Bilateral temporal glioma presenting as a paraneoplastic limbic encephalitis with pure cognitive impairment
Vincent Deramecourt1, Stéphanie Bombois, Stéphanie Debette
1Department of Neurology and Memory Clinic, University Hospital of Lille and Lille II University, France. v-deramecourt@chru-lille.fr
Introduction:
Memory impairment caused by bilateral hippocampal primitive brain tumor is rarely reported. Clinical and MRI features can mimic paraneoplastic limbic encephalitis (PLE), and the differential diagnosis between these 2 entities may be difficult.
Case Report:
We report the case of a 42-year-old woman presenting with amnesia without neurologic focal signs at clinical examination. The neuroimaging features consisted of bilateral limbic hyperintensities on T2-weighted and FLAIR brain MRI. Despite exhaustive biologic and radiologic investigations, no specific etiology was found. The diagnosis of paraneoplastic limbic encephalitis was suspected, although antineuronal antibodies were negative and no cancer was detected after the first evaluation. Eight months after onset, the memory complaint of the patient increased along with disability in activities of the daily living, the neurologic examination slightly changed with frontal neurologic signs and the brain MRI showed a new cystic lesion in the left hippocampus with enhancement after contrast administration. The left temporal tumor was resected and the neuropathological examination was consistent with gliomatosis cerebri with a focal high grade astrocytoma.
Conclusions:
This case highlights the need to consider the possibility of infiltrative gliomatosis in patients presenting with features of paraneoplastic limbic encephalitis.
Insights
Bilateral hippocampal primitive brain tumors can cause memory loss, mimicking paraneoplastic limbic encephalitis (PLE). Gliomatosis cerebri should be considered in such cases, even with negative antibody tests.
Area of Science:
- Neuroscience
- Oncology
- Radiology
Background:
- Memory impairment from bilateral hippocampal tumors is rare.
- Distinguishing these tumors from paraneoplastic limbic encephalitis (PLE) on MRI can be challenging.
- PLE mimics suggest autoimmune or neoplastic processes affecting the limbic system.
Observation:
- A 42-year-old woman presented with amnesia and bilateral limbic hyperintensities on MRI.
- Initial investigations for PLE were negative, including antibody testing and cancer screening.
- Progressive memory decline and new MRI findings led to surgical intervention.
Findings:
- Neuropathology revealed gliomatosis cerebri with a focal high-grade astrocytoma.
- The tumor infiltrated the hippocampus, causing the observed memory deficits.
- Initial presentation mimicked inflammatory or autoimmune limbic encephalitis.
Implications:
- This case underscores the importance of considering infiltrative gliomatosis cerebri.
- Early recognition of atypical presentations is crucial for accurate diagnosis and treatment.
- Differential diagnosis in limbic encephalitis requires a broad perspective, including neoplastic etiologies.
Related Concept Videos
Encephalitis l: Introduction
Encephalitis ll: Pathophysiology
Hepatic Encephalopathy
Dementia l: Introduction
Cryptococcal Meningitis
Brain Abscess l: Introduction

