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Rare disease
Acquired retrograde transsynaptic degeneration
  1. Marta Esteves Guedes,
  2. Ana Catarina Almeida,
  3. Maria Sara Patricio,
  4. João Marques Costa
  1. Ophthalmology Department, Hospital Egas Moniz, Lisbon, Portugal
  1. Correspondence to Dr Marta Esteves Guedes, marta.ce.guedes{at}gmail.com

Summary

Although retrograde transsynaptic degeneration in the visual pathway of monkeys has been described since 1963, data in humans are sparse. The authors present a 24-year-old female referred to a neuro-ophthalmology consult for assessment before neurosurgery for a right occipital ependymoma. Clinical examination was unremarkable, including visual fields evaluated by computerised static perimetry. Four years after tumour extraction, the patient showed a left homonymous haemianopia documented by computerised static perimetry and a bow-tie like atrophy on the left on funduscopy. MRI revealed right occipital cortex lobectomy and optic tract atrophy. The presence of left homonymous haemianopia, the characteristic pattern of optic disc atrophy and right optic tract atrophy 4 years after right occipital tumour excision, strongly suggest the presence of retrograde transsynaptic degeneration. To our knowledge, this is the first time that retrograde transsynaptic degeneration-associated optic tract atrophy is clearly demonstrated by MRI.

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.

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