J Neurol Neurosurg Psychiatry 80:581-583 doi:10.1136/jnnp.2008.150292
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Vacuolar myelopathy associated with optic neuropathy in an HIV-negative, immunosuppressed liver transplant recipient

  1. S R L Stacpoole1,
  2. R Phadke2,
  3. T S Jacques3,
  4. T Revesz2,
  5. G T Plant1
  1. 1
    Department of Neurology, National Hospital for Neurology and Neurosurgery, London, UK
  2. 2
    Division of Neuropathology, UCL Institute of Neurology, London, UK
  3. 3
    Neural Development Unit, The Institute of Child Health, London, UK
  1. Dr G Plant, Institute of Neurology, Internal Box Number 93, The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK; gordon{at}
  • Received 26 March 2008
  • Revised 28 June 2008
  • Accepted 16 September 2008

We report a case of progressive visual failure and relentless, fatal paralysis in a 52-year-old man, immunosuppressed with mycophenolate mofetil. Neuropathological examination of the brain and spinal cord revealed the cause to be vacuolar myelopathy (VM). This condition is common in HIV-infected patients but is rarely reported outside this context. An association of VM with a progressive optic neuropathy has not previously been described. This report discusses the current hypotheses regarding the pathogenesis of HIV-associated VM, and lessons that can be learnt from the unusual cases occurring in HIV-negative individuals.


A 52-year-old gentleman of South Asian origin presented with a 2-month history of progressive visual failure. He had been blind in his left eye for some years due to glaucoma. He was immunosuppressed with mycophenolate mofetil (1 g twice daily), following an orthotopic liver transplant 10 years earlier for primary biliary cirrhosis. Subsequent to the transplant, he developed type II diabetes mellitus, controlled with gliclazide. While undergoing investigations to discover the cause of the visual failure, he developed a spinal cord syndrome that progressed relentlessly, leading to his death from respiratory failure less than 2 months after initial presentation.

Extensive investigations, including viral and bacterial serology, vasculitic screen, tumour markers, antineuronal antibodies, angiotensin-converting enzyme and multiple cerebrospinal fluid (CSF) analyses, revealed few positive findings. He had active shingles, reflecting his immunocompromised state, and a persistent thrombocytopenia. MRI of his brain and spinal cord showed atrophy of the optic chiasm and tracts, signal change in the …

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