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Published on: May 7, 2020
Primary diffuse leptomeningeal gliomatosis mimicking tuberculous meningitis
Muhammet Kosker1, Dicle Sener1, Omer Kilic2
1Division of Infectious Diseases, Clinical Immunology and Allergy, Department of Pediatrics, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
Abstract:
Primary diffuse leptomeningeal gliomatosis is a disease with an aggressive course that can result in death. To date, 82 cases have been reported. Here, the case of a 3-year-old male patient presenting with strabismus, headache, and restlessness is reported. Physical examination revealed paralysis of the left abducens nerve, neck stiffness, and bilateral papilledema. Tuberculous meningitis was tentatively diagnosed, and antituberculosis treatment was initiated when cranial imaging revealed contrast enhancement around the basal cistern. Craniocervical magnetic resonance imaging (MRI) was performed when there was no response to treatment, and it revealed diffuse leptomeningeal contrast enhancement around the basilar cistern, in the supratentorial and infratentorial compartments, and in the spinal region. Primary diffuse leptomeningeal gliomatosis was diagnosed by a meningeal biopsy.
Insights
Primary diffuse leptomeningeal gliomatosis is a rare aggressive brain tumor. This case report details a 3-year-old male diagnosed with this condition after initial misdiagnosis.
Area of Science:
- Neuro-oncology
- Pediatric Neurology
- Pathology
Background:
- Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare and aggressive central nervous system malignancy.
- It affects the leptomeninges, the membranes surrounding the brain and spinal cord.
- Fewer than 100 cases have been documented, highlighting its rarity.
Observation:
- A 3-year-old male presented with symptoms including strabismus, headache, and restlessness.
- Initial examination revealed left abducens nerve palsy, nuchal rigidity, and bilateral papilledema.
- Cranial imaging showed contrast enhancement around basal cisterns, initially leading to a tentative diagnosis of tuberculous meningitis.
Findings:
- Despite antituberculosis treatment, the patient showed no improvement.
- Craniocervical MRI demonstrated diffuse leptomeningeal contrast enhancement throughout the supratentorial, infratentorial, and spinal compartments.
- A meningeal biopsy confirmed the diagnosis of primary diffuse leptomeningeal gliomatosis.
Implications:
- This case underscores the importance of considering rare neurological conditions in pediatric patients with atypical presentations.
- Accurate and timely diagnosis through advanced imaging and biopsy is crucial for appropriate management.
- Further research into PDLG is needed due to its aggressive nature and limited reported cases.
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