- Department of Neurosurgery, University of California, Los Angeles, Los Angeles, California, USA
- Department of Pathology, University of California, Los Angeles, Los Angeles, California, USA
- Jonsson Comprehensive Cancer Center, University of California, Los Angeles, Los Angeles, California, USA
- Department of Neurosurgery, Yeungnam University College of Medicine, Daemyung-dong, Nam-gu, Daegu, Korea
Department of Neurosurgery, University of California, Los Angeles, Los Angeles, California, USA
Jonsson Comprehensive Cancer Center, University of California, Los Angeles, Los Angeles, California, USA
DOI:10.4103/sni.sni_423_16Copyright: © 2017 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Seung J. Lee, Minsu Kim, Carlito Lagman, Timothy T. Bui, William H. Yong, Isaac Yang. Corpora amylacea mimicking low-grade glioma and manifesting as a seizure: Case report. 26-Apr-2017;8:64
How to cite this URL: Seung J. Lee, Minsu Kim, Carlito Lagman, Timothy T. Bui, William H. Yong, Isaac Yang. Corpora amylacea mimicking low-grade glioma and manifesting as a seizure: Case report. 26-Apr-2017;8:64. Available from: http://surgicalneurologyint.com/surgicalint-articles/corpora-amylacea-mimicking-low%e2%80%91grade-glioma-and-manifesting-as-a-seizure-case-report/
Background:Corpora amylacea (CA) are accumulations of polyglucosan bodies typically found in astrocytic foot processes, and rarely, can mimic neoplasm. CA accumulation has also been associated with seizure disorders. We report the first case of a histologically confirmed intracranial, intraparenchymal CA lesion mimicking a low-grade glioma and manifesting as a seizure.
Case Description:A 43-year-old man presented after a general tonic–clonic (GTC) seizure. Brain magnetic resonance imaging (MRI) revealed a small lesion in the right mesial temporal lobe with radiologic features of a low-grade glioma. The patient underwent a right pteronial craniotomy for resection of the lesion. Histology demonstrated abundant polyglucosan bodies without neoplastic features. The patient tolerated the procedure well, was free from seizures without antiepileptic drugs at 2-week follow-up, and is undergoing serial surveillance.
Conclusion:The clinical manifestation of CA as a seizure in the context of an identified brain mass is extraordinarily rare. Nevertheless, CA should be considered in the differential diagnosis for patients with seizures and a radiologically identifiable low-grade lesion. Symptomatic CA lesions Mimicking a low-grade glioma should be surgically pursued with a goal of safe, maximal resection to confirm the diagnosis and to provide the patient with prognosis, which can significantly impact patient quality of life.
Keywords: Case reports, corpora amylacea, glioma, pathology, seizures
Corpora amylacea (CA) are aggregates of polyglucosan bodies consisting of insoluble polysaccharides and protein.[
History and examination
A 43-year-old, right-handed man without a prior history of epilepsy presented to his neurologist for evaluation of a GTC seizure. He had no other symptoms and was neurologically intact. He was prescribed an antiepileptic drug and underwent radiological evaluation.
Magnetic resonance imaging (MRI) of the brain revealed a nonenhancing lesion measuring 1.5 × 1.8 cm in the right mesial temporal lobe with minimal mass effect. The lesion appeared hyperintense on T2-weighted images [Figure
A right pteronial craniotomy was performed for resection of the mass. The lesion was noted to have a firm consistency, ill-defined planes, and significant pial adhesions. The lesion was meticulously resected under the operative microscope, and the specimen was sent to pathologic examination. The patient tolerated the procedure well. Immediate postoperative imaging demonstrated confirmed gross-total resection without evidence of ischemia or abnormal enhancement. At 2-week follow-up, the patient was free from seizures and no longer required antiepileptic drugs. The patient has not returned in the 13 months since the surgery.
Histological examination of the lesion revealed normal gray matter with focally prominent polyglucosan body accumulations in the brain parenchyma. Numerous spherical polyglucosan bodies were identified on Hematoxylin and Eosin (H and E) frozen section slides without features of neoplasia. Permanent section H and E slides [
Glucose monomers within neuroglial cells are thought to polymerize in the setting of cellular degeneration to form aggregates of polyglucosan bodies (hence the term corpora amalycea, Latin for body and starchy, respectively); however, the physiologic and pathologic significance of these lesions are not fully elucidated.[
CA accumulation can also have a genetic etiology. Adult polyglucosan body disease (APBD) is a rare autosomal recessive genetic disorder caused by a glycogen branching enzyme deficiency, leading to CA accumulation in the central and peripheral nervous system.[
MRI is highly sensitive for brain tumors, although it lacks the specificity to distinguish low-grade from high-grade neoplasms.[
PET imaging has also been proven to be useful in the diagnosis of low-grade gliomas. Chen et al. reported that the sensitivity in detecting brain tumors with 18F-DOPA is higher (sensitivity, 96%; 95% CI, 87–100%) than 18F-fludeoxyglucose (18F-FDG) PET imaging (sensitivity, 61%; 95% CI, 41–81%).[
CA accumulation has been described in patients with temporal lobe epilepsy and other seizure disorders.[
Abel et al.[
This case illustrates an extraordinarily rare instance in which CA aggregates appeared as a well-demarcated, spherical mass lesion causing a seizure. Although uncommon, CA should be considered in the differential diagnosis for lesions with radiological characteristics of a low-grade glioma.
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Conflicts of interest
There are no conflicts of interest.
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