Bilateral perisylvian ulegyria: clinicopathological study of patients presenting with pseudobulbar palsy and epilepsy.
Structural abnormalities related with pseudobulbar palsy have been gaining attention because of their characteristic symptoms and unique pathogenesis. We present five cases of bilateral perisylvian ulegyria (BPU) presenting epilepsy and pseudobulbar palsy with pathogenesis different from previously reported syndromes. All patients showed medically intractable seizures, complex partial seizures with secondary generalization and clinical symptoms of pseudobulbar palsy, including dysarthria, limitation of tongue movement and drooling. MRI revealed BPU in all patients, and BPU associated with hippocampal sclerosis in four patients. Intracranial EEG recording with subdural grip and stripe was helpful for localizing the area of ictal generation. Resective surgeries, including the temporal lobe, central area and parietal lobe, were performed depending on the localizing information. The surgical outcome was favorable after 9.8 years of follow-up. Characteristic features of ulegyria were confirmed on pathological examination. Ulegyria is considered to be another important perinatal or postnatal structural abnormality which can explain the etiological heterogeneity for pseudobulbar palsy, which results from bilateral perisylvian lesions. Awareness of this disorder can provide a useful strategy for evaluation and treatment which differs from that in perisylvian polymicrogyria.
Kim
Hyoung-Ihl
HI
Department of Neurosurgery, Presbyterian Medical Center, Chonju, Korea.
Lee
Min-Cheol
MC
Lee
Ji-Shin
JS
Kim
Hyung-Seok
HS
Kim
Myeong-Kyu
MK
Woo
Young-Jong
YJ
Kim
Jae-Hyoo
JH
Jung
Shin
S
Palmini
Andre
A
Kim
Seung U
SU
D000328:Adult; D001927:Brain Diseases / Q000151:congenital* / Q000473:pathology* / Q000601:surgery; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000601:surgery; D004569:Electroencephalography; D004827:Epilepsy / Q000209:etiology* / Q000601:surgery; D005260:Female; D007839:Functional Laterality; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019635:Neurosurgical Procedures; D020828:Pseudobulbar Palsy / Q000209:etiology* / Q000601:surgery