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Primary diffuse leptomeningeal gliomatosis diagnosed on CSF cytology: perseverance pays off
  1. Céline Louapre1,
  2. Virginie Desestret2,
  3. Karima Mokhtari2,
  4. Catherine Lubetzki1
  1. 1Department of Neurology, APHP, Hôpital Pitié-Salpêtrière, Paris, France
  2. 2Department of Neuropathology, APHP, Hôpital Pitié-Salpêtrière, Paris, France
  1. Correspondence to Céline Louapre, Département de Neurologie, Hôpital Pitié-Salpêtrière, 47 boulevard de l'hôpital, Paris 75013, France; celine.louapre{at} or celine.louapre{at}

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Primary diffuse leptomeningeal gliomatosis is a rare neoplasm defined by the infiltration of the subarachnoid spaces by glial neoplastic cells, without there being a detectable intra-axial glioma. Unlike the meningeal dissemination of glial tumour cells that might follow a previously diagnosed parenchymatous glioma, primary diffuse leptomeningeal gliomatosis probably originates from heterotopic leptomeningeal glial nests in the subarachnoid space. Its clinical symptoms usually result from intracranial hypertension and malignant invasion of cranial or spinal nerves. Cranial and spinal MRI imaging usually shows extensive leptomeningeal thickening with gadolinium enhancement. Cerebrospinal fluid (CSF) cytology generally fails to detect malignant cells and so a meningeal biopsy—guided by the MRI findings—is often needed to confirm the diagnosis.

Among about 80 reported cases of primary diffuse leptomeningeal gliomatosis, six involved the spinal cord1; in these, the presenting symptoms, such as limb weakness and back pain, suggested a likely spinal cord location. Here, we report a patient with encephalopathic symptoms who had no intracranial meningeal enhancement on her brain MRI, either at the start or during the disease course, despite having spinal cord meningeal enhancement. We confirmed the diagnosis of primary diffuse leptomeningeal gliomatosis by identifying glial malignant cells in the CSF after the third spinal tap.

Case report

A 79-year-old woman gave a 5-month history of progressive balance and gait …

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  • Contributors All authors have contributed to the acquisition and interpretation of data for the work. CLo has contributed to the preparation of the manuscript. All authors have contributed to revising the manuscript critically for important intellectual content.

  • Competing interests None.

  • Competing interests CLu received honorarium corresponding to consultancy boards for Roche, Vertex, Genzyme and for giving lecture from Novartis.

  • Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Robin Grant, Edinburgh, UK.

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