A not so incidental 'incidentaloma' - pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review

Paraneoplastic neurological disorders are rare in children, with paraneoplastic cerebellar degeneration (PCD) considered highly atypical. We describe a 13-year-old girl with progressive neurobehavioral regression, cerebellar ataxia, and intractable epilepsy presenting in super-refractory status epilepticus. After an extensive evaluation, her clinical picture was suggestive of probable autoimmune encephalitis (AE). Further diagnostic testing revealed a molecularly undefined neural-restricted autoantibody in both serum and CSF, raising suspicion over an adrenal mass previously considered incidental. Surgical resection led to a robust clinical improvement, and pathology revealed a benign ganglioneuroma. This report widens the spectrum of paraneoplastic manifestations of ganglioneuroma, reviews the diagnostic approach to antibody-negative pediatric AE, and raises important clinical considerations regarding benign and incidentally found tumors in the setting of a suspected paraneoplastic neurologic syndrome.

Automated summary

Paraneoplastic neurological disorders in children are rare, and paraneoplastic cerebellar degeneration (PCD) is considered highly atypical. This case report describes a 13-year-old girl with progressive neurobehavioral regression, cerebellar ataxia, and intractable epilepsy, who was diagnosed with probable autoimmune encephalitis (AE). Surgical resection of an adrenal mass led to significant clinical improvement, and pathology revealed a benign ganglioneuroma. This report expands the understanding of paraneoplastic manifestations of ganglioneuroma and highlights important clinical considerations in the management of paraneoplastic neurological syndromes.