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Published on: January 9, 2019
[Diagnostic value of brain biopsy in a pediatric multiple sclerosis mimicking brain stem glioma]
Yumiko Nakazawa1, Takanobu Maekawa, Shinji Oana
1Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development.
Abstract:
Diagnosis of multiple sclerosis (MS) is difficult when the lesion mimics glioma or cerebral enchephalitis. We report a case of pediatric MS initially suspected as brain stem glioma. An 11-year-old boy developed left foot joint pain followed by progressive symptoms such as left arm and leg weakness, dysarthria, paraplegia, and decreased level of consciousness. He subsequently developed respiratory distress requiring endotracheal intubation and mechanical ventilation. Magnetic resonance imaging showed a mass measuring 2 cm in the medulla oblongata. Although this mass was initially suspected as a glioma, the patient's acutely progressive disease course was not consistent with this diagnosis. Open biopsy revealed inflammation and demyelination, but no malignant cells were detected. He was treated with steroid pulse therapy, which showed dramatic effects. Nine months later, he developed another episode characterized by several neurological symptoms, and the diagnosis of MS was clinically confirmed. Open brain stem biopsy is technically demanding, but this case demonstrates that appropriate neurosurgical evaluation can play an important role in diagnosis by ruling out glioma and confirming MS.
Insights
Pediatric multiple sclerosis (MS) can mimic brain stem glioma. Early biopsy and steroid treatment were crucial for diagnosing MS in an 11-year-old boy with progressive neurological deficits.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuro-oncology
Background:
- Multiple sclerosis (MS) diagnosis can be challenging, especially in pediatric cases where symptoms may mimic other neurological conditions like glioma.
- Demyelinating diseases in children require careful differential diagnosis to distinguish from neoplastic or infectious etiologies.
Observation:
- An 11-year-old boy presented with rapid neurological decline, including weakness, dysarthria, paraplegia, and respiratory distress.
- MRI revealed a medulla oblongata mass initially suspected as brain stem glioma, but the acute progression was atypical for malignancy.
Findings:
- Open biopsy confirmed inflammation and demyelination, ruling out glioma and leading to a diagnosis of pediatric MS.
- The patient showed a dramatic response to high-dose steroid pulse therapy.
Implications:
- This case highlights the importance of neurosurgical evaluation in differentiating MS from brain stem glioma in pediatric patients.
- Timely diagnosis and appropriate treatment, including immunosuppressive therapy, are critical for managing pediatric MS and improving patient outcomes.
