case_images: PMC10621035_01
case_images: MRI Brain at age 6 years (left) and age 18 years (right), shows progressive changes with prominent diffuse cerebellar atrophy (A,B), Signal change in both mesial temporal regions with left predominant atrophy (C,D), and left parahippocampal atrophy (E,F).
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case_images: PMC10621035_01_fneur-14-1250261-g001.jpg
case_images: fig1
case_images: Brain magnetic resonance imaging (MRI) showed cerebellar and left posterior parahippocampal atrophy, with bilateral mesial temporal lobe T2 hyperintensities (Figure 1).
case_images: CT chest & abdomen shows non-enhancing mass in the left adrenal region (A,B) with corresponding histopathology demonstrating features of ganglioneuroma (C).
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case_images: fig2
case_images: AE, computed tomography (CT) scan of her abdomen was performed which re-demonstrated the left adrenal mass (Figure 2), and follow-up positron emission tomography (PET)/CT revealed corresponding hypermetabolism. Although her adrenal mass was hormonally inactive and stable in size from four years prior, a high suspicion for a paraneoplastic neurologic syndrome led to a multidisciplinary consensus to proceed with surgical resection. Pathology was consistent with adrenal ganglioneuroma (Figure 2).
case_images: Timeline outlining disease progression and response to treatment over the lifespan (A) and during hospitalization for SRSE (B). SRSE, super-refractory status epilepticus; GTC, generalized tonic clonic seizure; IVMP, intravenous methylprednisolone; PLEX, plasmapheresis; IVIG, intravenous immunoglobulin; ASM, anti-seizure medication.
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case_images: PMC10621035_01_fneur-14-1250261-g003.jpg
case_images: fig3
case_images: Due to continued seizure activity and associated hemodynamic instability, she first required preliminary treatment with three days of 1 g intravenous methylprednisolone (IVMP) followed by 40 mg of prednisone daily, one cycle of PLEX, two separate courses of 2 g/kg IVIG, two doses of 1 g rituximab, and six doses of 500 mg IV cyclophosphamide (Figure 3).