Paraneoplastic ataxia and dystonia secondary to a malignant fibrous histiocytoma

Ross Dolan, Ralph Thomas, Jonathan O'Riordan

    Research output: Contribution to journalArticle

    Abstract

    Paraneoplastic movement disorders are part of the spectrum of paraneoplastic syndromes caused by the production of onconeural antibodies such as anti-Hu by underlying tumours. These attack specific neurons depending on receptor aetiology. We report the case of a 53-year-old man who presented 8 years previously with symptoms of upper limb weakness, light headedness, dizziness and falls. His condition followed a progressive course. Two years after onset he had right-sided weakness, diplopia and generalised dystonia. Initial investigations identified a positive anti-Hu antibody, but an extensive search for a primary tumour was negative. A malignant fibrous histiocytoma in his right gluteal fold was subsequently identified. At this stage he was bed bound with severe ataxia, dystonia and spasticity. Following surgical excision and treatment with high dose steroids and pulse immunoglobulin, further progression was arrested and minor improvements occurred. He can now ambulate with bilateral assistance but remains severely disabled.
    Original languageEnglish
    JournalBMJ Case Reports
    DOIs
    Publication statusPublished - 12 Nov 2010

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    Dystonic Disorders
    Malignant Fibrous Histiocytoma
    Dystonia
    Dizziness
    Ataxia
    Paraneoplastic Syndromes
    Diplopia
    Movement Disorders
    Upper Extremity
    Antibody Formation
    Immunoglobulins
    Anti-Idiotypic Antibodies
    Neoplasms
    Steroids
    Neurons
    Therapeutics

    Cite this

    Dolan, Ross ; Thomas, Ralph ; O'Riordan, Jonathan. / Paraneoplastic ataxia and dystonia secondary to a malignant fibrous histiocytoma. In: BMJ Case Reports. 2010.
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    Paraneoplastic ataxia and dystonia secondary to a malignant fibrous histiocytoma. / Dolan, Ross; Thomas, Ralph; O'Riordan, Jonathan.

    In: BMJ Case Reports, 12.11.2010.

    Research output: Contribution to journalArticle

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