Related Experiment Video
Updated: Mar 28, 2026
Author Spotlight: Unveiling Cellular Functions and Potential Clinical Implications of Leptomeningeal Lymphatic Endothelial Cells
Published on: September 8, 2023
Primary leptomeningeal B-cell lymphoma with normal pressure hydrocephalus at diagnosis
Takuma Ishizaki1, Takeki Mitsui, Yuri Uchiyama
1Department of Medicine and Clinical Science, Graduate School of Medicine, Gunma University.
Abstract:
An 80-year-old man, presenting with gait disturbance and memory loss, had findings of normal pressure hydrocephalus. Primary leptomeningeal lymphoma (PLML) was diagnosed based on cytology and flow cytometry of cerebrospinal fluid obtained by examination. Gadolinium-enhanced MRI showed enhancement of the brain and spinal cord but FDG-PET/CT revealed no lymph node swelling. With intrathecal chemotherapy, meningeal lesions disappeared and the gait disturbance and memory loss improved. However, the disease recurred three months later, manifesting as left facial nerve palsy, but the symptoms disappeared in response to intrathecal chemotherapy and systemic rituximab administration. Although a tumor lesion in the spinal canal was suggested by MRI examination, the patient has maintained a good clinical course for four years with intrathecal chemotherapy every three months. PLML is a very rare disease and its diagnosis is difficult. Repeated intrathecal chemotherapy appeared to be effective against PLML in this case.
Insights
Primary leptomeningeal lymphoma (PLML), a rare condition, was diagnosed in an elderly man with neurological symptoms. Intrathecal chemotherapy and rituximab led to a four-year remission, showing treatment efficacy.
Area of Science:
- Neurology
- Oncology
- Hematology
Background:
- Normal pressure hydrocephalus symptoms like gait disturbance and memory loss can mask other serious conditions.
- Primary leptomeningeal lymphoma (PLML) is an exceptionally rare malignancy affecting the central nervous system's protective membranes.
Observation:
- An 80-year-old male presented with symptoms suggestive of normal pressure hydrocephalus.
- Cerebrospinal fluid analysis confirmed PLML, despite negative findings on lymph node imaging (FDG-PET/CT).
- Initial treatment with intrathecal chemotherapy resolved neurological symptoms and meningeal enhancement on MRI.
Findings:
- The patient experienced disease recurrence with left facial nerve palsy, which responded to further intrathecal chemotherapy and systemic rituximab.
- Despite an MRI suggesting a spinal canal tumor, the patient maintained a favorable clinical course for four years with maintenance intrathecal chemotherapy.
- This case highlights the diagnostic challenges and effective management of PLML.
Implications:
- Repeated intrathecal chemotherapy, combined with systemic agents like rituximab, can be an effective long-term strategy for managing primary leptomeningeal lymphoma.
- Early and accurate diagnosis of PLML, though challenging, is crucial for initiating timely and appropriate treatment.
- This case underscores the importance of considering rare diagnoses in patients with atypical neurological presentations.
