Epilepsia partialis continua associated with widespread gliomatosis cerebri.
We report an uncommon association of intractable epilepsia partialis continua that was the main presentation of widespread gliomatosis cerebri in two females. Both children had a preceding prolonged secondary generalized seizure 2-4 months before the evolution of epilepsia partialis continua, including recurrent clusters of left-sided myoclonic twitching and sensory impairment. During these events, the children remained fully alert. These seizures were corroborated by prolonged focal epileptic spike/wave discharges evident on the electroencephalograms. Cerebral magnetic resonance imaging in the first patient demonstrated a wide area of increasing signals over the right frontocentral regions, along with diffuse cortical-subcortical infiltration impinging on the left hemisphere. In the second patient a cortical lesion was suspected. Evaluation for Rasmussen's encephalitis, focal cortical dysplasia, or a gliomatous process was conducted; the patients underwent a stereotactic brain biopsy in which the histologic findings were compatible with gliomatosis cerebri with diffuse widespread infiltration of glioma cells with no constitution of a circumscribed tumor mass. The first patient was treated with cranial radiation, chemotherapy, steroids, and combined antiepileptic therapy. The focal seizures gradually but markedly decreased in frequency, and sensory impairment abated within 18 months after establishment of the diagnosis and ensuing therapy. Cognition remains intact. The second female died 2 years after presentation despite massive chemotherapy and antiepileptic medications. Although rare, gliomatosis cerebri should be taken into account in the differential diagnosis of epilepsia partialis continua in children to facilitate a rapid diagnosis and initiation of prompt treatment of this rare disorder that may respond to a concurrent effective combination of cranial radiation, chemotherapy, and antiepileptic medications.
Shahar
Eli
E
Child Neurology Unit and Epilepsy Service, Meyer Children Hospital, Rambam Medical Center, Haifa, Israel.
Kramer
Uri
U
Nass
Dvora
D
Savitzki
David
D
D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D004569:Electroencephalography; D004660:Encephalitis / Q000175:diagnosis; D017036:Epilepsia Partialis Continua / Q000175:diagnosis* / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009207:Myoclonus / Q000209:etiology; D018302:Neoplasms, Neuroepithelial / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy