[P1-105] Status Epilepticus in Gliomatosis Cerebri: A Case Report
Introduction: Gliomatosis Cerebri (GC) is a rare diffuse infiltrative primary neoplasm of brain affecting several lobes without destructing brain architecture and neurons. Symptoms and signs of GC include headache, lethargy, hemiparesis and/or seizures. Here we reported a case of GC who initially presented with seizures and status epilepticus.
Methodology: The male patient aged 12 years when he exhibited the first seizure with clinical manifestations of bilateral fixed upward gaze, facial twitching and clonic seizures of right upper limb. The seizure frequency was 4 to 8 times per day. In the following days, prolonged trance-like stare was noted. Neurological examinations revealed unremarkable findings except for positive Babinski signs. Basic laboratory tests showed nothing particular. EEG disclosed generalized background slowing and focal spikes arising from F4. Brain MRI revealed multiple high signal intensity lesions scattering over the cortex, subcortical white matter of bilateral fronto-temporo-parietal lobes, corpus callosum and brainstem. For diagnosis, stereotactic brain biopsy was arranged.
Results: Brain biopsy showed diffuse astrocyte infiltration in H&E stain, and fibrillary astrocytic cells proliferation and infiltration containing cellular fibrillary processes and nuclei in S-100 stain. The diagnosis of GC was made. The seizures were too refractory to control. His clinical condition deteriorated gradually. He died of respiratory failure secondary to brain stem dysfunction at age of 12 years and 6 months.
Conclusions: Clinical manifestations of GC vary greatly and are non-specific. It remains a challenge in the diagnosis and needs brain tissue biopsy for confirmation. Seizures and status epilepticus could be the first clinical features.
Methodology: The male patient aged 12 years when he exhibited the first seizure with clinical manifestations of bilateral fixed upward gaze, facial twitching and clonic seizures of right upper limb. The seizure frequency was 4 to 8 times per day. In the following days, prolonged trance-like stare was noted. Neurological examinations revealed unremarkable findings except for positive Babinski signs. Basic laboratory tests showed nothing particular. EEG disclosed generalized background slowing and focal spikes arising from F4. Brain MRI revealed multiple high signal intensity lesions scattering over the cortex, subcortical white matter of bilateral fronto-temporo-parietal lobes, corpus callosum and brainstem. For diagnosis, stereotactic brain biopsy was arranged.
Results: Brain biopsy showed diffuse astrocyte infiltration in H&E stain, and fibrillary astrocytic cells proliferation and infiltration containing cellular fibrillary processes and nuclei in S-100 stain. The diagnosis of GC was made. The seizures were too refractory to control. His clinical condition deteriorated gradually. He died of respiratory failure secondary to brain stem dysfunction at age of 12 years and 6 months.
Conclusions: Clinical manifestations of GC vary greatly and are non-specific. It remains a challenge in the diagnosis and needs brain tissue biopsy for confirmation. Seizures and status epilepticus could be the first clinical features.