2016
DOI: 10.1155/2016/5016840
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Primary Diffuse Leptomeningeal Gliomatosis: Radiological/Pathological Features

Abstract: We present the case of a 43-year-old lady who presented with headaches, visual impairment, and seizures, progressing rapidly over the course of a few weeks. Extensive workup excluded an inflammatory or infectious cause. Imaging studies revealed diffuse thickening of the leptomeninges and serial CSF analysis showed raised opening pressures and increased protein levels. A diagnostic biopsy of the lower thoracic dura confirmed the diagnosis of primary diffuse leptomeningeal gliomatosis (PDGL). She was managed sup… Show more

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Cited by 4 publications
(4 citation statements)
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References 11 publications
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“…In moyamoya disease, diffuse leptomeningeal enhancement is scarce, which is caused by an engorged pial network via leptomeningeal anastomosis [ 32 ]. Other neoplasms characterised by diffuse leptomeningeal enhancement consist of primary diffuse leptomeningeal gliomatosis and diffuse leptomeningeal glioneuronal neoplasm [ 8 , 33 ]. Notwithstanding, the main imaging differential diagnosis of DLGNTs includes chronic infectious meningitis, tuberculous meningitis, sarcoidosis, leptomeningeal intraparenchymal diffuse astrocytoma, and pilocytic astrocytoma.…”
Section: Discussionmentioning
confidence: 99%
“…In moyamoya disease, diffuse leptomeningeal enhancement is scarce, which is caused by an engorged pial network via leptomeningeal anastomosis [ 32 ]. Other neoplasms characterised by diffuse leptomeningeal enhancement consist of primary diffuse leptomeningeal gliomatosis and diffuse leptomeningeal glioneuronal neoplasm [ 8 , 33 ]. Notwithstanding, the main imaging differential diagnosis of DLGNTs includes chronic infectious meningitis, tuberculous meningitis, sarcoidosis, leptomeningeal intraparenchymal diffuse astrocytoma, and pilocytic astrocytoma.…”
Section: Discussionmentioning
confidence: 99%
“…Moyamoya disease seldom show diffuse leptomeningeal enhancement [9]. Other entities exhibiting diffuse leptomeningeal enhancement include primary diffuse leptomeningeal gliomatosis and diffuse leptomeningeal glioneuronal tumor [6,10]. We underscore the need for suspicion of DLGNT/DLGNT-like neoplasms in such cases demonstrating thick and nodular meningeal enhancement, as it has a bearing on the treatment approaches.…”
Section: Discussionmentioning
confidence: 76%
“…Because of the small number of cases reported in the literature due to the low incidence of GBM with direct cranial nerve involvement and no cases of gliosarcoma, sometimes this kind of lesion is not considered and is misdiagnosed, both during preoperative diagnostic evaluation and during the surgery, hypothesizing the tumor origin from the nerve with secondary extension to the near brain parenchyma, or from heterotopic neuroglial cell dissemination from leptomeningeal gliomas or gliomatosis [29] , or from primary brainstem GBM with extension along adjacent cranial nerve.…”
Section: Discussionmentioning
confidence: 99%