Abstract
A sixteen-year-old female with no past medical history presented with a one-week history of painless dysphagia to solids and liquids and a two-week history of bowels not opening. Neurological examination on admission revealed left-beating nystagmus but no other cranial nerve or peripheral neurological deficits. CT Head showed a longitudinal Cerebrospinal Fluid-density lesion in the Medulla and Cervical Cord. Within eight-hours of her initial neurological examination, the patient developed bilateral Glossopharyngeal, Vagus, Accessory and Hypoglossal Nerve palsies, bilateral nystagmus, and glove-and-stocking paraesthesiae. MRI spine showed an extensive, complex hydro-syringomyelic cavity extending from the junction of the Fourth Ventricle and central Spinal Canal to the T10 level, with associated cord expansion. The patient underwent neurosurgical excision of the tumour and decompression of the syrinx within three days of admission. She subsequently made a full neurological recovery. Histology revealed a Grade 1 haemangioblastoma. The patient is being investigated for other features of Von Hippel Lindau Syndrome. This case illustrates the importance of regular neurological examination to detect evolving signs which may indicate life-threatening rises in intra-cranial or intra-spinal pressure. It also demonstrates that an extensive spinal cord space-occupying lesion in a young patient may be part of an underlying condition such as Von Hippel Lindau Syndrome, and therefore prompt imaging of the Central Nervous System should be considered in any patient with a focal neurological deficit. Finally, it serves as a reminder that timely referral to other specialties can be life-saving for patients admitted on the Acute Medical Take.