Mavili Ertugrul, Coskun Abdulhakim, Per Huseyin, Donmez Halil, Kumandas Sefer, Yikilmaz Ali
Department of Radiology, Faculty of Medicine, Erciyes University, Melikgazi, Kayseri, Turkey.
Childs Nerv Syst. 2012 Jun;28(6):905-9. doi: 10.1007/s00381-012-1703-2.
The aim of this study is to evaluate the correlation between clinical presentation and the extent of cortical involvement in patients with polymicrogyria.
The magnetic resonance imaging findings of 26 patients were evaluated for the location and distribution of polymicrogyria. Presence of asphyxia at birth and serological tests for TORCH infections, the presence and type (spastic, flaccid) of motor deficits, mental development,microcephaly, and epilepsy were noted.
Nineteen patients had bilateral, whereas seven had unilateral involvement. Patients with unilateral polymicrogyria presented later with milder symptoms. The most encountered symptom in patients with bilateral involvement was mental motor retardation (MMR) (89%) and speech problems (84%).The clinical presentations of patients with asphyxia and positive serological tests for cytomegalovirus (CMV) were worse.All patients with positive serological tests for CMV had bilateral involvement. The perisylvian region was affected in five(71%) patients with unilateral involvement. The most encountered presenting symptom in these patients was epilepsy. Cerebral palsy was seen in three (43%) of the patients, and all of them had left hemiparesis. Microcephaly, MMR, and speech delay were detected in one (14%) of the patients.
Late presenting epilepsy may be a predictor of aunilateral polymicrogyria and is associated with relatively good prognosis. CMV infection and the presence of asphyxia are predictors of worse prognosis.
本研究旨在评估多小脑回患者的临床表现与皮质受累程度之间的相关性。
对26例患者的磁共振成像结果进行评估,以确定多小脑回的位置和分布。记录出生时是否存在窒息以及TORCH感染的血清学检测结果、运动功能障碍的存在情况及类型(痉挛性、弛缓性)、智力发育、小头畸形和癫痫情况。
19例患者为双侧受累,7例为单侧受累。单侧多小脑回患者症状出现较晚且症状较轻。双侧受累患者最常见的症状是精神运动发育迟缓(89%)和言语问题(84%)。窒息患者和巨细胞病毒(CMV)血清学检测呈阳性的患者临床表现更差。所有CMV血清学检测呈阳性的患者均为双侧受累。单侧受累患者中有5例(71%)大脑外侧裂周围区域受累。这些患者最常见的首发症状是癫痫。3例(43%)患者出现脑瘫,且均为左侧偏瘫。1例(14%)患者检测出小头畸形、精神运动发育迟缓及言语延迟。
迟发性癫痫可能是单侧多小脑回的一个预测指标,且与相对较好的预后相关。CMV感染和窒息的存在是预后较差的预测指标。