Cargando…

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 epil...

Descripción completa

Detalles Bibliográficos
Autores principales: Aboseif, Albert, Palmer, Kaitlyn, Abrams, Aaron W., Lachhwani, Deepak, Knight, Elia Margarita Pestana, Valappil, Ahsan Moosa Naduvil, Zeft, Andrew
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10621035/
https://www.ncbi.nlm.nih.gov/pubmed/37928156
http://dx.doi.org/10.3389/fneur.2023.1250261
Descripción
Sumario: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.