Primary Diffuse Leptomeningeal Gliomatosis Mimicking Meningeal Tuberculosis

Abstract

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare condition, with only 45 cases recorded to date, characterized by infiltration of the meninges by glial cells without evidence of primary tumor in the brain or spinal cord parenchyma. Here, we describe a patient with PDLG who was managed with tuberculostatic drugs owing to multiple findings that were suggestive of tuberculous meningitis. A 19-year-old woman presented with headaches and behavioral changes. A sudden decrease in visual acuity with papilledema, bilateral sixth nerve palsies, and neck stiffness developed. Lumbar puncture showed elevated opening pressure (50 cm H2O). Cerebrospinal fluid (CSF) analysis showed glucose 30 mg/dL, protein 26.5 mg/dL, white blood cell count 150 (60% lymphocytes, 40% neutrophils). The second sample of CSF provided adenosine deaminase activity 21.9 U/L. Polymerase chain reaction for Koch's bacillus was positive in the third CSF sample. Magnetic resonance imaging revealed meningeal thickening of the quadrigeminal cistern, tentorium cerebelli, cerebral convexity, and spinal cord, with gadolinium enhancement in nodular lesions. The patient died 22 weeks after symptom onset owing to brainstem infarction. Postmortem pathologic studies revealed PDLG. This entity should be included in the differential diagnosis of tuberculous meningitis that does not respond to treatment with antituberculous drugs. Surgical biopsy should be considered in contrast-enhanced areas in magnetic resonance imaging.

Primary Diffuse Leptomeningeal Gliomatosis: Radiological/Pathological Features

“…Primary diffuse leptomeningeal gliomatosis is a rare condition characterised by infiltration of the meninges by neoplastic glial cells without evidence of primary tumour in the brain or the spinal cord [1]. It is an ultimately fatal condition which is characterised by infiltration of meninges by tumour cells composed of nests of heterotopic glial cells.…”

Primary diffuse leptomeningeal gliomatosis: An autopsy case report

“…This condition may be commonly mistaken for tuberculous meningitis especially, in endemic areas. [3] It is challenging to have an ante mortem diagnosis as PDLG can have varying and non-specific clinical features, time course, and progression of the illness. According to Cooper and Kernohan,[1] the definite diagnosis of PDLG is based on the following criteria: No apparent attachment of extra medullary meningeal tumor to the neural parenchyma, no evidence of primary neoplasia within the neuraxis, and the existence of distinct leptomeningeal encapsulation around the tumor.…”

“…In a review of 24 published autopsy proven cases of PDLG, 16 patients underwent ante mortem biopsy of which histology missed the diagnosis in 5 cases. [3] We performed a meningeal with brain biopsy near the temporal lobe where there was thick meningeal enhancement. However, the biopsy did not show any tumor cells.…”

Primary Diffuse Leptomeningeal Gliomatosis in Children

“…2 Ophthalmic manifestations of PDLG include optic disk edema or pallor, bilateral sixth nerve palsy associated with visual loss, and diplopia. [3][4][5] Neurologic symptoms of PDLG include headache, cranial nerve involvement, meningismus, and decreased mental status. 6 PDLG is a rare disease with only 26 biopsy-proven cases reported in the MEDLINE-indexed English literature, including 10 cases involving children.…”

“…To our knowledge, this is the first case of PDLG presenting with bilateral proptosis in which a direct involvement of the optic nerve sheath is demonstrated as a working diagnosis and not on autopsy. [4][5][6][7] …”