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Anti Ma2-associated myeloradiculopathy: expanding the phenotype of anti-Ma2 associated paraneoplastic syndromes
  1. Sinéad M Murphy1,
  2. Usman Khan2,
  3. Constantine Alifrangis3,
  4. Steven Hazell4,
  5. David Hrouda5,
  6. Julian Blake6,7,
  7. Joanna Ball8,
  8. Carolyn Gabriel8,
  9. Pierre Markarian9,
  10. Jeremy Rees2,
  11. Abid Karim1,
  12. Michael J Seckl3,
  13. Michael P Lunn1,
  14. Mary M Reilly10
  1. 1MRC Centre for Neuromuscular Diseases, The National Hospital for Neurology and Neurosurgery and Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK
  2. 2The National Hospital for Neurology and Neurosurgery and Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK
  3. 3Department of Medical Oncology, Charing Cross Hospital, Imperial College NHS Trust, London, UK
  4. 4Department of Histopathology, Charing Cross Hospital, Imperial College NHS Trust, London, UK
  5. 5Department of Urology, Charing Cross Hospital, Imperial College NHS Trust, London, UK
  6. 6Department of Clinical Neurophysiology, The National Hospital for Neurology and Neurosurgery and Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK
  7. 7Department of Clinical Neurophysiology, Norfolk and Norwich University Hospital, Norwich, UK
  8. 8St Mary's Hospital, Imperial College NHS Trust, London, UK
  9. 9Nexience, Valence, France
  10. 10Department of Clinical Immunology, University of Birmingham, Birmingham, UK
  1. Correspondence to S M Murphy, MRC Centre for Neuromuscular Diseases, The National Hospital for Neurology and Neurosurgery and Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK; sinead.murphy{at}uclh.nhs.uk

http://dx.doi.org/10.1136/jnnp.2010.223271

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    Anti-Ma2 associated paraneoplastic syndrome usually presents as limbic encephalitis in association with testicular tumours.1 2 Only four patients have been reported with involvement outside the CNS, two of whom also had limbic or brainstem encephalitis.2 3 We report a man with anti-Ma2 associated myeloradiculopathy and previous testicular cancer whose neurological syndrome stabilised and anti-Ma2 titres fell following orchidectomy of a microscopically normal testis.

    Case report

    A 46-year-old dentist noticed weakness of pincer movement in the left hand. Six weeks later he developed sequential finger drop of the 4th, 5th and 3rd fingers of the left hand over days. During the subsequent weeks the fingers of his right hand also dropped. He had a prior history of left orchidectomy for stage I testicular seminoma and had been well on surveillance for 5 years. MRI brain, cervical spine and brachial plexii were normal. A diagnosis of multifocal motor neuropathy with conduction block was considered. Neurophysiology of the upper limbs demonstrated reduced motor amplitudes and acute and chronic denervation but no conduction block. Neurophysiology of the lower limbs was normal. He was given a trial of intravenous immunoglobulin without response. Some weeks later he developed an unusual itchy sensation spreading across his back and shoulders.

    On examination there was head drop with weakness of neck flexion. There was wasting of both forearms and intrinsic hand muscles, with some fasciculations in the biceps and triceps. Tone was normal. There was asymmetric patchy proximal and distal weakness of both arms with finger drop. Reflexes were brisk …

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    Footnotes

    • Funding MMR is grateful to the Medical Research Council (MRC) and the Muscular Dystrophy Campaign for their support. This work was undertaken at University College London Hospitals/University College London and Imperial College London NHS Healthcare Trust/Imperial College London, which both received a proportion of funding from the Department of Health's National Institute for Health Research Biomedical Research Centres funding scheme. MJS is grateful for funding from the Department of Health and Cancer Research UK Imperial College Experimental Cancer Medicine Centre grant.

    • Competing interests None.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.