Surgery for Catastrophic Epilepsy with Temporo‐Parieto‐ Occipital Cortical Dysplasia: Clinical Features and Surgical Outcome of Three Patients.
Authors: Shimomura Jiro , Kubota Yuko , Tottori Takayasu , Tottori Takayasu , Matsuda Kazumi , Mihara Tadahiro , Fujiwara Tateki , Yagi Kazuichi 
Published: August 2005

Purpose: Three children with temporo‐parieto‐occipital cortical dys‐ plasia manifesting refractory seizures and severe mental retardation were treated by surgical resection of the epileptogenic lesions. Their clinical features and surgical outcome are presented.

Case report: Case 1 was a boy who was operated at 2 years and 9 months of age. He had had intractable partial seizures since the second postnatal day and serial spasms since 3 months old. He was admitted to our hospital at 9 months of age. Interictal electroencephalograms (EEG) showed increased slow waves in the left hemisphere and spikes and sharp waves in either both hemispheres or the left hemisphere. Rhythmic theta activities originating in the left posterior temporal and occipital areas were accompanied by partial seizures with eye blinking, respiratory change, and automatism in the extremities. Partial seizures occurred 40 to 50 times a day. Serial spasms appeared several times a day and always following partial seizures.

Case 2 was a boy who was operated at 5 years and 10 months of age. He had had spasms since the third postnatal day. Partial seizures with eye blinking, eye deviation to the right, and rigidity of the extremities appeared at the age of 2 months. He was admitted to our hospital at 4 years and 9 months of age. Interictal EEGs showed hilateral diffuse spikes, and focal spikes and sharp waves in the left occipital, left frontal, and right occipital areas. Partial seizures occurred 50 to 70 times a day with rhythmic theta activities in the left occipito‐parietal area. Serial spasms occurred 2 to 7 times a day.

Case 3 was a girl who was operated at 3 years and 10 months of age. Onset of upper gazing was noted at I month old and spasms 2 weeks arterwards. She was admitted to our hospital at the age of 4 months. Interictal EEGs showed spikes in the right occipital area. At the onset of a partial seizure, EEGs revealed fast rhythms at the 02 and T4 clectrodes, which was followed by eye deviation to the left or right, eye blinking, and opisthotonus of the body. Partial seizures occurred 10 to 20 timcs a day and spasms appeared at a frequency of several series per day.

All cases had severe mental retardation, and Cases 1 and 2 showed hypotonia. MRI revealed abnormal gyrations and signal abnormalities in the left (Cases 1 and 2) or the right (Case 3) temporo‐parietooccipital areas. Temporo‐parieto‐occipital resection was performed on the lcft side in Cases 1 and 2, and on the right side in Case 3. Pathological findings of the resected tissues from all the patients showed abnormal cortical structures, ectopic neurons, and balloon cells. Cortical dysplasia was diagnosed.

Partial seizurcs reduced in frequency by 10 % in Cases 1 and 2, and disappeared in Case 3, after surgery. Spasms disappeared before surgcry in Cases 1 and 3, and immediately after surgery in Case 2.

All patients showed an improvement of neuropsychological devel‐ opment after surgery with no surgical sequel.

Conclusion: Patients with temporo‐parieto‐occipital cortical dyspla‐ sia and intractable seizures may be good candidates for surgical treat‐ ment, if the epileptogenic lesion can be defined.