Clinical Report: Rhombencephalitis in a Postpartum Patient – Diagnostic Challenges
Overview
Rhombencephalitis (RE) is a rare, life-threatening inflammation of the brainstem and cerebellum, often overlooked in postpartum patients. This case study details a 30-year-old woman presenting with neurological decline initially misdiagnosed as a brain tumor, ultimately confirmed post-mortem as RE, highlighting diagnostic and therapeutic challenges.
Background
Rhombencephalitis is caused by infections, autoimmune diseases, or paraneoplastic syndromes and predominantly affects vulnerable populations such as the elderly, immunosuppressed, cancer patients, and pregnant women. The most common infectious cause in younger patients is Listeria monocytogenes, with other agents including enterovirus 71 and herpes viruses. Autoimmune causes include Behcet’s disease and multiple sclerosis. Diagnosis is complicated by overlapping symptoms with other neurological conditions and often requires MRI, cerebrospinal fluid analysis, and histopathology.
Data Highlights
Parameter
Day 1
Day 2
Day 3
C-reactive protein (CRP, mg/L)
<4
91
71
Leucocytes (10E9/L)
16.4
21.1
15.3
Temperature (°C)
35.0 (hypothermic)
38.0 (sub-febrile)
Not specified
Key Findings
RE can present with nonspecific symptoms such as headache, neck pain, dizziness, vomiting, and impaired consciousness, complicating early diagnosis.
Brain MRI may mimic diffuse midline glioma with extensive FLAIR changes from brainstem to cervical spinal cord, leading to misdiagnosis.
Laboratory inflammatory markers (CRP and leucocytes) may rise but can be misinterpreted as non-infectious, delaying antimicrobial treatment.
Post-mortem pathology revealed lymphocytic inflammation predominantly in brainstem and meninges without evidence of tumor, vasculitis, or infectious agents by PCR and immunohistochemistry.
Empirical antibiotic and antiviral treatments were not initiated due to low suspicion of infection, contributing to fatal outcome.
Postpartum immune modulation may predispose to RE even in previously healthy, immunocompetent women.
Clinical Implications
Clinicians should maintain a high index of suspicion for rhombencephalitis in postpartum patients presenting with brainstem symptoms and atypical MRI findings. Early consideration of infectious and autoimmune etiologies with prompt CSF analysis and empirical antimicrobial therapy may improve outcomes. Awareness of postpartum immune changes is critical to avoid misdiagnosis and delayed treatment.
Conclusion
Rhombencephalitis is a rare but fatal diagnosis that can be overlooked in postpartum patients due to nonspecific symptoms and misleading imaging. Early recognition and treatment are essential to improve prognosis.
References
Rhombencephalitis: An Overlooked Diagnosis in Postpartum Patients - A Case Study
