Keddie Stephen, Jaunmuktane Zane, Brandner Sebastian, Shah Sachit, Maddison Paul, Rees Jeremy H, Hanna Michael G, Lunn Michael P T, Reilly Mary M, Rossor Alexander M, Carr Aisling S
Department of Molecular Neuroscience, MRC Centre for Neuromuscular Diseases, National Hospital of Neurology and Neurosurgery, UCL Institute of Neurology, London, UK.
Division of Neuropathology, National Hospital of Neurology and Neurosurgery, UCL Hospitals NHS Foundation Trust, London, UK.
Pract Neurol. 2018 Apr;18(2):137-142. doi: 10.1136/practneurol-2017-001801. Epub 2018 Jan 23.
We present a 26 year old male with a 6 year history of painful sensory symptoms, weakness and wasting in the legs alongside progressive facial weakness, slurred speech, dysphagia and ophthalmoplegia. There were no neurological symptoms or signs in the upper limbs. Previous medical history included traumatic corneal injury to the left eye in childhood and a cisterna magna choroid plexus papilloma which was removed surgically at age 22. Investigations localised the pathology to the cranial nerve and pre-ganglionic lumbar nerve roots. A dural meningeal biopsy demonstrated grade 1 choroid plexus papilloma, 4 years after presumed curative excision of the original lesion. This presentation with multifocal peripheral nervous system deficits is due to choroid plexus papilloma drop metastases. We summarise the case and discuss the significance of the neurophysiology and imaging findings contributing to diagnosis.