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.