Giant clival chordoma causing pathological laughter

Abstract

Background:Chordomas are rare slowly growing tumors that originate from remnants of the notochord. They have a malignant local behavior, causing symptoms due to bone infiltration and compression of neurovascular structures. Only a few cases of brain tumors associated with pathological laughter have been reported in the literature.Case Description:We report a case of a 42-year-old male patient with this atypical clinical presentation treated at our institution, and discuss the concerning literature.Conclusion:Although being a very rare presentation of chordomas, pathological laughter is usually expected to improve after brain stem decompression.

Proton beam therapy in the management of skull base chordomas: systematic review of indications, outcomes, and implications for neurosurgeons

“…[17] Skull base chordomas can present with epistaxis or intracranial haemorrhage [18,19] and, rarely, with endocrine impairment [8,20]. One case reports a giant clival chordoma that presented with pathological laughter [21] although this is extremely rare.…”