related Collections
Right arrow MRI
Right arrow Dystonia
Right arrow Parkinson's disease/Parkinsonism

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