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

被引:0
作者
Aboseif, Albert [1 ]
Palmer, Kaitlyn [1 ]
Abrams, Aaron W. [2 ]
Lachhwani, Deepak [3 ]
Knight, Elia Margarita Pestana [3 ]
Valappil, Ahsan Moosa Naduvil [3 ]
Zeft, Andrew [4 ]
机构
[1] Cleveland Clin, Dept Neurol, Cleveland, OH 44195 USA
[2] Cleveland Clin, Mellen Ctr Multiple Sclerosis, Cleveland, OH USA
[3] Cleveland Clin, Epilepsy Ctr, Cleveland, OH USA
[4] Cleveland Clin, Ctr Pediat Rheumatol, Childrens Hosp, Cleveland, OH USA
来源
FRONTIERS IN NEUROLOGY | 2023年 / 14卷
关键词
paraneoplastic neurologic syndrome; autoimmune encephalitis (AE); ganglioneuroma; incidentaloma adrenal tumor; cerebellar degeneration; super-refractory status epilepticus (SRSE); CONVULSIVE STATUS EPILEPTICUS; ANTIBODIES; ETIOLOGY;
D O I
10.3389/fneur.2023.1250261
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
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