Reference : Primary diffuse leptomeningeal gliomatosis: an autopsy case and review of the literature
Scientific journals : Article
Human health sciences : Neurology
http://hdl.handle.net/2268/74363
Primary diffuse leptomeningeal gliomatosis: an autopsy case and review of the literature
English
Somja, Joan mailto [Université de Liège - ULg > Département des sciences biomédicales et précliniques > Anatomie et cytologie pathologiques >]
Boly, Mélanie mailto [Université de Liège - ULg > Département des sciences cliniques > Neurologie >]
Sadzot, Bernard mailto [Université de Liège - ULg > > Neurologie Sart Tilman >]
Moonen, Gustave mailto [Université de Liège - ULg > > Neurologie Sart Tilman >]
Deprez, Manuel mailto [Université de Liège - ULg > Département des sciences cliniques > Neuropathologie >]
2010
Acta Neurologica Belgica
Acta Medica Belgica
110
4
325-33
Yes (verified by ORBi)
International
0300-9009
Bruxelles
Belgique
[en] Aged ; Autopsy ; Brain/pathology ; Disease Progression ; Meningeal Neoplasms/cerebrospinal fluid/pathology/radiography ; Neoplasms, Neuroepithelial/cerebrospinal fluid/pathology/radiography ; Tomography, X-Ray Computed/methods
[en] We report a case of primary diffuse leptomeningeal gliomatosis (PDLG) in a 76-year-old male presenting with confusion, dysarthria, diplopia, lumbal pain and headaches of recent onset. Neurological examination revealed nuchal rigidity and bilateral sixth cranial nerve palsy. The cerebrospinal fluid showed a marked hyperproteinorachia (4711 mg/L) and mild cytorachia (5-10 leucocytes/mm3) with a few atypical lymphoid cells. On admission, brain CT scan and MRI demonstrated diffuse and nodular leptomeningeal contrast enhancement predominant at the skull base and several osteolytic lesions in the right parietal bone. Extensive serological studies for infectious, autoimmune or neoplastic diseases were negative. The work-up diagnosis was neurosarcoidosis or multiple meningeal and osseous metastases of an unknown primary cancer. Surgical biopsy of the right parietal bone lesion showed only fibrous tissue with no evidence of tumour or inflammation. The patient was treated with high dose corticosteroids but its neurological status progressively worsened and he died of aspiration pneumonia 35 days after admission. Post-mortem examination revealed a PDLG, a rare fatal tumour with about 60 cases reported. PDGL is characterized by the diffusion of neoplastic glial cells throughout the leptomeninges without evidence of a primary intra-parenchymal lesion. Recognition of this rare brain tumour is important as recent reports suggest that radiotherapy and chemotherapy can improve patient survival.
Researchers ; Professionals ; Students
http://hdl.handle.net/2268/74363

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