Abstracts

Rationale: Actual natural history and therapeutic outcome of Sturge-Weber syndrome (SWS) is still not well defined. We present our clinical analysis of diagnostic evaluation of seizure severity and therapeutic results. Methods: We reviewed 101 cases with SWS at Juntendo University 1990-2015 according to their intracranial leptomeningeal angioma extent, classifying them into six groups: Gr.1-bilateral, Gr.2-hemispherical, Gr.3-posterior quadrant, Gr.4-anterior quadrant, Gr.5-single lobe and Gr.6-others. We analyze seizure onset age, seizure type, seizure frequency, therapeutic method and treatment results. We also evaluated the following diagnostic modalities: scalp EEG, MRI, and FDG-PET. Positive findings ratios were calculated for each modality. Results: Results in the six groups were as follows: Gr.1 - 9 cases had first occurrence of epileptic seizures (FES) at an average age of 3.8M continuing at least once a month. Six complete callosotomy cases had improvement to at least Engel class 3. Gr.2 - 31 cases had FES at an age average 4.7M with frequency at least once a month in 68% of patients. Twenty-one hemispherotomy cases resulted in 90.5% seizure free outcome. Gr.3: 39 cases with FES at 10.1 months had seizure frequency of less than once a year in 54.8% of them. Almost half of the patients in this group had well-controlled seizure by antiepileptic drugs (AEDs). Thirteen of 14 patients after posterior quadrant disconnection procedure (92.9%) resulted being seizure free, but lesional excision was not effective. Gr.4: Eleven cases with FES average age of 18.9M had 55.6% seizure frequency less than once a year. In two disconnection procedure cases seizures disappeared, but in another three lesion excision cases seizures remained. Gr.5: Eight cases had well-controlled seizures by AEDs. Leptomeningeal angioma area could be well defined in all cases using FLAIR with Gd and SWI MRI imaging. FDG-PET demonstrated high uptake in 56.8%, and EEG showed low amplitude changes in 87.7% and paroxysms in only 21.8% of the cases. Conclusions: Patients with wide distribution of leptomeningeal angioma had a severe form of epilepsy. Their seizures were manifested early and needed radical surgery, and only lesional excision was ineffective in controlling seizures. MRI FLAIR with Gd enhancement and SWI sequences were effective to delineate the lesion. Hyper-metabolism of FDG-PET demonstrated the epileptic activity. Those modalities were effective to decide surgical treatment. Funding: none