Agostini G, Mancini J, Chabrol B, Villeneuve N, Milh M, George F, Maurel B, Girard N
Service de neurologie pédiatrique, CHU La-Timone, Assistance publique-Hôpitaux de Marseille, 264 rue Saint-Pierre, Marseille, France.
Arch Pediatr. 2010 Jul;17(7):1008-16. doi: 10.1016/j.arcped.2010.03.002. Epub 2010 Apr 20.
Morphologic abnormalities of the hippocampal formations (MAHF) are more frequently observed in magnetic resonance imaging (MRI). We wished to specify the types of disorders associated with these malformations based on a retrospective case series by studying the language of the children presenting these abnormalities.
From the data of all the MRIs taken in the neuroradiology ward of our center over 16 months in patients under 18 years of age, we retrospectively selected the children with an MAHF, isolated or associated with other malformations. The MAHFs were defined and described according to criteria of shape or orientation defects of the hippocampal formations. We studied the files of the patients with isolated MAHF again. Those whose clinical presentation was compatible with language assessment were tested in a prospective approach.
Out of 2208 MRIs from 1 January 2007 to 30 April 2008, 96 (4.3%) showed an MAHF, including 61 (64%) boys and 35 (36%) girls, aged from 2 months to 17 years. Eighty-two (85%) had associated abnormalities, mainly including cerebral atrophy, corpus callosum agenesis or defect, and abnormal ventricular frontal horns. Fourteen (15%) had an isolated MAHF: 2 on the left hemisphere, 2 on the right hemisphere, and 10 on both. Of these 14, 9 were compatible with language assessment. From the test results, we divided these children into 2 groups, depending on the type and severity of the impairment. Four had very serious language disorders as part of mental retardation or autistic disorders; 4 others had language disorders predominantly in expression and phonology, with weak to pathological visual memory. This study showed no potential relation between the lateralization of MAHF and language disorders, nor between the existence of epilepsy and the severity of the language disorders. Of these 14 children, 9 had behavior and autism spectrum disorders and 7 were epileptic.
Even though language disorders are often part of a larger deficiency presentation, the results we obtained suggest that isolated MAHFs are not only causes of amnestic disorders, but they could also directly underlie language disorders, particularly in expression.
海马结构形态异常（MAHF）在磁共振成像（MRI）中更为常见。我们希望通过回顾性病例系列研究，根据出现这些异常的儿童的语言情况，明确与这些畸形相关的疾病类型。
从我们中心神经放射科病房16个月内对18岁以下患者进行的所有MRI数据中，我们回顾性选择了患有MAHF的儿童，这些MAHF可为孤立性或与其他畸形相关。MAHF根据海马结构的形状或方向缺陷标准进行定义和描述。我们再次研究了孤立性MAHF患者的病历。那些临床表现适合语言评估的患者采用前瞻性方法进行测试。
在2007年1月1日至2008年4月30日的2208份MRI中，96份（4.3%）显示有MAHF，其中包括61名（64%）男孩和35名（36%）女孩，年龄从2个月至17岁。82份（85%）伴有相关异常，主要包括脑萎缩、胼胝体发育不全或缺陷以及异常的脑室额角。14份（15%）为孤立性MAHF：2份在左半球，2份在右半球，10份双侧均有。在这14名患者中，9名适合进行语言评估。根据测试结果，我们将这些儿童分为两组，取决于损伤的类型和严重程度。4名儿童有非常严重的语言障碍，是智力发育迟缓或自闭症障碍的一部分；另外4名儿童主要在表达和语音方面有语言障碍，视觉记忆从弱到病理性。这项研究表明，MAHF的侧化与语言障碍之间、癫痫的存在与语言障碍的严重程度之间均无潜在关系。在这14名儿童中，9名有行为和自闭症谱系障碍，7名患有癫痫。
尽管语言障碍往往是更广泛缺陷表现的一部分，但我们获得的结果表明，孤立性MAHF不仅是遗忘症的病因，还可能直接是语言障碍的基础，尤其是在表达方面。
