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"Pseudo-BECRS": intracranial focal lesions suggestive of a primary partial epilepsy syndrome
M I Shevell1, B Rosenblatt, G V Watters
1Division of Pediatric Neurology, Montreal Children's Hospital, Quebec, Canada.
Insights
Benign epilepsy of childhood with rolandic spikes (BECRS) typically has a good prognosis. However, atypical cases may indicate underlying structural lesions requiring neuroimaging for accurate diagnosis.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neuroimaging
Background:
- Benign epilepsy of childhood with rolandic spikes (BECRS) is the most common primary partial epilepsy in children.
- It typically presents between ages 3-13 with characteristic EEG findings and a good prognosis.
- Neuroimaging is sometimes considered unnecessary due to the disorder's benign nature.
Purpose of the Study:
- To investigate cases initially suggestive of BECRS but found to have underlying structural lesions.
- To highlight atypical clinical features that may warrant neuroimaging in suspected BECRS cases.
Main Methods:
- Retrospective review of five patients with peribuccal seizures and EEG findings suggestive of BECRS.
- Analysis of clinical features, neuroimaging (CT, MRI), and histopathology.
Main Results:
- All five patients had focal lesions identified on neuroimaging.
- Low-grade astrocytoma was diagnosed in three patients, and a cavernous angioma in one.
- Atypical clinical features were noted in all reviewed cases.
Conclusions:
- While BECRS is typically benign, atypical presentations necessitate neuroimaging.
- Identifying underlying structural lesions is crucial for accurate diagnosis and management.
- Negative neuroimaging may be essential in confirming a definitive BECRS diagnosis.
Abstract:
Benign epilepsy of childhood with rolandic spikes (BECRS) is an electroclinical entity that is the most common primary partial epilepsy syndrome of childhood. Typically presenting between the ages of 3 and 13 years, it is characterized by a well-recognized seizure pattern arising in a normal child with EEG findings restricted to rolandic/centrotemporal regions. Seizure control is usually easily achieved and prognosis is believed to be uniformly good. Some authors have suggested that individuals fitting the electroclinical parameters of this entity need not undergo neuroimaging due to the benign evolution of this disorder. Five patients presenting over a 13-year period with peribuccal seizures, normal neurologic examinations, and EEG data initially suggestive of BECRS found to have focal lesions on neuroimaging are summarized. Independent bilateral centrotemporal epileptiform abnormalities were seen in 3 patients. Imaging studies (CT, MRI, or both) documented a mass lesion in all in variable locations. Histologic examination documented a low-grade astrocytoma in 3 patients and a cavernous angioma in another. The fifth patient refused treatment or biopsy. Careful retrospective review of the clinical features of these patients reveals variable atypical features in each. Therefore, despite an electroclinical phenotype initially suggestive of the BECRS presentation, the presence of atypical clinical features raises the possibility of an underlying structural lesion and thus a negative neuroimaging study may in some patients be essential to the definitive accurate diagnosis of BECRS.