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Gazzetta Medica Italiana Archivio per le Scienze Mediche 2014 January-February;173(1-2):63-6

Copyright © 2014 EDIZIONI MINERVA MEDICA

language: English

Papillary glioneuronal tumor as a cause of superficial siderosis. A case report

Puzzolante A., Prontera A., Dimitriadis S., Pavesi G.

Neurosurgery, Department of Neurosciences Nuovo Ospedale Civile Sant’Agostino Estense Modena, Italy


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Superficial siderosis (SS) of the Central Nervous System (CNS) is a rare progressive neurological disease due to chronic hemorrhage into the subarachnoid space leading to hemosiderin deposition and inducing neurotoxicity. The main clinical features are bilateral sensorineural hearing loss, cerebellar ataxia pyramidal signs. MR imaging is the investigation of choice for the diagnosis of SS detecting the distinctive hypointense signal around pial surfaces of cerebellum, brainstem, spinal cord and cortical sulci seen in T2-weighted scan, related with hemosiderin deposition. We present the case of a young patient with SS secondary to chronic subarachnoid hemorrage from a papillary glioneuronal tumor (PGNT), a WHO-classified low grade mixed glial-neuronal tumor. In our case, despite a well tolerated total tumor resection and despite a post-operative iron-alkilant therapy, pathological effects of SS are still present and even progressing at 16 months follow-up. PGNT with its chronic hemorrhagic pattern may cause SS, whose long lasting effects on CNS seem to be unaffected by tumor removal.

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