P0997 - 7 year old Male with 2 months of progressive left sided weakness

Introduction: Gliomas are the most common primary CNS tumors making up 50-60% of brain tumors in children. Prognosis for gliomas depends on histologic grade and location of tumor. Impingement on normal brain tissue or increase in intracranial pressure is caused by either obstruction of CSF flow or by direct mass effect. Symptoms can include lethargy, headache and vomiting. Signs of slow growing CNS tumors can include irritability, poor school performance and loss of developing milestones. Most arise with no known underlying risk factor or disorder.

Case Presentation: 7-year-old male with no significant past medical history presented with left sided weakness that began 8 weeks prior to admission. Initially mother noticed the patient to have an asymmetric smile. Patient was seen by PCP and was referred to ophthalmology and neurology at that time. 4 weeks later, he was noted to have left arm weakness and difficulty writing with his left hand. A week later he began having weakness in his left leg and was noticed to have increased instability. Patient was reevaluated by PCP who instructed him to complete a 5-day course of steroids and follow-up with neurology. On the day of admission he was seen by an outpatient neurologist using telemedicine due to COVID outbreak. It was noted the patient had left sided paralysis, a dilated left pupil, weakness of the left arm and leg with elevation. After seeing the physical exam findings through a webcam, the neurologist suggested for the patient to have urgent evaluation in the emergency department. CT brain with contrast revealed a heterogenous, complex mass in the region of the right hypothalamus. MRI brain showed mass that involved the right and left cerebral hemisphere, with infiltration of the left optic tract. A brain biopsy was performed by neurosurgery and pathology was GFAP positive confirming astrocytoma in the brainstem.

Discussion: About 25% of astrocytomas are aggressive or high grade. Brainstem gliomas make up 10-20% of CNS tumors in children less than 15 years old. Diffuse midline gliomas are infiltrative tumors, usually with astrocytic morphology and are located in the pons, thalamus or spinal cord. Most cases have high grade features and are histologically consistent with Grade 4. Treatment includes surgery, radiotherapy and chemotherapy. For high grade tumors surgery can be performed as well as radiotherapy and chemotherapy. But without reasonable resection chemotherapy is palliative. Brainstem gliomas cannot be removed, and therefore have a poor outcome.

Conclusion: Despite lack of in person evaluation, this case highlights the importance of prompt recognition and action when performing history and physical exams even in this new era of telemedicine. In the setting of the COVID-19 pandemic, the use of telemedicine proved to be prominent in the diagnosis of this patient.