Giant clival chordoma causing pathological laughter
A 42 year-old male patient was admitted with a history of headache, which started approximately one year ago. He had been followed in another institution as a pituitary adenoma and had not had surgery before coming to our consultation. In the past 3 months he complained of worsening of the headache, diplopia, and diminished bilateral visual acuity. He had at this time many episodes of unmotivated laugh without consciousness disruption. They were self-limited lasting for a few seconds only. At examination, an intense ataxia, global hyperreflexia, bitemporal hemianopia, and mild right VI nerve paresis were remarkable. Panhypopituitarism was evident in laboratory exams. Eletroencephalogram did not reveal seizure activity. Neuroimaging revealed a large clival mass invading the sphenoid sinus with destruction of the sella with a huge suprasellar and retrosellar extension, brain stem displacement and skull base bone destruction [Figures and ]. We performed a binostril, transnasal transphenoidal endoscopic approach with bilateral medial turbinectomy, wide opening of the anterior wall of the sphenoid sinus, and a radical resection of the lesion with partial resection of the infiltrated clival dura. Satisfactory brain stem decompression was accomplished [Figures and ]. Reconstruction of the skull base was achieved with abdominal fat, nasoseptal pediculated graft (Haddad) and fibrin glue. Postoperative period was uneventful with no cerebrospinal fluid leakage episodes and a great improvement in vision, with total recovering of the VI nerve deficit and ataxia. He has not had any paroxysm of pathological laughter since surgery. Histological examination confirmed the diagnosis of chordoma. Patient was submitted in the postoperative period to radiotherapy (IMRT) and has been asymptomatic in the past 14 months. Follow up magnetic resonance imaging (MRI) is shown in [Figures and ].