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Related Experiment Videos

Extrapyramidal symptoms in Wilson's disease are associated with olfactory dysfunction.

Antje Mueller1, Ulrike Reuner, Basile Landis

  • 1Smell & Taste Clinic, Department of Otorhinolaryngology, University of Dresden Medical School, Dresden, Germany. antje.mueller@uniklinikum-dresden.de

Movement Disorders : Official Journal of the Movement Disorder Society
|June 10, 2006
PubMed
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Patients with neurological Wilson's disease exhibit significant olfactory dysfunction, worsening with symptom severity. This deficit is linked to basal ganglia pathology, not treatment or imaging findings.

Area of Science:

  • Neurology
  • Metabolic Disorders
  • Neuroscience

Background:

  • Wilson's disease is a rare genetic disorder causing copper accumulation, primarily affecting the liver and brain.
  • Neurological manifestations include extrapyramidal symptoms like Parkinsonism, but olfactory function in these patients is understudied.
  • Investigating olfactory deficits can offer insights into neurodegeneration in metabolic disorders.

Purpose of the Study:

  • To investigate olfactory dysfunction in patients with Wilson's disease.
  • To correlate olfactory deficits with neurological symptoms, disease presentation (hepatic vs. neurological), and treatment.
  • To explore the relationship between olfactory function, brain imaging (MRI, PET), and copper deposition in the basal ganglia.

Main Methods:

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  • Twenty-four pharmacologically treated Wilson's disease patients were assessed.
  • Patients were categorized into hepatic-type (n=11) and neurological-type (n=13).
  • Olfactory function was tested using the Sniffin' Sticks test; MRI and [18F]FDG-PET were performed on 21 patients.

    • Main Results:

    • Neurological Wilson's disease patients demonstrated significant olfactory dysfunction compared to hepatic-type patients.
    • A more severe neurological presentation correlated with a more pronounced olfactory deficit.
    • Olfactory function was not significantly affected by penicillamine treatment, MRI lesions, or [18F]FDG-PET metabolism.

    Conclusions:

    • Olfactory dysfunction is a notable feature in neurologically affected Wilson's disease patients.
    • Basal ganglia degeneration and neuronal loss due to copper accumulation are implicated in the olfactory deficit.
    • Olfactory testing may serve as a sensitive indicator of neurological involvement in Wilson's disease.