RESUMEN
Chordoma is a rare tumor derived from notochord remnants, but can occur at any level of the craniovertebral axis, pri-marily in the sacrum, clivus, and cervical regions. Therefore, chordoma should be considered as a rare cause in the dif-ferential diagnosis of mass lesions around the cervicomedullary junction. A 62-year-old man suffered from a suboccipi-tal, nuchal pain and headache for 15 years and nasal obstruction, recently. In a neurological examination, dysarthria, uvula and tongue deviation, depressed soft palate, decreased gag reflex, and atrophic SCM were noted. The MRI find-ings included an isosignal, round, well- marginated, and nearly homogeneous tumor compressing the lower brainstem. A biopsy was done by the transoral approach with a permanent diagnosis of chordoma arising from the clivus. We report a rare case of chordoma arising from the inferior margin of the clivus in a patient with multiple lower cranial nerve palsies.
Asunto(s)
Humanos , Persona de Mediana Edad , Vértebra Cervical Axis , Biopsia , Tronco Encefálico , Cordoma , Fosa Craneal Posterior , Enfermedades de los Nervios Craneales , Diagnóstico , Disartria , Cefalea , Imagen por Resonancia Magnética , Obstrucción Nasal , Examen Neurológico , Notocorda , Paladar Blando , Reflejo , Sacro , Lengua , ÚvulaRESUMEN
Chordoma is a rare tumor derived from notochord remnants, but can occur at any level of the craniovertebral axis, pri-marily in the sacrum, clivus, and cervical regions. Therefore, chordoma should be considered as a rare cause in the dif-ferential diagnosis of mass lesions around the cervicomedullary junction. A 62-year-old man suffered from a suboccipi-tal, nuchal pain and headache for 15 years and nasal obstruction, recently. In a neurological examination, dysarthria, uvula and tongue deviation, depressed soft palate, decreased gag reflex, and atrophic SCM were noted. The MRI find-ings included an isosignal, round, well- marginated, and nearly homogeneous tumor compressing the lower brainstem. A biopsy was done by the transoral approach with a permanent diagnosis of chordoma arising from the clivus. We report a rare case of chordoma arising from the inferior margin of the clivus in a patient with multiple lower cranial nerve palsies.
Asunto(s)
Humanos , Persona de Mediana Edad , Vértebra Cervical Axis , Biopsia , Tronco Encefálico , Cordoma , Fosa Craneal Posterior , Enfermedades de los Nervios Craneales , Diagnóstico , Disartria , Cefalea , Imagen por Resonancia Magnética , Obstrucción Nasal , Examen Neurológico , Notocorda , Paladar Blando , Reflejo , Sacro , Lengua , ÚvulaRESUMEN
Melkersson-Rosenthal syndrome (MRS) is a rare form of hereditary angioedema characterized by a triad of orofacial swelling, relapsing facial paralysis, and a fissured tongue. However, the classic triad is not frequently seen in its complete form, and monosymptomatic or oligosymptomatic forms are more common. Case: A 20-year-old man presented with recurrent labial swelling 9 months ago and recently with a fissured tongue. The surface of the tongue showed deep furrows characteristic of lingua plicata, and an edematous enlargement was observed on the lower lips. Upon neurologic examination, a left sided facial palsy of a peripheral type was noted without abnormalities in the taste sense or lacrimation. An electromyography of the left frontal muscle showed positive sharp waves and fibrillation potentials. Biopsies performed on the lower lips revealed the congested vessels and perivascular inflammatory cells. We report a 20-year-old man with a classical triad of symptoms of Melkersson-Rosenthal syndrome.