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Acute Dysphagia as the Presenting Feature of Syringobulbia in a Sixteen-Year-Old Patient<br />

<strong>Case</strong> <strong>Report</strong><br />

Hong Gao<br />

King's College Hospital<br />

Anita Cheah<br />

Neli Avramova<br />

Martin Whyte<br />

Kholoud Al-Majali<br />

A sixteen-year-old female with no past medical history presented with a one-week history of painless<br />

dysphagia to solids and liquids and a two-week history of bowels not opening. Neurological examination<br />

on admission revealed left-beating nystagmus but no other cranial nerve or peripheral neurological deficits.<br />

CT Head showed a longitudinal Cerebrospinal Fluid-density lesion in the Medulla and Cervical Cord. Within<br />

eight-hours of her initial neurological examination, the patient developed bilateral Glossopharyngeal,<br />

Vagus, Accessory and Hypoglossal Nerve palsies, bilateral nystagmus, and glove-and-stocking<br />

paraesthesiae. MRI spine showed an extensive, complex hydro-syringomyelic cavity extending from the<br />

junction of the Fourth Ventricle and central Spinal Canal to the T10 level, with associated cord expansion.<br />

The patient underwent neurosurgical excision of the tumour and decompression of the syrinx within three<br />

days of admission. She subsequently made a full neurological recovery. Histology revealed a Grade 1<br />

haemangioblastoma. The patient is being investigated for other features of Von Hippel Lindau Syndrome.<br />

This case illustrates the importance of regular neurological examination to detect evolving signs which may<br />

indicate life-threatening rises in intra-cranial or intra-spinal pressure. It also demonstrates that an extensive<br />

spinal cord space-occupying lesion in a young patient may be part of an underlying condition such as Von<br />

Hippel Lindau Syndrome, and therefore prompt imaging of the Central Nervous System should be<br />

considered in any patient with a focal neurological deficit. Finally, it serves as a reminder that timely referral<br />

to other specialties can be life-saving for patients admitted on the Acute Medical Take.

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