Facial diplegia as the first manifestation of Burkitt lymphoma with a Guillain-Barré syndrome–like presentation: a case report

BACKGROUND: Guillain-Barré syndrome (GBS) is among the most common causes of acute inflammatory polyneuropathy and may present with cranial nerve involvement, including facial diplegia. Rarely, hematologic malignancies can produce a GBS-compatible or GBS-like neurological phenotype, creating a diagnostic challenge in the acute setting. CASE PRESENTATION: A 50-year-old immunocompetent woman presented with bilateral facial paralysis, dysarthria, dysphagia, areflexia, and mild left upper-limb weakness. Cerebrospinal fluid (CSF) analysis showed albuminocytologic dissociation. Baseline electromyography and nerve conduction studies (EMG/NCS), performed early in the course, showed non-specific sensorimotor polyneuropathy without definitive demyelinating features. Because the clinical syndrome and CSF findings were compatible with a time-sensitive working diagnosis of GBS, intravenous immunoglobulin was initiated. Follow-up EMG/NCS in the second week evolved to show demyelinating features with secondary axonal involvement, compatible with GBS. However, rapidly progressive leukocytosis, markedly elevated lactate dehydrogenase and ferritin levels, profound weight loss, splenomegaly, para-aortic lymphadenopathy, and blast-like cells on peripheral smear prompted parallel hematologic reassessment. Peripheral blood flow cytometry was consistent with Burkitt lymphoma, and repeat CSF flow cytometry demonstrated a CD10 + germinal center-derived B-cell neoplastic population, indicating central nervous system involvement. The patient subsequently deteriorated with aspiration pneumonia, sepsis/shock, and a fatal course before lymphoma-directed chemotherapy could be initiated. CONCLUSIONS: Burkitt lymphoma with central nervous system involvement can present with a GBS-compatible acute neuropathic phenotype, including facial diplegia. The initial diagnosis of GBS may be clinically reasonable in a time-sensitive setting; however, major systemic and hematologic red flags should prompt early parallel evaluation for malignancy. Peripheral blood and CSF flow cytometry may provide decisive diagnostic evidence when biopsy or further work-up is not feasible.

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Journal
BMC Neurology
Published
2026-07-18
DOI
https://doi.org/10.1186/s12883-026-05153-4
Primary Topic
CNS Lymphoma Diagnosis and Treatment
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article
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article

Facial diplegia as the first manifestation of Burkitt lymphoma with a Guillain-Barré syndrome–like presentation: a case report

Ahmet Kasım Kiliç, Banu Özen Barut, Irem Yagmur Demir
BMC Neurology
CNS Lymphoma Diagnosis and Treatment
article

Facial diplegia as the first manifestation of Burkitt lymphoma with a Guillain-Barré syndrome–like presentation: a case report

Ahmet Kasım Kiliç, Banu Özen Barut, Irem Yagmur Demir
article en

Abstract

BACKGROUND: Guillain-Barré syndrome (GBS) is among the most common causes of acute inflammatory polyneuropathy and may present with cranial nerve involvement, including facial diplegia. Rarely, hematologic malignancies can produce a GBS-compatible or GBS-like neurological phenotype, creating a diagnostic challenge in the acute setting. CASE PRESENTATION: A 50-year-old immunocompetent woman presented with bilateral facial paralysis, dysarthria, dysphagia, areflexia, and mild left upper-limb weakness. Cerebrospinal fluid (CSF) analysis showed albuminocytologic dissociation. Baseline electromyography and nerve conduction studies (EMG/NCS), performed early in the course, showed non-specific sensorimotor polyneuropathy without definitive demyelinating features. Because the clinical syndrome and CSF findings were compatible with a time-sensitive working diagnosis of GBS, intravenous immunoglobulin was initiated. Follow-up EMG/NCS in the second week evolved to show demyelinating features with secondary axonal involvement, compatible with GBS. However, rapidly progressive leukocytosis, markedly elevated lactate dehydrogenase and ferritin levels, profound weight loss, splenomegaly, para-aortic lymphadenopathy, and blast-like cells on peripheral smear prompted parallel hematologic reassessment. Peripheral blood flow cytometry was consistent with Burkitt lymphoma, and repeat CSF flow cytometry demonstrated a CD10 + germinal center-derived B-cell neoplastic population, indicating central nervous system involvement. The patient subsequently deteriorated with aspiration pneumonia, sepsis/shock, and a fatal course before lymphoma-directed chemotherapy could be initiated. CONCLUSIONS: Burkitt lymphoma with central nervous system involvement can present with a GBS-compatible acute neuropathic phenotype, including facial diplegia. The initial diagnosis of GBS may be clinically reasonable in a time-sensitive setting; however, major systemic and hematologic red flags should prompt early parallel evaluation for malignancy. Peripheral blood and CSF flow cytometry may provide decisive diagnostic evidence when biopsy or further work-up is not feasible.

BMC Neurology
Dr Lütfi Kırdar Kartal Eğitim ve Araştırma Hastanesi (TR), Sağlık Bilimleri Üniversitesi (TR), University of Health Sciences Antigua (AG)
Good health and well-being
Openalex Percentile: Top 8%
CNS Lymphoma Diagnosis and Treatment
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