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Isolated frontal disequilibrium as presenting form of anti-Hu paraneoplastic encephalomyelitis

Authors

  • Yaroslau Compta MD,

    1. Department of Neurology, Institute of Neurosciences, Hospital Clínic i Universitari de Barcelona, Barcelona, Catalonia, Spain
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  • Francesc Valldeoriola MD, PhD,

    Corresponding author
    1. Department of Neurology, Institute of Neurosciences, Hospital Clínic i Universitari de Barcelona, Barcelona, Catalonia, Spain
    • Department of Neurology, Institut de Neurociències, Hospital Clínic i Universitari de Barcelona, C/ Villarroel, 170, 08036-Barcelona, Catalonia, Spain
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  • Xabier Urra MD,

    1. Department of Neurology, Institute of Neurosciences, Hospital Clínic i Universitari de Barcelona, Barcelona, Catalonia, Spain
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  • Beatriz Gómez-Ansón MD, PhD,

    1. Department of Radiology, Diagnostic Imaging Center, Hospital Clínic i Universitari de Barcelona, Barcelona, Catalonia, Spain
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  • Lorena Rami PhD,

    1. Department of Neurology, Institute of Neurosciences, Hospital Clínic i Universitari de Barcelona, Barcelona, Catalonia, Spain
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  • Eduardo Tolosa MD, FRCP,

    1. Department of Neurology, Institute of Neurosciences, Hospital Clínic i Universitari de Barcelona, Barcelona, Catalonia, Spain
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  • Francesc Graus MD, PhD

    1. Department of Neurology, Institute of Neurosciences, Hospital Clínic i Universitari de Barcelona, Barcelona, Catalonia, Spain
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Abstract

Anti-Hu encephalomyelitis is one of the most frequent paraneoplastic syndromes, classically presenting with diffuse neurological involvement. We report a 69-year-old man presenting with a three-month isolated, progressive gait disorder with normal neurological examination, except for loss of balance and gait failure reminding frontal disequilibrium, only accompanied by a very mild rigidity of his right foot. MRI of the brain showed hyperintensities in both amygdale and left putamen. EMG study showed no abnormal continuous spontaneous fiber activity. Because of fast progression and MRI findings, anti-Hu antibodies were tested, resulting positive. Mediastinal biopsy of two adenopathies detected by body-PET, confirmed an oat-cell carcinoma. The patient received oral steroids and oncological therapy. One year later, the tumor is in remission. His gait and abnormal posture of right leg are normal. Only mild residual hyperintensities persist on follow-up MRI. A paraneoplastic syndrome should be considered in the differential diagnosis of subacute, fast progressive gait disorders. © 2007 Movement Disorder Society

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