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Pearls & Oy-sters: Salt and Pepper Sign, PLNTY for Drug-Resistant Epilepsy
Elma M Paredes-Aragón1, Norah A AlKhaldi2, Jorge G Burneo2
1From the Departments of Clinical Neurological Sciences (E.M.P.-A., J.G.B., D.L., L.C.A., D.S., S.M.M.), Department of Clinical Neurological Sciences and Oncology (M.M), Epidemiology and Biostatistics (J.G.B., D.S.), Pathology and Laboratory Medicine (L.C.A.), Medical Imaging (S.M.M.), Medical Biophysics (S.M.M.), and Psychology (S.M.M.), Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada; Department of Neurological Emergencies (E.M.P.-A.), National Institute of Neurology and Neurosurgery, Mexico City, Mexico; Department of Neurology (N.A.A.), King Fahad University Hospital, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia; and Epilepsy Department (M.H.-A.), Neurology Division, Instituto Nacional de Ciencias Neurológicas, Lima, Peru. elma.paredesaragon@lhsc.on.ca.
Abstract:
Drug-resistant epilepsy, defined as the failure of 2 or more antiseizure medications to achieve seizure freedom, is responsible for 2/3 of epilepsy cases. Tumors are responsible for up to 15% of all adult onset and up to 6% of childhood onset epilepsies. Among these tumors, commonly known subtypes DNET, ganglioglioma, and low-grade astrocytoma are often suspected. New advances in tumor classification have been made, with genetics playing a key role in tumor classification. Polymorphic low-grade neuroepithelial tumor of the young (PLNTY) is a highly epileptogenic subtype of tumors that may mimic low-grade gliomas but offer pathologic and genetic clues: oligodendroglioma-like cellular components and infiltration patterns and strong CD34-immunopositive stain. In addition, a key finding is radiologic: a unifocal abnormality best seen on MRI brain in FLAIR sequence as the "salt and pepper sign" and calcifications appreciated on CT head.
Insights
Drug-resistant epilepsy, often caused by tumors, can be challenging to treat. Polymorphic low-grade neuroepithelial tumor of the young (PLNTY) is a newly identified, highly epileptogenic tumor subtype with distinct genetic and imaging features.
Area of Science:
- Neurology
- Neuro-oncology
- Pathology
Background:
- Drug-resistant epilepsy affects two-thirds of epilepsy cases.
- Tumors account for a significant proportion of adult and childhood-onset epilepsies.
- Traditional tumor classifications for epilepsy are evolving with genetic insights.
Purpose of the Study:
- To introduce and characterize Polymorphic low-grade neuroepithelial tumor of the young (PLNTY) as a distinct, highly epileptogenic tumor subtype.
- To highlight the diagnostic challenges and unique features of PLNTY tumors.
- To differentiate PLNTY from other low-grade gliomas based on pathological and genetic markers.
Main Methods:
- Review of pathological and genetic findings in epilepsy-associated tumors.
- Analysis of imaging characteristics, including MRI FLAIR (salt and pepper sign) and CT head calcifications.
- Immunohistochemical staining for CD34.
Main Results:
- PLNTY tumors exhibit oligodendroglioma-like cellular components and infiltration patterns.
- A strong CD34-immunopositive stain is a key diagnostic clue for PLNTY.
- Characteristic radiologic findings include the 'salt and pepper sign' on MRI FLAIR and calcifications on CT.
Conclusions:
- PLNTY represents a distinct, highly epileptogenic tumor entity.
- Integrated pathological, genetic, and radiological analysis is crucial for accurate PLNTY diagnosis.
- Identification of PLNTY aids in understanding and potentially managing drug-resistant epilepsy cases linked to specific tumor types.
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