A Case of Primary Leptomeningeal Lymphoma Presenting with Hydrocephalus Characterized by Disproportionately Large Fourth Ventricle
Abstract
Primary leptomeningeal malignant lymphoma (PLML) is a rare variant of primary central nerve system malignant lymphoma (PCNSL) which is restricted to leptomeninges. The lesions of PLML can often be detected as abnormal enhancement on the surface of central nervous system or the ventricular wall on magnetic resonance imaging (MRIs). Cerebrospinal fluid (CSF) evaluation together with such MRI findings provides the definitive diagnosis of PLML. Here, we present a 45-year-old female case of PLML in which hydrocephalus with disproportionately large fourth ventricle was observed at presentation with gait instability. Head MRI revealed no abnormal enhancement and CSF cytology was negative, leaving the cause of hydrocephalus undetermined. Endoscopic third ventriculostomy (ETV) was effectively performed for hydrocephalus and her symptoms disappeared. Nearly 2 years later, she was brought to emergent room due to unconsciousness with the recurrence of hydrocephalus. MRI showed expanded fourth ventricle and abnormal enhancement on the ventricular wall. The endoscopic surgery for improving CSF flow was successful and inflammatory change was endoscopically observed on the ventricular wall involving aqueduct. Pathological diagnosis of the specimen from the ventricular wall proved B-cell lymphoma. Because neither brain parenchymal masses nor systemic tumors were identified, she was diagnosed with PLML and treated by high-dose methotrexate. She was in a stable state 2 years after the…
How strong is the evidence?
Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
Read the paper
Related studies
- Temporal profiles of viral load in posterior oropharyngeal saliva samples and serum antibody responses during infection by SARS-CoV-2: an observational cohort study
- Treatment and prognostic factors for long-term outcome in patients with anti-N-Methyl-D-Aspartate (NMDA) receptor encephalitis: a cohort study
- Effectiveness of mRNA BNT162b2 COVID-19 vaccine up to 6 months in a large integrated health system in the USA: a retrospective cohort study
- Postsurgical prescriptions for opioid naive patients and association with overdose and misuse: retrospective cohort study
- Ethnic and socioeconomic differences in SARS-CoV-2 infection: prospective cohort study using UK Biobank
- Timing of high-efficacy therapy for multiple sclerosis: a retrospective observational cohort study
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.