BILATERAL PERISYLVIAN ULEGYRIA, PSEUDOBULBAR PALSY, AND EPILEPSY: THE PERI/ POSTNATAL COUNTERPART OF THE BILATERAL PERISYLVIAN SYNDROMES
Abstract number :
2.400
Submission category :
Year :
2003
Submission ID :
4016
Source :
www.aesnet.org
Presentation date :
12/6/2003 12:00:00 AM
Published date :
Dec 1, 2003, 06:00 AM
Authors :
Andre L. Palmini, Hyoung-Ihl Kim, Min-Cheol Lee, Yun-Hee Kim, Young-Jong Woo, Pedro Rosa-Neto, Jaderson Costa da Costa, Eliseu Paglioli-Neto, Mirna Portuguez, Eduardo Paglioli Porto Alegre Epilepsy Surgery Program, Hospital S[atilde]o Lucas da PUCRS, Port
Growing interest in malformations of cortical development often leads to an [italic] a priori [/italic] diagnosis of bilateral perisylvian polimycrogyria in children presenting with pseudobulbar palsy and epilepsy. Interestingly, the possibility that the same clinical picture might be due to a destructive peri or post-natal lesion in the perisylvian regions is often neglected. The superficial resemblance between the anatomical features of malformative polymicrogyria and destructive [italic] ulegyria [/italic] may be a confusing factor, contributing to an erroneous diagnosis with potential therapeutic implications. The present study describes the eletroclinical and neuroimaging features of patients with epilepsy and pseudobulbar palsy associated with bilateral perisylvian [italic]ulegyria [/italic] (BPU).
Thirteen consecutive patients with epilepsy and MRI-defined perisylvian ulegyria were evaluated from 1993 to 2000. Twelve had bilateral lesions. Histopathological evaluation was available for 6 patients who underwent surgery due to refractory seizures
Traumatic perinatal events were present in 7 patients (54%). Signs of pseudobulbar palsy were seen in 10, four of which had severe speech restriction and inability to protrude the tongue. Nine patients had delayed acquisition of motor and language milestones, and only one did not have mental retardation. Four patients had associated unilateral hippocampal sclerosis, ipsilateral to the most severe epileptiform abnormalities. Six patients reported only occasional generalized seizures, while the other 7 had simple or complex partial seizures, secondary generalization, and a high degree of refractoriness to medication. Histopathology in 6 operated patients confirmed hippocampal sclerosis and showed subpial and subcortical gliosis, laminar cortical necrosis, glial nodules, amyloid bodies, and secondary demyelination in the resected ulegyric cortex.
BPU mimics the electroclinical picture of bilateral perisylvian polimicrogyria. However, the pathophysiological, imaging and histopathological features of BPU should be recognized, thus allowing a correct syndromic diagnosis, which probably has therapeutic and prognostic implications.