Brainstem Encephalitis in Neuropsychiatric Systemic Lupus Erythematosus Mimicking Malignant Lymphoma: A Case Report and Literature Review

We report a rare case of neuropsychiatric (NP) systemic lupus erythematosus (NPSLE) manifesting as brainstem encephalitis with generalized lymphadenopathy. A 32-year-old woman developed altered consciousness, fever, and pancytopenia, with diffusion-restricted brainstem lesions and elevated soluble interleukin-2 receptor (sIL-2R) levels in both serum and cerebrospinal fluid (CSF), accompanied by systemic lymphadenopathy and cytopenia, strongly mimicking malignant lymphoma. Histopathology excluded malignancy. The patient underwent immunosuppressive therapy with corticosteroids and cyclophosphamide, leading to marked neurological improvement. This case highlights the diagnostic challenge in differentiating NPSLE with brainstem involvement from lymphoproliferative disorders and underscores the importance of early biopsy and prompt immunosuppressive intervention to achieve favorable outcomes.

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Publication Details

Journal
Cureus
Published
2026-01-14
DOI
https://doi.org/10.7759/cureus.101551
Primary Topic
CNS Lymphoma Diagnosis and Treatment
Type
article
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article

Brainstem Encephalitis in Neuropsychiatric Systemic Lupus Erythematosus Mimicking Malignant Lymphoma: A Case Report and Literature Review

Tatsuya Ueno, Shuya Ochiai
Cureus
CNS Lymphoma Diagnosis and Treatment
article

Brainstem Encephalitis in Neuropsychiatric Systemic Lupus Erythematosus Mimicking Malignant Lymphoma: A Case Report and Literature Review

Tatsuya Ueno, Shuya Ochiai
article en

Abstract

We report a rare case of neuropsychiatric (NP) systemic lupus erythematosus (NPSLE) manifesting as brainstem encephalitis with generalized lymphadenopathy. A 32-year-old woman developed altered consciousness, fever, and pancytopenia, with diffusion-restricted brainstem lesions and elevated soluble interleukin-2 receptor (sIL-2R) levels in both serum and cerebrospinal fluid (CSF), accompanied by systemic lymphadenopathy and cytopenia, strongly mimicking malignant lymphoma. Histopathology excluded malignancy. The patient underwent immunosuppressive therapy with corticosteroids and cyclophosphamide, leading to marked neurological improvement. This case highlights the diagnostic challenge in differentiating NPSLE with brainstem involvement from lymphoproliferative disorders and underscores the importance of early biopsy and prompt immunosuppressive intervention to achieve favorable outcomes.

CureusVol. 18(1)
Aomori Prefectural Central Hospital (JP)
Good health and well-being
Openalex Percentile: Top 88%
CNS Lymphoma Diagnosis and Treatment
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Brainstem Encephalitis in Neuropsychiatric Systemic Lupus Erythematosus Mimicking Malignant Lymphoma: A Case Report and Literature Review — Tatsuya Ueno, Shuya Ochiai · Cureus (2026) | TGRS Research Map | TGRS