Dejérine-Roussy syndrome associated with unilateral thalamic glioma

Abstract

Context: Déjérine-Roussy Syndrome is a rare entity that occurs after an ischemia located in the ventral posterolateral nucleus, and it is characterized by hemiplegia, superficial hemianesthesia, mild hemiataxia and astereognosis, pain on the paretic side and choreoathetosis movements. This unusual condition can be caused by haemorrhage or neoplasm. Thalamic tumors make up less than 5% of all intracranial tumors. The rare clinical presentation of a thalamic tumor is a diagnostic and therapeutic challenge for neurology and neurosurgery practice and generally requires treatment without biopsy. Case report: A 54-years-old man presented complaining of burning and tingling paraesthesias, decreased sensitivity in left dimidium, associated with decreased visual acuity in the left eye. Physical examination showed complete left hemiparesis provided grade 4-, normoreflexia with athetoid movements of the left arm and hand, painful, thermal hemihipoesthesia and epicritic touch, allodyne in the left hemibody, pressure sensitivity present globally, visual campimetry by confrontation with heteronymous hemianopia without changes in the cranial nerves. Magnetic Resonance Imaging of the Skull Base showed an oval mass, with hyposignal in T1 and hypersignal in T2 and FLAIR, with peripheral contrast uptake in the thalamus and nuclei from the right base. The patient showed good clinical-surgical evolution after surgery with Glasgow Outcome Scale 4 and modified Rankin scale 2. Anatomical Pathology confirmed low-grade glioma. Conclusions: Early diagnosis and immediate therapy can delay a fatal outcome or decrease treatment-related morbidity.