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Primary Diffuse Leptomeningeal Gliomatosis: Radiological/Pathological Features
Ehtasham Ahmad1, Mohamed Mohamed1, Apostolos Vrettos2
1East Kent Hospitals University NHS Trust, Queen Elizabeth the Queen Mother Hospital, Department of General Internal Medicine, Margate, UK.
Case Reports in Neurological Medicine
|November 29, 2016
Summary
Primary diffuse leptomeningeal gliomatosis (PDGL) is a rare brain tumor. This case highlights its rapid progression and fatal outcome, emphasizing the need for accurate diagnosis despite challenging presentation.
Area of Science:
- Neurology
- Neuro-oncology
- Pathology
Background:
- Primary diffuse leptomeningeal gliomatosis (PDGL) is an uncommon condition.
- It involves the spread of glial tumor cells diffusely throughout the leptomeninges.
- PDGL often presents with nonspecific neurological symptoms, making diagnosis challenging.
Purpose of the Study:
- To report a rare case of primary diffuse leptomeningeal gliomatosis.
- To illustrate the clinical presentation, diagnostic workup, and outcome of PDGL.
- To highlight the challenges in diagnosing and managing this aggressive condition.
Main Methods:
- Case report of a 43-year-old female patient.
- Clinical evaluation including neurological examination, imaging (MRI), and cerebrospinal fluid (CSF) analysis.
- Histopathological confirmation via dural biopsy.
Main Results:
- The patient presented with acute onset of headaches, visual impairment, and seizures.
- Imaging revealed diffuse leptomeningeal thickening.
- CSF analysis showed elevated opening pressure and protein.
- Dural biopsy confirmed primary diffuse leptomeningeal gliomatosis.
Conclusions:
- Primary diffuse leptomeningeal gliomatosis is a rare and aggressive CNS malignancy.
- Early and accurate diagnosis is crucial, often requiring invasive procedures like biopsy.
- Despite supportive care, the prognosis remains poor, with rapid disease progression and mortality.

